Sickle cell anemia: Pathophysiology, management, and prospects for the future

Sickle cell anemia is present in about 1 in 600 Black Americans at birth. It claims the life of many infants, causes pain and suffering in adults, and usually culminates in premature death. We currently understand a great deal about this disease on a molecular, cellular, and clinical basis. The results of years of research are slowly being translated to innovative therapies and improvements in patient care. In this paper I review the pathophysiology of this disorder, highlight the currently available treatment, and discuss new forms of therapy that promise a more direct approach to avoiding the many complications of sickle cell anemia.