Ask Your Physician: Diversity Among Blood Donors Important for Treatment of Sickle Cell Disease

Q: What is sickle cell disease? Why do these patients need a blood transfusion?

A: Sickle cell disease occurs when a defect in the red blood cell gene is inherited from parents. Healthy red blood cells are round and flexible. As they move through blood vessels, they bend and carry oxygen throughout the body. In sickle cell disease, the red blood cells are hard, sticky, and look like a sickle or crescent. Sickle cells pass through blood vessels and cannot deliver oxygen where they are needed.

Two of the most serious complications of sickle cell disease are acute chest syndrome and stroke. Acute chest syndrome occurs when sickle cells block small blood vessels in the lungs. Sickle cells cannot pick up oxygen from the lungs and deliver it to the rest of the body, potentially leading to death. Stroke is caused by sickle cells that block the blood vessels that supply oxygen to the brain. This can lead to death of brain cells, which can lead to injury or death.

In both cases, the treatment is a blood transfusion that reduces the concentration of sickle cells and increases the number of healthy red blood cells. Special red blood cells are used in this process.

On the surface of red blood cells are various proteins inherited from their parents. Patients receiving blood transfusions may develop antibodies to these proteins, which can lead to reactions to the blood transfusion in the future. Patients with sickle cell disease have too many blood transfusions, which increases the risk of making antibodies and reacting.

To prevent this from happening, sickle cell patients receive special red blood cells that match not only the blood group but additional proteins. Because these proteins are inherited, matches are likely to be found within donors of the same ethnic group as the patient. This is why having diversity among blood donors is so important.

Liz Culler, MD, is the Dean of Blood Assurance and a member of the Chattanooga Hamilton County Medical Society.

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