MANAGEMENT OF PATIENTS WITH SICKLE-CELL DISEASE

The ever-increasing body of information regarding the molecular pathogenesis of sickle cell disease has raised expectations that a specific and effective therapy could be devised to inhibit polymerization of hemoglobin S. Despite an intense international research effort, this goal has not yet been realized. Supportive care continues to be the mainstay in the management of patients with sickle cell anemia. Empiric measures were used in early attempts to treat sickle cell anemia. Herrick reported gratifying improvement after 'rest, nourishing food, the administration of iodide, arsenic, thymol and the application of boric ointment to leg ulcers.' Many of these suggestions are similar to those of today. Several factors have significantly improved the prognosis of patients with this disease. Improved medical care, genetic counseling, and universal neonatal screening have directly resulted in improved outcome. Molecular biologic techniques have allowed us to approach this disease in a pathophysiologic way. Now we can envision antenatal diagnosis with the use of molecular probes and treatment by gene amplification. Still, however, the most important and challenging link in the chain between the biology of the disease and the clinical sequelae is an astute and interested physician who must remain objective at all times.