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
Abstract
Background While national guidelines for the management of
vaso-occlusive crisis (VOC) and acute chest syndrome (ACS) in sickle
cell disease (SCD) exist, institutional algorithms are tailored to their
clinical practices. This study aims to compare institutional algorithms
to each-other and to national recommendations for VOC/ACS in pediatric
SCD. Procedures Algorithms were collected from children’s
hospitals across the United States, and compared to recommendations made
by the National Heart, Lung, and Blood Institute (NHLBI) 2014 and the
American Society of Hematology (ASH) 2020 regarding management of
VOC/ACS in SCD. Results: 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, with individualized pain protocol and non-pharmacological
measures mentioned in 19 each. Incentive spirometry was included in 16
of 17 ACS algorithms, but only 11 of 37 VOC algorithms. Antibiotics were
recommended by all ACS algorithms, but 4 used regimens different from
the NHLBI recommendation. Most ACS algorithms had recommendations
regarding transfusion, but with considerable variability. Intravenous
fluid management strategies were also highly variable and hypotonic
fluids were recommended in 6 VOC and 4 ACS algorithms.
Conclusion: Internal algorithms for pediatric SCD show great
variability compared to each other and with national guidelines, likely
due to the lack of robust evidence supporting specific recommendations.
Prospective studies are crucial to fill these evidence gaps with the
overall goal to improve patient care.