Chronic Red Blood Cell Transfusions. Chronic red blood cell transfusions are used as a treatment for various complications of sickle cell disease. Transfusions are the major treatment for cerebrovascular accidents (stroke) in children with sickle cell disease. With chronic transfusions, blood is given every three to four weeks. Complications of chronic red blood cell transfusions include iron overload, infection, antibody formation, and transfusion reactions.
Hydroxyurea. All infants are born with fetal hemoglobin (HbF) in their blood cells; however, soon after birth the amount of HbF rapidly decreases. Hydroxyurea is a medication taken by mouth once a day to help increase the body’s production of HbF, thereby reducing sickling and many of the major complications of sickle cell disease.
Bone Marrow Transplantation. Bone marrow (or stem cell) transplantation is the only cure for sickle cell disease. The cure was first performed successfully in 1983, when a St. Jude patient with leukemia and sickle cell disease received a bone marrow transplant. The procedure cured both diseases. Red blood cells are made in the bone marrow. In a person with sickle cell disease, the bone marrow produces hemoglobin S instead of hemoglobin A. During a transplant, the patient’s bone marrow is replaced with marrow from a person who does not have sickle cell disease. Even though bone marrow transplant is a cure for sickle cell disease, its use is limited because of the difficulty in finding a matched donor, and the complications associated with transplant.
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