What is Homozygous Beta Thalassemia Major (Cooleys Anemia) disease?
Homozygous Beta Thalassemia Major, also known as Cooley’s Anemia, is an inherited blood disorder that affects a person’s ability to produce beta protein, causing anemia. Anemia is also known as having a low blood count. It is generally found in people of Mediterranean descent; such as Italians and Greeks, and is also found in Arabia, Iran, Africa, Southeast Asia, and China countries. Cooley’s anemia is the most severe form of beta thalassemia disease in which the complete lack of beta protein in the hemoglobin causes a life-threatening anemia that requires regular blood transfusions and extensive ongoing medical care. These extensive, lifelong blood transfusions lead to iron-overload that must be treated with chelation therapy to prevent early death from organ failure. Generally, chelation therapy is any drug treatment that helps the body rid itself of excessive levels of iron, a condition known as iron overload.
