Preventing cognitive decline in sickle cell disease: A good night's sleep.

Abstract

Among children with sickle cell disease (SCD), 40% of untreated children with elevated transcranial Doppler (TCD) velocities will develop stroke within 3 years.1 In 1998, the Stroke Prevention Trial in Sickle Cell Anemia (STOP) demonstrated that the use of chronic red blood cell transfusions in children with SCD with elevated TCD velocities in the middle cerebral artery decreased the risk of stroke by 92%.2 STOP, which revolutionized the outlook of children with SCD, relied on the assumption that TCD velocity predicts the risk for developing stenosing arteriopathy prior to the development of irreversible arteriopathy. Cerebral arteriopathy is a potent risk factor for primary and recurrent stroke in childhood, including among children with SCD.3,4