Sickle Cell Anemia

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Treatment

The goals with treatment for sickle cell disease are to relieve pain, minimize complications and organ damage, and prevent infection. Babies and children with sickle cell disease should have all of the regular childhood vaccines, plus influenza vaccine (every year) and pneumococcal vaccine (at 2 and 5 years of age). Newborns with sickle cell disease are often placed on long-term penicillin therapy (begins at 2 months of age and continues until 5 years of age) to prevent pneumonia and other infections. Blood transfusions are used to treat severe anemia. Children and adults may be treated with hydroxyurea, a medication that has been found to reduce the number of crises and lessen the severity of those that do occur. The number of crises can also be minimized by avoiding situations that frequently trigger episodes, such as overexertion, dehydration, temperature extremes, changes in altitude, smoking, and stress. Taking folic acid supplements is recommended for people with sickle cell disease because folic acid is necessary for the production of new red blood cells.

Stroke, aplastic crises, acute chest syndrome, swollen spleens, and occasionally severe crises may need to be treated with blood transfusions or exchange transfusions to raise the red blood cell count and reduce the number of sickle cells. Some people who have had multiple transfusions may require treatment to address iron overload. Those with organ damage and/or organ failure may require additional treatments, such as having their spleen removed. Full-match bone marrow transplants (stem cell transplants) have been shown to wipe out sickle cell disease, eliminating many of the painful associated symptoms; however, finding a complete bone marrow match may not be feasible due to a shortage of donors. In a recent John Hopkins study, partial-match bone marrow transplants successfully treated 60% of patients with sickle cell disease.

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