A hemoglobinopathy with a chronic hemolytic anemia and vaso-occlusive ischemia tissue injury. The Hb ranges form 5-10 g/dl and there is usually a neutrophilia Patietns suffer from jaundice, Splenomegaly, gall stones, and poorly healing ulcers over the lower tibia. Hemolytic crisis with severe pain in back, long bones and chest, an vaso-occlusive episodes leading to strokes, splenic infarctions, and priapism. Aplastic crisis precipitated by infections, and folate deficiency. Individuals with just the triat are usually healthy but still run the risk of sudden death with rigorous exrcise
Treatment
Painful crisis managed with rest, hydration and analgesics, and if very severe narcotics might be required. Folate supplementation is routine. Transfusion are to be given only for aplastic or hemolytic crisis and during the third trimester of pregnancy. Exchange transfusions are preferred for treatment of intractable crisis, priapism and stroke
