FitSugar

Gear Review: Evernew Collapsible Water Bottle

FitSugar — Fri, 29 Aug 2008 13:00:00 -0700

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Kidney stones

FitSugar — Wed, 08 Oct 2008 17:35:35 -0700

HEALTH GUIDE REFERENCE FROM A.D.A.M

Highlights

New Research:

Patients who have the most common type of gastric bypass surgery, the Roux-en-Y, are at increased risk for kidney stones, beginning 6 months after surgery, according to a study published in 2006.

Causes of Kidney Stones:

Calcium stones form when there is an imbalance in the urine substances that promote and block the formation of stones. Often, the cause of this imbalance is unknown.
Having acidic urine or too much uric acid in the body leads to the formation of uric acid stones.
Struvite stones are almost always caused by urinary tract infections due to bacteria that produce certain enzymes.
Other stones, including cystine and xanthine stones, are usually due to genetic abnormalities.

Treatments:

In about 85% of patients, the kidney stones are small enough that they pass through normal urination, usually within 2 - 3 days.
Certain medications can prevent recurrence of stones in people who are at high risk.
Extracorporeal shock wave lithotripsy (ESWL) is a technique that uses sound waves (ultrasound) to break up simple stones in the kidney or upper urinary tract. The shock waves are delivered from outside the body.
Surgery may be necessary if the stone or stones are too big to pass, and cannot be broken down through ESWL.
A change of diet and increased drinking of fluids, especially water, will help prevent a recurrence.

Introduction

Kidney stones are hard, solid rocks that form in the urinary tract. In many cases, the stones are very small and can pass out of the body without any problems. However, if a stone (even a small one) blocks the flow of urine, excruciating pain may result, and prompt medical treatment may be needed.

The process of urination begins in the kidneys. The kidneys filter out fluids and waste from the body, producing urine. The two kidneys are located deep behind the abdominal organs, below the ribs and toward the middle of the back.

Each kidney contains over a million nephrons. These are the tiny filtration units of the kidney.
Each nephron is composed of a tiny group of blood vessels (a glomerulus) enclosed in a funnel-like structure called Bowman's capsule.
Each glomerulus filters waste products, water, and salts out of the liquid part of the blood (plasma) that has entered the kidney.
About 1% of the plasma is converted into urine. The rest returns into the blood to prevent dehydration. Urine is primarily made of acids, urea, and creatinine (nitrogen compounds).
Urine passes from Bowman's capsule into tiny tubules, which lead to large collecting tubes in the center of the kidney. As the urine passes through this network, it becomes more concentrated.
Urine then flows from the kidney through thin tubes called ureters into the bladder.
The bladder's stretchy walls expand to store the incoming urine until it leaves the body through a tube called the urethra.

The kidneys are responsible for removing wastes from the body, regulating electrolyte balance and blood pressure, and stimulating red blood cell production.

Click the icon to see an image of the urinary tract.

Occasionally, various salts build up on the inside surfaces of the kidney and form crystals. Eventually these crystals become large enough to form stones in the kidney, a condition called nephrolithiasis. Kidney stones (renal calculi) may also form in the ureter or the bladder. Combinations of minerals and other chemicals, some derived from a person's diet, make up the salts in these stones.

Click the icon to see an image of the kidney stones.

Calcium Stones. About 70 - 90% of all kidney stones are made of calcium, usually combined with oxalate, or oxalic acid. A number of common vegetables, fruits, and grains contain oxalate.

About 6% of calcium stones are made of calcium phosphate (called brushite).

Uric Acid Stones. Uric acid is responsible for close to 10% of kidney stones. It is the breakdown product of purines, nitrogen compounds found in our bodies and in certain foods. The breakdown of purines to uric acid occurs in the liver, and from there uric acid enters the bloodstream, most of it passing into the kidneys. From the kidneys, uric acid leaves the body in the urine. Often, uric acid stones occur with calcium stones.

Struvite Stones. Struvite stones are made of magnesium ammonium phosphate. They are almost always associated with certain urinary tract infections. Worldwide, they make up to 30% of all kidney stones. In the United States, however, less than 15% of all stones are struvite. Most struvite stones occur in women. The rate of these stones may be declining in America, perhaps because of better control of urinary tract infections.

Cystine Stones. A build-up of the amino acid cystine, a building block of protein, causes 1% of kidney stones in adults and up to 8% of stones in children. The tendency to form these stones is inherited. Cystine stones are marked by rapid growth and recurrence, which, if not treated promptly, can eventually lead to kidney failure.

Xanthine Stones. Other kidney stones are composed of xanthine, a nitrogen compound. These stones are extremely uncommon and usually occur as a result of a rare genetic disorder.

Click the icon to see an animation about kidney stones.

Causes

The key process in the development of kidney stones is supersaturation.

The urine carries salts, including calcium oxalate, uric acid, cystine, or xanthine.
These salts can become extremely concentrated if there is not enough urine, or if unusually high amounts of crystal-forming salts are present.
When salt concentration levels reach the point at which they no longer dissolve, these salts form crystals.

Different factors may be involved in either reducing urine amount, or increasing the levels of the salts.

Deficiencies in Protective Factors. Normally, urine contains substances that may protect against stone formation, including:

Magnesium
Citrate
Pyrophosphate
Enzymes

These substances:

Allow salt in the urine to be at higher-than-normal concentrations without forming crystals
Prevent crystal formation
Coat the crystals and prevent them from sticking to the surface of kidney tubes

Not having enough of these protective substances can cause stones.

Changes in the Acidity of the Urine. Changes in the acid balance of the urine can affect stone formation.

Uric acid and cystine stones mainly form in acidic urine.
Calcium phosphate and struvite stones increase in alkaline urine.

Factors that Bind Crystals to the Kidney Tubules. Researchers are studying the cells lining the kidney tubules in order to understand how and why early crystals bind to the tubes long enough to form stones. Under investigation are elevated levels of substances that either cause crystals to stick to the tubes or deficiencies in those that prevent them from sticking.

In general, calcium stones form when there is an imbalance in the urine substances that promote and block the formation of stones. Often, the cause of calcium stones is not known, and the condition is then called idiopathic nephrolithiasis. Research suggests that nearly all stones result from problems in the breakdown and absorption of calcium and oxalate. Genetic factors may play a role in about half of these cases. A number of medical conditions and drugs can also affect digestion and intestinal absorption.

Excess Calcium in the Urine (Hypercalciuria). Hypercalciuria (too much calcium in the urine) is responsible for as much as 70% of calcium-containing stones. A number of conditions may produce hypercalciuria. Many are due to genetic factors, but most cases are idiopathic (due to unknown causes).

The following can lead to hypercalciuria and calcium stones:

Too much calcium absorption in the intestines: In most of these conditions, genetic factors lead to increased calcium absorption in the intestine. Researchers are investigating a possible defective gene that regulates calcitriol, a form of vitamin D, which, in excess levels, may increase intestinal absorption of calcium.
Excessive chloride: Chloride has a negative charge, and calcium has a positive one, so they balance each other in the body. Excess chloride may lead to excess calcium. A gene known as CLCN5, which regulates chloride in the urine, is defective in many patients with calcium stones.
Renal calcium leak: In this condition, the filtering processes in the kidney fail, causing an increase of calcium in the urine.
Excessive sodium: High urinary levels of sodium result in increased levels of calcium. Certain defects in the kidney tubules transport system, which cause imbalances in sodium and phosphate, can lead to high calcium levels in the urine. A diet high in salt can also produce this effect.

Excess Oxalate in the Urine (Hyperoxaluria). Oxalate is the most common stone-forming compound. Excessive oxalate in the urine (hyperoxaluria) is responsible for up to 60% of calcium stones and is a more common cause of stones than too much calcium in the urine.

Hyperoxaluria can be either primary or secondary.

Primary hyperoxaluria is an inherited disorder in which too much oxalate in the urine is the main problem.
Secondary hyperoxaluria results from specific conditions that cause high levels of urinary oxalate.

Secondary hyperoxaluria is usually caused by too much dietary oxalates (found in a number of common vegetables, fruits, and grains) or by problems in the body's breakdown of oxalates. Such defects may be due to various factors:

Severe vitamin B6 deficiencies (usually due to genetic disorders)
Deficiencies in Oxalobacter formigene, an intestinal bacteria that breaks down oxalate
Short bowel syndrome, a condition that makes the intestines unable to properly absorb fat and nutrients; calcium may bind to unabsorbed fat instead of oxalates, which causes a buildup of oxalate
Androgens (male hormones)

Female hormones (estrogens) actually lower the risk of hyperoxaluria. Estrogen may help prevent the formation of calcium oxalate stones by keeping urine alkaline, and raising protective citrate levels.

A study published in 2006 found that patients who undergo the most common gastric type of bypass surgery, the Roux-en-Y, were at increased risk for calcium oxalate kidney stones, beginning 6 months after surgery. The study found that patients who underwent the procedure developed hyperoxaluria, and the condition was common 12 months after surgery. The authors also noted an increased number of kidney stone incidents in this patient group.

Excessive Calcium in the Bloodstream (Hypercalcemia). Hypercalcemia generally occurs when bones break down and release too much calcium into the bloodstream. This is a process called resorption. It can occur from a number of different diseases and events:

Hyperparathyroidism: Overactive parathyroid glands cause about 5% of calcium stones. People with this disorder have at least a 20% chance of developing kidney stones. Women are more likely to have this disorder than men.
Immobilization: Lack of movement can lead to kidney stones.
Renal tubular acidosis: This disorder causes acidic and alkaline imbalance. Renal tubular acidosis not only increases calcium levels in the bloodstream but also reduces protective citrate levels.

Hyperuricosuria is a condition of high levels of uric acid in urine. It occurs in between 15 - 20% of people (mostly men) with calcium oxalate stones. Urate, the salt formed from uric acid, creates the center of a crystal (nidus), around which calcium oxalate crystals form and grow. Such stones tend to be severe and recurrent. They appear to be strongly related to a high intake of protein. (Hyperuricosuria also plays a major role in some uric acid stones.)

Low Urine Levels of Citrate (Hypocitraturia). Citrate is the main substance in the body that is responsible for removing excess calcium. It also blocks the process that turns calcium crystals into stones. Low levels of citrate in the urine (hypocitraturia) is a significant risk factor for calcium stones. In addition, hypocitraturia also increases the risk for uric acid stones. This condition most likely contributes to about a third of all kidney stones.

Many conditions can reduce citrate levels. Some causes include:

Renal tubular acidosis
Potassium or magnesium deficiency
Urinary tract infection
Kidney failure
Chronic diarrhea

Often, however, the cause of hypocitraturia-related stones is unknown.

Low Levels of Other Stone-Blocking Compounds. Several other compounds in the urine, including magnesium and pyrophosphate, also prevent the formation of calcium stones. If any of these compounds are lacking, stones may develop.

Nanobacteria Infection. Nanobacteria are tiny infectious organisms that can pass from the blood into urine. They coat themselves with mineral deposits that resemble the composition of kidney stones. Cells infected with these bacteria develop mineral deposits on the inside and outside. Researchers believe that nanobacteria may form the cores of the kidney stones in many people.

Human body tissues, certain foods, and certain alcoholic drinks contain substances called purines. Purine-containing foods include dried beans, peas, and liver. When the body breaks down purines, it produces uric acid. The presence of a certain level of uric acid in the body is normal.

The following conditions are usually seen in patients with uric acid stones:

Too much acid in the urine for a long period (the most important cause of uric acid stones)
Lower than normal amounts of urine produced.
Hyperuricosuria, a metabolic disorder that leads to high levels of uric acid in the urine

Note: Hyperuricosuria can also trigger calcium stones. Therefore, a combination of calcium and uric acid stones may be present in patients with hyperuricosuria.

A number of conditions and other factors may contribute to, or cause, uric acid stones:

Gout: Uric acid and other kidney stones develop in up to 25% of patients with primary gout, a painful form of arthritis that occurs when uric acid in the blood forms crystals in one or more joints.
Diabetes: New research has shown that people with type 2 diabetes have highly acidic urine that can lead to kidney stones, particularly uric acid stones. The findings were published in the May 2006 Journal of the American Society of Nephrology.
Insulin resistance: People with insulin resistance are at an increased risk for uric acid stones. The reason is unknown but may be related to the transport of certain salts through the kidneys. This transport changes in patients with insulin resistance.
Kidney abnormalities: Kidney problems that reduce the production of ammonia, particularly in people with diabetes or insulin resistance, may lead to uric acid stones.
Genetic factors: Genetic factors can increase a person's risk for uric acid stones.
Hypocitraturia: Hypocitraturia is a low amount of citrate in the urine.
Diet: Eating too much animal protein increases the risk of forming uric acid stones.

Other risk factors include:

Certain medications (chemotherapy drugs, diuretics, and salicylates)
Binge drinking
Not eating for long periods of time (fasting)
Lead poisoning
Blood cancers (leukemia, multiple myeloma, and lymphomas)
Some rare types of anemia (low levels of red blood cells in the blood)
Chronic diarrhea

Struvite stones are almost always caused by urinary tract infections due to bacteria that produce certain enzymes. These enzymes raise the concentration of ammonia in the urine. Ammonia makes up the crystals that form struvite stones. The stone-promoting bacteria are usually Proteus, but may also include Pseudomonas, Klebsiella, Providencia, Serratia, and staphylococci. Women are twice as likely to have struvite stones as men.

Other stones, including cystine and xanthine stones, are usually due to genetic abnormalities.

Causes of Cystine Stones. Cystine stones develop from genetic defects that cause abnormal transport of amino acids in the kidney and gastrointestinal system leading to a build-up of cystine, one of these amino acids. Researchers have identified two genes responsible for this condition: SLC3A1 and CLC7A9.

Causes of Xanthine Stones. In some cases, xanthine stones may develop in patients being treated with allopurinol for gout.

Risk Factors

Kidney stones are one of the most common disorders of the urinary tract. They are an ancient health problem. Evidence of kidney stones has been found in an Egyptian mummy estimated to be more than 7,000 years old.

An estimated 1.3 million Americans seek medical help for kidney stones each year. At this time, studies suggest kidney stones affect over 5% of Americans and that the rate has increased since the 1970s, perhaps because of increases in animal and dietary protein intake.

Men. The risk of kidney stones increases in a man's 40s and continues to rise until age 70. Caucasian men are at higher risk than other groups.

Women. The risk of kidney stones peaks in a woman's 50s. In younger women, stones are more likely to develop during the late stages of pregnancy. Pregnant women tend to have a higher calcium intake, but their kidneys do no handle the calcium as well as they did prior to pregnancy. Kidney stones are still a rare occurrence during pregnancy, however, affecting only 1 in 1,500 pregnancies.

Risk Factors in Children. Stones in the urinary tract in children are usually due to genetic factors. Most of the time, the cause is too much calcium in the urine (hypercalciuria). Deformities in the urinary tract pose a significant risk for kidney stones in children. Children with low birth weight who need to be fed intravenously are also at risk for stones.

Obesity and weight gain are both associated with an increased risk of kidney stones.

Men who weigh more than 220 lbs are 44% more likely to develop kidney stones than men who weigh less than 150 lbs.
Women who are obese are 90% more likely to develop kidney stones than women with a lower body mass index (BMI).

Higher BMIs and larger waist circumferences are both risk factors for kidney stones. Researchers think that there may be a link between fat tissue, insulin resistance, and urine composition. People with larger body sizes may excrete more calcium and uric acid, which increase the risk of kidney stone formation.

A family history of kidney stones increases one's risk for the condition. Researchers are looking into markers or other factors that might predict kidney stones in relatives, although none has yet been clearly identified. One report found that among the siblings of patients with calcium stones, sisters with higher urinary calcium levels and more acidic urine were more likely to develop stones. Brothers with high urinary calcium, low urinary potassium, and older age were more likely to have the problem. A family history of gout may also make a person vulnerable to stones.

According to a 2003 study of American ethnic groups, Caucasians have the highest incidence of kidney stones (5.9%) followed by Mexican Americans (2.6%). African-Americans have the lowest risk (1.7%).

Dietary factors, minerals in local water, or both may contribute to geographic differences that have been observed in the occurrence of kidney stones. Studies have reported the highest occurrence of kidney stones in the southern region of the United States and the lowest in the west. One study suggested that the higher risk may be due to a higher rate of high blood pressure in the southern states and certain dietary habits, particularly lower intake of magnesium and low use of calcium supplements.

Specific Foods. In general, certain foods increase the risk for stones only in people who have genetic or medical vulnerability. People whose diets are high in animal protein and low in fiber and fluids may be at higher risk for stones. A number of foods contain oxalic acid, but there is no proof that such foods make any major contribution to calcium oxalate stones in people without other risk factors. However, several studies have shown that increasing dietary calcium and restricting salt, animal protein, and foods rich in oxalate can help prevent calcium oxalate stones from returning.

Stress. One study reported that people who had a major, stressful life experience were more likely to develop stones than those who had not. Some experts speculate that this increased risk may be due to a hormone called vasopressin, which is released in response to stress. Vasopressin also increases the concentration of urine.

Sleep Position. Sleeping in the same position consistently may influence risk. A 2001 study reported that in people who had a history of kidney stones, recurrences tended to occur on the same side that people slept on. An earlier study suggested that people who had kidney stones were more apt to sleep on their stomachs. Movement during sleep did not appear to affect the risk.

Being Bedridden. Any medical or physical condition that keeps a person in bed or immobile increases blood levels of calcium from bone breakdown, thereby posing a risk for stone formation.

Gout. Patients with gout are at a high risk of uric acid stones. These patients have very acidic urine, and a 2002 study suggested that the two disorders may have a common source.

High Blood Pressure. Persons with high blood pressure are up to three times more likely to develop kidney stones. It is not entirely clear whether having high blood pressure increases the risk for a stone, whether stones lead to high blood pressure, or if there is an action linking both.

Inflammatory Bowel Disease: Crohn's disease and ulcerative colitis cause problems in absorption of substances in the intestines. These problems significantly increase the risk for kidney stones, particularly in men.

Urinary Tract Infections (UTIs): Urinary tract infections are almost always the cause of struvite stones.

Hyperparathyroidism: The parathyroid glands regulate calcium levels in the body through the parathyroid hormone. In hyperparathyroidism, one or more of these glands makes too much parathyroid hormone. Some people with hyperparathyroidism develop kidney stones. Surgery to remove the hyperactive parathyroid gland in such patients reduces the risk for stone formation, but the risk still remains high for some time after surgery.

Other Medical Conditions. Kidney disease, chronic diarrhea, certain cancers (such as leukemia and lymphoma), and sarcoidosis put people at higher risk for stones.

AIDS medications. Over 10% of persons with AIDS who take the medicine indinavir develop stones. The risk is even higher in patients with AIDS who also have hepatitis B, hepatitis C, or hemophilia, as well as those who are very thin or who take the antibiotic combination TMP-SMX. In one study of persons with AIDS who took a combination of indinavir, zidovudine, and lamivudine, 36% developed kidney stones.

Other Drugs. Kidney stones are a rare side effect of thyroid hormones and loop diuretics (drugs that increase urination). In fact, diuretics are also used to prevent calcium stones. Certain cancer chemotherapies can also cause kidney stones. Long-term use of medications, such as antacids, which change the acidic content of urine, may increase the risk for kidney stones.

Symptoms

In many cases, kidney stones do not produce symptoms. However, if a stone becomes stuck in the ureter (the thin tube between the bladder and the kidney), symptoms can be very severe. Often, they vary depending on the stone's location and its progress.

Kidney stone attacks tend to be most common late at night or in the early morning, possibly because of minimal urine output or constriction of the ureters during the early morning hours. Kidney stone attacks are least common during the late afternoon

Pain usually begins abruptly on one side and then usually continues as intense, constant pain. (In some cases it persists for a few minutes, disappears, and then returns after about 10 minutes.)
The patient cannot become comfortable and usually stands, sits, paces, or reclines in a vain search for a position that will bring relief.
If the stone is in the kidney or upper urinary tract, the pain usually starts in one flank area (to the side of the back near the waist). It typically moves to the groin as the stone passes down.
If the stone is too large to pass easily, the pain follows the muscle contractions in the wall of the ureter as they try to squeeze the stone along into the bladder.
Nausea and vomiting may occur.
Blood in the urine may be present.
As the stone passes down the ureter closer to the bladder, a person may feel the need to urinate more often or a burning sensation during urination.
If fever and chills accompany any of these symptoms, an infection may be present.

The size of the stone does not necessarily predict the severity of the pain; a very tiny crystal with sharp edges can cause intense pain while a larger round stone may not be as distressing. Struvite stones can often occur without symptoms.

Diagnosis

The doctor will perform a physical exam. This includes pressing against abdominal areas for tender locations that might indicate the presence of the stone.

The patient's age is a significant factor. Kidney stones that occur in children and young patients are more apt to result from inherited problems that cause cystine, xanthine, or, in some cases, calcium oxalate stones. In adult patients, calcium stones are most common.

A medical history may help predict which crystal has formed the stone. The doctor will need to know the following:

Any previous kidney stone attacks
Histories of cancer, sarcoidosis, or small bowel disease
Any medications being taken, including non-prescription substances, particularly high doses of vitamins D or C and calcium-containing antacids

Many conditions can cause symptoms similar to kidney stones. Usually the diagnosis is easily made because of the specific nature of the symptoms, but it is not always clear. Urinary tract infections can cause similar, but usually less intense, pain. In fact, infection may be present with a kidney stone. Other causes of pain that may mimic kidney stones include:

Gallstones
Diverticulitis (infection or irritation of abnormal pockets in the intestines)
Intestinal blockage
Blood clots
Irritable bowel syndrome
Appendicitis
Stomach ulcers
Hiatal hernia (when the upper part of the stomach bulges into the chest, through an opening in the diaphragm)
Pancreatitis (inflammation of the pancreas)
Hepatitis
Pelvic inflammatory disease
Inflammatory bowel disease (Crohn's and colitis)
Heart attack

Various imaging techniques are helpful in determining the presence of kidney stones. The best approach uses spiral (or helical) computed tomography scans. If it is not available, the patient will need ultrasound or standard x-rays. If no stones show up, but the patient has severe pain that suggests the presence of kidney stones, the next step is an intravenous pyelogram.

X-Rays. A standard x-ray of the kidneys, ureters, and bladder may be a good first step for identifying many stones, since many are visible on x-rays. Calcium stones can be identified on x-rays by their white color. Cystine crystals can also show up on x-rays.

Spiral (or Helical) Computed Tomography. A type of computed tomography (CT) scan, called a spiral or helical CT scan, is currently the best method for diagnosing stones in either the kidneys or the ureters. This test is fast, does not require instruments or foreign chemicals to enter the body, and provides detailed accurate images of even very small stones. If stones are not present, a spiral CT scan can often identify other causes of pain in the kidney area. It is better than x-rays, ultrasound, and intravenous pyelogram -- the previous standard test for detecting kidney stones. Experts hope spiral CT will eventually be able to identify the chemicals present in a stone.

Ultrasound. Ultrasound can detect clear uric acid stones and obstruction in the urinary tract. It is not useful for finding very small stones, but some research indicates that it may be a useful first diagnostic step in the emergency room to help predict the likelihood of a stone, including suspected stones in children.

Intravenous Pyelogram. With intravenous pyelogram (IVP), the doctor injects a special dye into the patient. A technician will then take x-rays as the dye enters the kidneys and travels down the urinary tract. IVP is invasive but, until recently, was the most cost-effective method for detecting stones. Where it is available, spiral CT is now preferred, since it gives a faster diagnosis, is more accurate, is safer, and is similar in cost.

In any case, IVP should not be used on patients with kidney failure. There is also a risk for an allergic reaction to standard dyes, although newer less allergenic ones are becoming available.

In the procedure intravenous pyelogram (IVP), the patient is injected with dye. X-rays are taken as the dye travels through the urinary tract. This procedure is done to confirm the presence of kidney stones, although some stones may be too small to see.

Magnetic Resonance Imaging. Magnetic resonance imaging (MRI) techniques are showing promise for diagnosing urinary tract obstruction but do not yet accurately reveal small stones, or ones that do not cause a blockage. Because no radiation is involved with MRI, however, it may prove to be a good option for pregnant women.

Urine samples are required to evaluate features of the urine, including its acidity and the presence of:

Red or white blood cells
Infection
Crystals
High or low levels of chemicals that inhibit or promote stone formation

Clean-Catch Urine Sample for Culturing. After determining that a kidney stone is present, the health care provider usually gives the patient a collection kit, including filters, to try to catch the stone or gravel as it passes out. The urine may also be tested (cultured) for the presence of infection-causing organisms. A clean-catch urine sample is almost always required for culturing. To provide a clean catch, do the following:

First, wash your hands thoroughly, then wash the penis or vulva and surrounding area four times with downward strokes, using a new soapy sponge each time.
Begin urinating into the toilet and stop after a few drops.
Position the container to catch the middle portion of the urine stream. Ideally, this urine will contain only the bacteria and other evidence of the stone.
Urinate the remainder into the toilet.
Tighten the cap on the container securely, being careful not to touch the inside of the rim.

Click the icon to see an image of a calcium urine test.

Twenty-Four Hour Urine Collection. A 24-hour urine collection may be needed to measure urine volume and levels of acidity, calcium, sodium, uric acid, oxalate, citrate, and creatinine.

You should not change any of your usual eating or drinking patterns when performing this test.
Discard the first urination on the day of the test.
Afterward all urine passed over the next 24 hours is collected, including the first urination on the morning of day two.
A second 24-hour urine collection may be needed to determine if treatment is working or if the first analysis was not conclusive and the doctor suspects a less common stone, such as a cystine or xanthine stone.

Click the icon to see an image of a uric acid urine test.

Urine tests that are used to determine the specific chemical and biological factors causing the stone should be performed about 6 weeks after the attack, since the attack itself may change the levels of such substances, including calcium, phosphate, and citrate. It should be noted that calcium levels in the urine may be abnormal even in many people without stones. In addition, high urinary concentrations of calcium may pose a greater or lesser risk depending on age. (In one 2001 study, middle-aged adults with high urinary calcium concentrations had a much greater risk than older adults with high levels.)

The kidney stones obtained from the urine sample are examined under a microscope. The crystal formations are often specific enough so that the doctor is able to identify the substance causing the stone.

Calcium oxalate crystals are eight-sided, while calcium phosphate crystals tend to have irregular shapes.
Uric acid stones are sometimes described as pear-shaped or diamond-shaped.
Some struvite stones have very specific shapes commonly described as "coffin lids." Struvite crystals may also occur in a formation known as a staghorn, which can be large and damaging to the kidney.

Testing whether urine is acidic or alkaline helps to identify the specific type of stone. The levels of acidity or alkalinity in any solution, including urine, are indicated by the pH scale:

A pH value of 7.0 is neutral.
A solution with a low pH (below 7.0) is acidic. (A low pH favors uric acid and cystine stones.)
A solution with a high pH is alkaline. (A high pH favors calcium phosphate and struvite stones.)

A dipstick test for blood in the urine (called hematuria) is typically performed when patients appear in the emergency room with flank pain (the primary symptom of kidney stones). About a third of kidney stone patients, however, do not show blood in the urine, so other tests may be needed.

Blood Tests for Stone Factors. Blood and urine tests help determine what substances form the crystals. This allows the doctor to determine the appropriate treatment and preventive measures.

Blood tests may help determine blood levels of urea nitrogen, creatinine, calcium, phosphate, and uric acid for patients with known or suspected calcium oxalate stones. Doctors will usually schedule these tests about 6 weeks after the attack, in order to measure these substances when the stone has been passed, and the patient has been stabilized. This is particularly true in patients with recurrent stones.

Parathyroid Tests. Tests to detect parathyroid hormone levels are given if the doctor suspects hyperparathyroidism, based on other signs and symptoms.

Tests for Infection. A test result that shows a high white blood cell count might indicate infection. Such results, however, could be misleading, since the number of white blood cells could also increase in response to the extreme physical stress of a kidney stone attack.

Tests for Metabolic Problems. About half of children with stones have an identifiable metabolic disorder, which increases their risk of stone recurrence five-fold. Experts argue whether tests for metabolic disorders are routinely needed once the stone composition has been determined. Studies suggest the following:

People with recurrent calcium stones have a wide range of irregular blood or urine test results, indicating a variety of possible metabolic disorders. For example, calcium stones in middle-aged women may be due to parathyroid abnormalities.
Calcium phosphate stones most likely result from renal tubular acidosis.
People with non-calcium stones generally have identifiable metabolic disorders.
Determining the stone composition may be sufficient for treatment, and may help avoid unnecessary metabolic tests.

Treatment

When tests show there is a kidney stone, the next step is to determine treatment. The patient should be admitted to the emergency room if they have severe vomiting, fever, or symptoms of infection.

Strong opioid painkillers, such as meperidine (Demerol), are often required for a severe kidney stone attack. However, doctors will usually not give such drugs until they confirm the presence of a kidney stone on an x-ray. In some cases, powerful nonsteroidal anti-inflammatory drugs (NSAIDs) may work just as well as opioids, and they have fewer side effects. However, they do take longer to work.

In about 85% of patients, the kidney stones are small enough that they pass through normal urination, usually within 2 to 3 days. In some cases, a stone may take weeks to months to pass, although pain usually goes away before that.

The patient should drink plenty of water (two to three quarts a day) to help move the stone along, and take painkillers as needed. The doctor usually provides a collection kit with a filter and asks the patient to save any passed stones for testing.

If the stone has not passed in 2 - 3 days, the patient will need additional treatments. In some severe cases, hospitalization may be necessary.

Specific procedures vary depending on the size of the stone or complexity of the situation. Noninvasive procedures are proving to be very beneficial in eliminating stones, and have largely replaced invasive surgeries.

For small stones that are lodged in the lower part of the ureter, ureteroscopy or shock wave lithotripsy are the procedures of choice.
For larger stones, ureteroscopy, percutaneous nephrolithotomy, and shock wave lithotripsy are all potentially useful. The choice of any of these procedures depends on a number of factors, including location of the stone and the presence of any problems that caused the stone in the first place.
In some complicated cases, standard open surgical procedures (called nephrolithotomy) may be required.

See "Other Treatments" section for more information on kidney stone surgery.


Stone Type	Diet and Lifestyle	Medications	Procedures
Calcium Oxalate	Plenty of fluids. (Choose water, lemon juice. Avoid grapefruit, apple, and cranberry juice.) Limit the amount of protein and salt in the diet. Increase fiber. Limit the amount of fats in the diet, particularly in people who have short bowel syndrome. Balance normal calcium intake with potassium- and phosphate-rich foods. Limit the amount of calcium in the diet (only in people who have genetic abnormalities that cause high intestinal absorption of calcium). Limit the amount of foods high in oxalates (only in patients with rare intestinal conditions that cause hyperoxaluria).	Diuretics ("water pills"), Citrate salts, phosphates, cholestyramine.	Lithotripsy, uteroscopy, percutaneous nephrolithotomy, open surgery.
Uric Acid	Plenty of fluids. (Choose water, blackcurrant juice. Avoid cranberry juice.) Increase calcium intake (be sure well-balanced with potassium and phosphates). Reduce protein and other foods with high-purine content.	Potassium citrate, sodium bicarbonate, allopurinol.	Lithotripsy, uteroscopy, percutaneous nephrolithotomy, open surgery.
Struvite stones	Plenty of fluids (water, cranberry juice). Reduce proteins.	Antibiotics to eliminate any infection. Acetohydroxamic acid (AHA) may be helpful in combination with antibiotics. In some cases, organic acids given through urinary tract.	May respond poorly to most lithotripsy procedures and require open surgery. Newer procedures may be helpful.
Cystine stones	Very high fluid intake (four quarts a day). Limit the amount of protein in the diet.	Alkalizing agents (such as bicarbonate). Sometimes d-penicillamine, tiopronine, or captopril useful for lowering cystine levels.	May respond poorly to most lithotripsy procedures and require open surgery. Newer procedures may be helpful.

Medications

Diuretics. Diuretics are medicines commonly used to treat high blood pressure and other disorders. They remove fluid and sodium from the body. Low doses of a class of diuretics known as thiazides are sometimes used to reduce the amount of calcium released by the kidneys into the urine. Thiazides include:

Hydrochlorothiazide (Esidrix, HydroDiuril)
Chlorothiazide (Diuril)
Trichlormethiazide (Metahydrin, Naqua)
Chlorthalidone (Hygroton)

However, thiazides also cause potassium loss, which reduces citrate levels and can increase the risk for stones. Patients taking thiazide pills should also take potassium citrate, to prevent citrate loss. Amiloride (Midamor) is a potassium-sparing diuretic, which may be used if a thiazide does not work.

Citrates. Citrate salts are often given to people with calcium oxalate or uric acid stones:

Potassium magnesium citrate is available over the counter. It is proving to be very beneficial in preventing kidney stones. In one study, potassium magnesium citrate reduced the risk for kidney stone recurrence by 85%.
Potassium citrate (K-Lyte, Polycitra-K, Urocit-K) is given as the only treatment to people with normal urine calcium levels. Between 70 - 75% of patients with recurrent stones have ongoing remission (no stone recurrence) with potassium citrate treatment. However, some people cannot tolerate potassium citrate because of side effects (stomach problems).
Magnesium citrate (Citroma, Citro-Nesia) may help people who develop calcium stones from impaired intestinal absorption due to short bowel disease.

None of these products should be used by people with struvite stones, urinary tract infections, bleeding disorders, or kidney damage. Patients who take citrate supplements containing potassium should not take any other medications that either contain this mineral or prevent its loss (such as so-called potassium-sparing diuretics). People with peptic ulcers should avoid citrate supplements, or discuss using non-tablet forms with their doctor.

Phosphates. Phosphates help reduce the breakdown of bone that releases calcium into the bloodstream. They are also involved in the kidney's reabsorption of calcium from the urine.

Phosphate compounds:

Neutral (nonacidic) sodium or potassium phosphate (K-Phos, Neutral, Neutra-Phos) is usually taken four times a day after meals to prevent kidney stones unless otherwise directed by the doctor. Diarrhea is a possible side effect.
Cellulose phosphate (Calcibind) is recommended only for severe hypercalciuria that is associated with recurrent calcium stones and is caused by excessive absorption of calcium from the intestines. However, this drug may increase oxalate levels and decrease magnesium levels, which can lead to different stones. Taking magnesium supplements and reducing dietary oxalates, calcium, and ascorbic acid may help offset these risks. Cellulose phosphate may also cause bloating.

Avoid acidic forms of phosphate, since they increase the risks for both hypocitraturia and hypercalciuria.

Cholestyramine (Questran, Questran Light) is a drug used to reduce cholesterol levels. However, it also binds with oxalate in the intestine, so it is also used to reduce high oxalate levels in urine (hyperoxaluria). The drug usually comes in a powder that is dissolved in liquid.

Bloating and constipation are common side effects of this drug. Cholestyramine also interferes with other medications, including digoxin (Lanoxin) and warfarin, and may contribute to calcium loss and osteoporosis. In order to prevent such interactions, take other drugs 1 hour before, or 4 - 6 hours after, taking cholestyramine.

Long-term use of cholestyramine may cause deficiencies of vitamins A, D, E, and K. Vitamin supplements may be necessary.

Sodium Bicarbonate. Patients whose persistently acidic urine causes uric acid stones may take sodium bicarbonate to reduce urine acidity. Patients taking sodium bicarbonate must test their urine regularly with pH paper, which turns different colors depending on whether the urine is acidic or alkaline. Too much sodium bicarbonate can cause the urine to become too alkaline. This increases the risk for calcium phosphate stones. Patients who need to reduce the amount of sodium they take in (as a result of other medical conditions) should not use sodium bicarbonate.

Potassium Citrate. Potassium citrate, which restores citrate to the urine, is useful for patients with high levels of uric acid in the urine.

Allopurinol. Allopurinol (Lupurin, Zyloprim) is very effective in reducing high levels of uric acid, and may be helpful for patients with uric acid stones. Allopurinol will not prevent calcium stones from forming. There is also a slight risk for the formation of xanthine stones with this drug. Side effects include diarrhea, headache, and fever. More severe complications include blood disorders that may produce fatigue, bleeding, or bruising. The drug may also increase the risk for cataracts.

About 2% of patients experience an allergic reaction to allopurinol that causes a rash. In rare cases, the rash can become severe and widespread enough to be life threatening. Allergic individuals who have experienced only a mild rash to sodium bicarbonate may be able to build up their tolerance for allopurinol by undergoing a desensitization process. In this process, patients start with small doses of allopurinol and gradually increase them, if no reaction develops.

Allopurinol reduces uric acid levels rapidly, so it may trigger an attack of gout in vulnerable people. To prevent this problem, patients taking allopurinol should also take a nonsteroidal anti-inflammatory drug (NSAID) for 2 or 3 months. Aspirin should not be taken, since it increases uric acid levels. Patients should discuss the appropriate NSAID choice with their doctor.

Before patients can receive any medical treatment for struvite stones, they must have surgery to completely remove the stones.

Antibiotics for Eliminating Infection. Persons with struvite stones receive ongoing treatment with antibiotics to keep the urine free of the bacteria that cause urinary tract infections. Careful follow-up and urine testing is extremely important. A high-pH urine indicates low acidity and an increased risk of infection.

Acetohydroxamic Acid (AHA). Acetohydroxamic acid (AHA or Lithostat) is beneficial when used with long-term antibiotics. AHA blocks enzymes that bacteria release, and has been effective in preventing stones even when bacteria are present. Side effects, however, can be severe. The drug reduces iron levels in the body, so anemia is a common problem. Patients may need to take iron supplements. Other side effects include nausea, vomiting, depression, anxiety, rash, persistent headache, and, rarely, small blood clots in the legs.

Experts recommend this drug only for patients with healthy kidneys who have chronic diseases caused by specific struvite-causing organisms.

Patients taking this medicine should avoid alcohol. Pregnant women should not take acetohydroxamic acid.

Organic Acids. Medical treatments to dissolve stones may be useful in patients who do not respond to other medications, or in combination with surgeries. Acidic urine dissolves struvite stones, so the doctor may wash the urinary tract with a solution of organic acids (such as Renacidin). Candidates for such washes must have sterile urine (no bacteria or other organisms in the urine) and healthy kidney function. In surgical patients, the wash is performed 4 or 5 days after the operation. The wash starts with saline (salt solution) for 1 - 2 days and, if there are no problems, the organic acid solution follows for another 1 or 2 days, until all stones dissolve. Regular urine tests are necessary to ensure that the bacteria do not return.

Aluminum Hydroxide Gel. An aluminum hydroxide anti-acid gel may reduce phosphate levels that are important in struvite stone formation, but it has a long-term risk of causing aluminum toxicity. Long-term reduction of phosphorus can also increase the risk for calcium oxalate stones. Experts recommend limiting phosphorus through a low-protein diet, rather than through the use of this gel.

The first-line treatment for cystine stones is increasing the alkalization of urine so the stones can dissolve. If alkalization fails, drug treatments may include d-penicillamine, alpha-mercaptopropionylglycine (tiopronine), or captopril. These medications lower cystine concentration.

Patients with cystine stones must drink plenty of fluids, much more than patients with other stones. The patients should drink at least four quarts of water over a 24-hour period.

Other Treatments

Surgery is usually needed if the stone is too large to pass on its own, if there are signs that the stone is growing, or if the stone is blocking the urine flow and causing a urinary tract infection or kidney damage.

Until recently, the procedure to remove a stone was a very painful, major surgery, requiring 4-6 weeks of recovery. Today, treatments for stones are much less invasive. Major surgery is performed in less than 2% of patients.

Stone removal procedures:

Extracorporeal shock wave lithotripsy (ESWL) is used for small stones (less than one centimeter, or slightly less than half an inch) that occur in the upper part of the ureter and do not pass on their own. One study indicated lithotripsy might even be safe and effective for patients whose stones are associated with malformed kidneys, although such patients are at higher risk for stone recurrence and should be carefully monitored.
Percutaneous nephrolithotomy (PNL). PNL can be used for very large stones in the upper urinary tract, when ESWL fails, for kidney transplant patients, or when the kidneys or surrounding areas are malformed. PNL is the preferred procedure for drug-resistant cystine stones, which are usually also resistant to shock wave therapy.
Ureteroscopy. For stones in the lower tract, ureteroscopy is generally the best procedure, although lithotripsy is also usually feasible and patients ordinarily prefer it.
Standard open surgery (nephrolithotomy) may be required if any of these procedures fail or are not appropriate, or in special cases, such as when the patient is very obese.

Most procedures are more effective for calcium and uric acid stones and less effective for struvite and cystine stones, although new techniques may be improving their effects on all stones.

Extracorporeal shock wave lithotripsy (ESWL) is a technique that uses sound waves (ultrasound) to break up simple stones in the kidney or upper urinary tract. ("Extracorporeal" means "outside the body," and "lithotripsy" means stone-breaking.) ESWL is not used for cystine stones. The procedure generally does not work for stones larger than three centimeters in diameter (which is slightly over an inch). There are several variations of ESWL. The following is a typical procedure:

Most ESWL procedures use some anesthesia, although they are often done on an outpatient basis.
The patient is positioned in a water bath. (In some procedures the patient lies on a soft cushion.)
The procedure uses ultrasound to generate shock waves that travel through the skin and body tissues until they hit the dense stones. (The doctor pinpoints the stone during treatment by using x-rays or ultrasound.)
The shock waves crush the stones into tiny sand-like pieces that usually pass easily through the urinary tract.
The shattered stone fragments may cause discomfort as they pass through the urinary tract. In such cases, the doctor may insert a small tube called a stent through the bladder into the ureter to help the fragments pass. This practice, however, has not proved to speed up passage of the stones in most cases and is not used routinely.

Extracorporeal shock wave lithotripsy (ESWL) is a procedure used to shatter simple stones in the kidney or upper urinary tract. Ultrasonic waves are passed through the body until they strike the dense stones. Pulses of sonic waves pulverize the stones, which are then more easily passed through the ureter and out of the body in the urine.

Success rates of ESWL range from 50 - 90%, depending on the location of the stone and the surgeon's technique and level of experience. Recovery time is short, and most people can resume normal activities in a few days.

Complications. Complications may include the following:

The most common complication is blood in the urine, which lasts for a few days after treatment. To reduce the chances of bleeding, doctors usually tell patients to avoid taking aspirin and other NSAIDs, which can promote bleeding, for 7 - 10 days before the treatment.
Bruising and minor discomfort due to the shock waves are common in the back or abdomen.
Sometimes the stone does not completely break up with one treatment, and additional treatments may be required. Inability to pass stone fragments may also be a particular problem in patients who have cysts or other kidney problems.
Higher risk for diabetes later. A 2006 study published in the journal Urology found that 17% of patients who received shock-wave lithotripsy developed diabetes later in life. The diabetes risk was related to the number and intensity of shocks.
Higher risk for hypertension (high blood pressure). The same study that linked ESWL to diabetes also showed that people who received shock-wave lithotripsy treatment were 47% more likely to develop high blood pressure than those who had their stones treated without surgery.

ESWL appears to be safe for children, although a 2001 study reported temporary damage in the kidney tubules after treatment. It is unclear if this complication has any long-term consequences. Experts recommend using the least amount of shocks and impact possible in young people. If more than one treatment is needed, there should be a waiting period of at least 15 days between treatments.

Percutaneous nephrolithotomy may be used when ESWL is not available or effective (such as if the stone is very large, in an inaccessible location, or is a cystine stone). It is also preferred over ESWL for stones that have remained in the ureter for more than 4 weeks.

It is more effective than ESWL for patients with severe obesity, and appears to be safe for the very elderly and the very young. Success rates are nearly 98% for kidney stones and 88% for ureteral stones. They may vary by the technique used and the specific patients. For example, success rates are slightly lower in children, although the procedure can be done safely in young patients. Long-term effects are unknown.

A typical procedure is as follows:

The surgeon makes a tiny incision in the back and creates a tunnel directly into the kidney.
The surgeon then inserts an instrument called a nephroscope through the tunnel.
The stone is located and removed. If it is large, it is destroyed using ultrasound, lasers, or other devices. The surgeon then removes the fragments. An advantage of percutaneous nephrolithotomy over ESWL is that the surgeon is able to remove the stone fragments directly, instead of relying on their natural passage from the kidney.
Generally, patients stay in the hospital for 5 or 6 days and may need a small device called a nephrostomy tube left in the kidney during the healing process.

Devices Used to Destroy Stones. For large stones, some type of energy-delivering device may be needed to break the stone into small pieces. They are referred to as intracorporeal lithotripsy devices (meaning stone breakers within the body). The device may be one of the following:

Ultrasound is currently the preferred method. It results in a stone-free rate of 94%. A rigid nephroscope delivers the ultrasound waves.
Pneumatic (compressed air) lithotripsy uses a probe that comes in direct contract with a stone. Compressed air causes a piston to collide rapidly with the probe, and the result is a "jackhammer" action against the stone, causing the stone to break up. This method, however, can send stone fragments into other parts of the urinary tract.
A more recent device uses a combination pneumatic probe and ultrasound, with stone-free rates of 80 - 89%. It may prove to be superior to ultrasound alone and be effective against stones of all types.
The holmium laser literally melts the stones and destroys up to 100% of stones of any composition. It uses a flexible nephroscope and has an excellent safety record. It should be used sparingly, however, with particular caution against large uric acid stones until more is understood about its effect. Another device, the erbium: YAG laser, although showing promise in lithotripsy, is not currently practical.

Complications. Complication rates are about 3%. Major complications occur in about 1% of cases. These complications may include scarring of the tissue, but studies indicate that it does not impair kidney function, even if the patient requires repeat surgery. There is also a risk for blood loss during and after the procedure, which, in some cases, can be significant.

Because the procedure requires large volumes of fluid, fluid overload is a potential problem, particularly in children or patients with heart disease.

In some cases, infection may result. Other complications may include a collapsed lung and injuries to areas outside the kidney (but within the operative area), such as the abdomen or chest.

Ureteroscopy may be used for stones in the middle and lower ureter. With the arrival of smaller instruments, this procedure can be done successfully in children as well. The procedure involves the following:

The patient receives a general anesthetic, though no incision is required for the procedure.
The surgeon passes a small fiberoptic instrument called a ureteroscope through the urethra and bladder into the ureter.
The surgeon locates the stone or stones.
The surgeon can remove smaller stones by grasping them with small forceps. A laser or pneumatic device breaks up large stones.
The surgeon may decide to leave a small tube, or stent, in the ureter for a few days after treatment, to help the lining of the ureter heal.

Complication rates range from 10 - 20%, with major problems occurring in up to 6% of patients. In some cases, large stones are not broken up into small enough pieces. This can result in blockage of the urinary tract and possible kidney damage.

Imaging tests, such as ultrasound or spiral CT, are useful within 3 months to check for residual stones, and a second procedure may be required. The risk of complications is highest when the procedure is performed by less experienced surgeons, or if stones are found in the kidney. The risk for perforation of the ureter increases the longer the procedure takes.

Open surgery involves incisions through the patient's flank and into the kidney. The surgeon will cool the kidneys using ice. X-rays during the procedure help locate the stone. At the beginning of the surgery, the surgeon will isolate the arteries supplying the kidneys, ensuring they are not harmed during the surgery. The surgeon will then locate and remove the stone. The surgeon will also correct any blockage in the affected area. The surgery, called nephrolithotomy, is very invasive and is restricted to the following:

Patients with very large or complex stones that cannot be removed using less invasive measures
Very obese patients

Some centers report success with extracorporeal shock wave lithotripsy, however, in patients who would normally be nephrolithotomy candidates. Therefore, even these patients should discuss other options with their surgeon.

The procedure is not appropriate for patients with:

Bleeding or clotting disorders
Untreated widespread infection
Severe and chronic kidney insufficiency (unless removing the stone will improve kidney function)

Complications

Between 70 - 90% of crystals remain tiny enough so that they can travel through the urinary tract and leave the body in the urine without being noticed. When they do cause symptoms, however, kidney stones have been described as one of the most painful disorders to afflict humans. The pain they cause is sometimes called renal colic. ("Renal" means "kidney.")

Obstruction and Infection. Although kidney stones often lead to obstruction (blockage) of the urinary tract, the blockage is usually temporary and causes no lasting damage. In some cases, however, particularly if the obstruction progresses with no symptoms, infection may occur, which can be serious and need immediate attention.

Kidney Failure. It is very rare for kidney stones to cause kidney failure, although some people have risk factors that make them more vulnerable to this serious complication. Risk factors include the following:

Very frequent recurrences (such as in people with cystine stones or other inherited forms of kidney stone disorders)
Accompanying episodes of urinary tract infections with obstruction, a particular risk with struvite stones
A history of multiple urologic procedures for kidney stones
Greater size of the kidney stone gravel

Without preventive treatment, calcium stones recur in 10% of patients within a year of the first attack, and in half of patients within 5 - 7 years. Individual risk for recurrence, however, varies depending on the stone and the underlying condition. For example, a 15-year-old with inherited cystine stones has a very high risk for recurrence, while a middle-aged man with a first calcium oxalate stone has a good chance of never passing another.

Prevention

All individuals who have experienced kidney stones should take some specific preventive measures to prevent recurrence. The following are some general observations:

The most important dietary recommendations for reducing the risk for calcium stones are increasing fluid intake, restricting sodium, and reducing protein intake.
A lower risk for calcium stones is also associated with higher potassium intake.
A high calcium diet does not appear to increase the risk for kidney stones as long as it also contains plenty of fluids and dietary potassium and phosphate. (Increasing calcium alone may pose a modest risk for stones.)
Patients should try to correct any dietary habits that cause acidic or alkaline imbalances in the urine, which promote stone formation.

Because different kidney stone types may require specific dietary changes, patients should work with their doctors to develop an individualized plan. It is important to note that nutritional considerations are very important in preventing recurrence, and patients should be vigilant in complying with the proper diet.

Good voiding habits, particularly frequent urination, are important. Therefore, of all the preventive recommendations, drinking enough fluids is the most important guideline for people with any type of kidney stones.

In general, patients with calcium or uric acid stones should drink at least 10 full glasses of fluid each day (at least half should be water). This includes one with each meal and drinking fluids at night, even if it means getting up from sleep. Fluid intake should produce at least two and a half quarts of urine each day.
To prevent cystine stones, patients should drink even more water -- over a gallon, or 16 8-ounce cups, every day. Patients should drink this amount at regular intervals throughout the night and day.

In all cases, patients need more fluid after exertion and during times of stress. If they drink enough, the urine should be pale and almost watery, not dark and yellow.

Water. Although water is best, it may vary depending on its source. Variations in water itself may have different impacts. One study reported that drinking hard tap water increased urinary calcium concentration by 50% compared to soft bottled water. On the other hand, mineral water containing both calcium and magnesium may reduce several risk factors for both calcium and uric acid stone formation.

Juices and Specific Effects. Other beverages have various positive or negative effects, depending on the type of stone:

Lemon Juice: Drinking one-half cup of pure lemon juice (enough to make eight glasses of lemonade) every day raises citrate levels in the urine, which might protect against calcium stones. (While orange juice also increases citrate levels, it does not lower calcium and it raises oxalate levels. Therefore, it is not recommended.)
Cranberry and Apple Juice: Apple and cranberry juice contain oxalates, and both have been associated with a higher risk for calcium oxalate stones. Cranberry juice has properties that may increase the risk for both calcium oxalate and uric acid stones. On the other hand, cranberry juice helps prevent urinary tract infections and so may be helpful for reducing the risk for struvite and brushite stones. (These stones are far less common, however.)
Black Currant Juice: In one study, black currant juice reduced urine acidity and was associated with protection against uric acid stones.
Grapefruit Juice: A number of studies have found a risk for stones from drinking grapefruit juice. In one study, just one 8-ounce cup of grapefruit juice per day increased the risk for forming stones by 44%.

Other Beverages and Their Effects on Stone Formation.

Soft Drinks. Patients with stones should avoid cola drinks, since they can severely reduce citrate levels in the urine. Many soft drinks contain phosphoric acid, which increases the risk for stones. Some research shows that drinking one quart (less than three 12-ounce cans) of soda per week may increase a person's risk of developing stones by 15%.
Alcohol. Wine may be protective against kidney stones. A study conducted in Finland, suggests that the risk of developing stones also decreases with beer consumption. However, it is important to remember that beer is high in oxalates. Beer and other alcoholic beverages also contain purines, which may increase the specific risk for the less common uric acid stones in susceptible people. Binge drinking, in any case, increases uric acid and the risk for stones.
Coffee and Tea. Some research reported a lower risk for stones in people who drink tea and both regular and decaffeinated coffee.

A long-term 2002 study followed men with calcium oxalate stones and high levels of urinary calcium. The study found that a low-sodium, low-protein diet, containing normal levels of calcium, dramatically reduced the recurrence of stones compared to a diet that was simply low in calcium.

Salt Restriction. Because salt intake increases the amount of calcium in urine, patients with calcium stones should limit their sodium intake. Sodium may also increase levels of urate, the crystalline substance that can trigger formation of recurrent calcium oxalate stones. Although the relative contribution of limiting sodium intake has not been confirmed, some researchers believe that restricting sodium along with increasing fluid intake is the most important dietary measure for preventing stones.

Protein Restriction. Protein increases uric acid, calcium, and oxalate levels in the urine, and reduces citrate levels. Diets high in protein, particularly meat protein, have been consistently connected with kidney stones. (Meat protein has a higher sulfur content and produces more acid than vegetable protein.) A 2002 study of those following a high-protein, low-carbohydrate diet (such as the Atkins diet, for example), found dramatically increased levels of urinary uric acid and calcium after just several weeks. These effects put patients at higher risk for not just kidney stones, but possibly osteoporosis as well. According to Swiss studies, about a third of people at risk for calcium stones may have a sensitivity to meat proteins that causes mild hyperoxaluria.

Whether restricting meat protein alone has any protective value without restricting sodium as well is unknown. Most studies to date have found no difference in stone development between people with low and normal meat protein diets over four years. A 2000 study reported that only dramatic reductions in meat protein had any preventive effect against stone recurrence.

Although the precise role of dietary protein in kidney stones needs further clarification, it is reasonable for everyone to consume meat protein in moderation. People with struvite stones, who need to reduce phosphates in their diets, should also cut down on proteins.

Calcium from Foods. Dietary calcium recommendations for kidney stone prevention need to be determined on an individual basis. A doctor will suggest calcium guidelines based on a patient's age, gender, body size, and type of stone. Most studies indicate that dietary calcium (found in milk, yogurt, and cheese) protects against many types of calcium oxalate stones. Large studies of both men and women found that those with the highest intake of calcium from foods had a much lower risk for stones than those who had little calcium in their diets. A diet containing a normal amount of calcium, but reduced amounts of animal protein and salt, may protect against stones better than a low-calcium regimen. However, calcium metabolism changes as people age. Some studies suggest that a high calcium intake protects against kidney stones in men younger than age 60, but not in older men.

Dietary calcium may actually bind the oxalate in foods, preventing it from being absorbed into the blood and excreted into the urine. In a normal healthy diet, dairy products supply almost 80% of the daily calcium requirement. For people who have calcium stones associated with resorption (the breakdown of bone that releases calcium into the bloodstream), limiting calcium intake could cause further bone loss.

Calcium Supplements. Evidence on calcium supplements is mixed, although in general many studies suggest that they reduce oxalate levels and so help prevent calcium oxalate stones. One study suggested that taking 500 mg of calcium supplements a day regularly may "reprogram" the intestines to absorb less calcium and may therefore be protective. Experts generally agree that calcium supplementation within dosage recommendations (about 1,200 mg per day) remains safe. In one study, however, women who took calcium supplements had a 20% higher risk for stones. Research indicates that dosages of calcium above 2,000 mg per day are clearly associated with the formation of stones. Some experts speculate that this higher risk may occur because supplements are often taken in the morning, either without food or with breakfast, which is typically low in oxalates. Taking supplements with later meals may not produce the same risk.

Calcium Restriction in Certain Cases. Some patients, such as those whose stones are caused by genetic defects in which the intestine absorbs too much calcium, may need to limit calcium intake. More studies are needed to define this group precisely.

Fiber may be beneficial for people with kidney stones. In addition, some fiber-rich foods may contain compounds that help protect against kidney stones. A wide variety of high-fiber plant foods contain a compound called phytate (also called inositol hexaphosphate, InsP6, or IP6), which appears to help prevent crystallization of calcium salts, both oxalate and phosphate. Phytate is found in legumes and wheat and rice bran. (Soybeans are also rich in phytate but they are also very high in oxalates, so the overall effects of soy on kidney stones are not clear.)

A high intake of purines can increase the amount of uric acid in the urine. Those at risk for uric acid stones should reduce their intake of foods and beverages that contain purines. These include beer and other alcoholic beverages, anchovies, sardines, yeast, organ meats (such as liver and kidneys), legumes (including dried beans, peas, and soybeans), mushrooms, spinach, asparagus, cauliflower, and poultry.

Most people with calcium oxalate stones should not avoid oxalate-rich foods unless the doctor specifically recommends a restrictive diet. Oxalate binds with calcium in the intestine, which may actually reduce calcium absorption. Some studies, in fact, indicate that eating foods containing oxalates and calcium together may reduce the risk of stones. Most of the foods that contain oxalates are very important for good health. Limiting oxalates may be particularly harmful in people with bowel disorders marked by malabsorption.

Foods high in oxalic acid include beets, soy, beet tops, black tea, chenopodium, chocolate, cocoa, dried figs, ground pepper, lamb, lime peel, nuts, parsley, poppy seeds, purslane, rhubarb, sorrel, spinach, and Swiss chard.
Foods containing moderate amounts of oxalates include beans (green and wax), blackberries, blueberries, carrots, celery, coffee (roasted), concord grapes, currants, dandelion greens, endive, gooseberries, lemon peel, okra, green onions, oranges, green peppers, black raspberries, strawberries, and sweet potatoes.

Certain fats may play a beneficial or harmful role in specific cases of kidney stones.

Restricted Fats in Patients with Stones Associated with Bowel Disease. Patients who have stones associated with short-bowel syndrome should eat foods with lower amounts of fats and oxalates. If patients with short-bowel syndrome eat too much fat, calcium may bind to unabsorbed fat instead of to oxalates. This increased oxalate levels, resulting in increased risk of stone formation.

Fish Oil. Omega-3 fatty acids, found in oily fish like mackerel, salmon, and albacore tuna, have many health benefits, but the most current evidence suggests they do not help prevent kidney stones. A 2005 study of over 200,000 adults found that increased omega-3 fatty acid intake did not reduce kidney stone risk.

Vitamin B6. Vitamin B6, or pyridoxine, is used to treat people with primary hyperoxaluria, a severe inherited disorder. Patients should not try to treat themselves with vitamin B6. Very high doses (500 to 2,000 mg daily over long periods) can cause nerve damage, with loss of balance and numbness in the feet and hands. Food sources of vitamin B6 include meats, oily fish, poultry, whole grains, dried fortified cereals, soybeans, avocados, baked potatoes with skins, watermelon, plantains, bananas, peanuts, and brewer's yeast.

Vitamin C. Ascorbic acid (vitamin C) may change in the body to tiny crystals, called oxalates. These crystals do not dissolve. People with hyperoxaluria (too much oxalate in the urine) should avoid vitamin C supplements. Even for men with normal oxalate levels, higher consumption of vitamin C (more than 1,000 mg a day) may increase kidney stone risk.

Magnesium and potassium may help reduce the risk for kidney stones in men.

Because of an association between stress and kidney stones, relaxation and stress management techniques may also be beneficial.

Dietary Considerations. People with kidney stones appear to be more sensitive to certain foods than people who do not form kidney stones. Therefore, vulnerable people should make specific changes in their diet. They should work with their doctors to develop a dietary plan that fits their individual situation. Drinking plenty of fluids is important for preventing recurrence of any kidney stone.

Indications for Drug Treatments. If dietary treatments fail, drug therapy may be helpful. A number of drugs are available to prevent recurrences of calcium oxalate and other stones. Medications that inhibit the formation of stones include allopurinol, thiazide, potassium citrate, and potassium-magnesium citrate. In addition, drug treatments can sometimes also help prevent other complications related to stones, such as osteoporosis.

Correcting Underlying Conditions Known to Cause Kidney Stones. It is also important to treat and correct, if possible, any underlying disorder that may be causing stones to form. Such disorders include distal renal tubular acidosis, hyperthyroidism, sarcoidosis, and certain cancers. To prevent calcium stones that form in hyperparathyroid patients, a surgeon may remove the affected parathyroid gland (located in the neck). In most cases, only one of the glands is enlarged. Removing it ends the patient's problem with kidney stones.

Resources

www.kidney.niddk.nih.gov -- National Kidney and Urologic Diseases Information Clearinghouse
www.urologyhealth.org -- American Urological Association
www.kidney.org -- National Kidney Foundation
www.ohf.org -- Oxalosis and Hyperoxaluria Foundation

References

Cameron MA, Maalouf NM, Adams-Huet B, Moe OW, Sakhaee K. Urine composition in type 2 diabetes: predisposition to uric Acid nephrolithiasis. J Am Soc Nephrol. 2006 May;17(5):1422-8. Epub 2006 Apr 5.

Curhan GC, Willett WC, Knight EL, Stampfer MJ. Dietary factors and the risk of incident kidney stones in younger women: Nurses' Health Study II. Arch Intern Med. 2004;164(:885-891.

Finkielstein VA. Strategies for preventing calcium oxalate stones. CMAJ. 2006;174(10); 1407-1409.

Krambeck AE, Gettman MT, Rohlinger AL, Lohse CM, Patterson DE, Segura JW. Diabetes mellitus and hypertension associated with shock wave lithotripsy of renal and proximal ureteral stones at 19 years of followup. J Urol. 2006;175(5):1742-7.

Sinha MK, Collazo-Clavell ML, Rule A, et al. Hyperoxaluric nephrolithiasis is a complication of Roux-en-Y gastric bypass surgery. Kidney International. 2007;72:100-107.

Straub M, Hautmann RE. Developments in stone prevention. Curr Opin Urol. 2005;15(2):119-126.

Taylor EN, Stampfer MJ, Curhan GC. Dietary factors and the risk of incident kidney stones in men: new insights after 14 years of follow-up. J Am Soc Nephrol. 2004;15(12):3225-3232.

Taylor EN, Stampfer MJ, Curhan GC. Fatty acid intake and incident nephrolithiasis. Am J Kidney Dis. 2005;45(2):267-274.

Taylor EN, Stampfer MJ, Curhan GC. Obesity, weight gain, and the risk of kidney stones. JAMA. 2005;293(4):455-462.

Taylor EN, Stampfer MJ, Curhan GC. Diabetes mellitus and the risk of nephrolithiasis. Kidney Int. 2005 Sep;68(3):1230-5.

Wasserstein AG. Nephrolithiasis. American Journal of Kidney Diseases. 45(2);2005:422-28.

Review Date:
7/24/2007
Reviewed By:
Harvey Simon, MD, Associate Professor of Medicine, Harvard Medical School; Physician, Massachusetts General Hospital.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is an independent audit to verify that A.D.A.M. follows rigorous standards of quality and accountability. A.D.A.M. is among the first to achieve this important distinction for online health information and services. Learn more about A.D.A.M.'s editorial policy, editorial process and privacy policy. A.D.A.M. is also a founding member of Hi-Ethics and subscribes to the principles of the Health on the Net Foundation (www.hon.ch).

A.D.A.M. Copyright

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997-2009 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

adam.com

Stay Hydrated With Platypus

FitSugar — Tue, 07 Aug 2007 14:30:00 -0700

Since we all know how bad disposable water bottles are for the environment (since most of them end up in landfills because they don't get recycled), it's a great idea to use a reusable water bottle. Both Nalgene and SIGG bottles are great, but if your goal is to stay hydrated during some kind of strenuous activity, lugging around a hard bottle can be kind of annoying.

Check out these bottles made by a company called Platypus®. Their water bottles and carriers come in a range of sizes and shapes, and they're all collapsible, "taste-free," lightweight, easy to fill, and rugged enough to handle temperatures from freezing to boiling.

Want to know what else? Then read more

These are similar to Camelbak hydration packs, but more versatile. You can use Platypus bottles on their own or add accessories to them. I love the Neoprene insulted bottle holder to keep liquids hot or cold. You can also get a hands-free drink tube so you can tuck the bottle in any existing pack you're carrying and attach the tube to your shoulder strap.

These are perfect for hiking, biking, and backpacking because once the bottle is empty, it collapses so you can roll it up for easy, compact storage. Plus these are really inexpensive - an 80 oz bottle is only $9.95. You can check out all the Platypus gear at REI.com.

Exercise

FitSugar — Wed, 08 Oct 2008 17:35:02 -0700

In This Report

Highlights
Introduction
Recommended Exercise Method...
Exercise's Effects on the H...
Exercise's Effects on Diabe...
Exercise's Effects on Bones...
Exercise's Effects on the L...
Exercise's Effects on Weigh...
Exercise's Effects on Other...
Complications
Motivation
Resources
References

HEALTH GUIDE REFERENCE FROM A.D.A.M

Highlights

Chronic Conditions and Exercise:

A new study found that aerobic and resistance training significantly reduced fatigue in men undergoing radiation treatments for prostate cancer. Fatigue is a common side effect of such treatments.
Doctors at the Mayo Clinic found that exercise improves the physical and emotional well-being of patients with Alzheimer's disease. The patients exercised for as little as 60 minutes each week. Doctors noted improvements in areas ranging from depression to wandering.

Exercise and Smoking:

A 2007 review of existing studies found that moderate exercise, for as little as 5 minutes, can help combat the nicotine withdrawal symptoms people experience when they try to stop smoking.

Exercise and Aging:

A 2006 report found that older and elderly adults who exercised twice a week for 4 months significantly increased their body strength, flexibility, balance, and agility. The average age of the study participants was 83.5.

Before and After Exercising:

You should do warm-up exercises for 5 - 10 minutes at the beginning of an exercise session. Low-level aerobic exercise is the best warm-up.
To cool down, you should walk slowly until your heart rate is 10 - 15 beats above your resting heart rate. Stopping too suddenly may sharply reduce blood pressure or cause muscle cramping.
You must be careful when stretching during your warm-up to avoid injuring cold muscles.

Definitions:

Aerobic exercise: Aerobic exercise forces the heart and lungs to work harder for longer periods. It builds endurance, improves blood flow throughout the body, and increases the levels of "good" cholesterol.
Resistance Training: Resistance training works muscles against a force (usually weights). It burns fat and builds muscle.

Introduction

Everyone's goal of living a long and healthy life should include a healthy diet, regular exercise, and maintaining normal weight. The combination of inactivity and eating the wrong foods is the second most common preventable cause of death in the United States (smoking is the first).

Most research on the benefits of exercise focuses on heart protection. Studies clearly show that exercise helps the heart. In addition, new studies are reporting that even people at higher risk for heart disease may lower their risk of dying from it if they exercise.

Evidence suggests that our genes evolved to favor exercise. In other words, during prehistoric times, if a person couldn't move quickly and wasn't strong, he or she died. Those who were fit survived to reproduce and pass on these "fitter" genes. Some researchers believe that with our current inactive lifestyle, these genes produce a number of bad effects, which can lead to many chronic illnesses.

The benefits of exercise include:

Improved oxygen delivery throughout the body
Improved metabolic processes - the way the body breaks down and builds necessary substances
Improved strength and endurance
Decreased body fat
Improved movement of joints and muscles
Improved sense of well-being

In addition, exercise can help change other dangerous lifestyle habits. A 2007 review of existing studies found that moderate exercise, for as little as 5 minutes at a time, can help combat the nicotine withdrawal symptoms people experience when they try to stop smoking.

No one is too young or too old to exercise. The United States Surgeon General recommends at least 30 minutes of moderate exercise, such as brisk walking, nearly every day. However, vigorous exercise carries risks that people should discuss with a doctor. You should always check with your doctor before starting a new exercise program, especially if you have any of the following risk factors:

History of smoking
Obesity
Family history of a long-term disease
A symptom you haven’t told your doctor about
Chest pain
Shortness of breath
Heart palpitations
Blood clots
Infections
Fever
Unexplained weight loss
Foot or ankle sores that won’t heal
Joint swelling
Pain or trouble walking after a fall
Eye injury or eye surgery
Hernia
Hip surgery

Fifty percent of all people who begin a vigorous training program drop out within a year. The key to reaching and maintaining physical fitness is to find activities that are exciting, challenging, and satisfying.

Recommended Exercise Methods

A few simple rules are helpful as you develop your own routine.

Don't eat for 2 hours before vigorous exercise.
Drink plenty of fluids before, during, and after a workout.
Adjust your activity level according to the weather, and reduce it when you are fatigued or ill.
When exercising, listen to the body's warning symptoms, and consult a doctor if exercise causes chest pain, irregular heartbeat, undue fatigue, nausea, unexpected breathlessness, or light-headedness.

Heart rate is the standard guide for determining aerobic exercise intensity. It can be determined by counting one's own pulse or with the use of a heart rate monitor. To feel your own pulse, press the first two fingers of one hand gently down on the inside of the wrist or under the jaw on the right or left side of the front of the neck. You should feel a faint pounding as blood passes through the artery. Each pounding is a beat.

Click the icon to see how to take a radial pulse

Click the icon to see how to take a carotid pulse.

There are different types of heart rates.

Resting heart rate. The average heart rate for a person at rest is 60 - 80 beats per minute. It is usually lower for people who are physically fit, and often rises as you get older. You can determine your resting heart rate by counting how many times your heart beats in one minute. The best time to do this is in the morning after a good night’s sleep before you get out of bed.

Maximum heart rate. To determine your own maximum heart rate per minute subtract your age from 220. For example, if you are 45, you would calculate your maximum heart rate as follows: 220 - 45= 175.

Target heart rate. Your target rate is 50 - 75% of your maximum heart rate. You should measure your pulse off and on while your exercise to make sure you stay within this range. After about 6 months of regular exercise, you may be able to increase your target heart rate to 85% (but only if you can comfortably do so).

Certain heart medications may lower your maximum and target heart rates. Always check with your doctor before starting an exercise program.

Note: Swimmers should use a heart rate target of 75% of the maximum and then subtract 12 beats per minute. The reason for this is that swimming will not raise the heart rate quite as much as other sports because of the so-called "diving reflex," which causes the heart to slow down automatically when the body is immersed in water.


Age	Low	High
	(50% max.)	(75% max.)
20	100	150
30	95	142
40	90	135
50	85	127
60	80	120
Source: American Heart Association

VO2 Max. Serious exercisers may use a VO2 max calculation, which measures the amount of oxygen consumed during intensive, all-out exercise. The most accurate testing method uses computers, but anyone can estimate V02 without instrumentation (with an accuracy of about 95%):

After running at top pace for 15 minutes, round off the distance run to the nearest 25 meters.
Divide that number by 15.
Subtract 133.
Multiply the total by 0.172, then add 33.3.

Olympic and professional athletes train for VO2 max levels above 80. But for the average person interested in fitness, a VO2 max equaling between 50 and 80 is considered an excellent score for overall fitness.

Click the icon to see an image on exercise and heart rate.

Warming up and cooling down are important parts of every exercise routine. They help the body make the transition from rest to activity and back again, and can help prevent soreness or injury, especially in older people.

Warm-up exercises should be practiced for 5 - 10 minutes at the beginning of an exercise session. Older people need a longer period to warm up their muscles. Low-level aerobic exercise such as brisk walking, swinging the arms, or jogging in place, is the best approach.
To cool down, you should walk slowly until the heart rate is 10 - 15 beats above your resting heart rate. Stopping too suddenly can sharply reduce blood pressure, and is dangerous for older people. It may also cause muscle cramping.
Stretching may be appropriate for the cooling down period, but it must be done carefully for warming up because it can injure cold muscles. (There is no clear evidence, however, that stretching reduces muscle injuries.)

Warming up before exercise and cooling down after is just as important as the exercise itself. By properly warming up the muscles and joints with low-level aerobic movement for 5 - 10 minutes, one may avoid injury and build endurance over time. Cooling down after exercise by walking slowly, then stretching muscles, may also prevent strains and blood pressure fluctuation.

For most people, exercise may be divided into three general categories:

Aerobic or endurance
Strength or resistance
Flexibility

A balanced program should include all three. Speed training is also a major category, but generally only competitive athletes practice it.

Benefits of Aerobic Exercise. Regular aerobic exercise provides the following benefits:

Builds endurance
Keeps the heart pumping at a steady and high rate for a long time
Boosts HDL ("good") cholesterol levels
Helps control blood pressure
Strengthens the bones in the spine
Helps maintain normal weight
Improves one's sense of well-being

Types of Aerobic Exercise. Aerobic exercise is usually categorized as high or low impact. Examples of each include the following:

Low- to moderate-impact exercises: Walking, swimming, stair climbing, step classes, rowing, and cross-country skiing. Nearly anyone in reasonable health can engage in some low- to moderate-impact exercise. Brisk walking burns as many calories as jogging for the same distance and poses less risk for injury to muscle and bone.
High-impact exercises: Running, dance exercise, tennis, racquetball, squash. High-impact exercises should be performed no more than every other day, and less often for those who are overweight, elderly, out of condition, or have an injury or other medical problem that would rule out high-impact.

Click the icon to see an image of aerobic exercise.

Aerobic Regimens. As little as one hour a week of aerobic exercises is helpful, but 3 - 4 hours per week are best. Some research indicates that simply walking briskly for 3 or more hours a week reduces the risk for coronary heart disease by 65%. In general, the following guidelines are useful for most individuals:

For most healthy young adults, the best approach is a mix of low- and higher-impact exercise. Two weekly workouts will maintain fitness, but three to five sessions a week are better.
People who are out of shape or elderly should start aerobic training gradually. For example, they may start with 5 - 10 minutes of low-impact aerobic activity every other day and build toward a goal of 30 minutes per day, three to seven times a week. (For heart protection, frequency of exercises may be more important than duration.)
Swimming is an ideal exercise for many elderly and certain people with physical limitations, including pregnant women, individuals with muscle, joint, or bone problems, and those who suffer from exercise-induced asthma.
People who seek to lose weight should aim for six to seven low-impact workouts a week.

One way of gauging the optimal intensity of exercise is to aim for a "talking pace," which is enough to work up a sweat and still be able to converse with a friend without gasping for breath. As fitness increases, the "talking pace" will become faster and faster.

Shoes. All that's really necessary for a workout is a good pair of shoes that are made well and fit well. They should be broken in, but not worn down. They should support the ankle and provide cushioning for impact sports such as running or aerobic dancing. Airing out the shoes and feet after exercising reduces chances for skin conditions such as athlete's foot.

Clothing. Comfort and safety are the key words for workout clothing. For outdoor nighttime exercise, a reflective vest and light-colored clothing must be worn. Bikers, roller bladers, and equestrians should always wear safety devices such as helmets, wrist guards, and knee and elbow pads. Goggles are mandatory for indoor racquet sports. For vigorous athletic activities, such as football, ankle braces may be more effective than tape in preventing ankle injuries.

Aerobic-Exercise Equipment. Home aerobic exercise machines can be adapted to any fitness level and used day or night. Before investing in any exercise machine, however, it is wise to first test it at a gym. In addition, initial supervised training when using these machines can reduce the risk of injury that might occur with self-instruction.

Very inexpensive exercise machines tend to be flimsy and hard to adjust, but many sturdy machines are available at moderate prices. The higher-end models may utilize computers to record calories burned, speed, and mileage. While their readouts may provide motivation and gauge the intensity of a workout, however, they are not always accurate.

The following are a few observations on specific equipment:

A good floor mat is important to provide cushioning for all home exercises.
A simple jump rope improves aerobic endurance for people who are able to perform high-impact exercise. Jumping rope should be done on a floor mat plus a surface that has some give to avoid joint injury.
For burning calories, the treadmill has been ranked best, followed by stair climbers, the rowing machine, cross-country ski machine, and stationary bicycle. (Elliptical trainers, however, may be even better than treadmills for increasing heart rate, calorie expenditure, and oxygen consumption.)
Stationary bikes condition leg muscles and are fairly economical and easy to use safely. The pedals should turn smoothly, the seat height should adjust easily, and the bike's computer should be able to adjust intensity.
Stair machines also condition leg muscles. They offer very intense, low-impact workouts and may be as effective as running with less chance of injury.
Rowing and cross-country ski machines exercise both the upper and lower body.


Aerobic dancing	Sufficient cushioning to absorb shock and pressure that are many times greater than ordinary walking. Arches that maintain side-to-side stability. Thick upper leather support. Toe-box. Orthotics may be required for people with ankles that over-turn inward or outward. Soles should allow for twisting and turning.
Cycling	Rigid support across the arch to prevent collapse during pedaling. Heel lift. Cross-training or combination hiking/cycling shoes may be sufficient for casual bikers. Toe clips or specially designed shoe cleats for serious cyclers. In some cases, orthotics may be needed to control arch and heel and balance forefoot.
Running	Sufficient cushioning to absorb shock and pressure. Fully bendable at the ball of the foot. Sufficient traction on sole to prevent slipping. Consider insoles or orthotics with arch support for problem feet.
Tennis	Allow side-to-side sliding. Low-traction soles. Snug fitting heels with cushioning. Padded toe box with adequate depth. Soft-support arch.
Walking	Lightweight. Breathable upper material (leather or mesh). Wide enough to accommodate ball of the foot. Firm padded heel counter that does not bite into heel or touch ankle bone. Low heel close to ground for stability. Good arch support. Front provides support and flexibility.

Benefits of Strength Exercise. While aerobic exercise increases endurance and helps the heart, it does not build upper body strength or tone muscles. Strength-training exercises provide the following benefits:

Build muscle strength while burning fat
Help maintain bone density
Improve digestion

It is also associated with a lower risk for heart disease, possibly because it lowers LDL (the so-called "bad") cholesterol levels.

Click the icon to see an image of cholesterol.

Strength exercise is beneficial for everyone, even people in their 90s. It is the only form of exercise that can slow and even reverse the decline in muscle mass, bone density, and strength that occurs with aging. Please note: People at risk for cardiovascular disease should not perform strength exercises without checking with a doctor.

Types of Muscle Contractions. There are three types of muscle contractions involved in strength training:

Isometric contractions do not change the length of the muscle. An example is pushing against a wall.
Concentric contractions shorten muscles. An example is the "up" phase of a bicep curl.
Eccentric contractions lengthen muscles. An example is the "down" phase as weights are lowered.

Click the icon to see an image of isometric exercise.

Strength-Training Regimens. Strength training involves intense and short-duration activities. For beginners, adding 10 - 20 minutes of modest strength training two to three times a week may be appropriate. The following are some guidelines for starting a strength regimen:

The sequence of a strength training session should begin with training large muscles and multiple joints at higher intensity and end with small muscle and single joint exercises at lower intensities.
Both shortening and lengthening muscle actions should be performed. Emphasizing the movements that lengthen muscles is of increasing interest. This approach involves slowing and increasing the duration of these "down" movements. It appears to significantly increase blood flow, and some evidence suggests it may achieve stronger muscles more quickly. It may also improve heart function compared to standard movements. Exercises that lengthen muscles may be particularly beneficial for older people and some people with chronic health problems. This type of training increases the risk for muscle soreness and injury, however, and this approach is still controversial.
Strength training involves moving specific muscles in the same pattern against a resisting force (such as a weight) for a preset number of times. This is called a repetition. Students should first choose a weight that is about half of what would require a maximum effort in one repetition. In other words, if it would take maximum effort to do a single repetition with a 10-pound dumbbell, the person would start with a five-pound dumbbell. In the beginning, most people can start with one set of 8 - 15 repetitions per muscle group with low weights. As individuals are able to perform one or two repetitions over their routine, weights can be increased by 2 - 10%.
Breathe slowly and rhythmically. Exhale as the movement begins. Inhale when returning to the starting point.
The first half of each repetition typically lasts 2 - 3 seconds. The return to the original position lasts 4 seconds.
An alternative technique called "super slow" training stretches out one repetition to a 14-second count. This method places far more stress on the muscle group, so fewer repetitions are needed. A full week of recovery is required before repeating this workout. The goal is to initiate changes in the muscles so that the body continues to burn calories after the exercise. Some people report dramatic results from this approach, but scientific proof of these claims is not available. It is a very tedious workout, and people have a hard time sticking with it. People with high blood pressure should not use this approach.
Joints should be moved rhythmically through their full range of motion during a repetition. Do not lock up the joint while exercising it.
For maximum benefit, one should allow 48 hours between workouts for full muscle recovery.

Click the icon to see the proper way to breathe during exercise.

Strength-Training Equipment. Unlike aerobic exercise, strength training almost always requires some equipment. Strength-training equipment does not, however, have to cost anything.

Any heavy object that can be held in the hand, such as a plastic bottle filled with sand or water, can serve as a weight.
Dumbbells (1 - 10 pounds) and resistance bands are inexpensive, portable, and effective.
Wearable weights help strengthen and tone the upper body.
Ankle weights strengthen and tone muscles in the lower body. Wearable ankle weights should not be worn during high-impact aerobics or jumping.
Hand grips strengthen arms and are good for relieving tension.
A pull-up bar can be mounted in a doorway for chin-ups and pull-ups.

More elaborate and expensive home equipment for working body muscles is also available, costing from $100 to over $1,000. No one should purchase or use strength-training equipment without instruction from a professional.

Benefits of Flexibility Training. Flexibility training uses stretching exercises. Many stretching exercises are particularly beneficial for the back. In general, flexibility training provides the following benefits:

Prevents cramps, stiffness, and injuries
Improves joint and muscle movement (improved range of motion)

Certain flexibility practices, such as yoga and tai chi, also involve meditation and breathing techniques that reduce stress. Such practices appear to have many health and mental benefits. They may be very suitable and highly beneficial for older people, and for patients with certain chronic diseases.

Click the icon to see an image of flexibility exercise.

Flexibility Training Regiments. Doctors recommend performing stretching exercises for 10 to 12 minutes at least three times a week. The following are some general guidelines:

When stretching, exhale and extend the muscles to the point of tension, not pain, and hold for 20 - 60 seconds. (Beginners may need to start with a 5- to 10-second stretch.)
Breathe evenly and constantly while holding the stretch.
Inhale when returning to a relaxed position. Holding your breath defeats the purpose; it causes muscle contraction and raises blood pressure.
When doing stretches that involve the back, relax the spine to keep the lower back flush with the mat, and to work only the muscles required for changing position (often these are only the abdominal muscles).

Studies continue to show that it is never too late to start exercising. A report published in the February 2006 Journal of Aging and Health found that elderly adults who exercised twice a week for four months significantly increased their body strength, flexibility, balance, and agility. The exercise program included walking and lifting weights. The average age of the study participants was 83.5. The study adds further evidence that even small improvements in physical fitness and activity can prolong life and independent living.

Still, about half of Americans over 60 describe themselves as sedentary (inactive). According to a 2004 report by the Centers for Disease Control and Prevention, approximately 12% of people aged 65 - 75 years and 10% of people aged 75 years or older meet current recommendations for strength training.

The following tips for exercising may be helpful:

Any older person should have a complete physical and medical examination, as well as professional instruction, before starting an exercise program.
Start low and go slow. For sedentary, older people, one or more of the following programs may be helpful and safe: Low-impact aerobics, gait (step) training, balance exercises, tai chi, self-paced walking, and lower legs resistance training, using elastic tubing or ankle weights. Even in the nursing home, programs aimed at improving strength, balance, gait, and flexibility have significant benefits.
Strength training assumes even more importance as one ages, because after age 30 everyone undergoes a slow process of muscular erosion. The effect can be reduced or even reversed by adding resistance training to an exercise program. As little as one day a week of resistance training improves overall strength and agility. Strength training also improves heart and blood vessel health.
Power training, which aims for the fastest rate at which a muscle or muscle group can perform work, may be particularly helpful for older women in strengthening muscles and preventing falls.
Flexibility exercises promote healthy muscle growth and help reduce the stiffness and loss of balance that accompanies aging.
Chair exercises may be performed by people who are unable to walk.
Older women are at risk for incontinence accidents during exercise. This can be reduced or prevented by performing Kegel exercises, limiting fluids (without risking dehydration), going to the bathroom frequently, and using leakage prevention pads or insertable devices.

Exercise's Effects on the Heart

Inactivity is one of the major risk factors for heart disease. However, exercise helps improve heart health, and can even reverse some heart disease risk factors.

Like all muscles, the heart becomes stronger as a result of exercise, so it can pump more blood through the body with every beat and continue working at maximum level, if need be, with less strain. The resting heart rate of those who exercise is also slower, because less effort is needed to pump blood.

A person who exercises often and vigorously has the lowest risk for heart disease, but any amount of exercise is beneficial. Studies consistently find that light-to-moderate exercise is even beneficial in people with existing heart disease. Note, however, that anyone with heart disease should seek medical advice before beginning a workout program.

The heart is a large muscular organ that pumps blood throughout the body. Valves inside the heart open and close. This controls how much blood enters or leaves the heart.

Exercise has a number of effects that benefit the heart and circulation (blood flow throughout the body). These benefits include improving cholesterol and fat levels, reducing inflammation in the arteries, assisting weight loss programs, and helping to keep blood vessels flexible and open. Studies continue to show that physical activity and avoiding high-fat foods are the two most successful means of reaching and maintaining heart-healthy levels of fitness and weight.

The American Heart Association recommends that individuals perform moderately-intense exercise for at least 30 minutes on most days of the week. This recommendation supports similar exercise guidelines issued by the Centers for Disease Control and Prevention, and the American College of Sports Medicine.

Coronary Artery Disease. People who maintain an active lifestyle have a 45% lower risk of developing heart disease than do sedentary people. Experts have been attempting to define how much exercise is needed to produce heart benefits. In 2002, a well-conducted study on overweight adults confirmed previous research that reported beneficial changes in cholesterol and lipid levels, including lower LDL levels (bad cholesterol), even when people performed low amounts of moderate- or high-intensity exercise such as walking or jogging 12 miles a week. However, more intense exercise is required to significantly change cholesterol levels, notably increasing HDL (good cholesterol). An example of this kind of program would be jogging about 20 miles a week. Such benefits in the study occurred even with very modest weight loss, suggesting that overweight people who have trouble losing pounds can still achieve considerable heart benefits by exercising.

Some studies suggest that for the greatest heart protection, it is not the duration of a single exercise session that counts but the total daily amount of energy expended. Therefore, the best way to exercise may be in multiple short bouts of intense exercise, which can be particularly helpful for older people.

Resistance (weight) training has also been associated with heart protection. It may offer a complementary benefit to aerobics by reducing LDL levels. Exercises that train and strengthen the chest muscles may prove to be very important for patients with angina.

Effects of Exercise on Blood Pressure. Regular exercise helps keep arteries elastic (flexible), even in older people. This, in turn, ensures good blood flow and normal blood pressure. Sedentary people have a 35% greater risk of developing high blood pressure than athletes do.

Click the icon to see the risks associated with untreated hypertension.

It should be noted that high-intensity exercise may not lower blood pressure as effectively as moderate-intensity exercise. In one study, moderate exercise (jogging 2 miles a day) controlled high blood pressure so well that more than half the patients who had been taking drugs for the condition were able to discontinue their medication. However, a small study published in 2005 suggests that moderate exercise does not have a significant impact on systolic blood pressure (the top number) in older adults. While those who exercised did have notable drops in both the top and lower (diastolic) blood pressure levels, the only statistically significant change was the decrease in the lower number.

Experts recommend at least 30 minutes of exercise on most -- if not all -- days. Studies show that yoga and tai chi, an ancient Chinese exercise involving slow, relaxing movements, may lower blood pressure almost as well as moderate-intensity aerobic exercises.

Click the icon to see an image of someone practicing yoga.

Anyone with existing high blood pressure should discuss an exercise program with their doctor. Before starting to exercise, people with moderate-to-severe high blood pressure should lower their pressure, and be able to control it with medications. Everyone, and especially people with high blood pressure, should breathe as normally as possible through each exercise. Holding the breath increases blood pressure.

Effects of Exercise on Heart Failure. Traditionally, heart failure patients have been discouraged from exercising. Now, exercise performed under medical supervision is proving to be helpful for select patients with stable heart failure.

Studies continue to report benefits from exercise training. In one study, heart failure patients as old as 91 years old increased their oxygen use significantly, after 6 months of supervised treadmill and stationary bicycle exercises.
Progressive resistance training may be particularly useful for heart failure patients, since it strengthens muscles, which commonly weaken in this disorder. Even simply performing daily handgrip exercises can improve blood flow through the arteries.

Experts warn, however, that exercise is not appropriate for all heart failure patients.

All stroke survivors should have a pre-exercise evaluation done by their doctor before starting an exercise program.

The effects of exercise on stroke are less established than those on heart disease, but most studies show benefits. The following are some examples:

According to one major analysis, men cut their risk for stroke in half if their exercise program was roughly equivalent to about an hour of brisk daily walking 5 days a week. In the same study, exercise that involved recreation was more protective against stroke than exercise routines consisting simply of walking or climbing.
A 2000 study of women also found substantial protection from stroke in brisk walking or striding (rather than casual walking).

Anyone with heart disease or risk factors for developing heart disease or stroke should seek medical advice before beginning a workout program. Patients with heart disease can nearly always exercise safely as long as they work out under medical supervision. Still, it is often difficult for a doctor to predict health problems that might arise as the result of an exercise program. At-risk individuals should be very aware of any symptoms warning of harmful complications while they exercise.

Some experts believe that anyone over 40 years old, whether or not they are at risk for heart disease, should have a complete physical examination before starting or intensifying an exercise program. Some doctors use a questionnaire for people over 40 to help determine whether they require such an examination. The questions they use are as follows:

Has any doctor previously recommended medically supervised activity because of a heart condition?
Is chest pain brought on by physical activity?
Has chest pain occurred during the previous month?
Does the person faint or fall over from dizziness?
Is bone or joint pain intensified by exercise?
Has medication been prescribed for hypertension (high blood pressure) or heart problems?
Is the person aware of or has a doctor suggested any physical reason for not exercising without medical supervision?

Those who answer "yes" to any of the above questions should have a complete medical examination before developing an exercise program.

Stress Test. A stress test helps determine the risk for a heart problem resulting from exercise. Anyone with a heart condition or history of heart disease should have a stress test before starting an exercise program. Experts currently also recommend this test before a vigorous exercise program for older persons who are sedentary, even in the absence of known or suspected heart disease. The test is expensive, however, and some experts believe that it may not be necessary for many older people with no evident health problems or risk factors.

A small percentage of heart attacks occur after heavy physical work.

High-Risk Individuals. In general, the following people should avoid intense exercise or start it only with careful monitoring:

People who have certain medical conditions: These conditions include uncontrolled diabetes, uncontrolled seizures, uncontrolled high blood pressure, a heart attack within the previous 6 months, heart failure, unstable angina, significant aortic valve disease, or aortic aneurysm.
People with moderate-to-severe hypertension: Experts generally recommend that moderate or severe high blood pressure (systolic blood pressure over 160 mm Hg or diastolic (bottom number) pressure over 100 mm Hg) should be brought to lower levels before a person starts a vigorous exercise program.
Sedentary people should be cautious. One major study found that sedentary people who throw themselves into a grueling workout significantly increase their risk of heart attack.
Episodes of exercise-related sudden death in young people are rare but of great concern. Some are preceded by fainting, which is due to a sudden and severe drop in blood pressure. It should be noted that fainting is relatively common in athletes, and is dangerous only in people with existing heart conditions. Young people with genetic or congenital (present at birth) heart disorders should avoid intensive competitive sports.
Anabolic steroids or products containing ephedra have been associated with cases of stroke, heart attack, and even death.

The risk for heart attack from exercise should be kept in perspective, however. Some form of exercise, carefully personalized, has benefits for most of the individuals mentioned above. In many cases, particularly when the only risk factors are a sedentary lifestyle and older age, exercise can often be increased over time until it is intense.

Hazardous Activities for High-Risk Individuals. The following activities may pose particular dangers for high-risk individuals:

Intense workouts (snow shoveling, slow jogging, speed walking, tennis, heavy lifting, heavy gardening) may be particularly hazardous for people with risk factors for heart disease, especially older people. They tend to stress the heart, raise blood pressure for a brief period, and may cause spasms in the arteries leading to the heart. (See image: Coronary Artery Spasm)
Some studies suggest that competitive sports, which couple intense activity with aggressive emotions, are more likely to trigger a heart attack than other forms of exercise.

Listening for Warning Signs. It should be noted that according to one study, at least 40% of young men who die suddenly during a workout have previously experienced, and ignored, warning signs of heart disease. In addition to avoiding risky activities, the best preventive tactic is simply to listen to the body and seek medical help at the first sign of symptoms during or following exercise. These symptoms include the following:

Irregular heartbeat
Shortness of breath
Chest pain

Click the icon to see an image of a coronary artery spasm.

Click the icon to see an image of stable angina.

Exercise's Effects on Diabetes

Moderate aerobic exercise can lower your risk for type 2 diabetes. An important study found that adults who worked out 2 and 1/2 hours a week cut their risk by 58%.

Exercise has positive benefits for those who have diabetes. It can lower blood sugar, improve insulin sensitivity, and strengthen the heart. Strength training, which increases muscle and reduces fat, may be particularly helpful for people with diabetes, but more evidence is needed to confirm this theory. One study reported that yoga helped patients with type 2 diabetes reduce their need for oral medications.

In 2005, researchers found that people with type 2 diabetes who walked a minimum of 3 miles every day were in better health, and had lower medical expenses, after 2 years of such exercise. Those who remained sedentary for that time period experienced a decline in their overall health and higher health care-related expenses. Study participants who worked out for an average of 38 minutes per day lowered their blood pressure, cholesterol ,and A1C levels (glucose concentration over time). These participants also had lower heart disease risk, even if they didn't lose weight. The increase in the study participants' activity equaled about 2,200 extra steps a day. The findings were reported in the journal Diabetes Care.

An earlier study found that healthy lifestyle changes may work better than the prescription medication metformin (Glucophage), when it comes to preventing metabolic syndrome. Metabolic syndrome is a combination of risk factors including abdominal obesity, insulin resistance, high triglycerides, and hypertension.

The following are precautions for all people with diabetes, whether type 1 or 2:

Because people with diabetes are at higher than average risk for heart disease, they should always check with their doctors before starting a demanding exercise program. For best and fastest results, frequent high-intensity (not high-impact) exercises are best for people who are cleared by their doctor. For people who have been sedentary, or have other medical problems, lower-intensity exercises are recommended, using programs the patients designed with their doctors.
Strenuous strength training or high-impact exercise is not recommended for people with uncontrolled diabetes. Such exercises can strain weakened blood vessels in the eyes of patients with retinopathy (a common diabetic complication). High-impact exercise may also injure blood vessels in the feet.

Patients who are taking medications that lower blood glucose, particularly insulin, should take special precautions before starting a workout program.

Glucose levels swing dramatically during exercise. People with diabetes should monitor their levels carefully before, during, and after workouts.
Patients should probably avoid exercise if glucose levels are above 300 mg/dL or under 100 mg/dL.
To avoid hypoglycemia (low blood sugar), people with diabetes should inject insulin in sites away from the muscles they use the most during exercise.
People with diabetes should drink plenty of fluids. Before exercising, they should avoid alcohol, which increases the risk of hypoglycemia.
Insulin-dependent athletes may need to decrease insulin doses, or take in more carbohydrates, prior to exercise. However, they may need to take an extra dose of insulin after exercise. Stress hormones released during exercise may increase blood glucose level (in people without diabetes, insulin is released to control this increase). People with diabetes must regularly test their blood sugar, and take any medications as instructed by their doctor.

A person with diabetes must regularly check their blood sugar (glucose) level.

Exercise's Effects on Bones and Muscles

Exercise is critical for strong muscles and bones. Muscle strength declines as people age, but studies report that when people exercise they are stronger and leaner than others in their age group.

Exercise helps kids lower their risk of chronic pain in the future. Research has shown that it helps them prevent back and neck pain. The more flexible men are as teenagers, the lower their risk of neck tension in the future, according to a study published in the February 2006 British Journal of Sports Medicine. The same report found that women who had the greatest endurance strength as teenagers had a lower risk of tension neck than those with lower teenager endurance strength. However, men with the greatest endurance strength had higher rates of knee injuries later on.

Joints are complex structures. They are designed to bear weight and move the body. Above the knee is the femur (thigh bone). Below the knee is the tibia (shin bone) and fibula. The kneecap is also called the patella. It rides on top of the lower portion of the femur and the top portion of the tibia. The muscles and ligaments connect these bones and the space between them is cushioned by fluid-filled capsules (synovia) and cartilage. When you exercise, the muscles pull on the bones, strengthening them. The range of motion of a joint represents how far it can be flexed (bent) and extended (stretched).

Joints require motion to stay healthy. Long periods of inactivity cause the arthritic joint to stiffen and the adjoining tissue to weaken. A moderate exercise program that includes low-impact aerobics, power, and strength training has benefits for osteoarthritic patients, even if exercise does not slow down the disease progression. Many patients who start an exercise program report less disability and pain. They are also better able to perform daily chores, and remain independent longer than their inactive peers. Older patients and those with medical problems should always check with their doctor before starting an exercise program.

Click the icon to see an image of osteoporosis.

The following are useful exercises for osteoarthritis patients:

Strengthening exercises builds muscle strength. Some experts encourage patients to emphasize strengthening leg muscles as a first treatment step, even before using pain relievers. They fear that patients who rely on painkilling drugs may overuse knees, which do not have strong enough muscle tissue to protect the joints from further damage. Strengthening the thigh muscles is certainly protective for those who have not developed osteoarthritis.
Range-of-motion exercises increase the amount of movement in a joint and muscle. The best examples are yoga and tai chi, which focus on flexibility, balance, and proper breathing. In one 2001 study, older adults who practiced the gentle movement, breathing, and meditation exercises of tai chi for 10 weeks reported less pain than their peers who did not learn the technique.
Low-impact aerobic workouts help stabilize and support the joints. Cycling and walking are beneficial, and swimming or exercising in water is highly recommended for people with arthritis. Patients with arthritis should avoid high-impact sports, such as jogging, tennis, and racquetball.
Some researchers are now focusing on "power" training, which involves improving the muscle's ability to move more rapidly against resisting forces, such as gravity. For example, such training helps people stand up or climb stairs more quickly. Muscle power declines more rapidly than muscle strength, and may be particularly important in older people.

Exercise is very important for slowing the progression of osteoporosis, and extremely important for reducing the risk of falling, which causes fractures. Falls are one of the leading causes of death in people over the age of 65. Exercise helps build balance and flexibility, which reduces the risk of falling.

Specific exercises may be especially helpful for reducing the risk of fractures:

Weight-bearing exercise is very beneficial for bones in people of all ages, even older people. This approach applies tension to muscle and bone, and the body responds to this stress by increasing bone density, in young adults by as much as 2 - 8% a year. Careful weight training can also be very beneficial for elderly people, particularly women. In addition to improving bone density, weight-bearing exercise reduces the risk of fractures by improving muscle strength and balance, thus helping to prevent falls.
Regular brisk long walks improve bone density and mobility. In one 2002 study, for example, older women reduced their risk of hip fracture by over 40% by working out just four hours a week.
Exercises specifically targeted to strengthen the back can be beneficial in improving posture, and may even reduce kyphosis (hunchback) in people with osteoporosis.
Low-impact exercises, particularly yoga and tai chi, which improve balance and strength, have been found to decrease the risk of falling. In one study, tai chi reduced this risk by almost half.

Click the icon to see an image of the bone-building exercise.

Note on Female Athlete Triad. Some young female athletes who exercise very intensely, and are subject to intense pressure to remain thin, are at risk for the female athlete triad. This syndrome is a combination of three disorders -- an eating disorder, loss of menstrual periods, and osteoporosis.

People who do not exercise regularly face an increased risk for low back pain, especially during times when they suddenly have to perform stressful, unfamiliar activities. These activities may include shoveling, digging, or moving heavy items. Although no definitive studies have been done to prove the relationship between lack of exercise and low back pain, sedentary living is probably a primary nonmedical cause contributing to this condition.

Lack of exercise leads to the following conditions that may threaten the back:

Muscle inflexibility can restrict the back's ability to move, rotate, and bend.
Weak stomach muscles can increase the strain on the back and can cause an abnormal tilt of the pelvis (hip bones).
Weak back muscles may increase the load on the spine and the risk of disk compression.
Obesity puts more weight on the spine and increases pressure on the vertebrae and disks. Studies report only a weak association between obesity and low back pain, however.

Benefits for Chronic Back Pain. People in with sudden and severe back pain should not exercise. Exercise plays a very beneficial role in relieving chronic back pain, however. In one study, patients with back pain lasting for an average of 18 months were assigned eight 1-hour exercise sessions over 4 weeks. They showed greater improvement in nearly every area, including reduced pain, compared to patients who did not exercise.

Exercise should be considered as part of a broader program to return to normal home, work, and social activities. In this way, the positive benefits of exercise not only affect strength and flexibility but they also alter and improve the patients' attitudes toward their disability and pain.

Repetition is the key to increasing flexibility, building endurance, and strengthening the specific muscles needed to support the spine. Some exercise programs used for prevention or treatment of chronic low back pain include the following:

Low-impact Aerobic Exercises: Low-impact aerobic exercises, such as swimming, bicycling, and walking, can strengthen muscles in the abdomen and back without over-straining the back. Programs that use strengthening exercises while swimming may be a particularly beneficial approach for many patients with back pain. In one study, for example, pregnant women who engaged in a water gymnastics program had less back pain, and were able to continue working longer.
Lumbar Extension Strength Training: Exercises called lumbar extension strength training are proving to be effective. Generally, these exercises attempt to strengthen the abdomen, and improve lower back mobility, strength, and endurance. They also enhance flexibility in the hip and hamstring muscles, and in the tendons at the back of the thigh.
Yoga, Tai Chi, and Chi Kung: These exercises combine low-impact physical movements and meditation. They are based on principles of disciplining the mind to achieve a physical and mental balance, and can be very helpful in preventing recurrences of low back pain. In one study of Pilates, an exercise practice that uses yoga principles, the exercises were helpful in a woman with progressive and disabling severe low back pain resulting from early scoliosis. This approach deserves further research.
Flexibility Exercises: Whether flexibility exercises alone offer any significant benefit for chronic back pain is uncertain. One study suggested that any benefits derived from flexibility exercises are lost unless the exercise programs are sustained.
Retraining Deep Muscles: Studies are finding a link between low back pain and poor motor control of deep muscles in the back and trunk. According to these studies, contraction exercises specifically designed to retrain these muscles may be effective for patients with both acute and chronic pain.

It is important for any person who has low back pain to have an exercise program guided by professionals who understand the limitations and special needs of back pain and who can address individual health conditions. One study indicated that patients who planned their own exercise did worse than those in physical therapy or doctor-directed programs.

Hazardous Effects on the Back. Improper or excessive exercise can also cause back pain.

Exercise's Effects on the Lungs

Patients with chronic lung problems have difficulty exercising. Shortness of breath is a major limitation in most patients, but in about a third, muscle fatigue is an even greater problem. Although exercise does not improve lung function, training helps many patients with chronic lung disease by strengthening their limb muscles, thus improving endurance and reducing breathlessness.

In people who already have colds, exercise has no effect on the illness' severity or duration. People should avoid strenuous physical activity when they have fevers, muscle aches, or other symptoms of a widespread viral illnesses.

Long-term exercise may help control asthma and reduce hospitalization. One 2000 study found that aerobic exercise improves breathing capacity and function in patients with mild asthma. People with asthma who enjoy running should probably choose an indoor track, to avoid pollutants. Swimming is particularly excellent for people with asthma. Yoga practice, which uses both stretching, breathing, chest expansion, and meditation techniques may have specific benefits that include stress reduction as well as airway opening. One study reported that two thirds of patients who practiced yoga regularly were able to reduce or eliminate their asthma medications.

Exercise-Induced Asthma. About 40 - 90% of asthma cases are exercise-induced asthma (EIA), in which exercise triggers coughing, wheezing, or shortness of breath. It occurs most often in children and young adults and during intense exercise in cold dry air. EIA is triggered only by exercise. Unlike allergic asthma, there is no long-term increase in airway activity. People who only have EIA do not require long-term maintenance therapy. The warm-up and cool-down periods, which are important for any exercise regimen, may help reduce EIA events. A study of military recruits found that exercise-induced asthma attacks did not hinder their ability to perform or train, suggesting that EIA is not a reason to exclude people from physically demanding occupations.

Exercise-induced asthma is distinct from allergic asthma in that it does not produce long-term increase in airway activity. People who only experience asthma when they exercise may be able to control their symptoms with preventive measures such as warm-up and cool-down exercises.

Walking is the best exercise for people with emphysema. Patients should try to walk three to four times daily for 5 - 15 minutes each time. Devices that assist ventilation may reduce breathlessness that occurs during exercise.

Inspiratory muscle training involves exercises and devices that make inhaling (breathing in) more difficult, in order to strengthen breathing muscles. In a 2001 study, patients who took part in an inspiratory muscle training group improved their breathing, walking capacity, and quality of life. Yoga or martial arts exercises, such as tai chi, which emphasize breathing techniques and balanced movements, may be particularly beneficial for patients with emphysema.

Exercise's Effects on Weight

Exercising helps people reduce their weight, maintain weight loss, and fight obesity. Research has shown that women who regularly exercise but do not change their diet can lose significantly more weight than less active women.

Thirty minutes of moderate-intensity exercise may be adequate to maintain cardiovascular health, but it might not prevent weight gain. Recommendations published in 2003 and 2004 suggest that 45 - 60 minutes of exercise per day is necessary to promote weight loss. Children may need more activity.

Losing significant weight requires both exercise and calorie restriction. In addition, if a person exercises without dieting, any actual weight loss may be minimal because dense and heavier muscle mass replaces fat. Nonetheless, regardless of weight loss, a fit body will look more toned and be healthier.

People who exercise are more apt to stay on a diet plan. Exercise improves psychological well-being and replaces sedentary habits that usually lead to snacking. Exercise may even act as a mild appetite suppressant.

Exercising without dieting still adds health benefits. One study found that overweight but fit people have half the death rate of overweight, unfit people. Research suggests that people who have trained for a long time develop more efficient mechanisms for burning fat and are able to stay leaner.

Lifting weights builds muscle, which burns calories more efficiently than other body tissues.

The following are some suggestions and observations on exercise and weight loss:

The treadmill burns the most calories of standard aerobic machines. It may be particularly effective when used in short multiple bouts during the day. Exercise sessions as short as 10 minutes, which are done frequently (about four times a day), may be the most successful program for obese people.
The more strenuous the exercise, the longer the body continues to burn calories before returning to its resting level. This state of fast calorie burning can last for as little as a few minutes after light exercise, to as long as several hours after prolonged or heavy exercise.
Resistance (strength) training is excellent for replacing fat with muscles. It should be performed two or three times a week.
Fidgeting may be very helpful in keeping pounds off. Regular exercise is certainly the best course, but for people who must sit for hours at work, frequently shifting positions while sitting may have some benefit.
It is important to realize that as people slim down, they burn fewer calories per mile of walking or jogging. The rate of weight loss slows down, sometimes discouragingly so, after an initial dramatic head start using diet and exercise combinations. People should be aware of this trend and keep adding to their daily exercise routine.
Changes in fat and muscle distribution may differ between men and women as they exercise. Men tend to lose abdominal fat (which lowers their risk for heart disease faster than reducing general body fat). Exercise, however, does not appear to have the same effect on weight distribution in women. A study of women who practiced aerobic and strength training showed the training resulted in fat loss in the women's arms and trunk. However, they did not gain muscle tissue in those areas.

Because obesity is one of the risk factors for heart disease, anyone who is overweight must discuss their exercise program with a physician before starting.

Exercise's Effects on Other Conditions

Physical activity makes you healthier. It lowers your risk for cardiovascular disease and reduces bone loss. Physical activity also helps the body use calories more efficiently, which helps you eliminate body fat and lose weight. It also helps you maintain weight loss by increasing your metabolism and reducing your appetite.

A number of studies have indicated that regular exercise may reduce the risk of breast, colon, and possibly prostate cancers.

Studies confirm that exercise significantly reduces the risk of both colon cancer (by up to 50%) and breast cancer (by up to 30%).

A 2006 study found that, though protection from breast cancer may vary among the types of tumor, exercise offered the most marked protection from the more aggressive tumors. A second study, also done in 2006, supported this finding. Several studies also suggested that more intense exercise is more protective against breast cancer. Exercising consistently throughout life gives the best protection. Exercise not only lowers a woman's chance of getting breast cancer, it can help those who have received chemotherapy for the disease fight off fatigue.

While endurance athletes may suffer from stomach problems, low intensity exercise has a marked protective effect against colon cancer, according to studies, including the Nurses Health Study and the American Cancer Society's Cancer Prevention Study II. Furthermore, a 2006 study found that people with colon cancer who exercise reduce their risk of a recurrence.

Exercise also has a beneficial effect on people receiving treatment for prostate cancer. A new study found that aerobic and resistance training significantly reduced fatigue in men undergoing radiation treatments for prostate cancer. Fatigue is a common side effect of such treatments. In this study, 122 patients received supervised aerobic training, resistance training, or neither. At the end of 24 weeks, participants in both exercise groups noted significant improvement in their fatigue symptoms, compared to the control group. Participants in the resistance training group also lost a significant percentage of their body fat.

Endurance athletes often report stomach problems, such as bloating, diarrhea, and gas, even at rest. Experts suggest that moderate regular exercise might reduce the risk for some intestinal disorders. These disorders include ulcers, irritable bowel syndrome, indigestion, and diverticulosis. Older people who exercise moderately may have a lower risk for severe gastrointestinal bleeding.

Patients with end-stage kidney disease who exercise four to five times per week have better survival rates than those who are less active, according to researchers involved in the Dialysis Morbidity and Mortality Wave 2 study. However, the majority of study participants said that severe physical limitations prevented them from exercising so often.

Studies have shown that regular exercise, particularly walking, helps reduce one's risk for memory loss. A 2005 study found that older men who walked less than a mile daily had a 71% higher risk of dementia than those who walked more than two miles a day. A 2006 study found that people older than 65 who exercise regularly had lower risk of developing dementia, particularly Alzheimer's disease. An earlier study found that walking regularly protects women from mental decline. To date, there are no clear explanations for this apparent benefit. A preliminary study in mice suggests that physical activity changes the way brain-damaging proteins are processed in the brain, thus slowing the development of Alzheimer's disease. Aerobic exercise has been linked with improved reaction time, perception, and math skills in people of all ages.

Doctors found that exercise improves the physical and emotional well-being of patients who already have Alzheimer's disease. The patients exercised moderately for as little as 60 minutes each week. Doctors noted patients who exercised were less depressed, wandered away less, suffered fewer falls, and were placed in nursing homes later, compared to patients who did not exercise.

People with existing neurological diseases, such as multiple sclerosis, Parkinson's disease, and Alzheimer's disease, should be encouraged to exercise. Specialized exercise programs that improve mobility are particularly valuable for patients with Parkinson's disease. Patients with neurological disorders who exercise experience less stiffness, as well as reduction in, and even reversal of, muscle wasting. In addition, the psychological benefits of exercise are extremely important in managing these disorders. Exercise machines, aquatic exercises, and walking are particularly useful.

Some research has suggested that exercise may have antidepressant effects. Although there is little strong evidence that exercise can help manage depression, a number of studies have suggested benefits. Research findings include:

Just 30 minutes of brisk exercise three times a week was as effective as medication in relieving symptoms, and reducing relapse, in many patients with mild-to-moderate depression.
Over half of older women with depression that did not respond to medication improved with 10 weeks of exercise. (About a third of women who did not exercise also improved during that time.)
Studies on elderly, depressed patients report modest benefits from exercise, even in those who do not response to antidepressants. Simply participating in a group activity may help improve mood.
Teenagers who are active in sports have a greater sense of well-being than their sedentary peers. The more vigorously they exercise, the better their emotional health.
Physical inactivity is strongly linked to depression in children 8 - 12 years of age.

Specific exercises may be particularly beneficial:

Aerobics. Either brief periods of intense training or prolonged aerobic workouts can raise levels of certain chemicals in the brain. These chemicals -- which include endorphins, adrenaline, serotonin, and dopamine -- produce the so-called runner's high. Weight loss and increased muscle tone can boost self-esteem.

Yoga. Yoga practice, which involves rhythmic stretching movements and breathing, has been found to positively affect mood. It may have clinical potential as a technique for improving and stabilizing mood. A study comparing yoga to aerobic exercise found that men have significantly lower levels of tension, fatigue, and anger after yoga, compared with levels after swimming. Yoga and swimming tended to produce equal benefits in women.

Click the icon to see an image of the benefits of yoga.

Moderate exercise in healthy pregnant women does not increase the risk for miscarriage, preterm labor, or rupture of the membrane. Not exercising increases the risk for complications, including low-birth weight babies. Exercising increases the fetal heart rate, which in turn protects the baby.

Healthy women with normal pregnancies should exercise at least three times a week, being careful to warm up, cool down, and drink plenty of liquids. Many prenatal calisthenics programs are available.

The following are specific exercises that may benefit the pregnant woman:

Swimming and water aerobics may be the best option for most pregnant women. Swimming has special benefits for those with fluid buildup. Water exercises involve no impact, overheating is unlikely, and swimming face down promotes optimum blood flow to the uterus.
Performing yoga exercises under the guidance of informed instructors can be very helpful.
Walking is also beneficial.

To strengthen pelvic muscles, women should perform Kegel exercises at least six times a day. This involves contracting the muscles around the vagina and urethra for three seconds 12 - 15 times in a row.

Experts generally recommend the following precautions for pregnant women who exercise:

Fit women who have exercised regularly before pregnancy may work out intensely as long as the doctor approves and no discomfort occurs.
As a rule for previously sedentary, low-risk expectant mothers, the pulse rate should not exceed 70 - 75% of the maximum heart rate, or more than 150 beats per minute. Any sedentary expectant mother should check with her doctor before starting an exercise program.
According to one study, vigorous exercise may improve the chances for a timely delivery. All pregnant women, however, should avoid high-impact, jerky, and jarring exercises, such as aerobic dancing, which can weaken the pelvic floor muscles that support the uterus.
During exercise, women should monitor their temperature to avoid overheating, a side effect that can damage the fetus. (Pregnant women should also not use hot tubs or steam baths, which can cause fetal damage and miscarriage.)

Note: Strenuous exercise may affect the flavor of breast milk for a short time afterward. Nursing mothers who engage in such activity might want to wait about an hour after exercising before they feed their infant.

Complications

Exercise may lead to injury if not done properly. Always exercise with care.

Competitive running or high-impact aerobics pose a high risk of a number of injuries to the bones and muscle. The effect of high-impact exercise on the back is not entirely clear. Some research suggests that over time, high-impact exercise may increase the risk for degenerative disk disease. A survey of people who played tennis, however, found no increased risk for low back pain or sciatica.

High-impact exercise can also cause dizziness, ringing in the ear, motion sickness, or loss of high-frequency hearing.

Some research further suggests that in people unused to exercise, intense activity increases production of harmful particles in the body called free radicals. These unstable oxygen particles injure muscle tissue. Muscle pain in this case does not occur until 24 - 48 hours after exercise.

Some people have a higher than average risk for injury:

About half of people at any age who participate in competitive running or high-impact aerobics experience minor injuries at least once a year. Young, intensely competitive athletes may be at risk for permanent injury. Studies are mixed over whether intensive high-impact sports in younger people cause long-term degenerative joint disease.
As the number of older people who start exercising increases, there has also been an increase in injuries for this age group. Between 1990 and 1996, injuries from active sports increased by 54% in people age 65 and older.
Women are far more likely than men to suffer knee injuries.
Urinary incontinence affects many female athletes who engage in high-impact exercise.
Tennis players are at high risk for injuries from repetitive force on the shoulder joint.

Preventing High-Impact Injuries. The following may be helpful for preventing injury:

Wear shock-absorbing footwear with weight-dampening inserts.
Combine weight lifting with jumping exercises. This may prevent injury by strengthening hamstrings and improving coordination.
Vary training and alternate easy and harder workouts.
Be careful to warm up, cool down, and stretch. Flexibility is the key to preventing many muscle strains.
Take days off now and then. The risk of injury increases when athletes train more than five times a week.

Because of the association between high-impact exercises and oxidation, some experts suggest eating foods rich in antioxidants, such as vitamins A, C, and E. Such foods, which may protect against damage from free radicals, include many fresh fruits and vegetables.

Treating Minor Injuries. Most mild or moderate injuries respond well to a simple, four-step treatment: rest, ice, compression, and elevation (RICE). This combination works well for both spot injuries and chronic problems. Ice packs, which reduce inflammation and pain, can help new injuries, and can be useful for the first few hours after a chronically injured area is exercised. How much or how long to compress the injury is unclear.

Evidence suggests that early movement is helpful, although taping or bracing in people with a recurrent ankle sprain is known to be protective. It may not be helpful in those without a previous ankle injury.

Minor injuries like sprains may be treated at home if broken bones are not suspected. The acronym RICE can help you remember how to treat minor injuries: "R" stands for rest, "I" is for ice, "C" is for compression, and "E" is for elevation. Pain and swelling should decrease within 48 hours. Gentle movement may help, but pressure should not be put on a sprained joint until pain is completely gone. This can take up to a few weeks.

Heat, ultrasound, whirlpool, and massage may speed healing if applied a day or two after the initial injury or for warm-up before another workout session.

Some young female athletes who exercise very intensely, and are subject to intense pressure to remain thin, are at risk for a syndrome known as the female athlete triad. This combination of symptoms includes loss of menstruation, eating disorders, and osteoporosis. Eating disorders among young female athletes are estimated at 15 - 62%. Women at higher risk include ballet dancers, gymnasts, and divers. Continued intense exercise causes a stress response in which estrogen (the primary female hormone) is lost. Estrogen loss can lead to infertility and osteoporosis. Iron loss and anemia may also be a problem in women who exercise frequently, even at moderate intensity. A doctor should be consulted for any of these concerns.

Incorrect movements can literally cause mechanical problems in the muscles. These problems are usually the result of improper exercise instruction, and lack of attention. A single jerky golf swing, or the incorrect use of exercise equipment (especially free weights, nautilus, and rowing machines), can cause serious back injuries.

Between 30 - 70% of cyclists experience low back pain. Pain may be improved by adjusting the angle of the bicycle seat.

Everyone should drink lots of fluid during intense exercise. Thirst is often a poor indicator of dehydration in people who exercise, particularly older people. During a tough workout in a hot environment, the body can lose two liters of fluid per hour through sweat.

Anyone who exercises intensely should take the following precautions:

Drink 6 - 8 ounces of fluid about 15 minutes before a workout, and then pause regularly during exercise to drink more.
Water is the best choice for replenishing body fluids. Glucose-sodium-potassium solutions, the so-called "sports drinks," which promise instant energy, appear to be no better than water at improving endurance during prolonged intense running.
Caffeinated beverages like coffee and soft drinks give short bursts of energy, but can actually cause fluid loss. Caffeine before a workout has been shown to temporarily raise blood pressure, and reduces blood flow to inactive limbs.

Contrary to popular belief, drinking fluids will not cause cramps. Drinking enough, in fact, helps prevent the painful involuntary muscle spasms that sometimes occur during exercise.

Overheating, or hyperthermia, can be a problem with hard exercise, or when working out in hot weather. Overheating can cause mild to life-threatening conditions. Heat exhaustion, a moderate form of hyperthermia, is characterized by the following symptoms:

Lightheadedness, nausea, headache, hyperventilation, fatigue, and loss of concentration
A high temperature (above 103° F), possibly accompanied by complaints of chills and clammy skin

Individuals should rest in a cool, dry place, drink plenty of fluids, and bring down their body temperature with ice packs pressed against the skin.

Heatstroke. Heatstroke is the most dangerous complication of hyperthermia. The victim may suddenly stop sweating, after which symptoms such as altered consciousness, seizures, and even coma may quickly follow. Heat stroke is a medical emergency and requires immediate cooling of the victim in an ice-water bath or with ice packs. One study suggests that risk for serious complications from exercising in high temperatures may persist as late as the following day, even if the weather has cooled down.

Click the icon to see an image of the dangers of heatstroke.

Precautions are also necessary in cold weather. When exercising in winter dress in layers, including gloves and socks, which create insulated air pockets that trap heat. In cold weather, wear shoes with less ventilation than those worn in the summer. Fingers, toes, ears, and nose are most susceptible to frostbite. Frostbite progresses from stinging or aching to numbness. Fingers and toes may become white. Soaking the hands and feet in warm water can help, but only once there is no risk of refreezing, since a second bout of frostbite after thawing can quicken tissue damage.

Hypothermia can be life-threatening and can occur even after long exposure to temperatures that are above freezing. The condition is characterized by extreme fatigue, mental confusion, apathy, and a lack of coordination. The victim should be warmed as soon as possible with blankets, body heat, and warm fluids.

Motivation

Motivation, or a lack thereof, is one reason many people stop exercising. Here are some tips for avoiding burnout:

Think of exercise as a menu rather than a diet. Choose a number of different physical activities that are personally enjoyable such as sports, dancing, or biking. Although experts say you should get 30 minutes of aerobic exercises at least five times a week, those times can be divided into shorter periods -- such as 10 minute sessions. In addition, people can achieve health benefits from other exercise programs, including weight training, yoga, or tai chi.
Stick to a prepared schedule and record progress.
Develop an interest or hobby that requires physical activity.
Adopt simple routines such as climbing the stairs instead of taking the elevator, walking instead of driving to the local newsstand, or canoeing instead of zooming along in a powerboat.
Try cross training (regularly switching from one type of exercise to another). Studies suggest it is more beneficial than focusing only on one form of exercise.
Exercise with friends.
Join a gym or take classes. Many affordable programs are available.
For those who can afford them, personal trainers can be very helpful and are available in many gyms and exercise clubs. Personal trainers without any connection to a well-reputed gym or fitness club should be certified by a major fitness organization, such as the Aerobics and Fitness Association of America (AFAA) or the American Council on Exercise.
Exercise videos may also be helpful, but people should be sure they are suited to their individual age and health needs, and bear the seal of the AFAA.
Consider getting a dog. A study in the February 2006 American Journal of Preventive Medicine found that dog owners in Canada walk almost twice as much as those who don’t own a dog. Regular walking is a good way to improve health.

Differences in Motivation Between Men and Women. Motivation factors may differ by gender, and women appear to have a harder time. In one study, weight loss was the greatest motivator to exercise for women, and muscle tone was the primary motivator for men. Unfortunately, effects on appearances may take a long time to show, discouraging people from continuing an exercise program even though their health is improving.

Overweight among children and adolescents has now become an epidemic in the United States. Experts say that children should be vigorously active for at least 20 - 60 minutes 3 - 5 days a week. Parents and schools must be imaginative and rigorous in encouraging children to exercise.

Role of Parents. Parents must make conscious efforts to limit sedentary activities, and to encourage physical ones for their children. This includes monitoring the time children spend on the computer, in front of the TV, or playing video games. Parents should suggest different forms of entertainment. Even children who aren't interested in joining a Little League team may enjoy a round of catch with their parents, walking in the park, or swimming in a local lake.

Role of Schools. Early school physical education programs can make a significant difference and the earlier these routines are learned, the more likely they will be carried forth into a healthy adulthood. Schools should emphasize team cooperation or individual improvement and self-mastery. Studies have shown that people tend to give up more quickly and feel less competent if their perceptions of success are based only on comparison to their peers.

People mature at different rates, and there seems to be a genetic component to coordination, strength, speed, and one's response to resistance exercise. Nonetheless, everyone should strive to be as fit as they possibly can, given their strengths and limitations.

The decision to adopt a healthier behavior -- whether it's more exercise, weight loss, or quitting smoking -- is not as simple as just deciding to do it. Behavior change expert James Prochaska and his colleagues outlined a theory, which has been supported by numerous studies, showing that people cycle through a variety of stages before a new behavior is successfully adopted over the long term. It may help you to understand how this works. As you read the description of each stage -- specifically as it relates to exercise -- you may find yourself nodding and saying to yourself, "Yes, that's me!"

Stage 1: Pre-Contemplation. People at this stage have no plans or desire to exercise. They aren't even considering exercising. They are generally unaware of the specific benefits that exercise can bring -- exercise may seem more like a hassle than something worth doing. Or, they may simply have "failed" in the past and have given up.

There's no point in talking about how to start an exercise program if you are at this stage. Instead, it is important to think about how exercise might be good for you personally -- by helping you to lose weight, feel better, have more confidence, live longer, sleep better, or reduce your stress levels. The benefits must be identified before a person will consider exercise.

If you are at this stage, a good activity is to ask four friends or family members why they exercise. Their answers may show you some real-life benefits, and inspire enough interest to compel you to take the next step.

Stage 2: Contemplation. A person at this stage is thinking, "I think I should probably exercise, but I need help getting started." People at this stage know that exercise is good for them, but it seems like a daunting task or they don't think they can pull it off. Some may have tried and "failed" in the past, but they are still receptive to another go-round.

It's important for people at this stage to consider some of the truths and falsehoods of exercise. For example, it is helpful to know that there are many forms of physical activity to select from, and that you can do your exercising in small chunks. It is not true that exercise has to be painful, or that you either succeed or fail. There is no such thing as "failure" -- people become more or less active at different stages of their lives, and it is never too late to get moving again. And people at this stage should find assurance that an exercise plan can be very simple.

If you are at this stage, a good activity is to write down all the things that you believe make exercise difficult -- and to learn strategies for overcoming or side-stepping those hurdles. People at this stage might benefit from making a pledge, contract, or other commitment that they are going to get more active in the near future. The goal is to get un-stuck by identifying the roadblocks and the ways to overcome these roadblocks. The final goal at this stage is to make a commitment.

Stage 3: Preparation. These folks are primed and motivated. They are ready to give exercise a try. The goal of this stage is to create a specific action plan that takes all factors into account, so that the "launch" is successful. People at this stage need to know how much they should be exercising, their target heart rate, and the types of exercises. They should explore the different kinds of exercises and decide which ones to try.

At this stage, people will evaluate exercise machines and health plans, if that interests them, pick the proper clothing or accessories, and consult a doctor if necessary. They also need to think about how they are going to fit their exercise plans into their daily and weekly schedule.

If you are at this stage, you should also consider some backup plans -- what to do if it rains, or if you don't feel like exercising. That way you are prepared to overcome that hurdle when you encounter it. You should be aware of what to expect realistically at the beginning -- for example, be aware that weight loss takes time, but health benefits begin immediately.

Stage 4: Action! People at this stage have just started exercising. This stage is where the biggest behavior change occurs -- these people have started to exercise but it is not yet a long-term, ingrained habit. This stage requires significant commitment and energy.

If you are at this stage, keep talking to friends and family for inspiration. Review your backup plans. Reward yourself for small achievements. Give yourself notes and reminders to exercise. Having a friend to exercise with can be very helpful as you get through this stage. You want to build and maintain momentum, because exercising gets easier once it is a habit!

Stage 5: Maintenance. The people at this stage have been exercising for at least 6 months. At this point, exercising has started to become a habit. The goal here is to prevent relapse. If you are at this stage, identify ways that you can fine-tune your program. Continue to identify roadblocks and improve your backup plans. Think about what you have found most enjoyable about exercising.

What benefits have you gained? Keep reminding yourself of these perks. If giving yourself a challenge was part of your initial motivation, set new goals and find new challenges. If you risk getting bored with your routine, find ways to vary it. Or maybe you have found a comfortable routine that you enjoy -- if it's working, great! There is no need to change it. You might want to read or learn more about your method of exercising, and develop a deeper level of understanding about it. Soon you'll be a pro!

One point about this theory is that people do not proceed from one stage to another in a simple, step-by-step fashion. They actually cycle or spiral back and forth, so that they may move from stage 1 to 2 to 3, and then back to 2 again. They may stay in maintenance mode for years and then fall back to stage 2. Remember that this is normal -- if you tried exercising in the past and didn't stick with it, don't consider yourself a failure. Just know that it's time to try again!

Resources

http://fitness.gov -- The President's Council on Physical Fitness and Sports
www.ncppa.org --National Coalition for Promoting Physical Activity
www.acefitness.org --American Council on Exercise
www.arthritis.org --The Arthritis Foundation offers tips on exercising with arthritis
www.justmove.org -- Just Move (American Heart Association)

References

Taylor, A.H., Ussher, M., & Faulkner, G. The acute effects of exercise on cigarette cravings, withdrawal symptoms, affect and smoking behaviour: a systematic review. Addiction. 2007;102:534-543.

Kruk J. Lifetime physical activity and the risk of breast cancer: a case-control study. Cancer Detect Prev. 2007;31(1):18- 28.

Tehard B, Friedenreich CM, Oppert JM, et al. Effect of physical activity on women at increased risk of breast cancer: results from the E3N cohort study. Cancer Epidemiol Biomarkers Prev. 2006 Jan;15(1):57-64.

Adams SA, Matthews CE, Hebert JR, et al. Association of physical activity with hormone receptor status: the Shanghai Breast Cancer Study. Cancer Epidemiol Biomarkers Prev. 2006 Jun;15(6):1170-8.

Larson EB, Wang L, Bowen JD et al. Exercise is associated with reduced risk for incident dementia among persons 65 years of age and older. Ann Intern Med. 2006 Jan 17;144(2):73-81.

Meyerhardt JA, Heseltine D, Niedzwiecki D, et al. Impact of physical activity on cancer recurrence and survival in patients with stage III colon cancer: findings from CALGB 89803. J Clin Oncol. 2006 Aug 1;24(22):3535-41.

Slattery ML. Physical activity and colorectal cancer. Sports Med. 2004;34(4):239-52.

Peters HP, De Vries WR, Vanberge-Henegouwen GP et al. Potential benefits and hazards of physical activity and exercise on the gastrointestinal tract. Gut. 2001 Mar;48(3):435-9.

Abbott, RD, White, LR, G. Ross, W, et al. Walking and Dementia in Physically Capable Elderly Men. JAMA. 2004;292:1447-1453

Calton BA, Lacey JV Jr, Schatzkin A, Schairer C, Colbert LH, Albanes D, Leitzmann MF. Physical activity and the risk of colon cancer among women: A prospective cohort study (United States). Int J Cancer. 2006 Feb 17; [Epub ahead of print]

Di Loreto C, Fanelli C, Lucidi P, et al. Make your diabetic patients walk: long-term impact of different amounts of physical activity on type 2 diabetes. Diabetes Care. 2005 Jun;28(6):1295-302.

Mikkelsson LO, Nupponen H, Kaprio J, Kautiainen H, Mikkelsson M, Kujala UM. Adolescent flexibility, endurance strength, and physical activity as predictors of adult tension neck, low back pain, and knee injury: A 25 year follow up study. Br J Sports Med. 2006 Feb;40(2):107-13.

Brown SG, Rhodes RE. Relationships among dog ownership and leisure-time walking in Western Canadian adults. Am J Prev Med. 2006 Feb;30(2):131-6.

Simons R, Andel R. The effects of resistance training and walking on functional fitness in advanced old age. J Aging Health. 2006 Feb;18(1):91-105.

Review Date:
4/30/2007
Reviewed By:
Harvey Simon, MD, Associate Professor of Medicine, Harvard Medical School; Physician, Massachusetts General Hospital.

A.D.A.M. Copyright

adam.com

Fibromyalgia

FitSugar — Wed, 08 Oct 2008 17:35:02 -0700

In This Report

Highlights
Introduction
Causes
Risk Factors
Diagnosis
Conditions with Similar Sym...
Prognosis
Treatment
Lifestyle Changes
Behavioral Therapy
Medications
Resources
References

HEALTH GUIDE REFERENCE FROM A.D.A.M

Highlights

Causes

People with fibromyalgia have decreased activity in opioid receptors in parts of the brain that affect mood and the emotional aspect of pain, researchers have found. This might explain why fibromyalgia patients are likely to experience depression, and are not very responsive to opioid painkillers.
Researchers have identified a conflict between sensory perception and nervous system processing in people with fibromyalgia. One study suggests that people with the condition might have greater awareness of, or less tolerance for, movement problems (such as tremor) that don't match with their expected sensory feedback. This mismatch in sensory signals might enhance the perception of pain.

Treatment

New research adds to the evidence that exercise relieves some of the symptoms of fibromyalgia. Women with fibromyalgia who took part in a program that combined aerobic, strength training, and flexibility exercises had better physical and emotional function, as well as reduced symptoms. Another study found that an at-home exercise program improved upper body pain and function, especially in women who were having functional difficulties at the beginning of the study.
An anti-convulsant medication, gabapentin (Neurontin), significantly improved pain in fibromyalgia patients compared to placebo. Patients who took gabapentin also reported that they slept better and felt less tired.
The selective serotonin-reuptake inhibitor paroxetine (Paxil) significantly lowered patient scores on a fibromyalgia symptom questionnaire, and was well-tolerated, although the drug do much for their pain.

Introduction

Fibromyalgia is a syndrome of unknown causes that results in lasting, sometimes debilitating, muscle pain and fatigue. Fibromyalgia is also known as fibrositis or fibromyositis.

Pain. The primary symptom of fibromyalgia is pain. The pain can be in one place or all over. The exact locations of the pain are called tender points. The pain of fibromyalgia is often is described as follows:

Tender point pain occurs in local sites, usually in the neck and shoulders. The pain then spreads out from these areas. The actual pain starts at the muscles. The joints are not affected. There are no lumps or nodes associated with these points of pain, and no signs of inflammation (swelling). People diagnosed with fibromyalgia feel pain in at least 11 of 18 specific tender points.
Widespread pain described as stiffness, burning, and aching. The pain also "radiates," or spreads, to nearby areas. Most patients report feeling some pain all the time. Many describe it as "exhausting." The pain can vary depending on the time of day, weather changes, physical activity, and the presence of stressful situations. The pain is often more intense after disturbed sleep.

Fatigue and Sleep Disturbances. Another major complaint is fatigue. Some patients report that fatigue is more unbearable than their pain. Sleep disturbances, particularly restless legs syndrome, are also very common. Fatigue and sleep disturbances are almost universal in patients with fibromyalgia. Some experts believe that if these symptoms are not present, doctors should seek a diagnosis other than fibromyalgia.

Depression and Mood. Up to a third of patients have depression. Disturbances in mood and concentration are also very common. These conditions often go undiagnosed in patients with fibromyalgia.

Other Symptoms. The following symptoms may also be present:

Digestive problems, including irritable bowel syndrome with gas, and alternating diarrhea and constipation
Dizziness
Painful menstrual periods
Tension or migraine headaches
Tingling or numbness in the hands and feet
Urinary frequency caused by bladder spasms

Symptoms in Children. In general, children with fibromyalgia most often have sleep disorders and widespread pain.

Causes

The most common type is primary fibromyalgia, in which the causes are not known. Many experts believe that fibromyalgia is not a disease, but rather a chronic pain condition brought on by several abnormal body responses to stress. Physical injuries, emotional trauma, or viral infections such as Epstein-Barr may be triggers of the disorder, but none have proven to be a cause of primary fibromyalgia.

Research published in the December 2006 issue of Current Pain and Headache Reports found that the areas in the brain that are responsible for the sensation of pain are different in fibromyalgia patients from the same areas in healthy people.

People with fibromyalgia have been found to have decreased activity in opioid receptors in parts of the brain that affect mood and the emotional aspect of pain. This reduced response might explain why fibromyalgia patients are likely to havedepression, and are less responsive to opioid painkillers, researchers say.

Sleep disturbances are common in fibromyalgia. Both adult and young patients with fibromyalgia have a higher-than-average rate of a sleep disorder called periodic limb movement disorder (PLMD). PLMD used to be called nocturnal myoclonus. Patients with PLMD involuntarily contract their leg muscles every 20 - 40 seconds during sleep. This may occasionally wake up the patient.

Some researchers believe that fibromyalgia does not lead to poor sleeping patterns, but that sleep disturbances come first. Researchers continue to investigate the link between fibromyalgia and sleep.

In one study, healthy volunteers reported fibromyalgia-like pain after they had been subjected to disrupted deep sleep. Disturbed sleep appears to trigger factors in the immune system that cause inflammation, pain, fatigue, and lower tolerance to pain. A 2004 study found that patients with fibromyalgia have increased rates of cyclic alternating sleep pattern (CAP). Increased CAP produced serious sleep problems, which were strongly linked to symptom severity. Previous studies have also suggested that CAP may be related to PLMD.
A 2004 report found that sleep disorders that cause breathing problems are common in women with fibromyalgia.
Other biological measures of troubled sleep, however, such as levels of the hormone melatonin, which helps regulate circadian rhythms and the sleep-wake cycle, appear to be normal in most people with fibromyalgia.

Many abnormalities of hormonal, metabolic, and brain chemical activity have been described in studies of fibromyalgia patients. Changes appear to occur in several brain chemicals, although no regular pattern has emerged that fits most patients. Since there has been no clear cause-and-effect relationship established, this may be a result of the effects of pain and stress on the central nervous system, and not a cause of fibromyalgia.

Serotonin. Of particular interest to researchers is serotonin, an important nervous system chemical messenger found in the brain, gut, and other areas of the body. Serotonin plays important roles in feelings of well-being, adjusting pain levels, and promoting deep sleep. Serotonin abnormalities have been linked to many disorders, including depression, migraines, and irritable bowel syndrome. Lower serotonin levels have also been noted in some patients with fibromyalgia.

Stress Hormones. Researchers have also found abnormalities in the hormone system known as the hypothalamus-pituitary-adrenal gland (HAP) axis. The HAP axis controls important functions, including sleep, response to stress, and depression. Changes in the HAP axis appear to produce lower levels of the stress hormones norepinephrine and cortisol. (By contrast, levels of stress hormones in depression are higher than normal.) Deficiencies in the levels of stress hormones produce impaired and weaker responses to psychological or physical stresses. (Examples of physical stress include infection or exercise.)

The hypothalamus is a highly complex structure in the brain that regulates many important brain chemicals.

Click the icon to see an image of the adrenal glands.

Low Growth Hormone Levels. Some studies have reported low levels of insulin-like growth factor-1 (IGF-1) in about a third of fibromyalgia patients. IGF-1 is a hormone that is controlled by the adult growth hormone, and promotes bone and muscle growth. Low levels of growth hormone are related to impaired thinking, lack of energy, muscle weakness, and intolerance to cold. Studies suggest that changes in growth hormone likely stem from the hypothalamus in the brain. While researchers did not find a link between IGF-1 levels and fibromyalgia, a 2005 study indicated that serum growth hormone levels may be a marker of the disorder.

Abnormal Pain Perception and Substance P. Some studies have suggested that fibromyalgia may involve too much activity in the parts of the central nervous system that process pain (the nociceptive system). Brain scans of fibromyalgia patients have suggested abnormalities in pain processing centers. For example, researchers have detected up to three times the normal level of substance P in the cerebrospinal fluid of fibromyalgia patients. Substance P, a chemical messenger of the nervous system, is associated with increased pain perception.

Some fibromyalgia patients may also be oversensitive to external stimulation, and overly anxious about the sensation of pain. This increase in awareness is called generalized hypervigilance. One study compared patients with fibromyalgia or rheumatoid arthritis to those without chronic pain. Researchers then measured the different groups' responses to pain and noise. Of the three groups, the fibromyalgia patients were least tolerant, and most aware, of such stimuli. However, one analysis of studies on fibromyalgia found no strong support for the hypervigilance theory.

A conflict between sensory perception and nervous system processing might occur in people with fibromyalgia. Fibromyalgia patients have been found to have greater awareness of, or less tolerance for, movement problems (such as tremor) that don't match with their expected sensory feedback. This mismatch in sensory signals might enhance the perception of pain.

Fibromyalgia has symptoms that resemble those of some rheumatic illnesses, including rheumatoid arthritis and lupus (systemic lupus erythematosus). These are autoimmune diseases in which a defective immune system mistakenly attacks the body's own healthy tissue, producing inflammation and damage. The pain in fibromyalgia, however, does not appear to be due to autoimmune factors, and there is little evidence to support a role for an inflammatory response in fibromyalgia.

Although not primary causes, psychological and social factors may contribute to fibromyalgia in three ways:

They could make individuals susceptible to fibromyalgia.
They may play some role in triggering the onset of the condition.
They may perpetuate, or be responsible for, the condition.

Studies have reported a greater number of severe experiences of emotional and physical abuse in patients with fibromyalgia, compared with the general population. Most often, the abuse came from family members or partners. This suggests that post-traumatic stress disorder (PTSD) or chronic stress may play a strong role in the development of fibromyalgia in some patients. PTSD, an anxiety disorder, is a reaction to a specific traumatic event. Symptoms of this condition, which can last for years after the traumatic event, include emotional withdrawal, hopelessness, irritability, mood swings, sleep problems, inability to concentrate, and an excessive startle response to noise. Some evidence indicates that PTSD actually results in changes in the brain, possibly from long-term over-exposure to stress hormones.

Some research found muscle abnormalities in fibromyalgia patients. These problems can be classified as the following:

Biochemical abnormalities: For example, one study reported that fibromyalgia patients had lower levels of the muscle-cell chemicals phosphocreatine and adenosine triphosphate (ATP). Such chemicals regulate the level of calcium in muscle cells. Calcium is an important component in the muscles' ability to contract and relax. If ATP levels are low, calcium is not "pushed back" into the cells, and the muscle remains contracted.
Functional abnormalities: The pain and stress of the disease itself may harm muscle function.
Structural and blood flow abnormalities: Some researchers saw overly thickened capillaries (tiny blood vessels) in the muscles of fibromyalgia patients. The abnormal capillaries could produce lower levels of compounds essential for muscle function, as well as reduce the flow of oxygen-rich blood to the muscles.

To date, none of these abnormalities have a clearly defined relationship with fibromyalgia.

Risk Factors

About 3.7 million Americans have fibromyalgia. The condition affects 2% of Americans, including 3.4% of women and 0.5% of men.

Some evidence suggests that several factors may make people more susceptible to fibromyalgia. These risk factors include:

Being female
Coming from a very stressful culture or environment
Having a psychological vulnerability to stress
Having had difficult experiences in childhood

Nine out of 10 fibromyalgia patients are women. Women may be more prone to develop fibromyalgia during menopause.

The disorder usually occurs in people ages 20 - 60 years, though it can occur at any time. Some studies have noted peaks around age 35. Others note that fibromyalgia is most common in middle-aged women. In one study, cases of fibromyalgia increased with age, and reached a frequency of more than 7% among people in their 60s and 70s.

Juvenile Primary Fibromyalgia. This type of fibromyalgia appears in adolescents, typically after age 13, with a peak incidence at age 14. It is uncommon, but studies indicate that its incidence may be increasing. One study found that 1.2% of school children, all girls, met the criteria for fibromyalgia. Other studies have found an even higher frequency of fibromyalgia in children. Symptoms are similar to adult fibromyalgia, but outcomes may be better in young people.

Studies report a higher incidence of fibromyalgia among family members. It is not clear if genetic or psychological factors, or both, are involved.

One study reported that 28% of the children of mothers with fibromyalgia also develop the disorder. Offspring who developed fibromyalgia were no more likely to have psychological disorders than those who did not.
Another study noted that 66% of parents of children with fibromyalgia reported some sort of chronic pain. About 10% of them had fibromyalgia.

Diagnosis

There is no obvious, objective method for diagnosing fibromyalgia. The criteria used for studying fibromyalgia are very helpful, particularly if the patient does not have any accompanying disorder, such as depression or arthritis, which could complicate the diagnosis. Failure to meet the criteria, however, does not rule out fibromyalgia. Fibromyalgia should be suspected in any person with muscle and joint pain with no identifiable cause.

In 1990, the American College of Rheumatology (ACR) set the following criteria for the classification of fibromyalgia:

A. Widespread pain must be present for at least 3 months. This pain must appear in all of the following locations:

Both sides of the body
Above and below the waist
Along the length of the spine

B. Pain in at least 11 of 18 specific areas called tender points on the body. The pain experienced when pressing on a tender point is very localized and intensely painful (not just tender). Tender points are located in the following areas:

The left or right side of the back of the neck, directly below the hairline
The left or right side of the front of the neck, above the collar bone (clavicle)
The left or right side of the chest, right below the collar bone
The left or right side of the upper back, near where the neck and shoulder join
The left or right side of the spine in the upper back between the shoulder blades (scapula)
The inside of either arm, where it bends at the elbow
The left or right side of the lower back, right below the waist
Either side of the buttocks below the hip bones
Either kneecap

Other Factors. The ACR classification provides a guideline, but doctors will also use a patient's medical history and other symptoms to reach a diagnosis. Fibromyalgia is often diagnosed when other diseases have been excluded. Long-term symptoms that may indicate fibromyalgia include:

Fatigue
Headache
Morning stiffness
Numbness or tingling in the hands and feet
Sleep disturbance

The 18 fibromyalgia tender points are located throughout the body. According to the American College of Rheumatology, a diagnosis of fibromyalgia requires widespread body pain plus localized pain in 11 of these 18 specific points.

A doctor should always take a careful personal and family medical history, which would include a psychological profile and a history of any factors that might indicate disorders other than fibromyalgia. Such factors might include:

Infectious diseases
Muscle weakness
Physical injuries
Rashes
Recent weight change
Sexual, physical, or substance or alcohol abuse

Patients should report any drugs they take, including vitamins and over-the-counter or herbal medications.

Pressure on Tender Spots. Any physical examination for fibromyalgia requires that the doctor press firmly on all potential tender spots. They must be painful when pressed, not simply tender. In addition, for a doctor to reach a diagnosis of fibromyalgia, these tender sites should normally not show signs of inflammation (redness, swelling, or heat in the joints and soft tissue). The tender points may also change in location and sensitivity over time. A doctor, then, may recheck tender points that do not respond the first time, in patients who have other significant symptoms.

Detection of Other Causes of Symptoms. A health care provider will also examine nails, skin, mucous membranes, joints, spine, muscles, and bones to help rule out arthritis, thyroid disease, and other disorders.

No blood, urine, or other laboratory tests can provide a definitive diagnosis of fibromyalgia. If such tests show abnormal results, the doctor should look for other disorders. Tests for specific diseases depend on family histories and other symptoms. They may include:

Blood count
Sedimentation rate
Tests of certain antibodies
Thyroid and liver function tests

The doctor may suggest follow-up psychological profile testing, if laboratory results do not indicate a specific disease.

Conditions with Similar Symptoms

Between 10 - 30% of all doctor office visits are due to symptoms that resemble those of fibromyalgia, including fatigue, malaise, and widespread muscle pain. Since no laboratory test can confirm a diagnosis of fibromyalgia, doctors will usually first test for similar conditions. It should be noted that a diagnosis of many of the disorders below may not always rule out fibromyalgia, since it can accompany other common and similar conditions.

Several conditions overlap or often coexist with fibromyalgia, and have similar symptoms. It is not clear if these conditions cause fibromyalgia, are risk factors for the disorder, have causes in common with fibromyalgia, or have no relationship at all with it.

Chronic Fatigue Syndrome. There is a significant overlap between fibromyalgia and chronic fatigue syndrome (CFS). In a 2003 study, for example, 43% of CFS patients also had a diagnosis of fibromyalgia. As with fibromyalgia, the cause of CFS is unknown. A doctor can diagnose either disorder based only on symptoms reported by the patient. The two disorders share most of the same symptoms. They are also treated almost identically. The differences are primarily the following:

Pain with tender points is the primary symptom in fibromyalgia. Some patients with CFS exhibit similar tender pressure points. However, muscle pain is less prominent in patients with CFS.
Fatigue is the dominant symptom in CFS. It is severe and not relieved by rest or sleep, and it is not the result of excessive work or exercise.

Some doctors believe that fibromyalgia is simply an extreme type of chronic fatigue syndrome. There Some physical evidence, however, indicates that the two disorders are distinct, with treatments that are specific to each.

Myofascial Pain Syndrome. Myofascial pain syndrome can be confused with fibromyalgia and may also accompany it. Unlike fibromyalgia, myofascial pain tends to occur in trigger points, as opposed to tender points, and typically there is no widespread, generalized pain. Trigger-point pain occurs in tight muscles, and when the doctor presses on these points, the patient may experience a muscle twitch. Unlike tender points, trigger points are often small lumps, about the size of a pencil eraser.

Major Depression. The link between psychological disorders and fibromyalgia is very strong and problematic. Certain studies report that 50 - 70% of fibromyalgia patients have a lifetime history of depression. Only 18 - 36% of fibromyalgia patients, however, also have major depression, a severe form of depression.

Some studies found that people who have both psychological disorders and fibromyalgia are more likely to seek medical help, compared with patients who simply have symptoms of fibromyalgia. If this is the case, study results may be biased, favoring a higher-than-actual association between depression and fibromyalgia.

Depression most likely does not cause fibromyalgia, but it may increase susceptibility. Depressed feelings in people with fibromyalgia can certainly be normal responses to the pain and fatigue caused by this syndrome. Such emotions, however, are temporary and related to the situation a person is in. They are not considered to be a depression disorder. Unlike ordinary periods of sadness, an episode of major depression disorder can last many months.

Symptoms of major depression include the following:

Depressed mood every day
Feeling worthless or inappropriately guilty
Inability to concentrate or make decisions
Insomnia or excessive sleeping
Low energy every day
Restlessness or a sense of being slowed down
Significant weight gain or loss (of 10% or more of an individual's typical body weight)
Suicidal thoughts

If several of the above symptoms are present, and none of the physical symptoms (particularly the tender points) of fibromyalgia exist, the condition is most likely major depression.

Chronic Headache. Chronic primary headaches such as migraines are common in fibromyalgia patients. Some experts believe that migraine headaches and fibromyalgia may even share common defects in the systems that regulate certain chemical messengers in the brain, including serotonin and epinephrine (adrenaline). Low levels of magnesium have also been noted in patients with both fibromyalgia and migraines. In fact, chronic migraine sufferers who fail to benefit from usual therapies may also have fibromyalgia.

Symptoms of a migraine attack may include heightened sensitivity to light and sound, nausea, vision problems (auras), speech difficulty, and intense pain predominating on one side of the head.

Multiple Chemical Sensitivity. Multiple chemical sensitivity (MCS) is a term that describes conditions in which certain chemicals can cause symptoms similar to CFS or fibromyalgia in some people. Still, as with CFS and fibromyalgia, some experts are uncertain whether MCS is a medical condition or if it is psychologically based.

In one study, for example, CFS patients who believed their problem was chemically triggered were exposed to either an active chemical or a placebo (an inactive substance). Both groups reported symptoms, including those only exposed to a placebo. Because everyone is exposed to many chemicals on a daily basis, it is very difficult to determine whether chemicals are responsible for specific symptoms.

Experts have come up with criteria to help recognize MCS:

Symptoms can be produced by exposure to the chemical at levels lower than previously or usually tolerated.
Symptoms can be triggered by multiple substances that are chemically unrelated.
Symptoms involve multiple organ systems.
The condition is chronic.
The symptoms always happen with repeated exposure to a chemical. (These are often common chemicals found in popular products, such as perfumes, fabric softeners, and air fresheners.)
The symptoms improve when the chemical is removed.

Restless Legs Syndrome. About 15% of people with fibromyalgia have restless legs syndrome. Restless legs syndrome is an unsettling and poorly understood movement disorder that is sometimes described as a sense of unease and weariness in the lower leg that is aggravated by rest and relieved by movement.

Disorders Affected by the Sympathetic (also called Autonomic) Nervous System. Other conditions that commonly accompany fibromyalgia include:

Chest pain and heart palpitations
Mitral valve prolapse
Sudden drop in blood pressure

Certain stress-related disorders commonly occur with fibromyalgia, and have overlapping symptoms. In fact, some experts believe these disorders so often interact that they may all be part of one general condition.

Chemicals and environmental toxins -- exposure to various chemicals and environmental toxins such as solvents, pesticides, or heavy metals (cadmium, mercury, or lead) can cause fatigue, chronic pain, and other symptoms of fibromyalgia.
Irritable bowel syndrome
Osteoarthritis -- a common form of arthritis than can coexist with fibromyalgia. The two conditions may be confused, particularly in elderly people. Osteoarthritis, however, causes joint pain, not widespread or generalized pain.

Osteoarthritis is a chronic disease of the joint cartilage and bone. It is often thought to result from "wear and tear" on a joint, although there are other causes, such as congenital defects, trauma, and metabolic disorders. Joints appear larger, are stiff and painful, and usually feel worse the more they are used throughout the day.

Temporomandibular joint disorders

Some tests may be positive for one or more of these diseases. However, if the results are uncertain or weak, or if these conditions have been treated successfully, fibromyalgia should not be ruled out if the patient still meets the criteria for it.

Multiple sclerosis. This condition may have symptoms similar to those of fibromyalgia. Magnetic resonance imaging (MRI) scans often detect patches of tissue in the brain that confirm the presence of multiple sclerosis (MS). MRI findings combined with other tests and clinical findings usually make this diagnosis fairly certain. However, some patients may have symptoms that suggest MS, but diagnostic tests cannot confirm the diagnosis. Some of these patients may have symptoms similar to those of fibromyalgia.

Click the icon to see an image of multiple sclerosis.

Sjogren syndrome. This condition, characterized by dry eyes and mouth, is sometimes mistaken for fibromyalgia.

Autoimmune diseases. Rheumatoid arthritis, systemic lupus erythrometosis, and Sjogren syndrome are usually easy to diagnose but may develop slowly and be difficult to diagnose at first. Even if a doctor determines that a patient is most likely to have fibromyalgia, the doctor should keep track of any changes in symptoms over time in case one of these other illnesses is actually present.

Lyme Disease. Lyme disease is a bacterial disease transmitted by ticks. Health care providers can usually diagnose early Lyme disease correctly, but a delayed response or recurrence of this disorder may be mistaken for fibromyalgia. Some experts believe that 15 - 50% of patients referred to clinics for Lyme disease actually have fibromyalgia. Late Lyme disease can usually (but not always) be ruled out using blood tests that identify the organism that causes this disease. If fibromyalgia patients are incorrectly diagnosed and treated for Lyme disease with prolonged courses of antibiotics, the drugs may have serious side effects.

Drugs and Alcohol. Fatigue is a side effect of many prescription and over-the-counter medications, such as antihistamines. In addition, symptoms of dependency on, or abuse of, alcohol or drugs appear as constant fatigue. Health care providers should consider medications as a possible cause of fatigue if an individual has recently started, stopped, or changed medications. Withdrawal from caffeine can produce depression, fatigue, and headache.

Polymyalgia Rheumatica. Polymyalgia rheumatica is a condition that causes pain and stiffness, and generally occurs in older women. Tender points are also present with this disorder, although they almost always occur in the hip and shoulder area. Morning stiffness is common, and patients may also experience fever, weight loss, and fatigue. A higher-than-normal erythrocyte sedimentation rate (ESR) can suggest polymyalgia rheumatica. Elevated ESR, however, also occurs with other conditions. Polymyalgia rheumatica often gets better in about a year, but there is a risk of persistent disease. Worse, it is sometimes associated with a rare condition called temporal arteritis, which may cause blindness if not treated, so an accurate diagnosis of polymyalgia rheumatica is important.

Prognosis

Fibromyalgia can be mild or disabling, and the emotional toll can be substantial. About half of all patients have difficulty with routine daily activities, or are unable to perform them. An estimated 30 - 40% of patients have had to quit work or change jobs. In a 2003 study, patients with either CFS or fibromyalgia were more likely to suffer losses of jobs, possessions, and support from friends and family than were people suffering from other conditions that caused fatigue.

The pain, emotional consequences, or sleep disturbances that come with fibromyalgia may lead to self-medication and overuse of sleeping pills, alcohol, drugs, or caffeine.

Outlook in Adults. Some studies show that fibromyalgia symptoms remain stable over the long term, while others report a better outlook, with 25 - 35% of patients reporting improvement in pain symptoms over time. Studies suggest that regular exercise specifically improves the outlook. Those with a significant life crisis, or who were on disability, had a poorer outcome than others. Outcome was determined by improvements in the patients' ability to work, their own feelings about their condition, pain sensation, and levels of disturbed sleep, fatigue, and depression. Although the disease is life-long, it does not get worse and is not fatal.

Outlook in Children. Children with fibromyalgia tend to have a better outlook than adults with the disorder. Several studies reported that more than half of children with fibromyalgia recover in 2 - 3 years.

Treatment

Many patients with fibromyalgia are treated first with medication; however, the American Pain Society Fibromyalgia Panel recommends a combined approach using cognitive-behavioral therapy, education, medication, and exercise.

Fibromyalgia is a mysterious condition. Its causes are still largely unknown, as is how it inflicts damage. No strong evidence indicates that any single treatment (or combination of treatments) has any significant effect for most patients. However, in 2007 the U.S. Food and Drug Administration approved pregabalin (Lyrica) as the first drug treatment for fibromyalgia after a study showed the medicine reduced fibromyalgia pain by at least 50% in 63% of patients.

Treatment usually involves not only relieving symptoms but also changing a pateint's attitude about their disease. Treatment should also teach patients behaviors that help them cope.

Treatments usually involve trial and error:

Patients may start with physical therapy, exercise, stress reduction techniques, and cognitive-behavioral therapy.
If these methods fail to improve symptoms, an antidepressant or muscle relaxant may be added to the treatment. Doctors usually prescribe these drugs because they can may improve pain tolerance.
Patient education and programs that encourage coping skills are an important part of any treatment plan.

According to a 2005 study published in the Clinical Journal of Pain, a combination of non-drug therapies works just as well as drug therapy in improving pain, depression, and disability. This combination includes exercise, stress management, massage, and diet.

Patients must have realistic expectations about the long-term outlook of their condition, and their own individual abilities. It is important to understand that fibromyalgia can be managed, and patients can live a full life. The following tips may be helpful when starting a treatment program for fibromyalgia:

The goal of therapy is to relieve symptoms, not cure them.
Treatment must be tailored to each patient, and a combination approach is often needed.
Patients must begin all treatments with the attitude that these treatments are trial-and-error. There is no clear treatment solution. Patients and doctors need to work together to make the best choices for individual symptoms and concerns.
Treatments are long-lasting, in some cases life-long, and patients should not be discouraged by the return of symptoms (relapses).
Enlisting family members, partners, and close friends, particularly to help with exercise and stretching programs, can be helpful.
Becoming involved with support groups of fellow patients also benefits many patients. Support groups may also benefit family members, particularly parents of children with fibromyalgia. One study noted that the severity of the disorder increased in children whose parents were less able to cope with their child's pain.

The definition of improvement is personal. For example, some patients are pleased with only a 10% reduction in pain and other symptoms.

Lifestyle Changes

Many studies have shown that exercise is the most effective component in managing fibromyalgia, and patients must expect to take part in a long-term exercise program. Physical activity prevents muscle wasting, increases well-being, and, over time, reduces fatigue and pain. Many studies have also demonstrated the exercise can improve physical and emotional function, as well as reduce symptoms, including pain.

Programs often combine aerobic, strength-training, and flexibility exercises with self-management education. Some studies have shown improvements lasting for up to 9 months after the exercise program.

Graded Exercise. The basic approach used for fibromyalgia is called graded exercise. Graded exercise means you slowly increase the amount of your physical activity.

In general, graded exercise involves:

A very gradual program of activity, beginning with mild exercise and building in intensity over time.
Stretching exercises before exercising. A daily stretching routine can help relax tense muscles and prevent muscle soreness.
Walking, swimming, and using equipment such as treadmills or stationary bikes. Swimming and water therapy are good because they don't require putting weight on the joints.

Patients who try hard exercises too early actually experience an increase in pain, and are likely to become discouraged and quit.

Every patient must be prepared for relapses and setbacks, but should not get discouraged. Patients who do not respond to one type of exercise might consider experimenting with another form.

Physical therapy can be very helpful. Studies suggest that physical therapy may reduce muscle overload, lessen fatigue from poor posture and positioning, and help condition weak muscles.

Sleep is essential, particularly since sleep disruptions worsen pain. Many patients with fibromyalgia have trouble getting a restful and healing night's sleep. Those who are unable to sleep consistently have low improvement. Swing shift work, for example, is extremely hard on fibromyalgia patients. Poor sleep habits can add to sleep problems. Tips for good sleep habits include:

Avoid caffeine or alcohol 4 - 6 hours before bedtime.
Avoid drinking fluids right before bedtime so that needing to uriniate does not disturb your sleep.
Avoid exercising 6 hours before bedtime.
Avoid large meals before bedtime. A light snack, however, may help you sleep.
Avoid naps, especially in the evening or late afternoon.
Establish a regular time for going to bed and getting up in the morning. Maintain this schedule even on weekends and during vacation.
If you are unable to fall asleep after 15 or 20 minutes, go into another room and start a quiet activity. Return to bed when you feel sleepy.
Minimize light and maintain a comfortable, moderate temperature in the bedroom. Keep the bedroom well ventilated.
Use the bed only for sleep and sexual relations.

[For more information see In-Depth Report #27: Insomnia.]

Fibromyalgia patients should maintain a healthy diet low in animal fat and high in fiber, with plenty of whole grains, fresh fruits, and vegetables. Although everyone should be careful about calories from fats, some are healthy.

Omega-3 Fatty Acids. Oils containing omega-3 fatty acids are of particular interest for arthritic pain. Such oils are found in cold-water fish. You can also purchase these oils as supplements called EPA-DHA or omega 3.

Omega-3 fatty acids are a form of polyunsaturated fat that the body gets from food. Omega-3s are known as essential fatty acids (EFAs) because they are important for good health. These healthy fatty acids can be found in certain fish, dark green leafy vegetables, and some oils. Omega-3 fatty acids have anti-inflammatory properties, which help prevent blood clots, lower cholesterol and triglyceride levels, and reduce blood pressure. Omega-3s may also reduce the risks and symptoms of diabetes, stroke, rheumatoid arthritis, asthma, inflammatory bowel disease, ulcerative colitis, some cancers, and mental decline.

Vegetarian Diet. A vegan diet has no meat, dairy, or eggs and includes uncooked fruits, vegetables, nuts, and germinated seeds. The actual benefit of various vegetarian diets remains unproven.

Relaxation and stress-reduction techniques are proving to be helpful in managing chronic pain. Evidence shows that people with fibromyalgia have a more stressful response to daily conflicts and encounters than those without the disorder. Several relaxation and stress-reduction techniques may be helpful in managing chronic pain:

Biofeedback
Deep breathing exercises
Hypnosis
Massage therapy
Meditation
Muscle relaxation techniques

Biofeedback. Evidence from controlled trials does not suggest that biofeedback techniques may be very helpful for fibromyalgia patients. During a biofeedback session, electric leads are taped to a subject's head. The person is encouraged to relax using any method that works. Brain waves are measured and an audio signal sounds when alpha waves are detected. Alpha waves are brain waves that occur with a state of deep relaxation. By repeating the process, people using biofeedback connect the sound with the relaxed state, and learn to achieve relaxation on their own.

Meditation. Meditation, used for many years in Eastern cultures, is now widely accepted in this country as an effective relaxation technique. A number of studies are reporting its benefits for fibromyalgia patients who practice on a continued and regular basis. The practiced meditator can achieve the following physical benefits:

Reduced heart rate, blood pressure, adrenaline levels, and skin temperature while meditating.
Improved well-being.
Better sleep -- some research has reported an increase in melatonin levels in experienced meditators. Melatonin is important in regulating the sleep-wake cycle.
Less pain, possibly from reductions in levels of cortisol, a stress hormone.

An important goal for both religious and therapeutic meditation practices is to quiet the mind, essentially to relax thought. This redirection of brain activity from thoughts and worries to the senses disrupts the stress response and prompts relaxation and renewed energy. Several meditation techniques are available. Some may be more useful for fibromyalgia than others.

Breath meditation. Other meditative forms involve focusing on the present moment and observing (but not examining or judging) one's thoughts. During breath meditation, one sits upright with the spine straight and the eyes closed. The subject begins to breathe regularly and continues to observe the outward exhalation of the breath. As the mind wanders, one simply notes the thoughts as a fact and returns to the breath. A variant of this technique called mindfulness meditation has been helpful for fibromyalgia patients. It involves focusing on the present moment and letting thoughts pass without the accompanying breathing exercises.
Fixed point meditation involves focusing on a stationary object, mental image (such as a candle flame), or internal sound (such as a mantra). When the mind begins to wander, the meditator gently brings concentration back to the central image or sound. This exercise promotes focus, but it is often experienced as a thinking exercise. A popular variety of this type of meditation is known as transcendental meditation, or TM.
Mini-meditation. This method involves heightening awareness of the immediate surrounding environment. One should first choose a simple routine activity when alone. For example, while washing dishes concentrate on the feel of the water and dishes. Allow the mind to wander to any immediate sensory experience, such as sounds outside the window, smells from the stove, or colors in the room. If the mind begins to think about the past or future, abstractions, or worries, redirect it gently back.

People who try meditation for the first time should understand that it can be difficult to quiet the mind, and should not be discouraged by lack of immediate results. Some recommend meditating for no longer than 20 minutes in the morning after awakening and then again in the early evening before dinner. Even once a day is helpful. A person should probably not meditate before going to bed, because it causes some people to wake up in the middle of the night, alert and unable to return to sleep.

Hypnosis. In one small, short-term controlled study, hypnosis was more effective than physical therapy in improving function and reducing pain.

Massage Therapy. Massage therapy is thought to stimulate the parasympathetic nervous system, which slows down the heart and relaxes the body. In one study, patients who were given 30-minute massage sessions twice a week experienced lower stress and anxiety and less pain after 5 weeks compared to a group receiving an alternative therapy called transcutaneous electrical stimulation (TENS).

Because of the difficulties in treating fibromyalgia, many patients seek alternative therapies. Everyone should be wary of those who promise a quick cure or urge the purchase of expensive but potentially dangerous treatments.

Although some studies have reported benefit from these treatments, there is not enough evidence to recommend them. In one analysis, evidence was weakest on the advantages of so-called manipulative ("hands-on") approaches, such as chiropractic treatments.

Acupuncture. Studies continue to report conflicting results on acupuncture's ability to relieve pain. Several small studies suggest that it offers some benefit, especially to those who cannot take medicines because of their side effects. A larger controlled study found that inserting needles at fibromyalgia-related pressure points was no better at relieving pain for fibromyalgia than randomly inserting needles ("sham acupuncture"). A 2006 review of five randomized, controlled trials did not find enough evidence to support the use of acupuncture for fibromyalgia.

Click the icon to see an image of acupuncture.

Chiropractic or Osteopathic Manipulation. Chiropractic or osteopathic manipulation may also help some patients. While some studies have reported pain relief and improved sleep with osteopathic manipulation, larger controlled studies are needed to clearly identify whether manipulation is an effective treatment. Osteopathic techniques may include manipulation of the spine or muscle tissue release. Note that there is always some very small risk for adverse effects from any of these techniques. For example, in rare cases manipulation of the neck has caused stroke or damage to the large blood vessels in the neck.

Hydrotherapy and Similar Treatments. Hydrotherapy, also called balneotherapy, involves soaking in water, such as hot tubs, pools, or baths, to help relieve pain.

Herbal or Natural Remedies. Some alternative agents are being investigated for fibromyalgia:

Melatonin, a natural hormone associated with the sleep-wake cycle, may have benefits for some patients with fibromyalgia.
S-adenosylmethionine (SAMe) is a natural substance that has antidepressant, anti-inflammatory, and analgesic properties. It has shown some benefit in controlled studies.

It is extremely important for patients to realize that any herbal remedy or natural medicine that has positive effects most likely has negative side effects and toxic reactions, just as any conventional drug does. You should consult a doctor before using any untested products or dietary supplements. You should also discuss with your doctor any potential interactions between the supplements and any medications you take.

Generally, manufacturers of herbal remedies and dietary supplements do not need approval from the U.S. Food and Drug Administration to sell their products. Just like a drug, herbs and supplements can affect the body's chemistry, and therefore have the potential to produce side effects that may be harmful. There have been a number of reported cases of serious and even deadly side effects from herbal products. Always check with your doctor before using any herbal remedies or dietary supplements.

Behavioral Therapy

Studies continue to show that fibromyalgia patients feel better when they deal with the specific conditions of their disorder and their lives. Cognitive-behavioral therapy (CBT) enhances a patient's belief in their own abilities and helps them develop methods for dealing with stressful situations. CBT, also called cognitive therapy, is a known, effective method for dealing with chronic pain from arthritic conditions. Some evidence also suggests that cognitive-behavioral therapy can help some patients with fibromyalgia.

Although the effects of CBT and other non-medication treatments for fibromyalgia do not always last over the long-term, they may help certain groups of people, particularly those with a high level of psychological stress.

CBT may be particularly useful for addressing insomnia, one of the hallmark symptoms of fibromyalgia. Patients who received CBT for insomnia woke up 50% less at night, and had fewer symptoms of insomnia and improved mood.

The Goals of CBT. The primary goals of CBT are to change any unclear or mistaken ideas and self-defeating behaviors. Using specific tasks and self-observation, patients learn to think of pain as something other than a negative factor that controls their life. Over time, the idea that they are helpless against the pain goes away and, instead, they learn that they can manage the pain.

Cognitive therapy is particularly helpful in defining and setting limits -- a behavior that is extremely important for these patients. Many fibromyalgia patients live their lives in extremes. They first become heroes or martyrs, pushing themselves too far until they collapse. This collapse reverses the way they view themselves, and they then think of themselves as complete failures, unable to cope with the simplest task. One important aim of cognitive therapy is to help such patients discover a middle route. Patients learn to prioritize their responsibilities and drop some of the less important tasks or delegate them to others. Learning these coping skills can eventually lead to a more manageable life. Patients learn to view themselves and others with a more flexible attitude.

The Procedure. Cognitive therapy usually does not last long, typically 6 - 20 one hour sessions. Patients also receive homework, which usually includes keeping a diary and trying tasks they have avoided because of negative attitudes.

A typical cognitive therapy program may involve the following measures:

Keep a Diary. Patients are usually asked to keep a diary, a key part of cognitive therapy. The diary serves as a general guide for setting limits and planning activities. Patients use the diary to track any stress factors, such as a job or a relationship that may be making the pain worse or better.
Confront Negative or Discouraging Thoughts. Patients are taught to challenge and reverse negative beliefs. For example, "I'm not good enough to control this disease, so I'm a total failure" becomes the coping statement, "Where is the evidence that I can control this disease?"
Set Limits. Limits are designed to keep both mental and physical stress within manageable levels, so that patients do not become discouraged by getting "in over their heads." For example, tasks are broken down into incremental steps, and patients focus on one at a time.
Seek out Pleasurable Activities. Patients list a number of enjoyable low-energy activities that they can conveniently schedule.
Prioritize. Patients learn to drop some of the less critical tasks or delegate them to others.

Patients should learn to accept that relapses occur, and that over-coping and accomplishing too much too soon can often cause a relapse of symptoms. Patients should respect these relapses and back off. They should not consider them a sign of failure.

Research also shows that patient education can be effective in treating fibromyalgia, especially when combined with CBT, exercise, and other therapies. Educational programs can take the form of group discussions, lectures, or printed materials, although there isn't any clear evidence on which type of education works best.

Cognitive therapy may be expensive and not covered by insurance. Alternative and effective approaches that are free or less costly include strong, intelligently managed support groups or group psychotherapy. In one center, educational discussion groups were as effective, or even more so, than a cognitive therapy program. Such results are not typical in all centers, of course. Therapeutic success varies widely depending on the skill of the therapist.

Medications

Typically the first choice in drug treatment of fibromyalgia has consisted of an antidepressant or a muscle relaxant. The goal has been to improve sleep and pain tolerance. Medications from other drug classes (such as sleeping aids and pain relievers) may also be prescribed. Patients receive drug treatments in combination with exercise, patient education, and behavioral therapies. In 2007 the Food and Drug Administration approved Pregabalin (Lyrica) as the first drug for the treatment of fibromyalgia.

Pregabalin is an anti-epileptic. Also called anti-seizure drugs and anti-convulsants, these medicines affect the chemical messenger gamma aminobutyric acid (GABA), which helps prevent nerve cells from over-firing.

Pregabalin was previously approved in 2004 to treat nerve pain and diabetic peripheral neuropathy. A 2005 study of 529 patients with fibromyalgia reported that 450 mg per day of pregabalin reduced pain and improved sleep quality and fatigue symptoms. Study results presented in November 2006 showed pregabalin cut fibromyalgia pain by at least 50% in 63% of patients, and the effect was long-lasting. The study, lasting 6 months, was one of the longest controlled studies of pregabalin in fibromyalgia to date. The most common side effects include mild-to-moderate dizziness and sleepiness. Pregabalin can impair motor function and cause problems with concentration and attention. Patients should talk to their doctor about whether pregabalin may impair their ability to drive.

Studies have shown that another anti-convulsant, gabapentin (Neurontin), which is approved for treatment of postherpetic neuralgia, affects pain transmission pathways and may relieve pain associated with fibromyalgia when compared with placebo. Patients who took gabapentin also reported that they slept better and were less tired.

The main classes of antidepressants used for treating fibromyalgia are tricyclics, selective serotonin-reuptake inhibitors (SSRIs), and serotonin-norepinephrine reuptake inhibitors (SNRIs). Although these drugs are antidepressants, doctors prescribe them to improve sleep and relieve pain in non-depressed patients with fibromyalgia. The dosages used for managing fibromyalgia are generally lower than dosages prescribed for treating depression. If a patient has depression in addition to fibromyalgia, higher doses may be required.

Tricyclics. Tricyclic antidepressants cause drowsiness and can be helpful for improving sleep. The tricyclic drug most commonly used for fibromyalgia is amitriptyline (Elavil, Endep), which produces modest benefits with pain, but which can lose effectiveness over time. Other tricyclics include desipramine (Norpramin), doxepin (Sinequan), imipramine (Tofranil), amoxapine (Asendin), and nortriptyline (Pamelor, Aventyl).

Generally, only small doses of tricyclic antidepressants are needed to relieve fibromyalgia. Therefore, although tricyclics have several side effects, these side effects may be less frequent in fibromyalgia patients than in those taking tricyclics for depression. Side effects most often reported include:

Blurred vision
Difficulty urinating
Dizziness
Drowsiness
Dry mouth
Heart rhythm disturbances
Sexual dysfunction
Weight gain

As with all medications, tricyclics must be taken as directed. An overdose can be life-threatening.

Unfortunately, not all patients respond to tricyclics, and their effects wear off in some patients, sometimes after only a month.

Selective Serotonin-Reuptake Inhibitors. Selective serotonin-reuptake inhibitors (SSRIs) increase serotonin levels in the brain, which may have specific benefits for fibromyalgia patients. Commonly prescribed SSRIs include fluoxetine (Prozac), sertraline (Zoloft), paroxetine (Paxil), and fluvoxamine (Luvox). Studies suggest they may improve sleep, fatigue, and well-being in many patients. Studies are mixed on whether they improve pain. SSRIs should be taken in the morning, since they may cause insomnia. Common side effects are agitation, nausea, and sexual dysfunction, including delay or loss of orgasm and low sex drive.

Serotonin-Norepinephrine Reuptake Inhibitors. Serotonin-norepinephrine reuptake inhibitors (SNRIs) are also known as dual inhibitors because they act directly on two chemical messengers in the brain -- norepinephrine and serotonin. These drugs include:

Duloxetine (Cymbalta) is gaining attention as a treatment for fibromyalgia. In a 2004 study, 207 patients with fibromyalgia were randomized to receive either 60 mg of duloxetine twice a day or placebo for 12 weeks. Duloxetine significantly improved pain and tenderness and was effective for both depressed and non-depressed patients. Duloxetine was most effective for women, but very few men were enrolled in this trial.
Venlafaxine (Effexor) is similar to fluoxetine (Prozac) in effectiveness and tolerability for most patients. As with SSRIs, and unlike other newer antidepressants, venlafaxine impairs sexual function. Although clinical trials have shown that the drug is safe and effective in most people, there have been reports of changes in blood pressure. There have also been reports of problems with the electrical system of the heart when taking this drug. These side effects may cause serious problems in elderly patients. Some patients report severe withdrawal symptoms, including dizziness and nausea.
Milnacipran (Ixel) is under investigation and is not yet approved in the United States. It is specifically being researched for helping people with fibromyalgia and similar pain syndromes. In a 2004 study of 125 patients, milnacipran improved fibromyalgia pain and other symptoms, including fatigue, sleep, and depression.

Cyclobenzaprine (Flexeril) relaxes muscle spasms in specific locations without affecting overall muscle function. Cyclobenzaprine is related to the tricyclic antidepressants and has similar side effects, including drowsiness, dry mouth, and dizziness. A 2004 review of five randomized controlled trials found that patients who received cyclobenzaprine were three times more likely to report improvement in fibromyalgia symptoms than patients who received placebo.

Zolpidem (Ambien) or other newer sleep medications such as zaleplon (Sonata) and eszopiclone (Lunesta) may improve sleep for patients who suffer from insomnia.

Pain relief is of major concern for patients with fibromyalgia. Pain relievers for fibromyalgia include:

Tramadol (Ultram), used alone or in combination with acetaminophen (Tylenol), is commonly prescribed for relief of fibromyalgia pain. Its most common side effects are drowsiness, dizziness, constipation, and nausea. Tramadol should not be used in combination with tricyclic antidepressants.
For relief of mild pain, acetaminophen is most often recommended. Anti-inflammatory drugs, which are commonly used for arthritic conditions, are less useful for the pain of fibromyalgia, since the pain is not caused by muscle or joint inflammation. Anti-inflammatory drugs include corticosteroids and nonsteroidal anti-inflammatory drugs (NSAIDs), such as aspirin and ibuprofen (Advil).
Capsaicin (Zostrix) is an ointment prepared from the active ingredient in hot chili peppers. Capsaicin is helpful in relieving painful areas in other disorders. It may have some value for fibromyalgia patients.
Opioids, or narcotics, may be used occasionally by certain patients with moderate-to-severe pain, or those with significant problems performing everyday tasks. Such patients should use narcotics only if they cannot find relief with other, less potent treatments. Some patients may get combinations of narcotic pain relievers and acetaminophen for periodic pain. Some physicians prescribe opioids, such as oxycodone (Roxicodone) or morphine sulfate (Duramorph), for patients who need ongoing relief. However, the benefit of opioids in fibromyalgia treatment is highly controversial. Physicians should take a careful medical and psychological profile of the patient before prescribing opioids. The patients should be evaluated periodically for continuing pain relief, side effects, and indications of dependence.
Pramipexole, a drug used to treat Parkinson’s disease and restless legs syndrome, may help relieve pain and fatigue in people with fibromyalgia, according to one study. Pramipexole stimulates production of dopamine, a chemical messenger in the brain. Researchers compared pramipexole with a dummy pill (placebo). After 3.5 months, 36% of those who took pramipexole said they felt much better, compared to 9% of those who received a dummy pill. Overall, patients had a 50% or greater decrease in pain.
One small 2005 study conducted in Spain suggests that the atypical antipsychotic olanzapine (Zyprexa) may be a beneficial add-on therapy for patients with fibromyalgia. Although proven effective for some chronic pain conditions, olanzapine and other antipsychotics cause unpleasant and potentially serious side effects.

Tropisetron. Tropisetron (Navoban) is a drug used to reduce vomiting during chemotherapy. European studies suggest that it may also help patients with fibromyalgia by reducing pain, dizziness, and depression, and by improving sleep. Fatigue and dizziness are the most common side effects.

Much of the pain patients experience occurs where muscles join tendons or bones, particularly when the muscles are stretched. Stretching or flexibility exercises are part of the warm-up and cool-down routines of any regular exercise program. Stretching techniques may also use injections or cooling agents to inactivate the pressure points so that muscles can be more effectively stretched. These techniques must be performed by a person other than the patient, usually a family member or close friend. With either injections or the spray, the benefits may last from a few days to weeks. Neither the spray nor the injection is useful without muscle stretching.

Spray and Stretch. One technique is known as "spray and stretch." This method uses the following approach:

The patient must be in a comfortable position.
The partner presses on suspected tender points and the patient reports any pain.
The points, when targeted, are sprayed with either ethyl chloride (Chloroethane) or Fluori-Methane. These chemicals are not numbing medicines. They cool the blood vessels in the skin to inactivate the tender points. Numbing skin creams do not appear to be effective for this treatment.
The spray bottle is held upside-down about 12 - 18 inches from the targeted area. The patient's face should be covered if the spray is being used near the head.
The patient's partner then slowly stretches the affected muscle.

After the procedure, the muscle should feel looser, and the patient should have a greater range of motion with that muscle.

Trigger-Point Injections. In some cases, "trigger-point injections" of a numbing drug, such as lidocaine, may be used for particularly painful tender points as an aid to stretching.

The injection causes intense, but brief, pain in the trigger point. After the medication has taken effect, however, the muscle's ability to stretch is much greater.
There is some soreness afterward, which can be severe. After an injection, spraying the whole muscle with cooling agents may inactivate less severe tender points.
In some cases, injections may be needed several times over 6 - 8 weeks.

Resources

www.rheumatology.org -- American College of Rheumatology
www.niams.nih.gov -- National Institute of Arthritis and Musculoskeletal and Skin Diseases
www.arthritis.org -- Arthritis Foundation
www.fmaware.org -- National Fibromyalgia Foundation
www.fmpartnership.org -- National Fibromyalgia Partnership
www.fmnetnews.com -- Fibromyalgia Network
www.aapainmanage.org -- American Academy of Pain Management
www.ampainsoc.org -- American Pain Society
www.medicalacupuncture.org -- American Association of Medical Acupuncture
www.asch.net -- American Society of Clinical Hypnosis
www.aabt.org -- Association for Advancement of Behavior Therapy
www.clinicaltrials.gov -- Find a clinical trial

References

Arnold LM, Goldenberg DL, Stanford SB, Lalonde JK, Sandhu HS, Keck PE, et al. Gabapentin in the treatment of fibromyalgia: a randomized, double-blind, placebo-controlled multicenter trial. Arthritis & Rheumatism. 2007;56:1336-1344.

Assefi NP, Sherman KJ, Jacobsen C, Goldberg J, Smith WR, Buchwald D. A randomized clinical trial of acupuncture compared with sham acupuncture in fibromyalgia. Ann Intern Med. 2005; 143(1): 10-9.

Da Costa D, Abrahamowicz M, Lowensteyn I, Bernatsky S, Dritsa M, Fitzcharles MA, Dobkin PL. A randomized clinical trial of an individualized home-based exercise programme for women with fibromyalgia. Rheumatology. 2005;44:1422-1427.

Harris RE, Clauw DJ. How Do We Know That the Pain in Fibromyalgia Is "Real"? Current Pain and Headache Reports. 2006;10:403-7.

Harris RE, Clauw DJ, Scott DJ, McLean SA, Gracely RH, Zubieta JK. Decreased central u-opioid receptor availability in fibromyalgia. J Neurosci. 2007;27:10000-10006.

Holman AJ, Myers RR. A Randomized, Double-Blind, Placebo-Controlled Trial of Pramipexole, a Dopamine Agonist, in Patients With Fibromyalgia Receiving Concomitant Medications. Arthr Rheum. 2005; 52(: 2495-2505.

Mannerkorpi K, Henriksson C. Non-pharmacological treatment of chronic widespread musculoskeletal pain. Best Pract Res Clin Rheumatol. 2007;21:513-534.

McCabe CS, Cohen H, Blake DR. Somaesthetic disturbances in fibromyalgia are exaggerated by sensory-motor conflict: implications for chronicity of the disease? Rheumatology. 2007;46:1587-1592.

Mease P. Fibromyalgia syndrome: review of clinical presentation, pathogenesis, outcome measures, and treatment. J Rheumatol Suppl. 2005;32(10):2063.

Rico-Villademoros F, Hidalgo J, Dominguez I, García-Leiva JM, Calandre EP. Atypical antipsychotics in the treatment of fibromyalgia: a case series with olanzapine. Prog Neuropsychopharmacol Biol Psychiatry. 2005; 29(1): 161-4.

Rooks DS, Gautam S, Romeling M, Cross ML, Stratigakis D, Evans B, et al. Group exercise, education, and combination self-management in women with fibromyalgia. Arch Intern Med. 2007;167;2192-2200.

Van Koulil S, Effting M, Kraaimaat FW, van Lankveld W, van Helmond T, Cats H, et al. Cognitive-behavioural therapies and exercise programmes for patients with fibromyalgia; state of the art and future directions. Ann Rheum Dis. 2007;66:571-581.

Zheng L, Faber K. Review of the Chinese medical approach to the management of fibromyalgia. Curr Pain Headache Rep. 2005;9(5): 307-12.

Review Date:
12/17/2007
Reviewed By:
Harvey Simon, MD, Editor-in-Chief, Associate Professor of Medicine, Harvard Medical School; Physician, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

A.D.A.M. Copyright

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Sickle cell disease

FitSugar — Wed, 08 Oct 2008 17:35:29 -0700

In This Report

Highlights
Introduction
Risk Factors
Symptoms
Diagnosis
Outlook
Complications
Treatment
Prevention and Lifestyle Ch...
Resources
References

HEALTH GUIDE REFERENCE FROM A.D.A.M

Highlights

Screening for Sickle Cell Disease

The United States Preventive Services Task Force’s 2007 guidelines recommend that all newborn infants be screened for sickle cell disease. (In the United States, most states require hospitals to perform this test.) Early detection of sickle cell disease ensures that babies will be given treatment to prevent infections. Sickle cell disease is an inherited condition. About 1 in 375 African-American babies are born with sickle cell disease, but children of other ethnicities are also at risk.

Infections and Sickle Cell Disease

Children with sickle cell disease are highly susceptible to many life-threatening infections, including those caused by the pneumococcus bacterium. Pneumococcal vaccinations are an important protection against this bacterium. Research published in 2007 in Clinical Infectious Diseases indicates that the introduction of the pneumococcal conjugate vaccine has helped reduce by 90% the rate of pneumococcal infections in children with sickle cell disease. Four doses of this vaccine are given from age 2 - 15 months. A second type of pneumococcal vaccine, pneumococcal saccharide, is given when the child reaches 2 years of age.
Daily antibiotics given from age 2 months through 5 years can help prevent many other types of bacterial infections, such as meningitis and blood infections.

Introduction

Blood has two major components:

Plasma is a clear yellow liquid that contains proteins, nutrients, hormones, electrolytes, and other substances. It constitutes about 55% of blood.
White and red blood cells and platelets make up the balance of blood. The white cells are the infection fighters for the body, and platelets are necessary for blood clotting. The important factors in anemia, however, are red blood cells.

Red blood cells (RBCs), also known as erythrocytes, carry oxygen throughout the body to nourish tissues and sustain life. Red blood cells are the most abundant cells in our bodies. Men have about 5.2 million red blood cells per cubic millimeter of blood, and women have about 4.7 million red blood cells per cubic millimeter of blood. To understand red blood cells and their role in anemia, it is useful to know certain facts about them.

Hemoglobin and Iron. Each red blood cell contains about 280 million hemoglobin molecules. Hemoglobin is a complex molecule and the most important component of red blood cells. It is composed of protein (globulin) and a molecule (heme), which binds to iron.

In the lungs, the heme component binds to oxygen in exchange for carbon dioxide. The red blood cells carry the oxygen to the body's tissues, where the hemoglobin releases the oxygen in exchange for carbon dioxide, and the cycle repeats. The oxygen is used in the mitochondria, the power source within all cells.

Structure and Shape. Red blood cells are extremely small and look something like tiny, flexible inner tubes. This unique shape offers many advantages:

It provides a large surface area to absorb oxygen and carbon dioxide.
Its flexibility allows it to squeeze through capillaries, the tiny blood vessels that join the arteries and veins.
Abnormally shaped or sized erythrocytes are typically destroyed and eliminated.

Blood Cell Production (Erythropoiesis). The actual process of making red blood cells is called erythropoiesis. (In Greek, erythro means "red" and poiesis means "the making of things.") The process of manufacturing, recycling, and regulating the number of red blood cells is complex and involves many parts of the body:

The body carefully regulates its production of red blood cells so that enough are manufactured to carry oxygen but not so many that the blood becomes thick or sticky (viscous).
Most of the work of erythropoiesis occurs in the bone marrow.
If the body needs more oxygen (at high altitudes, for instance), the kidney triggers the release of erythropoietin (EPO), a hormone that increases production of red blood cells in the bone marrow.
The lifespan of a red blood cell is 90 - 120 days. The liver and spleen remove old red blood cells from the blood.
When old red blood cells are broken down for removal, iron is returned to the bone marrow to make new cells.

Sickle cell disease occurs from genetic changes which causes a portion of the hemoglobin molecules to be abnormal:

Hemoglobin A (HbA). HbA is the hemoglobin molecule found in normal red blood cells during childhood and adulthood. People without sickle cell anemia have primarily this type of hemoglobin in their blood cells.
Hemoglobin S (HbS). HbS (S is for sickle) is the abnormal variant of hemoglobin A, which occurs in sickle-red blood cells and is the primary characteristic of the disease. The difference between hemoglobin A (HbA) and hemoglobin S (HbS) lies in only one protein out of about 300 that are common to both. This protein lies along an amino-acid chain called beta-globin, where even a tiny abnormality has disastrous results.

Hemoglobin is the most important component of red blood cells. It is composed of a protein called heme, which binds oxygen. In the lungs, oxygen is exchanged for carbon dioxide. Abnormalities of an individual's hemoglobin value can indicate defects in red blood cell balance. Both low and high values can indicate disease states.

Hemoglobin F (HbF) is a form of hemoglobin that is produced during fetal development in the womb. (The F in HbF stands for fetal.) It is usually present for only a short time after birth. Normally, most HbF is later replaced by HbA, although some HbF may persist throughout life. Importantly, HbF is able to block the sickling action of red blood cells. Infants who have inherited sickle cell disease do not develop symptoms of the illness while they still have HbF present in their blood. People with the sickle cell gene who continue to carry some fetal hemoglobin are better protected, therefore, from severe forms of the disease. This knowledge is being used as the basis for therapies used in treating sickle cell disease.

The symptoms and problems of sickle cell disease are a result of the hemoglobin S (HbS) molecule:

When the sickle hemoglobin molecule loses its oxygen, it forms rigid rods called polymers that change the red blood cells into a sickle or crescent shape.
These abnormally sickle-shaped cells are both rigid and sticky. They stick to the walls and cannot squeeze through the capillaries. Blood flow through tiny blood vessels becomes slowed or stopped throughout the body. This deprives tissues and organs of oxygen.
In the immediate setting, oxygen deprivation (hypoxia) can cause severe pain (the sickle cell crisis). Over time, it leads to gradual destruction in organs and tissues throughout the body.

Click the icon to see an image of sickle cells.

In a vicious cycle, oxygen deprivation in cells leads to more polymerization and increased production of sickle cells. The higher the concentration of sickle hemoglobin and the more acidic the environment, the faster the sickle cell process.
Cell dehydration (not enough water molecules) is another major destructive factor in the sickling process of red blood cells. Dehydration increases the density of hemoglobin S within the cell, thereby speeding up the sickling process.
Sickle cells also have a shorter life span (10 - 20 days) than that of normal red blood cells (90 - 120 days). Every day the body produces new red blood cells to replace old ones, but sickle cells become destroyed so fast that the body cannot keep up. The red blood cell count drops, which results in anemia. This gives sickle cell disease its more common name, sickle cell anemia.

The severity of sickle cell disease generally depends on a number of factors:

The extent of oxygen loss. Prolonged oxygen deprivation contributes to the severe pain experienced as a sickle cell crisis. It also produces both short- and long-term organ damage. The lungs are specifically critical targets of the disease process. Because they supply oxygen, they can restore the sickle molecules to a normal form. Unfortunately, once the process occurs, the lungs become major sites for sickle cell damage, particularly for dangerous acute episodes of chest pain.
The acidity of the environment. The lower the better. The organs most seriously affected are those with an acidic environment (such as the spleen and bone marrow).
The concentration of hemoglobin S within the cell. The lower the better.
The amount of a protective hemoglobin F (for fetal). The more the better.

Risk Factors

Sickle cell disease is inherited. People at risk for inheriting the gene for sickle cell descend from people who are or were originally from Africa and parts of India and the Mediterranean. The sickle cell gene also occurs in people from South and Central America, the Caribbean, and the Middle East. The high incidence of the sickle cell gene in these regions of the world is due to the sickle cell's ability to make red blood cells resistant to the malaria parasite:

People who inherit just a single gene are referred to as having the sickle trait. These people are protected against malaria and do not develop sickle cell disease. About 40% of people in certain parts of Africa and about 9% of African-Americans have the trait.
Those who inherit both copies of the HbS gene develop sickle cell disease. They are not protected from malaria, however. In fact, malaria is more serious in these individuals. An estimated 1 in 500 African-Americans and 1 in 1,000 - 1,400 Hispanic Americans are born with sickle cell disease.

The sickle cell gene for hemoglobin S (HbS) is the most common inherited blood condition in America. About 72,000 Americans -- mostly African-Americans -- have sickle cell disease. The risk for inheriting sickle cell disease from parents with the sickle cell gene is as follows:

One parent has only one copy of the sickle cell gene and the other parent has two normal hemoglobin genes, and the child inherits a healthy gene from each parent. The child will not inherit either the disease or the trait.
The child inherits one copy of the sickle cell gene. The child has the trait (HbS) only. The other, healthy hemoglobin gene overrides HbS and blocks the development of sickle cell disease. Such people lead normal lives.
The child inherits the hemoglobin S gene from both parents (HbSS). The child develops the full-blown disease. (If each parent has one copy of the gene, the child has a 25% chance of acquiring the disease.)
The child inherits one hemoglobin S gene and one abnormal hemoglobin gene from other causes (such as one form called HbSC). Such children may develop a form of sickle cell disease. It is often a milder variant, but children can experience severe symptoms. They are also at risk for some of the complications of sickle cell disease, although their risks for serious problems are lower than in children with the full-blown disease.

Symptoms

General Symptoms in Infants. In infants, symptoms do not usually appear until late in the baby's first year. Most commonly, they include:

Fever
Swelling of the hands and feet
Pain in the chest, abdomen, limbs, and joints
Nosebleeds and frequent upper respiratory infections

General Symptoms in Childhood. Pain is the most common complaint. It can be acute and severe or chronic, usually from orthopedic problems in the legs and low back. Other symptoms include:

Anemia
Fatigue
Irritability
Jaundice (yellowish discoloration of the skin and eyes)
Bedwetting

Additional Symptoms in Adolescence or Adulthood. Symptoms of childhood continue in adolescence and adulthood. In addition, patients may experience:

Delayed puberty (in young teenagers)
Severe joint pain
Progressive anemia
Leg sores
Gum disease

The hallmark of sickle cell anemia is a group of devastating symptoms known collectively as a sickle cell crisis (also sometimes known as a vaso-occlusive crisis). Sickle cell crises are episodes of pain that occur with varying frequency and severity in different patients and are usually followed by periods of remission. Severe sickle cell pain has been described as being equivalent to cancer pain and more severe than postsurgical pain. It most commonly occurs in the lower back, leg, abdomen, and chest, usually in two or more locations. Episodes usually recur in the same areas.

The risk for a sickle cell crisis is increased by any activity that boosts the body's requirement for oxygen, such as illness, physical stress, or being at high altitudes. In more than half the cases, however, the trigger is unknown. Acute chest syndrome is a particularly serious complication of sickle cell crisis. It occurs in the lungs and can be extremely serious and even life threatening.

Diagnosis

Prenatal diagnosis of sickle cell disease is now possible for women who may be at risk for having a child with the disease. A positive result for sickle cell disease, however, poses extremely difficult questions even for parents who are not opposed to abortion.

A genetic test known as preimplantation genetic diagnosis (PGD) may prove to determine the presence or absence of the sickle cell mutation in embryos (fertilized eggs) before they are implanted in the mother during assisted fertilization techniques. This genetic tool may eventually help avoid the often emotionally devastating effects of abortion.

In the United States, most hospitals screen newborn babies for sickle cell disease. To perform the test, a blood sample is taken from the baby's heel using a simple needle prick. Early detection of sickle cell disease can help reduce the risk for life-threatening infections and increase the odds for survival. Babies who are diagnosed with sickle cell disease are given daily antibiotics to help prevent infections.

Unfortunately, no tests can definitely determine which children are at highest risk for a stroke and, therefore, would be candidates for ongoing blood transfusions. The following are diagnostic tools currently used or under investigation:

Transcranial Doppler (TCD) ultrasonography measures the speed of blood flow in the brain and is the most sensitive method to date for identifying children at risk for stroke. However, high-risk children are still vulnerable to stroke even if the TCD screening diagnosed normal blood flow velocities.
The use of follow-up magnetic resonance imaging (MRI) to detect small blockages in blood vessels may help confirm high risk in patients identified by TCD ultrasound.
Some patients may need to undergo angiography, an invasive diagnostic technique useful for detecting aneurysms.
Researchers are also beginning to uncover possible genetic markers that may eventually be used to help identify sickle cell patients at higher risk for stroke.

Outlook

New and aggressive treatments for sickle cell disease are prolonging life and improving its quality. As recently as 1973, the average lifespan for people with sickle cell disease was only 14 years. Currently, life expectancy for these patients can reach 50 years and over. Early studies showed that women had a greater risk for death from sickle cell disease than men, but experts now believe this was due to high mortality during pregnancies before the mid-1970s. Women with sickle cell disease now actually live longer than their male counterparts.

The damage and durability of sickle cell disease occurs because the logjam that sickle cells cause in the capillaries slows the flow of blood and reduces the supply of oxygen to various tissues. Not only does pain occur when body tissues are damaged by lack of oxygen, but serious and even life-threatening complications can result from severe or prolonged oxygen deprivation. Sickle cell disease is referred to in some African languages as "a state of suffering," but the disease has a wide spectrum of effects, which vary from patient to patient. In some people, the disease may trigger frequent and very painful sickle cell crises that require hospitalization. In others, it may cause less frequent and milder attacks.

Children with sickle cell disease are very susceptible to infections, usually because their damaged spleens are unable to protect the body from bacteria. A recent study suggested that signs of impaired lung function occur even in very early years. As medical progress has increased the lifespan of children with sickle cell disease, older patients are now facing medical problems related to the long-term adverse effects of the disease process. The most serious dangers are acute chest syndrome, long-term damage to major organs, stroke, and complications during pregnancy such as high blood pressure in the mother and low birth weight.

Complications

There is still no cure for sickle cell disease other than experimental transplantation procedures, but treatments for complications of sickle cell have prolonged the lives of many patients who are now living into adulthood.

The hallmark of sickle cell disease is the sickle cell crisis (also sometimes known as a vaso-occlusive crisis), which is an episode of pain. It is the most common reason for hospitalization in sickle cell disease. The pattern may occur as follows:

In general, the risk for a sickle cell crisis is increased by any activity that boosts the body's requirement for oxygen, such as illness, physical stress, or being at high altitudes. In more than half of episodes, however, the trigger is unknown.
Episodes typically begin at night and last 3 - 14 days, accelerating to a peak over several days and then declining.
The pain is typically described as sharp, intense, and throbbing. Severe sickle cell pain has been described as being equivalent to cancer pain and more severe than postsurgical pain. Shortness of breath is common.
Pain most commonly occurs in the lower back, leg, hip, abdomen, or chest, usually in two or more locations. Episodes usually recur in the same areas. Pain in the bones (usually occurring symmetrically on both sides) is common because blood obstruction can directly damage bone and because bone marrow is where red blood cells are manufactured.
The liver or spleen may become enlarged, causing pain in the upper right or upper left sides of the abdomen. Liver involvement may also cause nausea, low-grade fever, and increasing jaundice.
Males of any age may experience prolonged, often painful erections, a condition called priapism.

Episodes cannot be predicted, and they vary widely among different individuals. In one study, nearly 40% of patients reported no painful episodes over a 5-year period. About 5% of patients experienced severe and frequent episodes (more than three a year). They sometimes become less frequent with increasing age. Generally, people can resume a relatively normal life between crises. Most patients are pain-free between episodes although pain can be chronic in some cases.

Acute chest syndrome (ACS) occurs when the lungs are deprived of oxygen during a crisis. It can be very painful, dangerous, and even life threatening. It is a leading cause of illness among sickle cell patients and is the most common condition at the time of death. At least one whole segment of a lung is involved, and the following symptoms may be present:

Fever of 101.3°F degrees (38.5°C) or above
Rapid or labored breathing
Wheezing or cough
Acute chest pain

Pain often lasts for several days. In about half of patients, severe pain develops about 2 - 3 days before there are any signs of lung or chest abnormalities. Acute chest syndrome is often accompanied by infections in the lungs, which can be caused by viruses, bacteria, or fungi. Pneumonia is often present. A dull, aching pain usually follows, which most often ends after several weeks, although it may persist between crises.

Air is breathed in (inhaled) through the nasal passageways, and travels through the trachea and bronchi to the lungs.

Causes of Acute Chest Syndrome. Primary causes of acute chest syndrome include:

Infection. Infection from viruses or small atypical organisms (Chlamydia and Mycoplasma) is the most common cause of the oxygen deprivation that leads to acute chest syndrome.
Blockage of blood vessels. Blockage in the blood vessels (called infarction) that cuts off oxygen in the lungs is another important cause of acute chest syndrome. Blockage may be produced by blood clots or fat embolisms. (Fat embolisms are particles formed from fatty tissue in the bone marrow that enter and travel through the blood vessels.)
Asthma. Asthma can increase the frequency and pain of acute chest syndrome episodes in children, according to an important 2006 study. The researchers recommended that all children with sickle-cell disease who have frequent acute chest syndrome attacks should be evaluated for asthma.

In about 45% cases, the cause cannot be established. Some cases of acute chest syndrome may result from treatments of the crisis, including from administration of opioids (which reduce oxygen) or excessive use of intravenous fluids. Other lung diseases may also trigger ACS.

Severity of Acute Chest Syndrome. The mortality rates for ACS are around 2% in children and 4% in adults. The syndrome and its long-term complications are the major causes of death in older patients. The condition is four times more deadly in adults than in children. The longer a patient survives, the greater is the damage done by repetitive sickle cell crises in the chest and lungs.

The following destructive effects can occur:

Damage in the chest area from recurrent episodes increases susceptibility to invading infections, even those that are ordinarily not harmful. Infections frequently clear up if they are limited to small areas of the lung, but if they spread, they can progress very quickly and become life threatening.
Lung damage over time can lead to obstruction in the airways in lungs, causing asthma-like conditions.

Infections are common and an important cause of severe complications in sickle cell patients. Before early screening for sickle cell disease and the use of preventive antibiotics in children, 35% of infants with sickle cell died from infections. Fortunately, with screening tests for sickle cell now required for newborns in most states, and with the use of preventive antibiotics in babies who are born with the disease, this terrible mortality rate has dropped significantly.

Infections in Infants and Toddlers with Sickle Cell Disease. The most common organisms causing infection in children with sickle cell disease include:

Streptococcus pneumoniae (can cause blood infections or meningitis)
Haemophilus influenza (a cause of meningitis)

Such infections pose a grave threat to infants and very young children with sickle cell disease. They can progress to fatal pneumonia with devastating speed in infants, and death can occur only a few hours after onset of fever. The risk for pneumococcal meningitis, a dangerous infection of the central nervous system, is also significant.

Infections in Children and Adults. Infections are also common in older children and adults with sickle cell disease, particularly respiratory infections such as pneumonia, kidney infections, and osteomyelitis, a serious infection in the bone. (The organisms causing them, however, tend to differ from those in young children.) Infection-causing organisms include:

Chlamydia and Mycoplasma pneumoniae. These are the important infections in acute chest syndrome (see above).
Gram-negative bacteria. This group of bacteria mostly infects hospitalized patients and can cause serious pneumonias and other infections.

About 30% of patients with sickle cell disease have pulmonary hypertension. Pulmonary hypertension is a serious and potentially deadly condition that develops when pressure in the arteries of the lungs increases. It is an often unrecognized complication and cause of death in sickle cell disease. Many doctors recommend that all adults with sickle cell disease undergo echocardiographic testing to identify if they are at risk for pulmonary hypertension and require treatment.

Researchers are developing new types of tests that may help with early identification of pulmonary hypertension. For example, some studies indicate that a simple blood test for the hormone brain natriuretic peptide (BNP) could help identify patients with sickle cell pulmonary hypertension. Higher levels of BNP are associated with increased pressure in the pulmonary (lung) arteries. A blood test measuring levels of the enzyme lactate dehydrogenase (LDH) may also help identify patients at risk for pulmonary hypertension, as well as leg ulcerations and priapism (persistent and painful erection of the penis). Echocardiography or other tests would still need to be performed to confirm results from these blood tests.

The primary symptom of pulmonary hypertension is shortness of breath, which is often severe. Pulmonary hypertension can be very serious and life threatening in the short- and long-term. If pulmonary hypertension develops suddenly it can cause respiratory failure, which is life threatening. Over time, pulmonary hypertension may cause a condition called cor pulmonale, in which the right side of the heart increases in size. In some cases, this enlargement can lead to heart failure.

Click the icon to see an image of cor pulmonale.

After acute chest syndrome, stroke is the most common killer of patients with sickle cell disease who are older than 3 years old. Between 8 - 10% of patients suffer strokes, typically at about age 7. Patients may also suffer small strokes that may not be immediately noticeable. However, patients who have many of these small strokes may over time start behaving differently or have worsening mental functioning.

Strokes are usually caused by blockages of vessels carrying oxygen to the brain. Patients with sickle cell disease are also at high risk for stokes caused by aneurysm, a weakened blood vessel wall that can rupture and hemorrhage. Multiple aneurysms are common in sickle cell patients, but they are often located where they cannot be treated surgically.

Click the icon to see an image of stroke.

Anemia is a significant characteristic in sickle cell disease (which is why the disease is commonly referred to as sickle cell anemia).

Severe worsening of anemia. Children, adolescents, and possibly young adults may experience what is called splenic sequestration. This happens when a large amount of the sickled red blood cells collect in the patient's spleen. Symptoms may include pain in the right abdomen below the ribs and a large mass (the swollen spleen) may be felt.

Chronic Anemia. Because of the short lifespan of the sickle red blood cells, the body is often unable to replace red blood cells as quickly as they are destroyed. This causes a particular form of anemia called hemolytic anemia. Most patients with sickle cell disease have a hemoglobin levels of 8 g/dL, much lower than people without sickle cell anemia. Chronic anemia reduces oxygen and increases the demand on the heart to pump more oxygen-bearing blood through the body. Eventually, this can cause the heart to become dangerously enlarged, with an increased risk for heart attack and heart failure.

On occasion, patients may experience what is called an aplastic crisis. This happens when the cells in the bone marrow that are normally trying to make new red blood cells suddenly stop working. This sudden stopping is often triggered by a virus called human parvovirus B19.

The kidneys are particularly susceptible to damage from the sickling process. Persistent injury can cause a number of kidney disorders, including infection. Problems with urination are very common, particularly uncontrolled urination during sleep. Patients may have blood in the urine, although this is usually mild and painless and resolves without damaging consequences. Kidney failure is a major danger in older patients and accounts for 10 - 15% of deaths in sickle cell patients. Renal medullary carcinoma is an aggressive, rapidly destructive tumor in the kidney that is rare but can occur as a result of sickle cell disease.

Click the icon to see an image of kidney anatomy.

A reported 38 - 42% of males, including children, with sickle cell disease suffer from priapism. Priapism causes prolonged and painful erections that can last from several hours to days. Experts think that priapism in sickle cell disease may be caused by the destruction of red blood cells and subsequent reduction of nitric oxide. If priapism is not treated, partial or complete impotence can occur in 80% of cases.

Click the icon to see an image of the male reproductive anatomy.

Enlargement of the liver occurs in over half of sickle cell patients, and acute liver damage occurs in up to 10% of hospitalized patients. Because sickle cell patients often need transfusions, they have been at higher risk for viral hepatitis, an infection of the liver. This risk, however, has decreased since screening procedures for donated blood have been implemented.

About 30% of children with sickle cell disease have gallstones, and by age 30, 70% of patients have them. In most cases, gallstones do not cause symptoms for years. When symptoms develop, patients may feel overly full after meals, have pain in the upper right quadrant of the abdomen, or have nausea and vomiting. Acute attacks can be confused with a sickle cell crisis in the liver. Ultrasound is usually used to confirm a diagnosis of gallstones. If the patient does not have symptoms, no treatment is usually necessary. If there is recurrent or severe pain from gallstones, the gallbladder may need to be removed. Minimally invasive procedures (using laparoscopy) reduce possible complications. [For more information, see In-Depth Report #10: Gallstones.]

Click the icon to see an image of cholithiasis.

The spleen of most adults with sickle cell anemia is nonfunctional due to recurrent episodes of oxygen deprivation that eventually destroy it. Injury to spleen causes problems in immune function and increases the risk for serious infection. A very serious anemic condition called acute splenic sequestration crisis (sudden spleen enlargement) can occur if the damaged spleen suddenly becomes enlarged from trapped blood.

Click the icon to see an image of an enlarged spleen.

In some children with sickle cell disease, excessive production of blood cells in the bone marrow causes bones to grow abnormally, resulting in long legs and arms or misshapen skulls. Sickling that blocks oxygen to the bone can also cause bone loss and pain. Sickling that affects the hands and feet of children causes a painful condition called hand-foot syndrome. A condition called avascular necrosis of the hip occurs in about half of adult sickle cell patients when oxygen deprivation causes tissue death in the bone. Eventually adult patients may require surgery to remove diseased and dead bone tissue. Joint replacement may be required in severe cases. X-rays are not very useful for detecting early disease in the bones. MRI may be important.

Click the icon to see an image of the blood supply to bone.

Leg sores and ulcers occur in up to 10% of sickle cell patients and usually affect patients older than 10 years.

Women with sickle cell disease who become pregnant are at higher risk for complications, but serious problems have dropped significantly over the past decades. One study reported a higher risk for premature birth and low birth weight in the baby, and a higher risk for infections and hospital visits in the mother after delivery. Pain crises occurred in nearly half of the women, and nearly 60% required transfusions. The study also reported, however, that, in general, the outcome for pregnancy is favorable. Still, pregnancy during sickle cell is high-risk and carries a mortality rate of about 1%.

Older children and adult patients with sickle cell are subject to other medical problems, including impaired physical development, gum disease, and scarring and detachment of the retina.

Treatment

Research is ongoing toward identifying the biologic and chemical activities that promote or protect against the sickle cell process. Currently, experimental treatments focus on the basic processes that cause the red blood cells to sickle in the first place. There are three basic modes of treatment:

Stimulation of production of healthy fetal hemoglobin in order to inhibit the sickling process
Blocking dehydration in the cells
Transplantation of bone marrow or stem cells from healthy donors so that normal hemoglobin is produced rather than hemoglobin S

Hemoglobin F (HbF), also called fetal hemoglobin, is the form of hemoglobin in the fetus and small infants. Most HbF is later replaced by the hemoglobin that is present in the growing child and adult, although some HbF may persist. Fetal hemoglobin is able to block the sickling action of red blood cells so that infants with sickle cell disease do not develop symptoms of the illness while they still have hemoglobin F. Adults who have sickle cell disease but still retain high levels of hemoglobin F generally have mild disease.

Studies now suggest that the severity of sickle cell disease can be reduced by using drugs that stimulate production of HbF. Even increases as modest as 4% may have significant benefits for these patients.

Hydroxyurea. Hydroxyurea (Droxia, Hydrea) destroys cells in the bone marrow, which results in an increase in special cells that can produce HbF. It is currently the only drug in general use to prevent acute sickle cell crises.

Hydroxyurea is used to treat adults and adolescents with moderate-to-severe recurrent pain (occurring three or more times a year). Hydroxyurea reduces sickling crises and pain, priapism, the number of transfusions, and life-threatening complications in this group. The benefits appear to be long-lasting. Hydroxyurea is not a cure-all. Not all patients respond to hydroxyurea, and the best candidates for the treatment are not yet clear. Small studies have reported no protection from damage in the spleen or bones and joints. Effects on stroke and complications in the eye or kidney are not yet known.

Hydroxyurea is still being investigated in young people. To date, the response to the drug in children and teenagers with sickle cell disease is similar to the response in adults, and few severe adverse effects are being reported. Recent research also suggests that hydroxyurea is safe and beneficial for infants. A 2005 study indicated that long-term hydroxyurea treatment can improve height, weight, and spleen function, and reduce episodes of acute chest syndrome. Patients in the study started the treatment as babies, and most patients took the drug for at least 4 years. The drug was given by mouth in a flavored liquid form.

Side effects include gastrointestinal problems, headache, drowsiness, and skin and nail changes. In rare cases, there have been reports of hallucinations and seizures. The drug may also cause leg ulcers and gangrene in some patients. Patients should handle hydroxyurea with care and wash their hands before and after touching the bottle or capsules. Household members who are not taking hydroxyurea (such as caregivers) should wear disposable gloves when handling the medicine or its bottle.

Cytidine Analogues. Cytidine analogues increase HbF production by affecting the genes that regulate it. Decitabine is one such drug that was developed to treat leukemia and other blood malignancies. Early studies are suggesting that it significantly increases HbF production, even in patients in whom treatment with hydroxyurea failed. Only minor toxic side effects have been reported to date.

Butyrates. Butyrates are natural fatty acids, the end-products of fermented carbohydrates in the intestinal tract that are also metabolized from fiber. One derivative, arginine butyrate, has been under investigation for some time in sickle cell for its role in stimulating production of HbF. Because its actions are different from hydroxyurea, experts hope the two drugs may eventually be used in combination. However, arginine butyrate is difficult to administer, and different forms that might make it simpler to use are needed.

General Guidelines for Managing a Sickle Cell Crisis. The basic objectives for managing a sickle cell crisis are control of pain and rehydration by administration of fluids. Oxygen is typically given for acute chest syndrome. Effective pain medications are available to help reduce the severe pain of sickle cell crises.

Accurate and continually updated assessment of pain determined by patient input and participation is at the crux of effective care for children with sickle cell disease. Often, however, patients are not given the treatment they require.

Many patients, their families, and even doctors are hesitant to use opioids aggressively because of fear of addiction. This fear, however, is nearly always unwarranted. Addiction occurs in only about 1 - 3% of patients with sickle cell disease who are taking opioids.
Many patients use emergency rooms of large hospitals for treating acute pain. Waiting times are long, and there is no single health care provider who knows the patient and can offer consistent assessment and management of pain.
Many doctors do not understand the nature of sickle cell pain. For example, early phases of sickle cell crisis can cause severe pain before test results confirm a diagnosis of a crisis. In such cases, health professionals may question the patient's self-reporting and withhold appropriate pain medication.
Patients may behave normally (talking on the phone, sleeping) and not appear to be in pain, but have actually developed coping behaviors to allow them to function in spite of severe pain.
Children and adults report pain differently, with children tending to report less pain than they actually feel. (One way of determining the severity of pain that a child feels is to show pictures of faces demonstrating degrees of pain and asking the child to point to the one that best expresses his or her experience.)

Adult patients and parents of children with the disease should insist on aggressive pain-relief treatment. If doctors show any reluctance to administer medications after the onset of pain, patients or caregivers should not hesitate to seek a more responsive health care professional.

All patients should have a treatment plan that helps guide them and their families during a pain episode. Plans should outline which medicines to take and when to seek medical help. Patients and families should learn to recognize symptoms early and begin managing with an appropriate amount of pain medication.

Opioids. Severe pain should be treated with strong painkillers, usually opioids. Opioids are generally given orally to adults and adolescents and intravenously to children. Nevertheless, there are exceptions. Studies indicate that oral medications are also effective in children.

Morphine is often used for frequent or prolonged episodes of pain. Unfortunately, its effectiveness is not as long-lasting in sickle cell patients as it is in other patients with severe pain, such as those with cancer.
The opioid meperidine (Demerol) is also used for sickle cell crises. Meperidine is not as powerful as morphine, however, and, if used for prolonged periods, may cause twitches, tremors, and disturbed mental states including seizures.
Some newer synthetic opioids such as fentanyl (Duragesic) or hydromorphone(Dilaudid) have a rapid onset and possibly fewer side effects than morphine. Fentanyl can be applied using a patch, which may help some patients who have difficult receiving intravenous drugs. It takes 12 hours to be effective, however.
Oral drugs, such as methadone, oral morphine, codeine, and oxycodone, are useful for home management of chronic pain and for transitional treatments between the hospital and home. Tramadol (Ultram) is a potent oral painkiller that has opioid-like properties but is not as addictive. (Dependence and abuse have been reported, however.) It may be very useful for sickle cell patients who need painkillers outside the hospital. It has minimal effects on respiratory function and has a low potential for addiction.

Possible side effects of opioids are vomiting and nausea, itching, constipation, itching, skin rashes, and problems urinating. If the patient vomits or becomes nauseated, the doctor may prescribe prochlorperazine (Compazine). Devices have been developed to allow patients to administer their own painkillers as needed.

Anti-Inflammatory Drugs. Because of the potentially serious side effects of opioids, doctors are constantly searching for safer and easier ways of reducing the severity of pain of sickle cell crises. Because experts believe that inflammation is a major contributor to the pain of sickle cell disease, drugs that reduce inflammation are being studied:

Prescription-strength NSAIDs include diflunisal (Dolobid) and ketorolac (Toradol). Ketorolac may be particularly helpful in relieving bone pain, and may be effective for individuals who cannot tolerate opioids. In one study, it was superior to meperidine and had fewer side effects. Studies have suggested, however, that when used as first-line therapy in an acute crisis, ketorolac is effective only in about half of episodes.
Corticosteroids are powerful anti-inflammatory drugs that are commonly used to treat pain caused by inflamed muscles and joints. Such drugs include methylprednisolone (Medrol) and dexamethasone (Decadron, Hexadrol). Studies suggest that using these drugs along with opioids may help some sickle cell patients. Because steroids can suppress the body's infection fighters, they should not be given to patients with bacterial infections or any serious medical complication.

Epidural Anesthesia. An epidural analgesia (injection of an anesthetic into the spinal fluid) may be very effective for pain that is unresponsive to the usual therapies.

Initial Management. Acute chest syndrome can be fatal and must be treated immediately. Basic treatments include the following:

Supplementary oxygen -- this is critical and life saving.
Administration of fluids -- overhydration should be avoided to reduce the risk of fluid in the lungs.
Pain relievers
Bronchoscopy (a diagnostic procedure involving insertion of a tube into the lower airways) may be needed to identify infection.

Other Treatments. Other treatments include:

High-dose intravenous corticosteroids (usually dexamethasone) may hasten recovery from acute chest syndrome and reduce the duration of hospitalization. They are also important if fat embolisms develop.
Antibiotics that specifically target the organisms ( Chlamydia, Mycoplasma) that commonly trigger acute chest syndrome. Such antibiotics include erythromycin, azithromycin, clarithromycin, and various tetracyclines.
Transfusions are important early on for rapid improvement in severe cases, especially if fat embolisms have developed.

To increase oxygen levels in children hospitalized for acute chest syndrome, a simple breathing technique known as incentive spirometry may also be beneficial. A spirometer is a hand-held plastic device commonly used by asthma patients to measure their lung capacity and by patients after surgery to increase intake of oxygen. Patients with sickle cell disease are asked to inhale and exhale into this device every 2 hours during the day and when wake at night until their chest pain subsided. This device forces more air into the lungs, and may help prevent the serious drop in oxygen levels and the risk for infection caused by acute chest syndrome. Spirometry leads to slower rates of collapsed lung tissue and infections. This very inexpensive and simple treatment might have beneficial long-term effects.

General Approach to Treating Infections. Fever in any sickle cell patient should be considered an indication of infection. Temperatures over 101°F in children warrant a call to the doctor. Adults with sickle cell should call the doctor if they have a have fever over 100°F and any signs of infection, including chest pain, productive cough, urinary problems, or any other symptoms. Some approaches for treating infections include:

Hospitalization for infections. When sickle cell patients develop infections, they are nearly always hospitalized immediately and treated with intravenous or high-dose injections of antibiotics in order to prevent septicemia, the dangerous spread of the infection throughout the body. Antibiotics called cephalosporins [cefotaxime (Claforan), ceftriaxone (Rocephin), or cefuroxime (Ceftin)] are typically used. Repeated hospitalizations are very disruptive for both children and adults. Studies have found that older children whose fever is below 38.5°C (101°F) and who have no serious infection or other complications may not need hospitalization. Children who have indications of serious complications of infection (higher fevers, pain, a history of pneumonia, and signs of dehydration) should remain in the hospital.
Treatment of osteomyelitis. If osteomyelitis, an infection in the bone, occurs, a 6-week antibiotic course is needed, most of it intravenous. An accurate diagnosis of osteomyelitis is sometimes difficult to make, because bone damage from sickling can cause similar symptoms. It should be strongly considered in children with signs of pain and swelling in the legs, a high white blood cell count, high fever, and high levels of a test that measures so-called sedimentation rates. It is important, however, to confirm the presence of an actual infection before administering antibiotics, because the antibiotic treatment required for osteomyelitis is so intensive and prolonged. The most common cause of osteomyelitis in children is Salmonella.
Treatment of urinary tract infections. Urinary tract infections may be difficult to manage and can be a serious problem for pregnant women with sickle cell disease. Doctors should take a urine culture before beginning antibiotic treatment and another culture 1 - 2 weeks after treatment to be sure the infection has cleared up.

Bosentan (an endothelin receptor antagonist) and other drugs are used to treat this condition. Investigational therapies include nitric oxide, L-arginine (which converts to nitric oxide), blood transfusions, warfarin, vasodilators, and sildenafil (Viagra). Hydroxyurea does not appear to help.

Folic acid and possibly iron supplements are often given to help treat the anemia that occurs in patients with sickle cell disease. (Patients who are given multiple transfusions may experience iron overload, and iron supplements should be avoided in such cases. Also, folic acid can mask pernicious anemia, which is caused by deficiency of vitamin B12 and is more common in African-Americans than other populations.)

Kidney damage in patients with sickle cell disease can cause bleeding into the urine. Mild episodes can usually be treated with bed rest and fluids. Severe bleeding may require transfusions. ACE inhibitors are drugs commonly used to control high blood pressure and are proving to be important for preventing hypertension and kidney failure in sickle cell patients. Such drugs include captopril (Capoten), enalapril (Vasotec), quinapril (Accupril), benazepril (Lotensin), and lisinopril (Prinivil, Zestril).

Priapism causes prolonged and painful erections that can last from several hours to days. It is best to relieve this problem within 12 hours. Relief within 36 hours is important to avoid permanent impotence. Pain relief and intravenous fluids are the initial steps. Exchange transfusions may be used to reduce the hemoglobin S and sickling that cause this condition. Drugs used to prevent priapism include terbutaline and phenylephrine, which help restrict blood flow to the penis. Hormonal treatments such as leuprolide (Lupron) and diethylstilbestrol may prevent repetitive and prolonged episodes of priapism in severely affected teenage boys with sickle cell disease. A surgical procedure that implants a shunt to redirect blood flow is sometimes performed. Inflatable penile implants may help maintain potency without causing priapism. Researchers are also investigating other treatments including inhaled nitric oxide, arginine, and sildenafil (Viagra).

The spleen is often removed (splenectomy) in children who have one or two acute splenic sequestration crises. Transfusion therapy is an alternative for preventing acute splenic sequestration in high-risk patients. At this time there are no studies comparing overall survival and benefits between the two approaches.

Leg ulcers are difficult to treat. Simple treatment with a moist dressing usually provides the best results. To treat mild ulcers, the leg should be gently washed with cotton gauze soaked in mild soap or a solution of one tablespoon of household bleach to one gallon of water. A dressing soaked in diluted white vinegar may be applied every 3 - 4 hours.

More severe ulcers require debridement, which is the removal of injured tissue until only healthy tissue remains. Debridement may be accomplished using chemical (enzymes), surgical, or mechanical (irrigation) means. Hydrogels (Nu-Gel, Intrasite Gel, Scherisorb, Clearsite, Duoderm, Geliperm) are helpful in healing ulcers and are noninvasive and soothing. Topical antibiotics, saline or zinc oxide dressings, or cocoa butter or oil are also used depending on severity. The leg should be elevated. Bed rest for a week or more is sometimes required for severe ulcers.

Skin grafts and transfusions have been helpful in some extreme cases. In one promising study administering arginine butyrate for many weeks improved ulcer healing by 10-fold. (This drug is also under investigation for other beneficial effects in patients with sickle cell disease.)

Women who are pregnant should be treated at a high-risk clinic. They should take folic acid in addition to multivitamins and iron. Standard treatment is given for sickle cell crises, which may occur more frequently during pregnancy. The benefits of transfusions to prevent crises during pregnancy are not yet clear and experts recommend them only for women who experience frequent complications during pregnancy.

Women with sickle cell disease should talk to their doctors before becoming pregnant. Sexually active women should use contraception at all times.

At this time, the only true cure for sickle cell disease is bone marrow or stem cell transplantation. The bone marrow nurtures stem cells, which are early cells that mature into red and white blood cells and platelets. By destroying the sickle cell patient's diseased bone marrow and stem cells and transplanting healthy bone marrow from a genetically-matched donor, normal hemoglobin may be produced. Clinical studies using a few carefully selected patients have reported very successful results.

Up to 80 - 85% of patients who meet criteria for receiving a transplant receive remain disease free. Unfortunately, only about 7% meet the criteria for transplantation, including those who:

Are age 16 or younger (generally considered the better candidates, but patients in their 20s have had successful transplants)
Have severe symptoms but no long-term organ or neurologic damage
Have a genetically matched brother or sister who will donate their marrow

Complications. Bone marrow transplant carries its own dangers and limitations. About 10% of those who have bone marrow transplants die from the treatment. Some complications include:

In patients who do not receive a bone marrow donation from a matched sibling, the transplanted cells from a donor (called allogeneic grafts) may attack the patient's own tissues, a potentially fatal condition called graft-versus-host disease (GVHD). Drugs that destroy bone marrow and suppress immunity must be administered before the procedure so that the body's immune system does not attack the transplanted tissue. Still, this does not always prevent the problem.
Other very serious complications include bleeding, pneumonia, and severe infection.
Those who live but are not cured face long-term problems caused by the drugs used in transplantation and by the disease itself.
Even in those who are cured, long-term consequences may include a higher risk for cancer and infertility.

The use of umbilical cord blood and cells from placentas is showing promise for providing healthy stem cells to patients who do not have genetically matched donors for bone marrow transplant. Cord blood has certain advantages over stem cell transplantation, including the capacity to produce more cells quickly. Because immune factors in cord blood are immature, the risk and severity of graft-versus-host disease may be reduced.

Early clinical trials are also reporting some success with a process called partial chimerism, in which a mixture of the patient's and a donor's bone marrow is used. The procedure has far fewer side effects because all the bone marrow is not destroyed. Although some sickle blood cells remain, small studies indicate that the patients are still free of the typical infections and pain of the disease.

Transfusions are often critical for treating sickle cell disease. In some cases, they may be given on a regular basis to prevent stroke or other life-threatening complications of the disease. Ongoing transfusions can reduce episodes of pain and acute chest syndrome. They can also help improve height and weight in children with sickle cell disease. Regular transfusions, however, can have severe side effects. Normal hemoglobin levels for patients with sickle cell disease are around 8 g/dL. Doctors will try to keep the hemoglobin level no higher than 10 g/DL after transfusion.

Transfusions may be required by sickle cell patients either for specific episodes (used only for specific events) or as chronic transfusions (ongoing transfusions).

Episodic Transfusions. Episodic transfusions are needed in the following situations:

To manage sudden severe events, including acute chest syndrome, stroke, widespread infection (septicemia), and multi-organ failure.
To manage severe anemia, usually caused by splenic sequestration (dangerously enlarged spleen) or aplasia (halting of red blood cell production, most often caused by parvovirus). Transfusions are generally not required for mild or moderate anemia.
Before major surgeries. Some evidence suggests that a conservative transfusion regime is as effective as aggressive transfusions in these cases, but more research is needed. Transfusions are generally not required for minor surgeries.

Chronic Transfusions. Chronic (on-going) transfusions are used for:

Stroke Prevention. Chronic transfusions are also used to prevent first or recurrent strokes. Evidence shows that regular (every 3 - 4 weeks) blood transfusions can reduce the risk of a first stroke by 90% in high-risk children. The objective of such transfusions is to reduce hemoglobin S concentrations to less than 30% of total hemoglobin. In addition, studies indicate that as many as 90% of patients who have experienced a stroke do not experience another stroke after 5 years of transfusions. In 2004, the National Heart, Lung, and Blood Institute (NHLBI) issued a clinical alert strongly advising doctors against terminating regular transfusions for high-risk children.
Pulmonary hypertension and chronic lung disease
Heart failure
Chronic kidney failure and severe anemia
Unusually severe and protracted episodes of pain

Chronic blood transfusions carry their own risks, including iron overload, alloimmunization (an immune response reaction), and exposure to bloodborne pathogens. Still, data from large-scale trials suggest that the risks for stroke outweigh the risks associated with transfusions. Researchers are working on ways to reduce the side effects associated with transfusion treatment.

Kinds of Transfusions. Transfusions may be either simple or exchange.

Simple Transfusion. Simple transfusions involve the infusion of one or two units of donor blood to restore blood volume levels and oxygen flow. It is used for moderately severe anemia, severe fatigue, and nonemergency situations when there is a need for increased oxygen. It is also used for acute chest syndrome.
Exchange Transfusion. Exchange transfusion involves drawing out the patient's blood while exchanging it for donor red blood cells. It can be done as manual procedure or as automatic one called erythrocytapheresis. Exchange transfusions should be used promptly if there is any evidence that the patient's condition is deteriorating. It prevents stroke and also may be used in patients with severe acute chest syndrome and to reduce the risk of iron overload in patients who require chronic transfusion therapy. Studies suggest that it may improve oxygenation and reduce hemoglobin S levels. Exchange transfusion may also reduce the risk of heart failure and help prevent fat embolism, a life-threatening condition in which fatty tissue from the bone marrow travels to blood vessels in the lungs and cuts off oxygen.

Iron Overload and Chelation Therapy. Iron overload increases risk for complications, including liver cancer and heart failure. A liver biopsy accurately determines whether excess iron levels are present. A non-invasive test called a superconducting quantum interference device (SQUID) should be used if available.

Chelation therapy is used to remove excess iron stores in the body that can harm the liver, heart, and other organs. The drug deferoxamine (Desferal) is commonly used during such therapy. Unfortunately, deferoxamine has some severe side effects and must be used with a pump for about 12 hours each day. Many patients do not continue treatment. In 2005, the drug deferasirox (Exjade) was approved for the treatment of transfusion-related iron overload in patients ages 2 and older. It is taken once a day by mouth. Patients mix the pills in liquid and drink the mixture. This new treatment may make chelation therapy much easier and less painful for patients.

Other Complications of Transfusion Therapy.

Immune reactions. An immune reaction may occur in response to donor blood. In such cases, the patient develops antibodies that target and destroy the transfused cells. This reaction, which can occur 5 - 20 days after transfusion, can result in severe anemia and may be life-threatening in some cases. It can be generally prevented with careful screening and matching of donor blood groups before the transfusion.
Hyperviscosity. With this condition, a mixture of hemoglobin S and normal hemoglobin causes the blood to become sticky. The patient is at risk for high blood pressure, altered mental status, and seizures. Careful monitoring can prevent this condition.
Transmission of viral illness. Before widespread blood screening, transfusions were highly associated with a risk for hepatitis and HIV. This complication has decreased considerably.

Nitric oxide, a soluble gas, is a natural chemical in the body that relaxes smooth muscles and expands blood vessels. Hemoglobin removes nitric oxide. Because sickle cells release hemoglobin, patients with the disease are deficient in nitric oxide. This lack of nitric oxide constricts blood vessels and causes pain in sickle cell diseases. In adult patients, men may be more susceptible to this effect than women. Some studies indicate that inhaling nitric oxide may slow the disease process and improve symptoms in acute sickle cell crises. It is difficult to administer, however. More studies are needed. (Nitric oxide is not the same substance as nitrous oxide, the so-called laughing gas used in dentistry.)

Sickle cell disease can cause red blood cells to break apart. This process is called hemolysis. Hemolysis causes a lack of the amino acid arginine. Arginine is involved in producing nitric oxide. Recent research suggests that a lack of arginine may contribute to the development of pulmonary hypertension, a leading cause of death in patients with sickle cell disease. Pulmonary hypertension causes high blood pressure in the arteries that carry blood to the lungs.

A 2005 study found that patients with sickle cell who had low levels of arginine were 3.6 times more likely to die than patients with high arginine levels. Most patients in the study died from pulmonary hypertension. Scientists are working on developing a blood test that could measure amino acid levels and help identify patients at greatest risk of death. They are also working on developing drugs that could block arginase, a protein in cells that is released during hemolysis, which consumes arginine. There is no evidence indicating that arginine nutritional supplements are helpful or harmful for patients with sickle cell disease. Patients should talk to their doctor before taking these or other supplements.

Researchers are studying the mechanisms behind cell membrane damage, dehydration, and potassium loss in order to develop drugs that will inhibit these processes. Drugs under investigation include those that specifically block the Gardos channel, which is an important route for potassium loss and dehydration. Researchers are also studying specific types of mineral supplements, such as magnesium pidolate and zinc sulfate. Initial studies have shown promising results for zinc’s efficacy in preventing red blood cell dehydration, but more research is needed.

Prevention and Lifestyle Changes

No o proven methods prevent either sickle cell crises or long-term complications of sickle cell disease. By taking precautions and aggressively managing problems that occur, however, patients are now living longer, with a better quality of life.

To prevent or reduce the severity of long-term complications, a number of precautions may be helpful:

Have regular physical examinations every 3 - 6 months.
Have periodic and careful eye examinations.
Have sufficient rest, warmth, and increased fluid intake. (These are critical precautions for reducing oxygen loss and the risk for dehydration.)
Avoid conditions, such as crowds, that increase risk for infections.
Avoid excessive demands on the body that would increase oxygen needs (physical overexertion, stress). Low impact exercise (leg lifts, light weights) may be useful and safe for maintaining strength, particularly in the legs and hips, but patients should consult their doctor about any exercise program.
Avoid high altitudes if possible. If flying is necessary, be sure that the airline can provide oxygen.
Do not smoke, and avoid exposure to second-hand smoke. Both active and passive smoking may promote acute chest syndrome in patients with sickle cell disease.

Vaccinations. Everyone with sickle cell disease should have complete regular immunizations against all common infections. Children should have all routine childhood vaccinations. The following are important vaccinations for everyone with sickle cell disease:

Pneumococcal vaccines. All sickle cell patients should be vaccinated with the pneumococcal vaccine. There are two types of pneumococcal vaccines; the choice between them depends on the age of the patient. Infants and children less than 2 years of age should receive 4 doses of the pneumococcal conjugated vaccine (Prevnar) between 2 - 15 months of age. (This vaccine has helped reduce the rate of serious pneumococcal disease by more than 90%.) The pneumococcal polysaccharide vaccine should be administered at age 2 years or older, repeated after 3 - 5 years for patients younger than age 10, or in 5 years for patients older than age 10.
Vaccination against Haemophilus influenza, the major cause of childhood meningitis, starting at age 2 months.
Influenza vaccines should be given every winter, starting at age 6 months.
Meningococcal vaccination for patients age 5 and older.
Hepatitis B vaccine. Anyone starting transfusion therapy should receive this vaccine.

Tuberculosis skin testing should be performed every year.

Antibiotics. In addition to regular immunizations, preventive (prophylactic) antibiotics are the best approach for protection against pneumonia and other serious infections among children with sickle cell disease. Babies diagnosed with sickle cell are given daily antibiotics, starting at 2 months of age and continuing through 5 years of age. Penicillin is usually the antibiotic given, unless a child is allergic to it.

Many patients stop taking their antibiotics or the parents stop giving them to their children. Doctors are concerned about developing bacterial resistance to common antibiotics and researchers warn that patients might experience breakthrough infections as resistance becomes more frequent.

Foods. Good nutrition, while essential for anyone, is critical for patients with sickle cell disease. Some dietary recommendations include:

Fluids are number one in importance. The patient should drink as much water as possible each day to prevent dehydration.
Diet should provide adequate calories, protein, fats, and vitamins and minerals. Patients and families should discuss vitamin and mineral supplements with their doctors and nurses.
Studies on omega-three fatty acids, found in fish and soybean oil, suggest that they might make red blood cell membranes less fragile, and possibly less likely to sickle, although no studies have proven this definitively. Fish and soy products have health benefits in any case. In one small study, fish oil supplements reduced the frequency of painful episodes over the course of a year.

Vitamins. Patients should take daily folic acid and vitamin B12 and B6 supplements. Vitamin B6 may have specific anti-sickling properties. Some experts recommend 1 mg folic acid, 6 microgram vitamin B12, and 6 mg vitamin B6. Foods containing one or all of these vitamins include meats, oily fish, poultry, whole grains, dried fortified cereals, soybeans, avocados, baked potatoes with skins, watermelon, plantains, bananas, peanuts, and brewer's yeast. Of note, folic acid can mask pernicious anemia, which is caused by deficiency of vitamin B12 and is more common in African-Americans than other populations.

Click the icon to see an image of vitamin B6 sources.

Note on Iron. Although sickle cell disease is often referred to as anemia, patients should avoid iron supplements or iron rich foods when receiving multiple transfusions, which increase the risk for iron-overload.

In assessing the seriousness of this disease, no one should underestimate its emotional and social impact. For the family, nothing is more heartbreaking than watching their child endure extreme pain and life-threatening medical conditions. The patient endures not only the pain itself but also the emotional strain from unpredictable bouts of pain, fear of death, and lost time and social isolation at school and work. Academic grades among patients average less than C, even in children with a low frequency of hospitalization (averaging 17 days a year).

These problems continue over the years, and both children and adults with sickle cell disease often suffer from depression. The financial costs of medical treatments combined with lost work can be very burdensome.

Any chronic illness places stress on the patient and family, but sickle cell patients and caregivers often face great obstacles in finding psychological support for the disease. Communities in which many sickle cell patients live generally lack services that can meet their needs, and professionals who work in their medical facilities are often overworked. In a study comparing patients with different kinds of long-term illnesses, those with sickle cell disease gave the lowest scores to their doctors and other professional caregivers for compassion, and were least satisfied with their medical care.

It is very important for patients and their caregivers to find emotional and psychological support. No one should or can endure this life-long disease alone. Unfortunately, studies indicate that most patients do not receive even basic supportive care that could help reduce the anxiety and intensity of pain that occurs when a sickle cell crisis erupts.

The following are some measures that some people find helpful in dealing with this disease:

Stress Reduction. Stress reduction techniques and relaxation methods appear to be helpful. Breathing and mediation techniques may be very helpful.
Cognitive-Behavioral Therapy. Studies suggest that cognitive behavioral therapies that teach coping skills can result in less negative thinking and even less pain. Coping skills refer to the patient's ability to respond to symptoms, such as pain.
On-Line Support Help. Computer on-line services are now valuable sources of support groups and access to research. They are particularly valuable for patients who cannot easily leave home or for patients who are ill.
Support Associations. Parent and professional support associations still offer the best and least expensive sources of help.

Other important factors are those that help maintain positive attitudes including spirituality, humor, or having important life goals (such as having children or pursuing a career).

Resources

www.sicklecelldisease.org -- Sickle Cell Disease Association of America
www.nhlbi.nih.gov -- National Heart, Lung, and Blood Institute (NHLBI)
www.scinfo.org -- Sickle Cell Information Center
www.sicklecellsociety.org -- Sickle Cell Society (UK)
www.sicklecell-info.org -- NHLBI Comprehensive Sickle Cell Centers
www.clinicaltrials.gov -- Find clinical trials

References

Adams RJ, Brambilla D; Optimizing Primary Stroke Prevention in Sickle Cell Anemia (STOP 2) Trial Investigators. Discontinuing prophylactic transfusions used to prevent stroke in sickle cell disease. N Engl J Med. 2005 Dec 29;353(26):2769-78.

Al Hajeri AA, Fedorowicz Z, Omran A, Tadmouri GO. Piracetam for reducing the incidence of painful sickle cell disease crises. Cochrane Database Syst Rev. 2007 Apr 18;(2):CD006111.

Bernaudin F, Socie G, Kuentz M, et al Long-term results of related myeloablative stem-cell transplantation to cure sickle cell disease. Blood. 2007 Oct 1;110(7):2749-56. Epub 2007 Jul 2.

Dunlop RJ, Bennett KC. Pain management for sickle cell disease. Cochrane Database Syst Rev. 2006 Apr 19;(2):CD003350.

Fathallah H, Atweh GF. Induction of fetal hemoglobin in the treatment of sickle cell disease. Hematology Am Soc Hematol Educ Program. 2006:58-62.

Halasa NB, Shankar SM, Talbot TR, et al. Incidence of invasive pneumococcal disease among individuals with sickle cell disease before and after the introduction of the pneumococcal conjugate vaccine. Clin Infect Dis. 2007 Jun 1;44(11):1428-33. Epub 2007 Apr 18.

Hankins JS, Wynn LW, Brugnara C, Hillery CA, Li CS, Wang WC. Phase I study of magnesium pidolate in combination with hydroxycarbamide for children with sickle cell anemia. Br J Haematol. 2008 Jan;140(1):80-5. Epub 2007 Nov 7.

Lee MT, Piomelli S, Granger S, et al. Stroke Prevention Trial in Sickle Cell Anemia (STOP): extended follow-up and final results. Blood. 2006 Aug 1;108(3):847-52.

Mehta SR, Afenyi-Annan A, Byrns PJ, Lottenberg R. Opportunities to improve outcomes in sickle cell disease. Am Fam Physician. 2006 Jul 15;74(2):303-10.

Singh PC, Ballas SK. Drugs for preventing red blood cell dehydration in people with sickle cell disease. Cochrane Database Syst Rev. 2007 Oct 17;(4):CD003426.

Tanabe P, Myers R, Zosel A, et al. Emergency department management of acute pain episodes in sickle cell disease. Acad Emerg Med. 2007 May;14(5):419-25. Epub 2007 Mar 26.

U.S. Preventive Services Task Force. Screening for Sickle Cell Disease in Newborns: U.S. Preventive Services Task Force Recommendation Statement. AHRQ Publication No. 07-05104-EF-2, September 2007. Agency for Healthcare Research and Quality, Rockville, MD.

Review Date:
3/11/2008
Reviewed By:
A.D.A.M. Editorial Team: David Zieve, MD, MHA, Greg Juhn, MTPW, David R. Eltz, Kelli A. Stacy, ELS. Previously reviewed by Harvey Simon, MD, Editor-in-Chief, Associate Professor of Medicine, Harvard Medical School; Physician, Massachusetts General Hospital (1/1/2008).

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Glaucoma

FitSugar — Wed, 08 Oct 2008 17:35:34 -0700

In This Report

Highlights
Introduction
Causes
Symptoms
Outlook
Risk Factors
Diagnosis
Treatment
Medications
Surgery
Lifestyle Changes
Resources
References

HEALTH GUIDE REFERENCE FROM A.D.A.M

Highlights

Drug Approval

Bimatoprost (Lumigan) has been approved as a first-line treatment for open-angle glaucoma.

Glaucoma Cases Increasing Worldwide

About 60 million people worldwide will have glaucoma by 2010, and the number will increase to nearly 80 million by 2010, according to a recent study in the British Journal of Ophthalmology.

Weightlifting May Increase Glaucoma Risk

Weightlifting can cause a temporary increase in intraocular eye pressure (IOP), and holding your breath while lifting weights further increases IOP, suggests a 2006 study in the Archives of Ophthalmology. Doctors should ask patients with normal-tension glaucoma if they engage in weightlifting exercise.

IOP and Posture

IOP increases in some people when they are lying prone during sleep, yet glaucoma exams measure IOP in patients while they are sitting upright and awake, notes a 2006 study in the Archives of Ophthalmology. The researchers caution that posture may affect the interpretation of IOP readings.

Pregnancy and Glaucoma

The course of glaucoma is unpredictable during pregnancy -- IOP may remain stable in some women and increase in others, indicates a 2006 study. Although glaucoma eye drops can increase the risk of some pregnancy problems, especially during the first trimester, some pregnant women may need to continue to take glaucoma medication. Be sure your ophthalmologist carefully evaluates your individual case and explains the risks and benefits of taking medication during pregnancy.

Diabetes and Glaucoma

Type 2 diabetes increases the risk for open-angle glaucoma (the most common type of glaucoma). People with type 2 diabetes need to get regular glaucoma screenings.

Glaucoma Surgery

Tube shunts may work better than trabulectomy surgery for some patients with glaucoma, suggests a 2007 study.
Phacoviscocanalostomy, a surgery procedure that combines phacoemulsification (used for cataract surgery) and viscocanalostomy (used for glaucoma surgery), is safe and effective for patients who have both glaucoma and cataracts, indicates a 2006 study.

Introduction

Glaucoma is defined as a disease of the optic nerve, in which the nerve cells in the front of the optic nerve (the ganglion cells) die. The process is irreversible. Previously, it was believed that glaucoma was almost always due to increased intraocular pressure. However, glaucoma has been observed in many patients with normal and even low eye pressure, so the definition now rests on the damage to the optic nerve.

The Aqueous Humor. In understanding of glaucoma, it is important to first consider aqueous humor, the clear, watery fluid that circulates continuously through the front (anterior) chamber of the healthy eye and is a primary focus of glaucoma research. (This fluid is not related to tears, nor is it the dense jelly-like substance called vitreous humor that is contained in the rear chamber.) It serves two important functions in the eye:

It nourishes the area around the colored iris and behind the cornea.
It exerts pressure to help maintain the eye’s shape.

Draining the Fluid and Intraocular Pressure. The aqueous fluid is continuously produced within the front of the eye, causing pressure known as intraocular pressure (IOP). To offset the in-flowing fluid and to maintain normal IOP, the fluid drains out between the iris and cornea (an area known as the drainage angle). It does so through two channels within this angle:

The trabecular meshwork, a sponge-like, porous network, and its connecting passageways are referred to as the "conventional" outflow pathway. Most of the eye fluid outflow occurs in this region and flows from the trabecular meshwork to a group of vessels encircling the anterior chamber, called Schlemm's canal. From here, the fluid enters collection chambers and then flows out into the general blood circulatory system of the body.
The uveoscleral pathway is located behind the trabecular meshwork and is called the "unconventional" pathway. Up to 30% of the fluid flows out through this channel.

Intraocular Eye Pressure. Previously, it was believed that glaucoma was almost always due to an abnormal rise in intraocular pressure.

Glaucoma is a condition of increased fluid pressure inside the eye. The increased pressure causes compression of the retina and the optic nerve which can eventually lead to nerve damage. Glaucoma can cause partial vision loss, with blindness as a possible eventual outcome.

Increased IOP is, indeed, present in most cases of glaucoma, but some patients have normal IOP, which is usually maintained at measurements of 10 - 20 mm Hg. Measurements above this, however, do not necessarily predict glaucoma. For example, only about 10% of people with IOP levels between 21 - 30 mm Hg will actually develop glaucoma. This still puts such individuals at considerable risk for glaucoma, however.

Most people with glaucoma have the form called primary-open-angle glaucoma (also called chronic open-angle glaucoma). Open-angle glaucoma is essentially a plumbing problem.

The disease process may occur as follows:

The drainage angle remains open, but tiny drainage channels in the trabecular meshwork pathway become clogged. This pathway is responsible for most aqueous humor fluid outflow. An imbalance then occurs as fluid continues to be produced but does not drain out efficiently. Experts have still not definitely determined the precise area in the pathway where the blockage is most likely to occur. (In rare instances the pressure is high because the eye produces too much aqueous humor.)
The fluid in the eye’s anterior chamber builds up and increases pressure within the eye. This is called intraocular pressure (IOP).
The intraocular pressure exerts force on the optic nerve at the back of the eye.
Over time, the persistent pressure or other factors irreversibly damages the delicate long fibers of the optic nerve, called axons, which convey images to the brain.
As these axons die, the small cup-like head of the optic nerve may eventually collapse into an enlarged irregular shape.

Optic nerve damage is the basic glaucoma condition. If it is untreated, eventually the nerve deteriorates until a person loses sight, first in the peripheral vision (the vision in the "corner of the eyes"). If it becomes severe, the person loses central vision (in the middle of the eyes), and may eventually become blind. (Blindness is fortunately nearly always preventable with early treatment.)

Primary open-angle glaucoma tends to start in one eye but eventually involves both. In about half of patients the damage in the eye is diffuse, that is the nerve damage is generalized. In the other half the disease is localized, causing wedge-shaped abnormalities in the nerve fiber layers of the retina.

Intraocular eye pressure is normal (between 12 - 22 mmHg) in about 25 - 30% of U.S. glaucoma cases, a condition known as normal-tension glaucoma. (In Japan, the rates may be as high as 70%.) Other factors are present that cause optic nerve damage but do not affect IOP.

Closed-angle glaucoma (also called angle-closure glaucoma) is responsible for 15% of all cases. It is less common than open-angle glaucoma in the U.S., but it constitutes about half of the world's glaucoma cases because of its higher prevalence among Asians. The iris is pushed against the lens, sometimes sticking to it, closing off the drainage angle. This can occur very suddenly, resulting in an immediate rise in pressure. It often occurs in genetically susceptible people when the pupil shrinks suddenly. Closed-angle glaucoma can also be chronic and gradual, a less common condition.

Congenital glaucoma, in which the eye's drainage canals fail to develop correctly, is present from birth. It is very rare, occurring in about 1 in 10,000 newborns. This may be an inherited condition and often can be corrected with microsurgery.

The Light-Processing Parts. To understand sight, one begins with light and its passage through the eye's sensitive camera-like structures:

Light first passes through the cornea, a clear tissue at the front of the eye.
Behind the cornea, the iris (the colored tissues of the eye) opens and closes like a camera shutter to regulate the passage of light.
The lens, located behind the iris, focuses the light, which then hits the retina.
The retina is an electric fragile membrane of nerve cells called photoreceptors that receive light and translate it into signals.
A layer of cells, called the retinal ganglia, receive signals from the retina. These nerve cells are the front ends of the optic nerve cable, which, in turn, receive the signals.
The optic nerve is actually a cable of about 1.2 million nerve fibers called axons. It carries the signals to the brain, which interprets them as images.
They exit the eye through the optic disc, located in the back of the eye.

The Supportive Chambers. To help support and protect these sensitive structures, the eye contains two fluid-filled chambers:

The posterior (rear) chamber is the large area behind the iris.
Fluid passes from the posterior into the anterior (forward) chamber located in the bulging area between the iris and the front of the eye.

Causes

No single factor has been identified as a cause of primary open-angle glaucoma. A number of conditions, alone or in combination, are needed to trigger the processes leading to pressure in the first place and then to the nerve damage that destroys sight. The damage done to the optic nerve in glaucoma is triggered in most cases by the excessive pressure on the optic nerve that, over time, causes damage. Because optic nerve damage occurs in patients with normal as well as high intraocular pressure, however, researchers are investigating several other abnormal events that occur and can damage the optic nerve.

A number of genes have now been identified as possible factors in many cases of glaucoma. A gene called MYOC is of particular interest. Defects in this gene occur in between 3 - 6% of patients with adult-onset and juvenile open-angle glaucoma. They appear to overproduce a sticky protein called myocilin, which clogs the trabecular meshwork. The genes WDR36 and OPTN may cause primary open-angle glaucoma. Researchers hope that identification of genes will help improve screening of high-risk patients.

Specific syndromes have been identified with glaucoma. Many have an inherited component, although in most cases other factors must be present to activate the disease process.

Pseudoexfoliation Syndrome. Pseudoexfoliation (PEX) syndrome (also known as exfoliation syndrome) is the most common identifiable condition associated with glaucoma. In one study, 9% of patients with open-angle glaucoma had the syndrome. PEX occurs when dandruff-like matter flakes off the outer layer of the lens and collects in the drainage angle. The substance is composed of proteins produced by the lens, iris, and other parts of the eye. People can have this condition and not develop glaucoma, but they are at high risk. In one Australian study, 14% of the people with this condition had glaucoma compared to 2% of those without exfoliation. PEX has a strong genetic component but other factors (possibly sunlight, an autoimmune response, or slow virus) may be needed to trigger the disease.

Pigment Glaucoma. Pigment glaucoma starts with a condition called pigment dispersion syndrome, an inherited condition in which granules of pigment (the substance that colors the iris) flakes off into the intraocular fluid. In about 30% of cases, these fragments clog the trabecular meshwork and pressure builds up, causing glaucoma. In one study, 2% of patients had this form of glaucoma.

Irido Corneal Endothelia Syndrome. In irido corneal endothelial syndrome (ICE), cells on the back surface of the cornea spread to the drainage angle, sometimes forming scars that connect the iris to the cornea.

Neovascular Glaucoma. Neovascular glaucoma is always associated with other disorders, usually diabetes, that result in abnormal formation of new blood vessels on the iris and in the drainage system.

Aniridia. Aniridia is a rare inherited disorder (in which the iris is abnormal and increases the risk for glaucoma) that is difficult to treat. (A surgical approach called goniosurgery may help prevent glaucoma in young people with aniridia.)

Congenital Glaucoma. When an infant is born with glaucoma (congenital glaucoma), it is usually caused by an inherited abnormality in the drainage canal. Researchers have identified the gene responsible for 85% of these cases.

A natural process called apoptosis (cellular self-destruction) may contribute to damage in the retinal ganglion nerve cells, the nerve cells that are the front line of the optic nerve. Cell death can occur with or without elevated eye pressure. It is not clear what triggers apoptosis and cell death in such cases, but there are a number of suspects.

Excess Glutamate. Researchers have observed abnormally high levels of glutamate in people and animals with glaucoma. Glutamate is an amino acid that excites nerve cells. In the eye this occurs during vision. Some experts theorize that in glaucoma, either reduced blood flow or increased pressure on nerve cells triggers the release of excess glutamate. In large amounts, glutamate causes the nerve cells to fire intensively, which eventually destroys them.

Reduced Blood Flow. Researchers have observed reduced blood flow to the optic nerve in patients with glaucoma associated with both high and normal IOP. Less blood flow suggests oxygen loss, which may play a role in the destructive process. Some studies suggest that the greatest risk factor for nerve damage in patients is when blood pressure to the eye drops during the night. Ocular pressure at this time is highest, so the risk for nerve damage becomes intensified. Of interest in this regard are reports finding a significant reduction in eye blood pressure at night in patients with normal-tension glaucoma.

Excess Nitric Oxide. Elevated levels of nitric oxide, another nerve-stimulating compound, also plays a role in the nerve-damaging process. Nitric oxide is critical for nerve function and flexible blood vessels, but excess amounts may be toxic to nerves.

Glaucoma and Alzheimer's Disease. Some research has pointed out similarities in the process leading to cell death in glaucoma and Alzheimer's disease. Specifically, in both diseases activation of certain enzymes called caspases occurs and leads to accumulation of fragments of beta amyloid, an insoluble protein that forms sticky patches.

Autoimmunity. Some experts are studying the possibility that normal tension glaucoma may be an autoimmune disease; that is, factors in the immune system, including antibodies, attack cells in the person's own body as if they were foreign substances. In the case of glaucoma, such antibodies would damage parts of the optic nerve.

H. pylori Infection. Some research indicates that glaucoma is associated with Helicobacter (H.) pylori, the bacterium implicated as a major cause of peptic ulcers. Studies have reported over 87% of patients with glaucoma are infected with this bacterium.

People with acute closed-angle glaucoma often have a structural defect that causes a narrow angle between the iris and cornea where the aqueous humor circulates. Conditions that suddenly dilate the pupils may cause this shallow angle to close and precipitate attacks of acute glaucoma in susceptible people. Such conditions may include:

Certain drugs such as antihistamines, tricyclic antidepressants, some asthma medications (nebulized ipratropium), some anti-seizure drugs (topiramate)
Darkness
Emotional stress

When intraocular pressure leading to glaucoma is caused by other diseases or conditions, it is known as secondary glaucoma. Secondary glaucoma may be chronic or acute, mild or severe.

Medical Conditions. A number of diseases can contribute to the development of intraocular pressure leading to glaucoma:

Diseases that affect blood flow to the optic nerve (diabetes, high blood pressure, migraine; people with type 2 diabetes should be regularly screened for glaucoma.)
Hypothyroidism
Sleep apnea
Physical injury in the eye
Extreme nearsightedness (myopia)
Previous eye surgery
Other disorders, including leukemia, sickle cell anemia, and some forms of arthritis

Corticosteroids. Corticosteroids, commonly called steroids, have multiple effects on the trabecular meshwork and may even cause genetic changes. In fact, studying the effects of steroids on the eye is helping researchers understand the glaucoma disease process. Steroids pose a higher or lower risk depending on the form:

Taking topical steroid treatments in the eye poses the highest risk. It must be monitored carefully since, in some cases, damage may be permanent.
Taking oral corticosteroids, particularly in high doses or for long periods, increases the chance of glaucoma. In such cases, the eye disorder typically develops almost immediately and reverses within 2 weeks after the drug has been withdrawn.
Inhaled steroids were not thought to cause glaucoma, but there is some risk in people with a family history of glaucoma and other risk factors.

Symptoms

Chronic glaucoma is insidious. If the pressure increases slowly, it will not produce any symptoms until it has done irreversible damage. In such cases, people may notice visual problems at first only when light is dim. Patients are often sensitive to glare. Eventually they may lose contrast sensitivity; that is, they might have trouble differentiating between varying shades and brightness.

In acute closed-angle glaucoma, the pressure inside the eye increases quickly, and the symptoms are dramatic. Intense pain in the eyebrow area and blurred vision develop usually in one eye, and the patient often feels like the eye will burst (although it won't). The eye usually reddens. A person may see rainbow-like halos around lights. Sometimes nausea and vomiting occur. These symptoms may occur on and off and not appear as a full attack. In either case, they indicate a medical emergency. In chronic closed-angle glaucoma, the process is gradual and painless.

Although congenital glaucoma is usually present at birth, symptoms generally don’t develop in the infant for a few months. If parents notice that an infant’s eyes are enlarging, becoming cloudy, often watering, or tending to close in the presence of light, they should have an ophthalmologist examine the child’s eyes. Port-wine stains on an infant’s face could indicate the Sturge-Weber syndrome, a disorder that occasionally causes glaucoma.

Outlook

Worldwide, glaucoma ranks as one of the leading causes of blindness. Even if people with glaucoma do not become blind, vision can be impaired. In developed countries, most people get treatment in time to preserve their vision. Even so, glaucoma causes between 3 - 6% of blindness cases in Caucasians, and even more cases in African Americans.

In a 20-year study of Caucasian patients with glaucoma, blindness in at least one eye occurred in 27% of patients and blindness in both eyes occurred in 9% of patients. The blindness rates in African Americans are most likely higher. In fact, glaucoma is the leading cause of blindness in African Americans. Despite this higher prevalence, this ethnic group receives surgical treatment at half the rate of Caucasians.

The Process Leading to Vision Loss. Chronic glaucoma is often called “the silent thief of sight," because the afflicted person has no warning sign, no hint that anything is wrong. Untreated, the destruction develops slowly over time:

Over years or decades, the increased pressure compresses nerves at the back of the eyes.
Glaucoma gradually destroys first the outer fibers of the optic nerve, which reduces peripheral vision (the top, sides, and bottom areas of vision), but not central vision.
By the time a person notices that peripheral vision has been lost, permanent damage has already occurred.
If the eye pressure remains high, the destruction can progress until tunnel vision develops, and the person is only able to see objects that are straight ahead.
The last nerve fibers destroyed are those responsible for central vision; if this occurs, the glaucoma victim becomes totally blind.

Although there is no cure for open-angle glaucoma, a number of treatments are available that lower intraocular pressure and slow progression of vision loss.

Risk Factors for Vision Loss. Estimates of progression rates in vision deterioration range from 9 - 30% over a 2 - 7 year period.

According to a study on patients with elevated IOP, for every 1-mm Hg increase in IOP, there is a 10% higher risk of disease progression. A very elevated IOP (above 30 mm Hg) is certainly hazardous. An elevated IOP that is below 30 mm Hg, however, is not necessarily the most important factor in determining the risk for disease progression. Some evidence suggests that frequent and large daily fluctuations in intraocular pressure, not simply high IOP, are associated with the greatest risk for loss of vision. Having normal-tension glaucoma with optic nerve damage also carries a high risk for progression, even if eye pressure is reduced.

In any case, factors other than IOP play a role in increasing the chances for progression and vision loss in patients with slightly elevated IOP and normal tension glaucoma:

Both eyes affected
Pseudoexfoliation (PEX) syndrome. PEX occurs when proteins produced in the eye flake off the outer layer of the lens and collects in the drainage angle.
Bleeding in a specific region called the peripapillary nerve fiber layer
Thin corneas. (People who have thick corneas and elevated IOP may only need to be monitored if they have no other risk factor for vision loss.)
Larger cup-to-optic disc ratio. (The cup of the optic disc is the center portion, which enlarges as nerve damage progresses.)

Non-eye related factors associated with disease progression include being elderly, African American, female, or having a history of migraines.

Acute closed-angle glaucoma is a medical emergency; if the high pressure is not reduced within hours, it may permanently damage vision. Anyone who experiences its symptoms should immediately contact an ophthalmologist or go to a hospital emergency room.

Risk Factors

About 2 million Americans have open-angle glaucoma, but an exact count is unclear. Experts estimate that by 2010, over 60 million people worldwide will have glaucoma, with 74% of these cases due to open-angle glaucoma. Half of people with glaucoma are unaware of this problem because the condition causes no symptoms.

Elevated intraocular pressure in the eye occurs in 5 - 10 million Americans, but only about 10% of such people develop glaucoma because of this pressure. And, in 15% of actual glaucoma cases, IOP is normal. Major studies are helping to clarify the people who are at highest risk for glaucoma and optic nerve damage, including those with normal tension glaucoma.

Elderly. The prevalence of chronic glaucoma increases with age. In a major study, 0.6% of people age 60 - 64 had primary open-angle glaucoma. Among people who were 10 years older, the prevalence had more than doubled to 1.3%, and among those who were age 80 - 84, it had more than doubled again to 3%.

People of African Descent. Across all age groups, according to a 2000 report, the prevalence of glaucoma in African Americans is about 3.5% compared to about 1% in Caucasians. In addition, U.S. studies suggest that glaucoma develops earlier in African American population groups (starting at age 45 instead of age 60 in Caucasians). And, their risk for blindness once they have glaucoma is 14 - 17 times that of Caucasians with glaucoma. African American men are at higher risk than women. African American children who are extremely near-sighted and have relatives with glaucoma should begin regular eye examinations for glaucoma as early as possible.

In a major glaucoma study in Barbados, where most people are of African descent, over 10% of those age 50 and older had open angle glaucoma, and over 15% were afflicted after age 70. About half of the cases had normal or lower eye pressure. An interesting 2001 study suggested that African Americans tend to have significantly thinner central corneas than Caucasians. This could lead to misleadingly lower pressure scores in African American patients who actually may have high IOPs.

Family History. Glaucoma tends to run in families. Brothers and sisters of patients with open angle glaucoma are 5 times more likely to develop glaucoma by the time they are 70 years old than people whose siblings do not have the disease. Previous studies have also found that people with family histories of glaucoma are more likely to already have some vision loss when they are first diagnosed with glaucoma.

Effects of Blood Pressure. The association between a person's blood pressure and intraocular pressure in the eye is not entirely clear. A number of studies have found a higher risk for glaucoma in people with high blood pressure. A 2002 study suggested, however, that people with blood pressure that is low relative to their intraocular pressure may be at higher risk for glaucoma. The same study found no higher risk for glaucoma in people with hypertension, and in fact, high blood pressure was associated with a lower risk.

Having Certain Medical Disorders. Individuals with certain medical or physical conditions, including diabetes, migraine, nearsightedness, and sleep apnea, appear to have a higher risk. Conditions that require the use of any oral or inhaled steroid, particularly high doses for prolonged periods of time, can cause glaucoma. Previous eye surgery also puts people at risk.

Weightlifting. According to a 2006 study, holding your breath while weightlifting can increase the risk for developing normal-tension glaucoma. Weightlifting causes temporary increases in eye pressure; holding your breath during this exercise leads to even greater intraocular pressure.

Risk Factors for Closed-Angle Glaucoma. Chronic closed-angle glaucoma tends to be more common in people of Asian and African descent. Those who have this condition are often extremely farsighted. Acute closed-angle glaucoma occurs much more frequently in women than in men.

Risk Factors for Normal Tension Glaucoma. Risk factors for normal tension glaucoma include Japanese ancestry and a family history of the disease. It is more common in women than in men. A family history of cardiovascular disease also increases the risk.

Risk Factors for Pigmentary Glaucoma. Pigmentary glaucoma occurs three times more often in men then in women and at a younger age.

Risk Factors for Irido Corneal Endothelial Syndrome. This condition occurs more often in light-skinned women.

Diagnosis

A diagnosis of glaucoma no longer simply relies on the presence of pressure within the eye. It requires that there be optic nerve damage or a strong suggestion of damage, which can be clearly seen during a dilated eye examination of the optic nerve. In general, the hallmark sign of this condition is a loss of peripheral vision. With peripheral vision loss, a person can see in front of him- or herself but has lost the vision to the side.

The optic nerve carries the information of vision from the eye to the brain.

Because chronic glaucoma has no warning symptoms, half of its victims are unaware that they have the condition. Early diagnosis, however, is the key to successful treatment of glaucoma. One study reported that the more years since the last visit to an eye professional, the greater the risk for having visual loss.

Everyone over age 65 and African Americans over 40 years old should have periodic eye exams, including tests for glaucoma, every other year.
African Americans between ages 20 - 39 should have eye examinations every 3 - 5 years.
Other people at higher risk (people with diabetes, history of eye injuries, a family history of glaucoma, or those taking corticosteroid medications) should have eye examinations every year after age 35.
People with known glaucoma should have frequent examinations to check peripheral vision and to be sure treatment is maintaining a safe eye pressure. After such examinations, the ophthalmologist will assess current treatment and make necessary adjustments.

Doctors determine the intraocular pressure (IOP) of the aqueous humor inside the eye using tonometry, which measures the force necessary to make an indentation in the eye. There are several methods:

In the Schiotz method, the doctor first anesthetizes the eye with drops, then presses very lightly against it with tonometer, a tiny smooth instrument that is used to measure the pressure.
In the applanation method, the doctor touches a strip of orange-dyed paper to the side of the eye. The stain helps with the examination and rinses out with tearing. The doctor uses a slit-lamp, which is moved forward toward the patient's face until the tonometer touches the eye.
The noncontact approach applies a puff of air and measures the force needed to indent the eye.

Attempting to close the eyelids during the test can increase eye pressure and produce errors in the results.

In general, normal IOP is usually maintained at measurements of 10 - 20 mm Hg. Glaucoma pressure over 21 mm Hg indicates a potential problem. The test is not completely accurate, however. Only about 10% of people with IOP levels between 21 - 30 mm Hg will actually develop glaucoma and optic nerve damage. On the other hand, many people with glaucoma have normal pressure, at least for part of the time.

Changes in posture may also affect IOP. A 2006 study indicated that IOP increases during sleep or when a person is lying down. As IOP tests are generally given in a doctor’s office when a patient is sitting up, they may not provide a completely accurate evaluation of eye pressure.

The cornea thickness may be an important indicator of disease progression in patients with elevated IOP. According to some research, patients with thinner corneas have a significant risk for developing damage from glaucoma, while those with thicker corneas have a low risk.

In order to determine early damage in the optic nerve, a number of diagnostic instruments have been developed to assess the nerve fiber layers at the back of the eye (the fundus) and to check for optic disk cupping. (The cup of the optic disc is the center portion, which enlarges as nerve damage progresses.) The two most common procedures for identifying nerve damage are ophthalmoscopy and fundus photography. Other instruments have been developed, including those that use laser technology and computers, but none have proved to be infallible. No test has proven to be completely accurate, however, and none is routinely performed by all eye professionals.

In order to be accurate, the tests require a skilled professional and there are certain common factors:

The pupils must also be widely dilated using eye drops before the procedure.
Even mild cataracts and a slightly less-than-optimally dilated pupil can degrade the results. Such conditions are common in elderly people, who are the most likely to develop glaucoma.
If the back of the eye is lightly pigmented (colored), the area under observation is less distinct.
If the glaucoma is diffuse and there is a generalized loss of nerve fiber (which occurs in half of patients), it is more difficult to detect than if the glaucoma is more localized.

If IOP is low or normal and tests report optic nerve damage and peripheral visual loss, doctors should also check for other conditions before starting any treatment for glaucoma. Such problems include steroid use, anemia, and previous hemorrhage or severe low blood pressure.

Ophthalmoscopy. The eye professional (or even a primary care doctor) uses an ophthalmoscope to peer through the pupil directly at the optic nerve. The examiner can then check the shape and color of the nerve fibers to evaluate whether they have been damaged by the high pressure of glaucoma. Damaged nerve fibers may be indicated by:

An asymmetrical or elongated cupped optic nerve
The optic nerve color may be pale or an unhealthy-pink

If results show no optic nerve damage in patients who have mild elevations in pressure, the ophthalmologist may want to retest frequently but delay drug treatment, unless the patient has significant risk factors.

Fundus Photography. Fundus photography may be used to take pictures of the optic nerve and can reveal changes years in advance of vision loss. It is an unpleasant procedure requiring drops and a bright flash. This procedure has the same limitations as ophthalmoscopy.

Laser Polarimetry. Polarimetry uses laser technology to scan the eye and does not require any response from the patient. It is reported to be able to measure nerve fiber thickness in the eye and so be able to reveal early signs of deterioration. Preliminary studies have indicated that it has a diagnostic accuracy of over 90% for both confirming and ruling out glaucoma. One study, however, reported that laser polarimetry was sensitive enough to detect glaucoma in only up to 57% of patients with early glaucoma, 71% of those with moderate disease, and 81% of those with severe glaucoma. More research is needed.

Other Devices. Computer-assisted devices, such as the confocal scanning laser ophthalmoscope, are now available that may be useful for evaluating the retinal nerve layer. Another instrument, the optical coherence tomograph, measures the echo time delay of light that is scattered back from different layers in the retina. The value of these tests has not yet been determined.

If there is indication of optic nerve damage, the eye professional will conduct tests of the visual fields (the areas that the patient can see). In most people with glaucoma, the first areas to become noticeably impaired are the peripheral visual fields (areas of sight that are not directly in front of a person but more to the sides).

Click the icon to see an image of the visual field test.

Standard Perimetry Tests. Perimetry tests are used to check peripheral vision. One variation of this test is as follows:

A person sits closely facing a large computer-like monitor.
Small bright white lights flicker on and off hundreds of times, at different places on the screen, while the patient clicks a button whenever one of the lights is seen.
The machine prints out a report that maps any blanked-out areas in the person’s vision.

The test is complex and lengthy; elderly people and those with short attention spans may be inappropriate candidates. Other perimetry tests, some requiring less time to administer and some using "virtual reality" techniques, are currently being developed.

Other Tests. Other visual field tests are being developed that can detect abnormalities years before they can be detected by standard perimetry. Experts recommend some of these tests in selected patients with suspected glaucoma.

For example, a screening test called frequency doubling technology (FDT) checks for changes in particular cells in the retina that are indications of early glaucoma. It takes less than a minute to perform.

Another test called short wave automated perimetry (SWAP) uses colors (blue-on-yellow) and also detects very early abnormalities in the visual field. Testing time is longer than with FDT, however, and the presence of certain types of cataracts can interfere with its accuracy.

ELAM-1. Endothelial leukocyte cell adhesion molecule 1 (ELAM-1) is a molecule that has been found in glaucoma but not in healthy eyes. This molecule may prove to be a "marker" and its presence may be helpful in diagnosing glaucoma.

A simple test using a penlight helps determine the risk for acute closed-angle glaucoma. A beam of light is directed from the side of the face toward the patient's iris. If no shadow appears on the nose, then most likely the angle is wide enough to dilate. Using an instrument called a gonioscope, ophthalmologists can also inspect the front of the eyes and assess the drainage angle between the cornea and the iris and the channels in the trabecular meshwork. This test can differentiate between closed- and open-angle glaucoma.

Treatment

Most treatments for glaucoma aim to reduce ocular pressure and its fluctuations. Early treatment with medications, surgery, or both can nearly always maintain safe pressure of the aqueous humor, thus preventing optic nerve damage and blindness. The choice between surgery and medications and when to start treatment is not always straightforward. For example, with the introduction of beta blockers and newer glaucoma drugs, there has been a decline in surgeries. It is not clear, however, which drugs are more effective than others and if, over time, any will actually prevent surgery. Patients should discuss all issues with their doctors and ophthalmologists.

Many people have high IOP but no sign of nerve damage. Over the course of 20 years, only between 10 - 30% of these people will actually develop glaucoma. Nevertheless, once glaucoma has destroyed optic nerve fibers, no known treatment can reverse the damage.

Indeed, studies suggest that in people with glaucoma, even very small differences in pressure may mean the difference between disease progression and stability. An important trial reported that, on average, treating patients when their glaucoma was first detected reduced IOP by 25%. In addition, treatment reduced the risk for progression by 17%. This study confirmed previous findings supporting early treatment for glaucoma. Another study found that treatment with eye drops halved the risk of developing open-angle glaucoma in African Americans who had elevated IOP. Some evidence suggests that early treatment to lower IOP may be beneficial even in patients with normal tension glaucoma.

However, not all individuals with early signs of glaucoma (elevated IOP or normal-tension glaucoma) develop optic nerve damage and serious vision problems. Nor does treatment prevent progression in a large minority of patients. Medications used for glaucoma also can carry significant side effects and risks.

Some experts suggest that treatment is warranted only in people with early signs of glaucoma who have risk factors for progressive disease and vision loss (thinner corneas, larger cup to optic disc ration, older age, and elevated pressure).

A number of effective drugs are now available for treating glaucoma. The drugs reduce pressure in the eye but all have a number of side effects that affect other parts of the body. Some of these side effects can be quite severe. Many of the drugs used for glaucoma also interact with common medications for other conditions. To compound the difficulties, many patients require multiple drugs. As a result, only about half of patients comply with their treatments.

Experts generally recommend topical drugs first (those that can be used as eye drops or ointments rather than taken by mouth).

Topical beta blockers are the standard first-line drugs, most commonly timolol (Timoptic). Newer beta blockers include betaxolol (Betoptic), levobunolol (Betagan), carteolol (Ocupress), and metipranolol (OptiPranolol). Timolol has been used for years, and these other drugs are also well tolerated.
Topical prostaglandins are alternatives if beta blockers fail. They include latanoprost (Xalatan) and unoprostone (Rescula). Of the standard drugs used for glaucoma, these drugs have the greatest effect on lowering IOPs. They also have fewer widespread effects than the beta blockers.
Topical carbonic anhydrase inhibitors (CAIs) are less effective than standard beta blockers or prostaglandins but have fewer widespread effects than the beta blockers. They may be helpful in certain cases. Topical forms are dorzolamide (Trusopt) and brinzolamide (Azopt). (Oral CAIs are available and more effective, but they have severe side effects and are rarely used for the long term.)
Alpha2-adrenergics, also called selective alpha adrenergics, are effective but may not be as well tolerated as timolol. They include brimonidine (Alphagan).
Miotics, which include pilocarpine and others, were the standard drugs before the introduction of topical beta blockers. They have now been largely replaced by timolol and others, although they are sometimes used in combinations.
Beta blockers and newer drugs (prostaglandins, topical CAIs, and selective alpha adrenergics) are now preferred over the older drugs, which include miotics, oral CAIs, and nonselective alpha adrenergics.

Combinations. Combinations of these drugs can be very effective, because they tend to have different actions. Single medications that contain two drugs are becoming available. For example, Cosopt combines timolol and dorzolamide; Timpilo is a combination of timolol and pilocarpine. Studies of these and other combinations compared to each other to single drugs are ongoing. To date, results on any superior combinations have been mixed. It should be noted that the side effects of each drug apply to any combination.

Treating the Pregnant Patient. Considerations for a pregnant woman with glaucoma can be complicated. All of the drugs used for glaucoma are absorbed by the body, cross the placenta, and are excreted in breast milk. Many have effects that can interfere with or adversely affect pregnancy.

Women should discuss going off medication, particularly during the first trimester, and be monitored during that time for increasing eye pressure. IOP tends to drop during pregnancy, although usually not to a significant degree. In addition, changes in IOP and visual loss vary greatly. Some women experience no IOP change or visual loss during pregnancy, while others may experience an increase in IOP or worsening of visual loss. It is important that your ophthalmologist carefully considers your individual case and discusses with you the risks and benefits of continuing glaucoma medication during pregnancy.

If women need to take medications, they should try to achieve the lowest dose possible. Some drugs have fewer side effects than others. Pregnant women must also be very careful about administering eye drops to allow as little medication as possible to enter the body. When taking eye drops, press your index finger against the corner of the eye near your nose. This helps prevent the eye drop from passing down into the tear duct where it is easily absorbed through the rest of the body. Even this approach, however, does not guarantee complete safety. Women with glaucoma who are planning to become pregnant might want to consider surgery before they conceive.

The object of standard glaucoma surgery is to reduce pressure in the eye by increasing the outflow of the aqueous fluid. Two methods are commonly used:

Filtration surgery (trabeculectomy). This uses standard surgical instruments to open a passage in the eye for draining fluid.
Laser trabeculoplasty. This procedure uses a laser to burn 80 - 100 tiny holes in the drainage area.

Both are effective, but certain patient groups may respond to one more than the other. For example, African Americans may do better with laser surgery while trabeculectomy may be a better choice for Caucasians with no serious medical problems.

In general, surgery is a last resort. Doctors may, however, recommend surgery before drug therapies for patients unlikely to comply with difficult drug regimens or for patients who may have severe reactions from the glaucoma drugs. Women who plan on becoming pregnant should also discuss surgery with their doctor.

Some studies indicate that laser treatment performed as the initial treatment for glaucoma is as effective as medications in some cases. Findings in 2003 from a major comparison study suggested that 4 years after surgery there was little difference in visual field loss between trabeculectomy and medical treatment. There was, however, a higher risk for cataracts and loss of vision sharpness with surgery. On the other hand, side effects from medications may be ongoing and troublesome. It is important to note that even surgery does not cure glaucoma, and over half of patients will require medication within 2 years. Experts who are against early surgeries also argue that studies on their success often omitted serious postoperative problems, such as late-onset infection, and quality of life assessments.

Medications

Nearly all glaucoma medications are prescribed for reducing eye pressure. Lowering IOP is even proving to be beneficial for about two-thirds of patients with normal-pressure glaucoma.

Topical beta adrenoceptor blockers (common called beta-blockers) are the drugs most often prescribed to treat glaucoma. They lower the pressure inside the eye by inhibiting the production of aqueous humor.

Brands. These drugs are categorized as either nonselective or selective beta-blockers:

Nonselective adrenoceptor beta-blockers. Timolol (Timoptic, Betimol) has been the standard beta-blocker for years. Newer nonselective drugs include levobunolol (Betagan), carteolol (Ocupress), and metipranolol (OptiPranolol). A few studies suggest some are more beneficial than timolol with similar side effects.
Selective beta1-adrenoceptor blockers. Betaxolol (Betoptic) and levobetaxolol (Betaxon) are selective beta-blockers. These drugs appear to have fewer adverse effects on the heart than the nonselective beta-blockers, although they still have widespread effects. Studies also suggest that they slow progression more than timolol, although timolol is more effective at lowering IOP. selective beta-blockers may also have nerve-protecting properties.

All beta-blockers are effective and generally well tolerated. Because they cause less eye irritation than many other glaucoma medications, they are often prescribed for patients who also have cataracts.

Side Effects and Complications. After the beta-blocker is administered, only a tiny amount of the drug is absorbed by the cornea. Most of it enters in the bloodstream. These drugs, therefore, can cause side effects in parts of the body other than the eyes ("systemic" side effects):

Common systemic side effects include reduced sexual drive, fatigue, depression, anxiety, severe nausea and vomiting, and breathing difficulties.
Beta-blockers affect the heart. They lower heart rate and reduce blood pressure. (The newer selective beta-1 blockers may not have as bad effects on the heart as the nonselective beta-blockers.) They may also cause unhealthy cholesterol and triglyceride changes.
All beta-blockers can worsen severe asthma or other lung diseases. Beta-blockers should only be used very cautiously or not at all by anyone with asthma, emphysema, bronchitis, or heart disease. In one study, lung function was reduced in 40% of elderly people who took timolol, even those without previous symptoms of lung problems. (Selective beta-blockers may produce fewer of these adverse effects.)
If the patient is switching to a beta-blocker from other glaucoma medication, there may be a sudden rise in eye pressure. It is important that the pressure be checked shortly after the other drug has been withdrawn.
When beta-blockers are used to treat one eye, the other (contralateral) eye also experiences a lesser, but still significant reduction in IOP.

Interactions with Other Medications. The effects of the eye medication may be additive to other oral medications, such as oral beta-blockers, calcium-channel blockers, or the antiarrhythmic drug quinidine. People with diabetes who take insulin or hypoglycemic medications should realize that timolol side effects may resemble and mask the symptoms of hypoglycemia (low blood sugar).

Prostaglandins are hormone-like substances that help open blood vessels. Drugs that resemble prostaglandins increase outflow of aqueous humor (the watery substance in the eye). Drainage of aqueous humor helps reduce intraocular pressure.

Brands. Latanoprost (Xalatan) and unoprostone (Rescula) are the standard brands. Latanoprost was the first prostaglandin to be approved as first-line treatment for elevated eye pressure. Two newer prostaglandins, travoprost (Travatan) and bimatoprost (Lumigan), may help some patients who do not respond to latanoprost. These drugs may also benefit patients with normal-tension glaucoma. Latanoprost, travoprost, and bimatoprost need to be taken only once daily. Unoprostone needs to be taken twice a day and is not as effective as others, but it still can reduce IOP significantly and is the least expensive of these drugs.

Latanoprost has been shown to reduce pressure by between 45 - 70%. Some, but not all studies, have suggested that newer prostaglandins travoprost (Travatan) and bimatoprost (Lumigan) are more effective than latanoprost, but the older drug appears to be better tolerated. All of these drugs may be work better than timolol in lowering IOP. The newer prostaglandins may be especially superior to timolol in treating African American patients. In comparison studies, latanoprost achieved better IOP pressure reduction than brimonidine. Studies have suggested that bimatoprost is more effective in lowering eye pressure than a combination of timolol and dorzolamide (Cosopt). Studies have been mixed on whether latanoprost is superior to the combination.

Side Effects. These drugs do not slow down the heart rate and also appear to be safe for people with asthma. Side effects include itching, redness, and burning during administration. Muscle and joint pain may also occur. All of these drugs may permanently change eye color from blue or green to brown. To date, such color changes do not seem to be hazardous. (The only significant problem may be cosmetic in people who treat only one eye, since the color may differ from the other.) These drugs can increase blood flow in the eye and also make eyelashes become thicker and longer in some patients. (These latter effects are more common with bimatoprost and travoprost than with latanoprost.)

Carbonic anhydrase inhibitors (CAIs) decrease eye pressure by reducing the fluid in the chambers of the eye (aqueous humor). Research suggests that CAIs reduce aqueous humor fluid by as much as 40%. These drugs are used for glaucoma when other drugs do not work. They may be combined with other medications.

CAIs may also improve blood flow in the retina and optic nerve (beta-blockers do not). Improving blood flow can keep the disease from getting worse.

Brands and Side Effects. CAIs are available in the following forms:

Eye-drop CAIs include dorzolamide (Trusopt) and brinzolamide (Azopt). About 10% of patients report fatigue, stinging in the eye, and loss of appetite using dorzolamide. Taste changes can occur. Research suggests that dorzolamide can be helpful for children with glaucoma, including those younger than 6 years old. Brinzolamide is a newer medication that was chemically designed to be closer in pH to human tears and may cause less stinging than dorzolamide.
Oral forms include acetazolamide (Diamox), methazolamide (Neptazane), and dichlorphenamide (Daranide). Although they are more effective than eye drops, they have significantly more side effects and are rarely used for long-term treatment. The oral forms have very unpleasant side effects that include frequent urination, depression, stomach problems, fatigue, weight loss, sexual dysfunction, and, in infants, failure to thrive. Long-term use of the oral forms, in rare cases, can cause serious anemia and kidney problems, including the risk for stones. They can also produce a toxic reaction when taken with large doses of aspirin.

Adrenergic agonists activate muscles in the eye that dilate pupils and, therefore, increase outflow of aqueous fluid. Newer variations called alpha 2-adrenergic agonists reduce production of aqueous humor and also increase outflow through the uveoscleral pathway (the alternative channel to the trabecular meshwork). Older adrenergic agonists include epinephrine.

Alpha 2-Adrenergic Agonists. Apraclonidine (Iopidine) and brimonidine (Alphagan) are alpha 2-adrenergic agonists. These have generally been used before glaucoma surgery, but a number of studies are indicating that they may even be useful as primary therapy when used in combination with beta-blockers or other standard drugs.

Brimonidine is proving to be particularly effective for long-term therapy. (Apraclonidine is used for the short term.) It also may have nerve-protecting properties and may be safer than other drugs during pregnancy and for patients with asthma.

The most common side effects of brimonidine and apraclonidine are dry mouth and altered taste. They also commonly trigger an allergic reaction that causes red and itching eyes and lids, a major drawback. Brimonidine causes less of an allergic response than apraclonidine. Unlike apraclonidine, however, it can cause lethargy and mild low blood pressure. It also appears to remain effective longer.

Miotics, also called cholinergic agonists, narrow the iris muscles and constrict the pupil. This action pulls the iris away from the trabecular meshwork and allows the aqueous humor to flow out through the drainage channels, reducing the pressure inside the front of the eye.

Brands. Pilocarpine (Pilocar, Adsorbocarpine, Almocarpine, Isoptocarpine, Ocusert) was the most widely used anti-glaucoma drug before timolol was introduced. It is the preferred miotic. Because pilocarpine is used up by the body fairly quickly, however, patients must take it several times a day; many people, therefore, fail to take their medication regularly. A combination of timolol or latanoprost with pilocarpine is more effective than either drug used alone. Carbachol is another miotic.

Demecarium (Humorsol), isoflurophate (Floropryl), and echothiophate (Phospholine) are a group of long-acting drugs known as anticholinesterase miotics. Because of their potential for serious side effects, however, some authorities even prefer surgery to their use.

Epinephrine and its derivatives are the older anticholinergics. Epinephrine is now rarely prescribed because of side effects. Dipivefrin (Dipivefrin), a newer form of epinephrine, remains inactive until it reacts with enzymes in the cornea. It is effective in low doses and causes few systemic side effects.

Side Effects. Side effects include:

Teary eyes, brow-aches, eye pain, and allergic reactions.
A miotic narrows the pupil and so can cause nearsightedness. Vision can also become dim and it may difficult to see in darkened rooms or at night, when driving could be hazardous. A gel form administered once a day or wafer placed under the lid once a week may help reduce these side effects.
The anticholinesterase miotics increase the risk of cataract development and are therefore used mostly in patients in whom cataracts have already been removed. Retinal detachment is an uncommon but dangerous side effect in susceptible individuals. Excessive use of these miotics may cause toxic reactions, including convulsions, muscular paralysis, and even death from respiratory failure.
Epinephrine can produce burning in the eyes, enlarged pupils, and allergic reactions. Occasionally it can cause anxiety and headaches. Rare side effects include high blood pressure and disturbances in heart rhythm. It is rarely prescribed now. Although dipivefrin, the newer form of epinephrine, has fewer systemic side effects, it still causes problems in the eyes similar to those of epinephrine.

Cannabinoids. Cannabinoids, compounds in marijuana (cannabis), are being studied for their effects on glaucoma. For example, oral or inhaled tetrahydrocannabinol (THC), the active ingredient in marijuana, has been shown to reduce IOP in 60 - 65% of patients. The effects of smoking marijuana on IOP last only 3 hours, however. THC also increases the release of glutamate -- a nerve-protecting chemical. Experts are hoping that topical use of THC or other cannabinoids may help prevent optic nerve damage without the widespread effects of oral or inhaled administration.

Reasons for Noncompliance. Studies indicate that more than 40% of patients miss 10% of their doses, and 15% of patients miss more than 50% of their doses. Noncompliance is very high for many reasons:

People with chronic glaucoma who are on medication must use eye drops or take pills one or more times a day, usually for the rest of their lives.
Many people require a multi-drug regimen, two or more different kinds of medications that can be used in various combinations, such as eye drops, ointments, or time-release wafers inserted under the eyelid. Such regimens can be very confusing.
The side effects of the drugs are more unpleasant than the disease itself, which has no symptoms until vision is lost. Because the treatment does not usually produce any noticeable improvement, the consequence of not taking the drugs (blindness) may seem far in the future.
Skipping even a few doses can greatly increase the risk of visual loss. It is essential that patients tell their doctor if they are not regularly taking their medication. Otherwise, the doctor may increase the dosage, thereby causing unwelcome side effects.

Patients who do not regularly take their glaucoma medication are at high risk for blindness. If you have problems taking your medications or sticking to the dosing regimen, talk with your doctor.

Hints for Managing a Regimen.

Pharmaceutical manufacturers use colored tops, yellow for timolol, for example, and green for pilocarpine, to help prevent mix-ups. Creating a chart scheduling each drug by color can be helpful.
Small electronic timers are available that will signal times for taking the medications. The timing of these combinations is important. For example, the combination of pilocarpine with latanoprost is most effective when pilocarpine is taken four times a day and when the bedtime dose is administered an hour after latanoprost.
Some patients may be candidates for single medications that combine two drugs, such as Cosopt, which contains both dorzolamide and timolol. This medication requires only one drop twice per day. Patients who need additional glaucoma drugs, however, will need to take these two drugs separately.
When using any drug for a long period of time, side effects are a potential problem. If they become intolerable, patients should discuss with the doctor reducing the dosage or trying other drugs.

Administering Eye Drops. A common reason that medicine does not work is that patients do not take it correctly. Patients should ask the ophthalmologist to watch while they place the drops in their own eyes to make sure the procedure is being done correctly. The following are some recommended steps:

If you use both ointments and eye drops, take the eye drops first.
Wash your hands before applying eye drops.
Hold the bottle upside down.
Tilt your head back and, with one hand, pull the lower eyelid down to form a pocket.
With your other hand, hold the bottle as close as possible to your eye. Don’t let the bottle directly touch your eye or eyelid.
After you have placed the drop, close your eye or press your index finger against the corner of the eye near your nose. Gently move the lower lid upward until the eye is closed. Keep your eye closed for at least 1 minute. This prevents the drop from draining out.
Wait at least 5 minutes before applying another drop or a different medication

In this emergency situation, ophthalmologists may administer a combination of two or more anti-glaucoma medications to reduce eye pressure quickly before it can damage the optic nerve and cause visual loss. Apraclonidine (Iopidine) is a powerful drug used before and after laser surgery to prevent an increase in fluid pressure and is more effective than other medications. In addition to standard drugs, doctors may also administer glycerin (Glyrol, Osmoglyn) by mouth or mannitol or acetazolamide intravenously. Surgery is almost always performed once the pressure is reduced.

Most rare forms of glaucoma respond to the same medications and surgery used for open angle glaucoma. Irido corneal endothelial syndrome (ICE) is difficult to treat and if surgery is required, filtering surgery is the best choice. Neovascular glaucoma is also very hard to treat; researchers are investigating drainage implants for this disorder.

Surgery

If medications do not control eye pressure, or if they create intolerable side effects, surgery may be necessary in a small percentage of people with chronic glaucoma. It may be particularly helpful for patients with pseudoexfoliation glaucoma.

The standard procedures are usually one of the following:

Filtration surgery (trabeculectomy). This procedure opens the full thickness of the drainage area.
Laser trabeculoplasty. This procedure partially opens the drainage area. It does not reduce pressure to the extent of trabeculectomy but it has fewer adverse effects.

African Americans may respond better to initial laser surgery than to conventional trabeculectomy, while the opposite may be true in Caucasians. Some experts now recommend that, in most circumstances, African Americans should start with laser surgery and Caucasians who have no serious medical problems should have trabeculectomy first.

In addition, a number of experimental and less invasive procedures are under development.

The Procedure. Filtration surgery has been used for more than 100 years with only minor modifications. It employs conventional surgical techniques known as full-thickness filtering surgery or guarded filtering surgery (trabeculectomy).

The surgeon creates a sclerostomy, a passage in the sclera (the white part of the eye) for draining excess eye fluid.
A flap is created that allows fluid to escape but which does not deflate the eyeball.
The surgeon may also remove a tiny piece of the iris (called an iridectomy) so that fluid can flow backward into the eye.
A small bubble called a bleb nearly always forms over the opening, which is a sign that fluid is draining out. Although surgeons aim for a thick bleb, which poses less risk than a thin one for later leakage, paradoxically the ideal operation would have no bleb at all.

The procedure has a high success rate. About 50% of patients no longer need medication after surgery. Thirty-five to 40% of those who still need medication have better control of their glaucoma.

A new instrument called a trabectome has allowed for a less invasive type of trabulectomy surgery The trabectome procedure appears to be a safe and simple way to lower eye pressure. It can be performed before a traditional trabulectomy, if needed. Results from a small study, presented at the 2005 meeting of the American Academy of Ophthalmology, showed that the new approach successfully reduced eye pressure in 90% of patients with open-angle glaucoma.

Side Effects. Many of the serious side effects or complications that occur with filtration surgery involve blebs (blister-like bumps).

Bleb Leaks and Infections. Blebs, particularly thin ones, commonly leak. Leakage can occur early on or sometimes as late as months or years after surgery. Untreated, such leaks can be serious and even cause blindness. Late-onset leakage significantly increases the risk for infection as well as a number of other serious conditions, including bleeding, a flattening of the eye ball, and harmful inflammation. Surgical repair is the most effective way of managing leaking blebs, although drug therapies, pressure patching, and other nonsurgical techniques may be tried first. Due to the dangers of leaking blebs, experts recommend lifelong monitoring after surgery. Unfortunately, the incidence of late-onset leaking blebs is increasing due to the use of drugs used in filtration surgery to prevent scarring, another complication.
Scarring. In up to 20% of cases, scars form around the incision, closing up the drainage channels and causing pressure to rebuild. These scars are formed from fibroblasts, which are immature collagen cells that form at the surgical site. Scarring is a particular problem in young patients, African Americans, patients who have taken multiple drugs, have had an inflammatory disease, or have had cataract surgery. Releasing the surgical stitches used in the procedure may help prevent scarring and pressure build-up. A second procedure called bleb needling sometimes can open up the scarred area and restore drainage. With this technique, the tip of a very fine hypodermic needle is used carefully to cut loose the particles closing off the drainage area. A new technique that does not require sutures may prove to be effective and have fewer complications.
Cataracts. The procedure is highly associated with the development of cataracts over time. Because cataracts are associated with glaucoma anyway, it is not entirely clear whether the cataracts are caused by the surgery or would develop in any case.

Click the icon to see an illustrated series detailing cataract surgery.

Supportive Medication for Preventing Scarring. Specific drugs, usually mitomycin C, are often used in conjunction with the procedure to prevent scarring and closure. A large review of studies of mitomycin C supported its effectiveness in increasing surgical success in nearly all patients. Fluorouracil (5-FU) appears to be similar in effectiveness but has a high risk for complications and is not used as often as in the past.

The Procedure. Laser trabeculoplasty involves the following steps:

The procedure uses an instrument, usually a YAG laser, to burn 80 - 100 tiny holes in the drainage area.
A tiny scar forms, which increases fluid outflow.
The procedure takes 15 minutes, causes almost no discomfort, and has very few complications.

In a 2-year study, laser surgery of the trabecular meshwork reduced pressure by a third in 70 - 97% of patients. Patients still need to take anti-glaucoma eye drop medications every day.

Laser surgery is not a cure. Within 2 - 5 years, about half of patients need either additional surgery or new medications.

Complications. In about 35% of patients, pressure increases after surgery. In most cases it is temporary, but in rare cases the increased pressure is permanent and vision loss can occur. Use of the drug apraclonidine (Iopidine) or pilocarpine can help prevent this elevated pressure. About a third of patients also develop adhesive-like substances called peripheral anterior synechiae that cause the iris to stick to part of the cornea.

Drainage implants, also known as tube shunts, may be used to drain fluid in certain cases, such as if glaucoma is not responsive to any standard procedure or is caused by certain conditions. A 2007 study suggested that tube shunts work better than filtration surgery (trabulectomy) for some patients. In the study, patients who received tube shunts had more stable IOP over the course of a year than patients who underwent trabulectomy.

Candidates. Success rates are highest (75% pressure control over 5 - 7 years) in appropriate patients. Drainage implants may be useful in the following conditions:

Glaucoma caused by swelling in the iris
Glaucoma caused by abnormal vessel formations
Iridocorneal endothelial (ICE) syndrome

The Procedure. In general, the procedure involves:

An implant, most commonly a 1/2 inch silicone tube, is inserted into the eye's front chamber (anterior). The Molteno implant used with mitomycin C is currently the most effective approach, with reported success rates of 80%. Other implants, such as the Ahmed implant, may have fewer complications.
The tube drains the fluid onto a tiny plate that is sewn to the side of the eye.
Fluid collects on the plate and then is absorbed by the tissues in the eye.

Complications. Complications include:

Hypotony (very low eye pressure) is a serious complication that has been reduced using better techniques and improved implants.
Cataracts, detached retina, breakdown of the cornea, and bleeding are potentially significant complications.
There is also a risk for eye movement disorders, such as strabismus (crossed eyes) or diplopia (double-vision).

The implant often becomes blocked and repeated operations are needed. Some researchers are studying the use of a drug called tissue plasminogen activator (tPA) to open up tubes that have been blocked by blood or blood factors. (This so-called clot-busting drug is normally used to break up blood clots during heart attacks.) In one 2002 study, tPA prevented such blocks in 89% of eyes. Unfortunately, significant complications rates were high (11%).

Deep sclerectomy and viscocanalostomy are less invasive techniques than filtering surgery that leave the anterior chamber (front of the eye) intact and avoid creation of blebs.

In deep sclerectomy, the surgeon removes a deep piece of the sclera (the white part of the eye), part of the trabecular meshwork, and the front of Schlemm's canal (the vessels that return fluid into the bloodstream).

In both deep sclerectomy and viscocanalostomy, the surgeon first creates a flap in the outer part of the sclera (the white part of the eye) and then removes a deep piece of the sclera underneath. This opens up Schlemm's canal (the vessels that return fluid into the bloodstream) and exposes a layer above the anterior chamber called Descemet's membrane. A space has also been created between the inner and outer layers of the sclera.
In deep sclerectomy, this space now serves as a tiny reservoir for aqueous fluid that flows through the membrane and pools here. The fluid then flows out without the surgeon having to open the anterior chamber (as in standard filtering surgery).
In viscocanalostomy, the surgeon typically injects gel-like materials into the ends of Schlemm's canal in order to enlarge the canal for fluid outflow and lower IOP. The tiny reservoir is sewn tightly up.

Many variations are under investigation. In general, the procedures have fewer complications afterward than standard filtering surgery, although they require excellent surgical skill. Nonpenetrating techniques do not lower IOPs as much as conventional surgery does, however. In time, however, these nonpenetrating techniques are expected to be as effective as filtration surgery.

Cataracts and Glaucoma. For patients with both glaucoma and cataracts, experts recommend the following:

In patients with cataracts and poorly controlled glaucoma, a two-step procedure for both eye conditions is needed. Typically the patient will first have a trabeculectomy for glaucoma, followed by cataract surgery such as phacoemulsification (lens removal through ultrasound). Fluid leakage and the presence of blood in the back chamber of the eye are potential complications of this combined procedure.
Phacoemulsification is sometimes combined with viscocanalostomy in a procedure called phacoviscocanalostomy. A 2006 study suggested this approach is safe and effective. The study followed patients for 7 years after they underwent phacoviscocanalostomy and found that no serious complications occurred.
In patients who have cataracts plus either closed-angle glaucoma or open angle glaucoma that is stabilized with medication, the cataract may be able to be extracted and medication continued for the glaucoma.

A major 2002 analysis suggested that the combined approach generally offers better control over eye pressure for patients with both cataracts and glaucoma. However, it is still unclear which specific type of surgical procedure works best. [See In-Depth Report #26: Cataracts.]

Diode laser transscleral cyclophotocoagulation (TSCPC), also called laser cycloablation, reduces aqueous production by destroying the muscles that control the lens for near and far vision (the ciliary body ). There is a chance of vision loss with this procedure, so it is reserved for people with end-stage glaucoma or those who fail to benefit from any other therapies. Nevertheless, researchers continue to explore the possibilities for this effective procedure, especially for people who may not have access to expensive medications. Studies have suggested it may even be suitable as first-line surgery for some patients.

For an acute closed-angle glaucoma attack, emergency microsurgery is usually necessary after reducing pressure with medications.

Iridotomy or Iridectomy. Either laser (iridotomy) or conventional (iridectomy) surgery may be used. With either procedure an ophthalmologist makes a tiny opening in the iris to let the aqueous humor flow out more freely. Because acute glaucoma commonly occurs later in the other eye, surgeons will often recommend surgery in the unaffected eye to prevent a second attack.

Laser iridotomy almost never requires hospitalization, and postsurgical treatment includes only aspirin and eye drops. It has almost completely replaced conventional surgery, which requires anesthesia and hospitalization.

Vision will be blurred, and recovery can take 4 - 8 weeks. Once surgery has been performed, such patients can usually use previously restricted anticholinergic medications, such as antihistamines and certain antidepressants, with safety.

Phacoemulsification and Intraocular Lens Implantation. Phacoemulsification and intraocular lens implantation, a procedure ordinarily used for cataracts, may prove to be beneficial for some patients with acute angle-closure glaucoma requiring surgery. [See In-Depth Report #26: Cataracts.]

Lifestyle Changes

Studies suggest that patients with glaucoma who exercise regularly (at least 3 times a week) may be able to reduce their intraocular pressure by an average of 20%. If they stop exercising for more than 2 weeks, pressure increases again. In one study, those who walked briskly 4 times a week for 40 minutes were able to go off their medications. (Although not confirmed by any evidence, yoga or other exercises that involve head-down or inverted positions may be harmful for patients with glaucoma and should be discussed with the doctor.)

Exercise has no effect on closed-angle glaucoma. It may, in fact, increase eye pressure in patients with pigmentary glaucoma. Vigorous high-impact exercise may cause more pigment to be released from the iris in these patients. Patients should talk to their doctor about an appropriate exercise program.

Antioxidants in Foods and Supplements. Diet most likely plays very little role in glaucoma. For example, a 2003 study found no association between important nutrients associated with protection against other eye disorders, including vitamins C, E, A, and carotenoids.

Caffeine. Some studies have shown that large amounts of caffeine drunk in a short period of time can elevate eye pressure for up to 3 hours. One study suggested that such changes in eye pressure could be significant in patients with both normal eye pressure and high IOP.

Fluids. Drinking large amounts (a quart or more) of any liquid within a short time, about 30 minutes, appears to increase pressure. Patients with glaucoma should have plenty of fluids, but they should drink them in small amounts over the course of a day.

Glaucoma can cause the eyes to be very sensitive to light and glare. Medications can worsen this problem. Sunglasses solve this problem and are important for prevention of cataracts. Protective sunglasses do not have to be expensive. Sunglasses are classified into three categories based on protection against ultraviolet radiation (UV) A or B:

Cosmetic-purpose sunglasses block at least 70% UVB and up to 60% UVA. People should avoid these glasses if they have any risk for cataracts or eye problems.
General-purpose sunglasses block at least 95% UVB and a minimum of 60% UVA. At the very least, people should purchase general purpose sunglasses and they should be labeled "Meets ANSI Z80.3 General Purpose UV Requirements.” Labels should indicate that sunglasses block UV radiation up to 400 nm.
Special-purpose sunglasses block at least 99% UVB and a minimum of 60% UVA rays. These are the optimal sunglasses for people at risk for eye disease. Special purpose glasses should wrap around the head and block light coming from above, below, and both sides of the glasses. They should also fit snugly on the nose.
Lenses that are simply dark but not coated with UV-absorbing material may increase the risk of cataracts because the pupil widens to compensate for the shaded glass. This may allow more harmful ultraviolet waves to enter the eye. Polarized glasses cut glare but have no effect on UV radiation. Mirror finishes without additional processing for UV blockage also are not fully protective. There is some controversy over whether blue light is harmful to the eyes. Some people prefer amber lenses, which block out the blue spectrum.

Meditation, biofeedback, and relaxation methods can help counteract stress, and there are some reports that they may help some people with open-angle glaucoma. A number of herbal and nontraditional remedies have been advertised as glaucoma remedies. A few studies have reported that the herbal remedy ginkgo biloba may have properties that offer benefits to patients with glaucoma, including increasing blood flow in the eye without altering overall blood pressure, heart rate, or intraocular pressure. More research is, however, needed.

Generally, manufacturers of herbal remedies and dietary supplements do not need FDA approval to sell their products. Just like a drug, herbs and supplements can affect the body's chemistry, and therefore have the potential to produce side effects that may be harmful. There have been a number of reported cases of serious and even lethal side effects from herbal products. Always check with your doctor before using any herbal remedies or dietary supplements.

The following is of special concern for people with glaucoma:

Bilberry, a European blueberry (Vaccinium myrtillus), is sold in natural food stores as a glaucoma remedy. Studies indicate that it may help some people improve night vision and glare, but it is not at all effective in preventing or treating glaucoma.

Resources

www.glaucoma.org -- Glaucoma Research Foundation
www.nei.nih.gov -- National Eye Institute
www.glaucomafoundation.org -- The Glaucoma Foundation
www.aao.org -- American Academy of Ophthalmology
www.glaucomaweb.org -- American Glaucoma Society
www.lighthouse.org -- Lighthouse International

References

Bottaro M, Ritch R. Intraocular pressure variation during weight lifting. Vieira GM, Oliveira HB, de Andrade DT. Arch Ophthalmol. 2006 Sep;124(9):1251-4.

Brauner SC, Chen TC, Hutchinson BT, Chang MA, Pasquale LR, Grosskreutz CL. The course of glaucoma during pregnancy: a retrospective case series. Arch Ophthalmol. 2006 Aug;124(:1089-94.

Gedde SJ, Schiffman JC, Feuer WJ, Herndon LW, Brandt JD, Budenz DL. Treatment outcomes in the tube versus trabeculectomy study after one year of follow-up. Am J Ophthalmol. 2007 Jan;143(1):9-22.

Hara T, Hara T, Tsuru T. Increase of peak intraocular pressure during sleep in reproduced diurnal changes by posture. Arch Ophthalmol. 2006 Feb;124(2):165-8.

Higginbotham EJ. Managing glaucoma during pregnancy. JAMA. 2006 Sep 13;296(10):1284-5.

Pasquale LR, Kang JH, Manson JE, Willett WC, Rosner BA, Hankinson SE. Prospective study of type 2 diabetes mellitus and risk of primary open-angle glaucoma in women. Ophthalmology. 2006 Jul;113(7):1081-6.

Quigley HA, Broman AT. The number of people with glaucoma worldwide in 2010 and 2020. Br J Ophthalmol. 2006 Mar;90(3):262-7.

Wishart MS, Dagres E. Seven-year follow-up of combined cataract extraction and viscocanalostomy. J Cataract Refract Surg. 2006 Dec;32(12):2043-9.

Review Date:
3/3/2007
Reviewed By:
Harvey Simon, MD, Editor-in-Chief, Associate Professor of Medicine, Harvard Medical School; Physician, Massachusetts General Hospital.

A.D.A.M. Copyright

adam.com

Ho Ho Ho! Healthy Stocking Stuffers

FitSugar — Wed, 10 Dec 2008 10:00:13 -0800

Since the holidays are a time for giving, why not give something healthy to your friends and family? Seriously, health is the gift that keeps on giving. Here are 14 great ideas for stocking stuffers that encourage a healthy lifestyle.

A BPA-free water bottle from Sigg, Nalgene, or Klean Kanteen.
A collapsible travel hula hoop.
A year's subscription to Women's Health, Cooking Light, or other healthy magazine. Just slip the gift card in the stocking.
Larabars or other energy bars.

To check out my other stocking stuffer ideas, read more.

Wicking socks from Darn Tough for runners, hikers, skiers, and riders.
Gift cards to gear shops or health-food stores.
A handful of Emergen-C packets.
A lightweight merino wool or Capilene shirt for cold weather.
A session with a personal trainer, private chef, or dietitian.
Winter running gloves.
Homemade trail mix with cashews, almonds, dried blueberries, and dried mangoes.
Fitness DVDs or a copy of your favorite workout mix.
Resistance bands or Thera-bands.
A one-month pass to a gym or studio.

For more stocking stuffer ideas, check out all of our gift guides.

Heart failure

FitSugar — Wed, 08 Oct 2008 17:34:55 -0700

Overview

Signs and Symptoms
Causes
Risk Factors
Diagnosis
Treatment Approach
Supporting Research

HEALTH GUIDE REFERENCE FROM A.D.A.M

Heart failure (or congestive heart failure) doesn’t mean your heart has failed or stopped beating. It means that your heart, which is a muscle that pumps blood to all parts of your body, is not working as well as it should be and can’t pump as much blood as your body needs. As your heart's pumping action lessens, blood may back up in your lungs, liver, or legs. This can cause shortness of breath, leg swelling (called edema), and other problems. In addition, organs in your body may not get the oxygen and nutrients they need, meaning that they also can’t function properly.

Heart failure is a chronic (ongoing) condition that usually develops over time. It is usually caused by underlying conditions such as high blood pressure or heart disease. These conditions damage your heart, making the heart muscle stiff or thick. The damaged muscle either can’t relax properly to let the pumping chambers of the heart – the ventricles – fill with enough blood, or it can’t contract properly to let the ventricles pump out enough blood. The left ventricle is the main pumping chamber, and heart failure usually starts on the left side. When the left ventricle can’t contract enough, it’s called systolic heart failure. When the left ventricle can’t fill with enough blood, it’s called diastolic heart failure. You can have a combination of both types of heart failure.

Although some conditions that cause heart failure are irreversible, you can manage the condition and improve your health and quality of life with a combination of lifestyle changes and medications.

Signs and Symptoms

You may experience one or more of the following symptoms of chronic heart failure:

Swollen feet, ankles, and sometimes abdomen
Weight gain from fluid retention
Shortness of breath and cough
Racing or skipping heart beat (palpitations)
Stomach upset, nausea and vomiting, and loss of appetite
Difficulty sleeping
Fatigue, weakness, and a reduced ability to exercise
Lightheadedness
Difficulty concentrating or staying alert

The more advanced your heart failure, the more likely you are to have symptoms.

Acute heart failure occurs when something suddenly damages your heart (such as a heart attack, blood clot in the lungs, allergic reaction, or severe infection). Symptoms are similar to those for chronic heart failure, but are more serious and get worse quickly. Acute heart failure is life-threatening and you should seek immediate emergency medical attention.

Causes

The most common causes of heart failure are high blood pressure and coronary artery (heart) disease. Other causes of heart failure include:

Heart attack
Damaged heart valves (valves separate the chambers of the heart and keep blood flowing in the right direction)
Cardiomyopathy (weakening of the heart muscle, which may be from infection, diseases such as rheumatoid arthritis or lupus, alcohol or drug abuse, or sometimes for unknown reasons)
Congenital heart disease (heart defects at birth)
Myocarditis (inflammation of the heart from a virus)
Abnormal heart rhythms (arrhythmias)

Risk Factors

You are at risk for developing heart failure if you:

Have high blood pressure
Have diabetes
Have had a heart attack or have heart disease of any kind.
Have high blood pressure or diabetes.
Are overweight.
Have sleep apnea
Take certain medications
Abuse alcohol, smoke cigarettes, or use cocaine.

Diagnosis

Your doctor will take a detailed medical history and do a physical exam. He or she will examine your heart and lungs, checking for enlargement of the heart and fluid in the lungs. Other signs of heart failure that your doctor will look for include enlarged neck veins, swelling in your legs or abdomen, and tenderness of the liver. A chest x-ray can help to see if there is fluid on your lungs or enlargement of your heart – two factors that often go along with heart failure.

After the initial diagnosis, your doctor will look for the underlying cause of heart failure. He or she may order these tests:

Blood tests, to check kidney or thyroid function
Echocardiogram, to determine systolic heart failure or diastolic heart failure
Ejection fraction, to see how much blood you heart is pumping out
Electrocardiogram (ECG), to look for heart rhythm problems
Coronary catheterization (angiogram), to look for narrowed arteries

Treatment Approach

With proper treatment, you can control symptoms of heart failure and improve your health. Many lifestyle changes, such as losing weight, cutting down on salt, and getting regular exercise can make a difference in your condition. Medications are also available to help your heart better pump blood. Some complementary and alternative therapies can be helpful, too, when used along with standard medical treatment. However, heart failure is a serious condition and you should always seek medical care; do not take any herbs or supplements without your doctor’s supervision.

Lifestyle

Carefully monitoring your health and helping to manage your condition makes a big difference in keeping heart failure under control. To do this, track your weight on a daily basis. Weight gain can be a sign that you are retaining fluid and that the pump function of your heart is getting worse. Make sure you weigh yourself at the same time each day and on the same scale.

Other important measures include:

Take your medications as directed. Carry a list of medications with you wherever you go.
Cut down on salt. People with heart failure should consume no more than 2,000 mg per day. Keep in mind that most salt in your diet doesn’t come from the salt shaker, but from processed foods that already contains high levels of sodium. (See “Tips to lower your sodium intake” below.)
If you smoke, quit.
Exercise and stay active. Walk or ride a stationary bicycle, for example. One study showed that a walking program was safe for people with heart failure and helped improve symptoms. Talk to your doctor before starting any new exercise program; he or she can help you find one that’s right for you.
Lose weight if you are overweight.
Get enough rest, including after exercise, eating, or other activities. This allows your heart to rest, too. Keep your feet elevated to decrease swelling.

Tips to lower your sodium intake

Look for foods labeled "low-sodium," "sodium-free," "no salt added," or "unsalted." Check the total sodium content on food labels. Be especially careful of canned, packaged, and frozen foods.
Don't cook with salt or add salt to what you are eating. Try pepper, garlic, lemon, or other spices for flavor instead. Be careful of packaged spice blends as these often contain salt or salt products (like monosodium glutamate, MSG).
Avoid foods that are naturally high in sodium, like anchovies, meats (particularly cured meats, bacon, hot dogs, sausage, bologna, ham, and salami), olives, pickles, sauerkraut, soy and Worcestershire sauces, and cheese.
Take care when eating out. Stick to steamed, grilled, baked, boiled, and broiled foods with no added salt, sauce, or cheese.
Use oil and vinegar instead of bottled dressings on salads.
Eat fresh fruit or sorbet when having dessert.

Medications

ACE inhibitors - widen blood vessels and make it easier on your heart to pump blood. Side effects can include chronic cough. ACE inhibitors include

Benazepril (Lotensin)
Captopril (Capoten)
Fosinopril (Monopril)
Lisinopril (Zestril)
Enlapril (Vasotec)

ARBs - also dilate blood vessels and may be used in people who can’t take ACE inhibitors. They include

Irbesartan (Avapro)
Candesartan (Atacand)
Losartan (Cozaar)
Valsartan (Diovan)

Digoxin (Lanoxin) - helps your heart pump more blood by increasing the strength of its contractions.

Beta blockers - slow heart rate and lower blood pressure. Beta blockers include

Acebutolol (Sectral)
Atenolol (Tenormin)
Bisoprolol (Zebeta)
Carvedilol (Coreg)
Propanolol (Inderal)
Metoprolol (Lopressor, Toprol-XL)

Diuretics (water pills) - keep fluid from building up in your body by making you urinate more. There are different types of diuretics that can affect potassium and magnesium levels in your body, so your doctor will check your levels frequently.

Isosorbide dinitrate and hydralazine hydrochloride (BiDil) - BiDil combines two drugs that dilate blood vessels. It is approved for use in African-Americans who have heart failure, as an addition to standard therapy.

Nutrition and Dietary Supplements

Magnesium - Magnesium is essential to heart health. This mineral is particularly important for maintaining a normal heart rhythm and is often used by physicians to treat irregular heartbeat (arrhythmia). People with heart failure are often at risk for developing an arrhythmia. In addition, some diuretics (water pills) may cause your body to lose too much magnesium. For this reason, your doctor may recommend a supplement. Always ask your doctor before taking a magnesium supplement on your own if you have heart failure.
Carnitine (500 mg two times per day) - Some early studies suggest that L-carnitine supplements may reduce your chances of developing heart failure after a heart attack and improve exercise capacity if you already have heart failure. Carnitine is a nutrient that helps the body convert fatty acids into energy. This energy is used primarily for muscular activities throughout the body. Most studies used a special form of carnitine called propionyl-L-carnitine.
Coenzyme Q-10 (CoQ10, 100 to 200 mg per day) - Levels of CoQ10 are low in people with heart failure. Several research studies suggest that CoQ10 supplements can help reduce swelling in the legs, enhance breathing by reducing fluid in the lungs, and increase exercise capacity in people with heart failure. Not all studies agree, however. More research is needed to see if CoQ10 has any real benefit. In the meantime, talk to your doctor about whether this supplement would be good as well as safe for you.
Creatine - Creatine is a naturally occurring amino acid (protein building block) found mainly in muscles. In a few studies of people with congestive heart failure, injections of creatine (in addition to standard medical care) provided improvement in heart function and ability to exercise compared to those who received placebo. Taking creatine orally improved muscle function and endurance. More studies are needed to determine whether oral creatine has any benefit for people with heart failure.
Vitamin B1 (Thiamine) - Thiamine may be related to heart failure in several ways. First, low levels of thiamine can contribute to the development of congestive heart failure. On the flip side, people with severe heart failure can lose a significant amount of weight including muscle mass (called cachexia) and become deficient in many nutrients including thiamine. In addition, diuretics (water pills) can cause your body to lose too much thiamine. Talk to your doctor about measuring your level of vitamin B1 and whether you should take thiamine.
Amino acids - A few small studies suggest these amino acids might be helpful for heart failure, but more research is needed:
- Arginine (needed for the body to make nitric oxide, which helps blood flow)
- Taurine (helps heart muscle contract)

Herbs

The use of herbs is a time-honored approach to strengthening the body and treating disease. Herbs, however, can trigger side effects and can interact with other herbs, supplements, or medications. For these reasons, herbs should be taken with care, under the supervision of a healthcare practitioner.

Hawthorn - Hawthorn (Crataegus spp.), a member of the rose family, was used by physicians in the early 1800s to treat circulation and respiration (breathing) problems. The flowers and berries have been used traditionally to treat irregular heartbeat, high blood pressure, chest pain, atherosclerosis (hardening of the arteries), and heart failure. Several scientific studies suggest that hawthorn improves the heart’s ability to pump blood in people with heart failure. It also significantly improved symptoms (like reduced shortness of breath and fatigue) and helped people exercise longer. No studies have examined whether hawthorn can work safely with other medications, or how it compares to drugs such as ACE inhibitors. Talk to your doctor to see if hawthorn is right for you.
Berberine (300 to 500 mg four times per day) – Berberine, an active ingredient of goldenseal (Hydrastis canadensis) and other herbs, can dilate blood vessels. In one study, people who took berberine for eight weeks had better heart function and were more able to exercise than those who took placebo. A few other studies suggest that when berberine is combined with standard medicines for heart failure, it can improve heart function and quality of life. Talk to your doctor about whether it is safe and appropriate for you to take berberine in addition to your usual care.

Prognosis and Complications

Heart failure is a serious disorder that leads to a lower life expectancy. It is generally a chronic illness, but many forms of heart failure can be controlled by treating the underlying causes, making lifestyle changes, and taking medication.

Potential complications include:

Pulmonary edema (fluid buildup in the lungs)
Total failure of the heart to function (circulatory collapse or shock)
Arrhythmias (irregular heart rhythm) including fatal arrhythmias

Supporting Research

Al-Hasso. Coenzyme Q10: a review. Hosp Pharm. 2001;36(1):51-66.

Andrews R, Greenhaff P, Curtis S, Perry A, Cowley AJ. The effect of dietary creatine supplementation on skeletal muscle metabolism in congestive heart failure. Eur Heart J. 1998;19(4):617-622.

Arsenian, MA. Carnitine and its derivatives in cardiovascular disease. Progr Cardiovasc Dis. 1997;40:3:265-286.

Azuma J. Long-term effect of taurine in congestive heart failure: Preliminary report. Adv Exp Med Biol. 1994;359:425-33.

Baggio E, Gandini R, Plancher AC, Passeri M, Carmosino G. Italian multicenter study on the safety and efficacy of coenzyme Q10 as adjunctive therapy in heart failure. CoQ10 Drug Surveillance Investigators. Mol Aspects Med. 1994;15(Suppl):s287-294.

Bleske BE, Zineh I, Hwang HS, Welder GJ, Ghannam MM, Boluyt MO. Evaluation of hawthorn extract on immunomodulatory biomarkers in a pressure overload model of heart failure. Med Sci Monit. 2007 Dec;13(12):BR255-258.

Blumenthal M, ed. The Complete German Commission E Monographs: Therapeutic Guide to Herbal Medicines. Boston, Mass: Integrative Medicine Communications; 1998:120,142-144,162-163,171-172,197.

Brady JA, Rock CL, Horneffer MR. Thiamin status, diuretic medications, and the management of congestive heart failure. J Am Diet Assoc. 1995;95:541-544.

Churbasik S. Evidence of the effectiveness of hawthorn extract. Am J Med. 2003;115(7):585; author reply 585-586.

Degenring FH, Suter A, Weber M, Saller R. A randomized double blind placebo controlled clinical trial of standardized extract of fresh Crataegus berries (Crataegisan) in the treatment of patients with congestive heart failure NYHA II. Phytomedicine. 2003;10:363-9.

Eaton LJ, Kinkade S. Hawthorn extract improves chronic heart failure. J Fam Pract. 2003;52(10):753-753.

Evangeliou A, Vlassopoulos D. Carnitine metabolism and deficit – when supplementation is necessary? Curr Pharm Biotechnol. 2003;4(3):211-219.

Ferraro S, Codella C, Palumbo F. Hemodynamic effects of creatine phosphate in patients with congestive heart failure: a double-blind comparison trial versus placebo. Clin Cardiol. 1996;19(9):699-703.

Field ML. Creatine supplementation in congestive heart failure. Cardiovasc Res. 1996;31(1):174-176.

Fong HH, Bauman JL. Hawthorn. J Cardiovasc Nurs. 2002;16(4):1-8.

Fugh-Berman A. Herbs and dietary supplements in the prevention and treatment of cardiovascular disease. Prev Cardiology. 2000;3:24-32.

Gavagan T. Cardiovascular disease. Primary Care. 2002;29(2):323-338, vi.

Ghidini O, Azzurro M, Vita G, Sartori G. Evaluation of the therapeutic efficacy of L-carnitine in congestive heart failure. Int J Clin Pharmacol Ther Toxicol. 1988;26:217-20.

Holubarsch CJ, Colucci WS, Meinertz T, Gaus W, Tendera M. Survival and prognosis: investigation of Crataegus extract WS 1442 in congestive heart failure (SPICE) – rationale, study design and study protocol. Eur J Heart Fail. 2000;2(4):431-437.

Houston MC. Treatment of hypertension with nutraceuticals, vitamins, antioxidants and minerals. Expert Rev Cardiovasc Ther. 2007 Jul;5(4):681-91.

Ince C, Schulman SP, Quigley JF, et al. Usefulness of magnesium sulfate in stabilizing cardiac repolarization in heart failure secondary to ischemic cardiomyopathy. Am J Cardiol. 2001;88(3):224-229.

Khatta M, Alexander BS, Krichten CM, Fisher ML, Freudenberger R, Robinson SW et al. The effect of conenzyme Q10 in patients with congestive heart failure. Ann Int Med. 2000;132(:636-640.

Koh SG, Brenner DA, Korzick DH, Tickerhoof MM, Apstein CS, Sauper KW. Exercise intolerance during post-MI heart failure in rats: prevention with supplemental dietary propionyl-L-carnitine. Cardiovas Drugs Ther. 2003;17(1):7-14.

Lau CW, Yao XQ, Chen ZY, Ko WH, Huang Y. Cardiovascular actions of berberine. [review]. Cardiovasc Drug Rev. 2001;19(3):234-244.

Leslie D, Gheorghiade M. Is there a role for thiamine supplementation in the management of heart failure? Am Heart J. 1996;131:1248–1250.

Mendoza CE, Rodriquez F, Rosenberg DG. Reversal of refractory congestive heart failure after thiamine supplementation: a report of a case and review of literature. J Cardiovas Pharmacol Ther. 2003;8(4):313-316.

Miller AL. Botanical influences on cardiovascular disease. Altern Med Review. 1998;3(6):422-431.

Morelli V, Zoorob RJ. Alternative therapies: Part II. Congestive heart failure and hypercholesterolemia. [Review]. Am Fam Physician. 2000;62(6):1325-1330.

Overvad K, Diamant B, Holm L, Holmer G, Mortensen SA, Stender S. Review coenzyme Q10 in health and disease. Eur J Clin Nut. 1999;53:764-770.

Pauly DF, Pepine CJ. The role of carnitine in myocardial dysfunction. Am J Kidney Dis. 2003;41(4 Suppl 4):S35-43.

Pittler MH, Schmidt K, Ernst E. Hawthorn extract for treating chronic heart failure: meta-analysis of randomized trials. Am J Med. 2003;114(:665-674.

Rizos I. Three-year survival of patients with heart failure caused by dilated cardiomyopathy and L-carnitine administration. Am Heart J. 2000;139:S120-3.

Rotblatt M, Ziment I. Evidence-Based Herbal Medicine. Philadelphia, PA: Hanley & Belfus, Inc; 2002:231-235.

Rigelsky JM, Sweet BV. Hawthorn: pharmacology and therapeutic uses. Am J Health Syst Pharm. 2002;59(5):417-422.

Sarter B. Coenzyme Q10 and cardiovascular disease: a review. J Cardiovasc Nurs. 2002;16(4):9-20.

Schmidt U, Kuhn U, Ploch M, Hubner WD. Efficacy of the hawthorn (Crataegus) preparation LI 132 in 78 patients with chronic congestive heart failure defined as NYHA functional class II. Phytomedicine. 1994;1:17-24.

Singh RB, Niaz MA, Agarwal P, Beegum R, Rastogi SS, Sachan DS. A randomised, double-blind, placebo-controlled trial of L-carnitine in suspected acute myocardial infaction. Postgrad Med. 1996;72:45-50.

Singh U, Devaraj S, Jialal I. Coenzyme Q10 supplementation and heart failure. Nutr Rev. 2007 Jun;65(6 Pt 1):286-93. Review.

Sole MJ, Jeejeebhoy KN. Conditioned nutritional requirements: therapeutic relevance to heart failure. Herz. 2002;27(2):174-178.

Tauchert M. Efficacy and safety of crataegus extract WS 1442 in comparison with placebo in patients with chronic stable New York Heart Association class-III heart failure. Am Heart J. 2002;143(5):910-915.

Witte KK, Clark AL, Cleland JG. Chronic heart failure and micronutrients. J Am Coll Cardiol. [Review]. 2001;37(7):1765-1774.

Zeng XH, Zeng XJ, Li YY. Efficacy and safety of berberine for congestive heart failure secondary to ischemic or idiopathic dilated cardiomyopathy. Am J Cardiol. 2003;92(2):173-176.

Zenuk C, Healey J, Donnelly J, Vaillancourt R, Almalki Y, Smith S. Thiamine deficiency in congestive heart failure patients receiving long term furosemide therapy. Can J Clin Pharmacol. 2003 Winter;10(4):184-8

Review Date:
12/27/2007
Reviewed By:
Steven D. Ehrlich, N.M.D., private practice specializing in complementary and alternative medicine, Phoenix, AZ. Review provided by VeriMed Healthcare Network.

A.D.A.M. Copyright

adam.com

Chest tube insertion - series

admin — Thu, 04 Sep 2008 19:42:36 -0700

Back

Overview

Normal anatomy
Indication
Procedure
Aftercare

HEALTH GUIDE REFERENCE FROM A.D.A.M

Normal anatomy

The pleural space is the space between the inner and outer lining of the lung. It is normally very thin, and lined only with a very small amount of fluid.

Indication

If fluid, such as blood, or air, gets into the pleural space, the lung can collapse, preventing adequate air exchange. Chest tubes are used to treat conditions that can cause the lung to collapse, such as:

air leaks from the lung into the chest (pneumothorax)
bleeding into the chest (hemothorax)
after surgery or trauma in the chest (pneumothorax or hemothorax)
lung abscesses or pus in the chest (empyema).

Procedure

Chest tubes are inserted to drain blood, fluid, or air and allow full expansion of the lungs. The tube is placed in the pleural space. The area where the tube will be inserted is numbed (local anesthesia). The patient may also be sedated. The chest tube is inserted between the ribs into the chest and is connected to a bottle or canister that contains sterile water. Suction is attached to the system to encourage drainage. A stitch (suture) and adhesive tape is used to keep the tube in place.

The chest tube usually remains in place until the X-rays show that all the blood, fluid, or air has drained from the chest and the lung has fully re-expanded. When the chest tube is no longer needed, it can be easily removed, usually without the need for medications to sedate or numb the patient. Medications may be used to prevent or treat infection (antibiotics).

Aftercare

Recovery from the chest tube insertion and removal is usually complete, with only a small scar.

The patient will stay in the hospital until the chest tube is removed. While the chest tube is in place, the nursing staff will carefully check for possible air leaks, breathing difficulties, and need for additional oxygen. Frequent deep breathing and coughing is necessary to help re-expand the lung, assist with drainage, and prevent normal fluids from collecting in the lungs.

Review Date: 8/10/2007

Reviewed By: Allen J. Blaivas, DO, Pulmonary, Critical Care, and Sleep Medicine, Department of Veteran Affairs, VA System, East Orange, NJ. Review provided by VeriMed Healthcare Network.

A.D.A.M. Copyright

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