Multi-center retrospective study of children with sickle cell disease admitted to pediatric intensive care units in the United States

Abstract

Data on outcomes and interventions for children with sickle cell disease (SCD) admitted to a pediatric intensive care units (PICU) are unknown. We provide the first comprehensive multi-center report on PICU interventions associated with death, the need for invasive respiratory support or stroke among critically ill children with SCD. We collected retrospective multi-center cohort data from January 1, 2012 to December 31, 2019 utilizing the Virtual Pediatric Systems, LLC database. We identified 3388 unique children with SCD, accounting for a total of 5264 PICU admissions from 138 PICUs. The overall mortality rate for the PICU admissions cohort was 1.8% (95/5264 PICU admissions, 95/3388 [2.8%] of all unique patients), the rate of needing of needing Invasive Respiratory Support (IRS, a composite category of exposure) was 21.3% (872/4093 PICU admissions with complete data) and the overall rate of stroke (ischemic or hemorrhagic) was 12.5% (657/5264 PICU admissions). In multivariable analysis adjusting for admission age category, sex, race/ethnicity, PRISM-3 score at admission, exposure to IRS, quartile of unit volume of patients with SCD, and patient origin, admitted children who needed invasive respiratory support (IRS) had higher adjusted odds ratios for mortality (adjusted odds ratio [aOR], 19.72; 95% confidence interval [CI] 8.98-43.29; p < 0.001), although admitted children > 2 years old had decreased aOR for needing IRS (aOR 0.25-0.62; 95% CI 0.16-0.94; p < 0.001-0.025). By contrast, admitted children > 2 years old had a strikingly increased aOR for stroke (aOR 7.57-16.32; 95% CI 2.25-52.15; p < 0.001). These groups may represent PICU-specific subsets of patients with SCD who are at higher risk for more serious illness and should deserve early consideration for referral to a pediatric institution providing comprehensive care for patients with SCD.

Figure 1

Box-whisker plot of percent of deaths per center, percent of patients with need for invasive respiratory support and percent of patients with stroke by quartiles of center volume. The vertical axis represents percentage of all patients reported from each center from 2012 to 2019. (A) Mortality; (B) need for invasive respiratory support or (C) stroke. The horizontal axis represents the number of admissions per center displayed by quartiles (see Table 1 for numeric quartile cutoffs and number of centers per quartile). The data are presented as box-whisker plots with the grey boxes representing the 25–75% percentiles of the data and the whiskers representing the 10% and 90% percentiles accordingly. Data outside the 90% are plotted individually. For each plot, by Kruskal–Wallis testing, the distributions for each quartile are different (p < 0.001).