A comment on improving transcranial <scp>D</scp>oppler ultrasonography screening in children with sickle cell anemia
Authors: Margaret O. Lewen, Patricia L. Kavanagh, Amy E. Sobota
Journal: American Journal of Hematology (2017)

Regular screening with transcranial Doppler ultrasonography (TCD) is a crucial component of primary stroke prevention for children with sickle cell anemia (SCA).1 Despite being the standard of care for nearly two decades, TCD screening rates remain low.2 In a paper recently published in the American Journal of Hematology, Adams et al. reported TCD re-screening rates for 1896 eligible children receiving care at 19 of the 26 original sites that participated in the pivotal STOP and/or STOP II trials.2 Only 57% of eligible patients had any follow-up TCD screening performed over a five year period, with significant variation by site (18-91%). The authors expressed concern that TCD screening protocols were not fully implemented even in these experienced centers. Other sickle cell centers have reported similar experiences with TCD screening among children with SCA, with rates ranging from 22% to 45%.3, 4 For the past six years, we have utilized systems-based processes to achieve high TCD screening rates. We offer our results to demonstrate the efficacy of these methods, as our screening rates among eligible children with SCA have been ≥70% since 2011. Approximately 200 children with sickle cell disease receive care in our pediatric hematology practice, which is based in an urban safety net academic institution. As an HRSA Sickle Cell Disease Newborn Screening grantee from 2011-2015, we implemented an electronic health record (EHR)-based patient registry and used a part-time patient navigator to work with providers and families to achieve consistently high TCD screening rates.5 Even though our grant funding ended in 2015, we have maintained high TCD screening rates without additional staff, surpassing most screening rates reported to date (Table 1). To achieve and maintain these results, we have made two significant changes to our approach to patient care. First, we have incorporated our EHR-based patient registry reporting functions into regular clinic workflow, which has enabled us to provide better care to our entire patient population. Population-based lists are generated weekly for all children seen in our clinic and reviewed at our multidisciplinary hematology team meetings that include both clinical and administrative staff. All patients who are overdue for TCD are identified and contacted by administrative staff to schedule necessary screenings. In addition, an “opportunity report” is generated prior to each clinic session to easily identify patients who are not up to date on TCD and other screenings so that appropriate services can be provided. By focusing on our entire sickle cell patient population, rather than just those who attend clinic, we reduce the risk of neglecting the needs of our most at-risk patients. This change has mirrored recent trends toward enhancing population (rather than individual) health, and our EHR-based registry has been paramount in this effort.6 Children and families affected by SCD face numerous barriers to care, including time and money spent traveling to appointments and the challenge of missing work or school. Our second intervention is the development of an excellent working relationship with the vascular laboratory at our institution. Over the past three years, we have had the ability to co-schedule TCDs with clinic visits, therefore decreasing the hardship of multiple visits on families. Moreover, this close working relationship has allowed same-day TCD screenings to be arranged for patients who are overdue and considered high risk for loss to follow-up. Co-scheduling TCD screening with clinic visits also reinforces the notion that they are an integral component of routine SCD care, thereby enhancing patients' and families' understanding of the importance of this intervention. There is unequivocal evidence that annual TCD screening plays a crucial role in ischemic stroke prevention in pediatric SCA and therefore is an essential component of routine care. However, as Adams et al. demonstrate,2 implementing proven interventions in real-world, non-research settings is a slow process. Unfortunately, variable implementation of TCD screening recommendations puts children with SCA at risk of poor outcomes. Within our institution, we have taken concrete steps to achieve high TCD screening rates. By developing and maintaining an EHR-based patient registry, transforming our clinic into a team-based practice caring for an entire population of patients, and by co-scheduling TCDs with clinic visits, we have maintained excellent TCD screening rates without any additional clinic staff. We offer our experience as evidence that robust, systems-based interventions can be easily integrated into clinical care across the US and provide effective, sustainable methods of improving TCD screening, thereby reducing the risk of stroke in children with SCA. Nothing to report.