The prevention and management of stroke in sickle cell anaemia

Perhaps the most important clinical complication of sickle cell anaemia is stroke, an event that occurs in approximately 5-10% of children who inherit this disorder. To prevent recurrent or progressive CNS damage, the institution of regular red blood cell (RBC) transfusions is the standard of care. In addition, children at high risk of developing stroke, as screened by transcranial Doppler, also benefit from regular RBC transfusions for stroke prevention. In this review, standard and novel techniques of RBC transfusion, and also alternative therapies to treat children with or at risk for stroke are considered. In addition, haematopoietic cell transplantation, the only curative option for sickle cell anaemia, is considered, and speculation about its present and future application in this clinical setting is discussed.