PC3083: National Mortality and Outcomes of Children with Sickle Cell Disease Admitted to a Pediatric Intensive Care Unit in the United States

Background: Data on children with sickle cell disease (SCD) admitted to a pediatric intensive care unit (PICU) are limited. Prior studies demonstrate that overall mortality for SCD has improved, but the PICU demographic profile of patients with SCD, data on exposure to interventions in the PICU, and risk factors for mortality in the PICU are unknown. To address this gap, we conducted a retrospective analysis of prospectively collected multicenter national data from the Virtual Pediatric Systems, LLC (VPS) database. Our specific aims were to: (1) characterize the demographic profiles of and the interventions and outcomes experienced by pediatric patients with SCD in the PICU; (2) characterize inter-institutional variability in outcomes experienced by pediatric patients with SCD in the PICU; and (3) identify associations between clinical descriptor variables and mortality or the need for invasive respiratory support (IRS). We hypothesized that there would be significant institutional and regional variability in outcomes for pediatric patients with SCD admitted to a PICU.

Methods: To address this question, we conducted a retrospective, multicenter cohort analysis utilizing data voluntarily submitted by PICUs that contribute to the VPS database. We queried for all patients admitted to PICUs from January 1, 2012 to December 31, 2019. All PICU admissions with either a primary or secondary diagnosis of SCD were included. We excluded patients < 1 month of age and >18 years of age at the time of admission and any patient with a diagnosis of sickle cell trait or thalassemia alone.

Results: We found 3388 unique children with SCD who accounted for a total of 5264 PICU admissions. The distribution of admissions per center was skewed. A total of 1.8% of admissions died in the PICU. There was no variability in mortality rate among centers based on geographic region. PICU admissions with SCD who were < 2 years of age, who experienced acute respiratory failure requiring interventions such as a bronchoscopy or high-frequency oscillation ventilation, or who required continuous renal replacement therapy or intensive neurologic support modalities had higher odds ratios for mortality. In multivariate analysis, admissions with higher PRISM-3 scores and those who needed IRS all had a higher adjusted odds risks for mortality.

Conclusion: Our retrospective analysis of a large, multi-year, national cohort of patients with SCD admitted to a PICU demonstrated that while overall mortality for PICU patients with SCD remains low, there are certain PICU-specific “flags” that should trigger concern that a particular patient with SCD admitted to a PICU may be at higher risk for death or more serious illness.