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For some reason not yet understood, the red cell membranes of certain individuals become fragile and vulnerable to destruction. Red cells are prematurely destroyed and blood may be lost in the urine. People with this disorder usually require transfusions and, in very severe cases, should be considered for a bone marrow transplant.

A defect in the beta chain of hemoglobin causes people with sickle cell anemia toproduce an abnormal type of hemoglobin, called sickling hemoglobin (Hb S) Inheriting one gene for Hb S gives you sickle cell trait, which rarely causes any health problems. Inheriting two genes for Hb S causes sickle cell anemia. Like thalassemia major, sickle cell anemia is a very severe disorder; treatment must begin in early childhood.