We propose to establish a Comprehensive Sickle Cell Disease Center which will have as its major objectives: 1) Improved care for patients with sickle cell disease residing in the catchment area of Duke University Medical Center, 2) Clinical Research on the effects of sickle cell disease on: a) metabolism of glucose by the brain, b) cardiopulmonary function, c) aseptic necrosis of the hip, 3) Development and institution of information-education programs about sickle cell syndromes for patients, heterozygotes, families, physicians, nurses, other health professionals, and the community at large. These information-education programs will be linked to sickle cell screening directed at education guidance, and disease detection and will be interwoven in the presently active State Sickle Cell Program and community based Sickle Cell Programs. 4) Study of basic scientific problems in sickle cell disease including: a) characterization and influence of hemoglobin geneotype, b) study of allosteric effectors of hemoglobin function, 3) effects of HbS geleation on ionic red cell membrane transport, d) studies of red cell membrane fludity. In order to diffuse the relevant programs throughout the State and to obtain input from the community, we will work with the State Program and two active community-based Sickle Cell groups. We will cooperate fully with other Comprehensive Centers involved in the National Sickle Cell Program.
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