Practice patterns for neuroimaging and transfusion therapy for management of neurologic complications in sickle cell anemia: DISPLACE consortium

Background Children with sickle cell anemia (SCA) are at risk for neurologic complications (stroke and silent cerebral infarct). The 2014 National Heart, Lung, and Blood Institute (NHLBI) guidelines for sickle cell disease include recommendations for initiation and maintenance of chronic red cell transfusion (CRCT) therapy for children with SCA at risk for or with ischemic stroke. The guidelines do not include well-delineated recommendations for cerebral imaging for stroke screening. The purpose of this study was to evaluate current stroke risk screening, prevention, and intervention practices amongst the Dissemination and Implementation of Stroke Prevention Looking at the Care Environment (DISPLACE) study sites. Procedure A survey was administered to DISPLACE site principal investigators to identify current stroke prevention practices relative to the Stroke Prevention Trial in Sickle Cell Anemia (STOP) study protocols and the 2014 NHLBI guidelines. Data were analyzed using descriptive statistics and line-by-line analysis of comments. Results Sites consistently applied NHLBI recommendations to initiate CRCT for ischemic stroke and abnormal transcranial Doppler ultrasound (TCD) results. Similarly, nearly all sites reported obtaining an magnetic resonance imaging (MRI)/magnetic resonance angiography (MRA) following an abnormal TCD result. There was wide variation for other indications for MRI/MRA, frequency of MRI/MRA, and other neurological indications for initiating CRCT. Conclusions Guideline-based practices for preventing ischemic stroke through TCD and CRCT initiation were evident in nearly all sites. Wide variation in practices pertaining to MRI/MRA exists, potentially influenced by more recent stroke prevention trials. New guidelines from the American Society of Hematology were published in April 2020, which may reduce practice variation.