Sickle Cell Anemia

People with sickle cell anemia who live in the United States have an average life expectancy of 45 years. There are a variety of effective medications available to help reduce complications and improve survival. However, at this time, there is no universal cure, although a very few children worldwide have been cured through a blood stem cell transplant. The stem cells come from a sibling's bone marrow, or less frequently, from umbilical cord blood. However, most children with sickle cell anemia do not have a sibling who is a good genetic match. In addition, stem cell transplants are a risky procedure and don't always cure the disease.

Early treatment of newborns includes penicillin, vaccination against pneumococcus bacteria and folic acid supplementation.

Standard treatments for complications of sickle cell anemia include antibiotics, pain management, intravenous fluids, blood transfusion and surgery.

Hydroxyurea is a new treatment for sickle cell anemia that reduces the frequency of pain episodes, acute chest syndrome and the need for blood transfusions in adults. Studies are currently underway to determine the proper dosage of hydroxyeurea for children. Researchers also are investigating similar drugs for children to reduce the number of complications and improve survival, as well as new approaches to gene therapy.

Reviewed by health care specialists at UCSF Benioff Children's Hospital.

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