Variability in the Recommendations for Management of Vaso-occlusive Crisis and Acute Chest Syndrome in Sickle Cell Disease: Review of Institutional Algorithms of Pediatric Hospitals Across the United States

This study aims to review institutional algorithms for vaso-occlusive crisis (VOC) and acute chest syndrome (ACS) in pediatric sickle cell disease (SCD), comparing them to each other and to national recommendations. Algorithms collected from children's hospitals across the country were compared with recommendations made by the National Heart, Lung, and Blood Institute 2014 and the American Society of Hematology 2020 regarding management of VOC/ACS in SCD. Review of 37 VOC and 17 ACS algorithms from 40 children's hospitals showed that most followed national guidelines for diagnostic evaluation of VOC/ACS. Parenteral opioids and NSAIDs were recommended by all VOC algorithms, with variations in dosing and administration. Intranasal fentanyl was recommended by 31 algorithms. Incentive spirometry was included in 16 of 17 ACS algorithms, but only in 11 of 37 VOC algorithms. Antibiotics were recommended by all ACS algorithms, but 4 used regimens were different from national recommendations. Most ACS algorithms had recommendations regarding transfusion, but with considerable variability. Intravenous fluid management strategies were highly variable, and hypotonic fluids were recommended in 6 VOC and 4 ACS algorithms. Overall, internal algorithms for pediatric SCD showed variability compared with each other and with national guidelines, likely due to the lack of robust evidence supporting specific recommendations.