Definition of Sickle cell disease

Sickle cell disease: A genetic blood disorder caused by the presence of an abnormal form of hemoglobin. These hemoglobin molecules tend to aggregate after unloading oxygen forming long, rod-like strictures that force the red cells to assume a sickle shape. Unlike normal red cells, which are usually smooth and malleable, the sickle red cells cannot squeeze through small blood vessels. When the sickle cells block small blood vessels, the organs are deprived of blood and oxygen. This leads to periodic episodes of pain and damages the vital organs. Sickle red cells die after only about 10 to 20 days. Instead of the usual 120 days or so. Because they cannot be replaced fast enough, the blood is chronically short of red cells, causing anemia. The gene for sickle cell anemia must be inherited from both parents for the illness to occur in children. A child with only one copy of the gene may have sickle-cell traits but no symptoms of illness.