Hydroxyurea — An Essential Medicine for Sickle Cell Disease in Africa

Higher levels of fetal hemoglobin (HbF) ameliorate the clinical course of sickle cell disease. Hydroxyurea, an inhibitor of the enzyme ribonucleoside diphosphate reductase, increases HbF synthesis by erythroid cells (Figure 1). More than 20 years ago, a major clinical trial of hydroxyurea in the management of sickle cell disease proved successful.1 Subsequent studies, including eight randomized, controlled trials, that have involved adults, children, and specific clinical situations all showed benefit from hydroxyurea,2 with particular effects on pain reduction, prolonged survival, improvement in quality of life, and recently, prevention of end-organ damage. Hydroxyurea is now recognized in national and international guidelines . . .