The Cincinnati Comprehensive Sickle Cell Center serves 185 children at the Children's Hospital Medical Center where the administrative core of the Center is located, and 190 adults at the University of Cincinnati Hospital. Integration of the two branches of the Center is facilitated by the proximity of the two hospitals. Four research projects draw upon the clinical resources for both the pediatric and adult patients with SCA: 1) a study relating intra- arterial sickling, red cells physiology and the regulation of fetal hemoglobin production to clinical severity; 2) a study of blood oxygenation and intra-arterial sickling during sleep; 3) a study of abnormalities of hemostatic mechanisms and endothelial integrity; 4) and patient compliance and ability to cope during transfer of care from childrens' to adults' program. Children only will be involved in an evaluation of nutrition and caloric needs and a study of the immune responses to pneumococcal antigens 3) or 4) Projects will employ recombinant DNA techniques to investigate the molecular mechanisms governing developmental regulation of the globin genes. Another project will entail introducing anti- sickling agents into red cells to affect therapy of the disease. A diagnostic program for newborns, that has been at the Center for twelve years, will be extended to the remainder of the state and affected children detected will be given prophylactic penicillin. Methodology will be established to assess the effectiveness of counseling, and patient and professional education in the Center.
