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Sickle cell disease

Description

An in-depth report on the causes, diagnosis, and treatment of sickle cell disease.

Alternative Names

Sickle cell anemia

Lifestyle Changes

There are no proven methods for preventing 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.

General Precautions

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.

Dietary Factors and Supplements

Foods. Good nutrition is essential for anyone and 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. Female patients may want to include cranberry juice to help prevent urinary tract infections.
• It is important to have 5 - 9 daily servings of green, red, and yellow vegetables, fruits, or juices that are rich in antioxidants and other important nutrients. Some research suggests that antioxidant foods or supplements (such as vitamins E or C) may help inhibit the formation of the dense cells that trigger a sickle cell crisis. One medical group has created a "cocktail" of supplements and food extracts that are rich in antioxidants and iron-binding compounds that might have more protective effects on the sickling process than single antioxidants. It includes garlic extract, black and green tea extract, pycnogenol, alpha-lipoic acid, vitamin E, coenzyme Q10, and beta-carotene. In any case, patients might eat foods containing these extracts and take supplements of the antioxidant vitamins E and C if their diet does not adequately supply them.
• The chemical resveratrol, which is found in red grape skins, appears to have properties similar to hydroxyurea, the primary drug used in sickle cell disease. Drinking great amounts of grape juice is unlikely to make much difference, but adding it to a child's diet is unlikely to do harm.
• Protein is important for sickle cell patients.
• 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.

Minerals and Other Natural Substances .

• Zinc. Zinc sulphate appears to help reduce red blood cell dehydration. Important studies indicate that it helps prevent sickle cell crises and reduce pain and life-threatening complications. A study on children with sickle cell suggested that supplements may help improve growth and weight gain. It may also boost the immune system and help protect against bacterial infections. Zinc deficiency is a common nutritional problem in sickle cell disease, so supplements may important.
• Magnesium. Magnesium protects against potassium and water loss in sickle cells. Small studies are reporting promise for its use in preventing dehydration and increasing hemoglobin S concentration.
• Arginine. Arginine is an amino acid that the body converts to nitric oxide, a natural substance that relaxes blood vessels. The sickle cell process reduces nitric oxide levels, which may be responsible for much of the pain in these patients. Arginine and other substances that convert to nitric oxide are being studied in trials.
• L-glutamine is an ordinary amino acid that is heavily used by sickle cells. One study using supplements of this substance reported that after a month it caused positive changes in the blood. Another small study found that daily oral glutamine supplementation improved growth and nutritional measures in children and adolescents with sickle cell disease.

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.

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

Relief for Mild Pain

For mild pain relief, common medications such as acetaminophen (Tylenol) or the class of drugs known as nonsteroidal anti-inflammatory drugs (NSAIDs) are often sufficient. Aspirin is the most common NSAID, but there are many others, including ibuprofen (Advil, Motrin) and naproxen (Naprosyn, Aleve). Aspirin is not usually recommended for children because it can aggravate abdominal pain.

Managing the Emotional and Social Impact

In assessing the seriousness of this disease, no one should underestimate its emotional and social impact. For the family, there is nothing more heartbreaking than to watch 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. Those that use 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).

• Review Date: 1/17/2007
• Reviewed By: Harvey Simon, MD, Editor-in-Chief, Associate Professor of Medicine, Harvard Medical School; Physician, Massachusetts General Hospital

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