How I Treat How I treat and manage strokes in sickle cell disease

Strokes in children and adults with sickle cell disease (SCD) continue to be a major cause of morbidity. Understanding the epidemiology of overt strokes is critical to the multidisciplinary, and timely acute and long-term management of strokes. The first neurologic complication associated with SCD was described in a child with sickle cell anemia (SCA), seizures, and acute left hemiparesis in 1923. In the 1970s, a single center retrospective cohort study by Powars et al, described for the first time the high rate of overt ischemic strokes in children and adults with SCD (;6%), and their high recurrence rate (;50%) in the first 2 years after the initial event, and 66% in the 9 years following the initial stroke. In the 1990s, investigators from the Cooperative Study of Sickle Cell Disease provided the most definitive and comprehensive study of the natural history of strokes across the lifespan. Children and adults with SCA (hemoglobin SS [HbSS]) have a high prevalence (4.01%) and incidence (0.61 per 100 patient years) of cerebrovascular accidents. Among patients with SCA, ischemic strokes were observed to have a bimodal distribution, being more common in children and older adults, and lowest in adults aged 20 to 29 years. Risk factors associated with ischemic strokes include prior transient ischemic attack (TIA), low steady-state hemoglobin (Hb) concentration, rate of, and recent episode of acute chest syndrome (ACS), and elevated systolic blood pressure (BP). Among individuals with SCA, hemorrhagic stroke was most frequent in the 20to 29-year age group. Associated risk factors included low steady-state Hb and high leukocyte count. Similar to observations by Powars et al, in this cohort, hemorrhagic strokes were associated with a high mortality rate (24% overall and 26% in individuals with SCA). This review describes our multidisciplinary approach to acute and long-term management of strokes. For illustrative purposes, we describe 2 cases, a child and an adult with SCA, presenting with focal neurologic deficit, highlighting inherent challenges in care, while also emphasizing current approaches in the acute, sub-acute, and long-term management of strokes.