Sickle Cell Disease - Lifestyle Changes

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Chelation therapy is used to remove excess iron stores in the body. 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. A newer drug deferasirox (Exjade) is 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.

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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 usually be 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 too "thick". 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.

Bone Marrow or Stem Cell Transplantation

At this time, the only chance for 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.

Bone marrow transplantations have been performed successfully in select children with sickle cell disease. However, due to a lack of available donors and the risks of potential complications, bone marrow transplantations for sickle cell disease are not routinely performed. Complications can include the immune system’s rejection of the transplant (a condition called graft-versus-host-disease) and serious infections. Patients can suffer serious neurological damage if the procedure triggers bleeding in the brain. In general, younger children are considered better candidates for bone marrow transplantation than older children.