sickle cell disease

(SIH-kul sel dih-ZEEZ) An inherited disease in which the red blood cells have an abnormal crescent shape, block small blood vessels, and do not last as long as normal red blood cells. Sickle cell disease is caused by a mutation (change) in one of the genes for hemoglobin (the substance inside red blood cells that binds to oxygen and carries it from the lungs to the tissues). It is most common in people of West and Central African descent. Also called sickle cell anemia.

A blood condition seen most commonly in people of African ancestry. The disorder is caused by a single base pair change in one of the genes that codes for hemoglobin, the blood protein that carries oxygen. This mutation causes the red blood cells to take on a sickle shape, rather than their characteristic donut shape. Individuals who suffer from sickle cell disease are chronically anemic and experience significant damage to their heart, lungs, and kidneys. See also gene, cell, protein, recessive