(807) Mortality and Respiratory Failure for Children With Sickle Cell Disease Admitted to U.S. PICUs

Introduction: Data on outcomes for children with sickle cell disease (SCD) admitted to pediatric intensive care units (PICUs) are limited. Despite decreased mortality, little is published on pediatric critical care outcomes for patients with SCD. We provide the first comprehensive report on PICU mortality and factors associated with the need for invasive respiratory support (IRS) among critically ill children with SCD.

Methods: We conducted a retrospective, multicenter observational cohort analysis of Virtual Pediatric Systems, LLC (VPS) data (IRB#:H-47768) from 1/1/2012-12/31/2019. We included children with either a primary or secondary diagnosis of SCD and excluded patients < 1 mo and >18 yrs and any patient with a diagnosis of sickle cell trait or thalassemia. We found 5264 admissions, representing 3388 unique patients with SCD from 138 separate PICUs. To maximize the analytic power of the de-identified data, we analyzed the cohort at the level of admissions, adjusting for multiple admissions and center effect.

Results: The cohort was 47% female and >65% were > 6 yrs old. The top 2 most common admission categories were acute chest syndrome (ACS) and respiratory-related diagnoses. Overall cohort mortality was low (1.8% [95/5264] admissions). Need for IRS was associated with smaller median weight and height while children >2 yrs had unadjusted odds ratios (ORs) < 1, indicating a decreased need for IRS. Not surprisingly, HFOV or iNO was associated with mortality in unadjusted analyses, although HFNC or NIPPV was not. In multivariable analysis (adjusted for age, sex, race, PRISM-3, quartile of unit SCD volume, patient origin), the adjusted OR of mortality was 19.7x higher for patients who required IRS. Adjusted ORs for need for IRS for children older than 2 years were < 1.

Conclusions: In this first analysis of a multi-year, multi-center cohort of patients with SCD admitted to a PICU, overall PICU mortality was low. IRS and some associated interventions impart a higher likelihood of PICU mortality. The risk for IRS was not distributed equally across age groups with higher ORs for children < 2 yrs. These are challenging data to interpret as previous literature has characterized ACS to be more severe in older patients with SCD, highlighting a need to study and examine these associations more thoroughly.