Provider Perspectives on Diagnosis and Management of Acute Chest Syndrome

Introduction:Acute chest syndrome (ACS) is the leading cause of mortality, accounting for 25% of all deaths among individuals with sickle cell disease (SCD). The etiologies and clinical manifestations of ACS are variable, with a lack of clear risk stratification guidelines for the practicing clinician. Management of ACS is based on limited evidence and is currently guided primarily by expert opinion. Based on this gap in the diagnosis and management of ACS, we created a multicenter ACS work group in March 2022 that included pediatric and adult hematologists, and transfusion medicine physicians. We hypothesized that the risk stratification and management of ACS is significantly variable among providers involved in the care of SCD individuals.