During the past ten years, a comprehensive sickle cell program has emerged at the University of south Alabama. Since the grant award from the National Heart, Lung and Blood Institute in 1988, the center has developed exponentially. The goal of the Comprehensive Sickle Cell Center is to improve the lives of sickle cell patients in the region as well as in the international community. The region of the central Gulf Coast has a large population at risk of sickle cell disorders. The goal of improving the lives of sickle cell patients is being accomplished through (i) basic, clinical and applied research to understand the pathophysiology, diagnosis and management of sickle cell disease; (ii) education of health professionals, patients and their families, and community at large; (iii) clinical and laboratory diagnosis of sickle cell disease and its complications as well as carrier state, pre-natal diagnosis, newborn screening and follow-up; and (iv) counseling, both genetic and psychosocial. The objectives of basic research projects are to elucidate the mechanism of hemoglobin switch through cloning of transacting regulatory gene(s) and studies of a 70 Kd, DNA-binding protein; to study interactions of spectrin, actin and band 4.1 cytoskeletal proteins; to determine the role of a newly described cytoplasmic protein, Calpromotin, in dehydration of sickle cells and the role of oxidation in sickle erythrocyte membrane damage; to examine interaction of blood cells and adhesion-influencing molecules; and to investigate the mechanism of microvascular injury in lungs. The clinical research projects evaluate school readiness in young children with sickle cell disease, and the impact of comprehensive health care on quality of life of the patients and health care costs. A strong community program provides counseling and supportive services. In summary, the University of South Alabama Comprehensive Sickle Cell Center proposes to continue research, diagnosis, education, and counseling to improve the lives of those who have sickle cell disease.
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