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What is Sickle Cell Anemia?

Sickle cell disease is a genetic disorder that arises as a result of a point mutation in the beta-chain of normal human hemoglobin. In the presence of reduced oxygen tension, the red cells assume a shape referred to as sickle (or crescent-shaped), and move through the small capillaries with considerable difficulty. The abnormal cells block the flow of blood to tissues and cause severe pain, growth retardation, liver damage, hepatitis, jaundice, gallstones, and kidney failure. These symptoms arise when abnormal genes are inherited from both parents.

The treatment for sickle cell anemia centers around reducing the symptoms of pain. The excess accumulation of iron characteristic of this disorder requires a reduction in iron intake and avoidance of iron-rich foods for some people; however, some sickle cell patients are already iron-deficient and should not reduce iron further without physician approval.

Compared with other major diseases sickle cell has attracted relatively little research finding. It's a disease shrouded in ignorance and superstition, resulting in high infant mortality, particularly in lower social groups. In the more remote villages of West Africa, the disease is still seen as "God's curse" and babies who die needlessly are sometimes marked in the belief that if the child is reincarnated, the mark will remain to warn families of the "evil spirits" return.