Cooley's Anemia (Beta Thalassemia Major)
Cooley's anemia is an inherited genetic blood disorder due to defective beta globin genes. It affects the production of beta globin chains of hemoglobin and as a result red blood cells break down as soon as they are formed. That causes hemolytic anemia in the affected children, who require regular blood transfusion throughout their life just to stay alive.Defectives genes are inherited by the affected children from their parents. These parents are healthy carrier of the defective gene and are able to produce viable hemoglobin, and as such do not require blood transfusion. But marriage of two carriers may give birth to affected children. Cooley's anemia cause significant complications - bone deformities and cardiovascular illnesses due to iron overload from multiple blood transfusions. The affected children have poor immunity and suffer from various infections, which require appropriate antibiotic therapy. The most (80%) of affected children die due to heart failure.
Cost of Treatment:
The cost of blood transfusion and iron chelation therapy is around US$ 1200/- per year for an affected child.