Beth Hyperlink

Matthew M. Hsieh, M .D., Elizabeth M. Kang, M.D., Courtney D. Fitzhugh, M.D., M. Beth Hyperlink, R.N., Charles D. Bolan, M.D., Roger Kurlander, M.D., Richard W. Childs, M.D., Griffin P. Rodgers, M.D., Jonathan D. Powell, M.D., Ph.D., and John F. Tisdale, M.D.1,2 This noticeable modification causes a propensity toward polymerization of hemoglobin and, hence, sickle-shaped red cells. Anemia, elevated hemolysis, and chronic and acute vaso-occlusive complications that affect multiple organs are the main features of sickle cell disease. At present, allogeneic hematopoietic stem-cell transplantation is the only curative option.3-5 Approximately 200 children have this process after myeloablative conditioning with busulfan and cyclophosphamide undergone, with or without antithymocyte globulin, resulting in a rate of disease-free survival of 95 percent in the newest series.5 After transplantation, the donor’s hematopoietic cells completely replace those of the recipient generally in most children who undergo this procedure, but some continue steadily to have both recipient and donor cells in the blood vessels .6 This blend is enough to reverse the sickle cell disease phenotype.