Sickle Cell Disease

Sickle cell disease is an inherited blood disorder. Normally, red blood cells are disc-shaped and flexible. In sickle cell disease, however, hemoglobin (the chemical within red blood cells that carries oxygen around the body) is abnormal. This causes red blood cells to collapse into a crescent or sickle shape. It also causes the red blood cells to be abnormally stiff and fragile. These elongated, stiff red blood cells have a tendency to clump together and clog up small blood vessels throughout the body. When blood vessels are blocked by sickle-shaped red blood cells, parts of the body are deprived of oxygen. This can cause severe pain and damage to the organs and tissues that are deprived of oxygen. Furthermore, abnormal red blood cells are destroyed at an unusually high rate, causing a shortage of red blood cells (called anemia).