Variability in Internal Algorithms for Sickle Cell Disease

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.

Methods:

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. Exchange transfusion guidelines from either NHLBI or ASH were included in 14 of 17 ACS algorithms. Intravenous fluid management strategies were highly variable and hypotonic fluids were recommended in 6 VOC and 4 ACS algorithms.

Discussion:

This study is the first of its kind to review algorithms across the country regarding VOC and ACS management in children. Institutional algorithms generally aligned with national guidelines for management of VOC and ACS, but significant treatment variations were observed. Many algorithms did not include certain recommendations specified in the national guidelines, such as incentive spirometry, despite its widespread availability and effectiveness. Intranasal fentanyl (INF) was commonly recommended in VOC algorithms, aligning with its proven efficacy as an opioid analgesic. Variability in IVF recommendations likely stems from the lack of randomized controlled trials addressing IVF benefits and risks in VOC and ACS. Variations existed in transfusion indications in ACS, possibly due to ambiguity in definitions of severity of ACS in national guidelines. Despite technical challenges, most protocols recommended exchange transfusion for severe ACS, in line with national guidelines.

Conclusion:

Despite the presence of national guidelines, internal algorithms for VOC and ACS in pediatric SCD show great variability, likely due to the lack of robust evidence supporting specific recommendations in national guidelines. Prospective studies are crucial to fill these evidence gaps with the overall goal of improving patient care.

No relevant conflicts of interest to declare.