The Duke Comprehensive Sickle Cell Center (DCSCC) is the nucleus of a broad program in clinical care, clinical and basic research, and education for sickle cell anemia and the related hemoglobinopathies. All the clinical and research programs, as well as the design of the overall Center, are directed toward hastening the process whereby gains in basic scientific, clinical, psychosocial, and health policy research can be translated more rapidly and with better effect into the clinical arena, for the benefit of our patients and others affected worldwide with sickle cell disease. Our Center benefits from close collaborations with the University of North Carolina at Chapel Hill (UNC-CH) as part of the NIH-funded Duke-UNC Comprehensive Sickle Cell Center, as well as from the active and enthusiastic participation of community-based sickle cell programs, the North Carolina State Sickle Cell Syndrome Program, and the North Carolina Consortium for Sickle Cell Disease.
The objectives of DCSCC are:
� to perform, facilitate, and support research directed toward solving the basic, clinical, psychosocial, educational, and community health problems in sickle cell disease;
� to facilitate and coordinate interaction among individuals and institutions engaged in such research so that they may benefit one another in helping patients with sickle cell disease, their families, and their communities;
� to provide services to patients and their families and communities that will ameliorate their condition and to assess the effects of such services to determine optimal algorithms for their provision; and
� to build and maintain relationships between the Center and outside agencies and individuals —locally, state-wide, and nationwide—so that improvements and knowledge about sickle cell disease may be both imported into and exported from the Center.
This DCSCC has made major achievements in basic research and has embarked on new initiatives in clinical research.
� Members of the DCSCC have successfully used ribozymes to alter hemoglobin S mRNA so that it encodes hemoglobin F;
� Members of the DCSCC have identified two major adhesion receptor of sickle red cells and the signaling mechanisms whereby they become activated, thus identifying a new target for potential therapeutic interventions;
� Members of the Adult Clinical Service have embarked on several new clinical research initiatives to evaluate new pharmacologic interventions to prevent or treat vaso-occlusion in SCD.
� Members of the Genetics group have identified genes that predispose patients to the development of pulmonary hypertension, a major risk factor for early mortality in SCD.