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

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.

Hospital Use and Mortality in Transition-Aged Patients With Sickle Cell Disease.

OBJECTIVES: Childhood mortality in sickle cell disease (SCD) has decreased, but the transition period is associated with poor outcomes and higher mortality rates. We analyzed recent US hospitalizations and mortality trends in the transition-aged population and evaluated for differences between patients with and without SCD. METHODS: Nationwide Inpatient Sample database was used to analyze hospitalizations among individuals aged 16 to 24 years from 2003 to 2017. Diagnoses were coded by using International Classification of Diseases, Ninth Revision, Clinical Modification and International Classification of Diseases, 10th Revision, Clinical Modification. We performed bivariate analyses to assess associations between sociodemographic characteristics and SCD hospitalizations, joinpoint regression analysis to describe mortality rate trends in SCD hospitalizations, and adjusted survey logistic regression to assess associations between patient characteristics and in-hospital mortality among transition-aged SCD and non-SCD-related hospitalizations. RESULTS: There were 37 344 532 hospital encounters of patients aged 16 to 24 years during 2003-2017; both SCD and non-SCD hospitalizations increased with age. Female patients accounted for 78% of non-SCD and 54.9% of SCD hospitalizations. Although there was a +3.2% average annual percent change in SCD hospitalizations, total SCD in-hospital mortality rates did not have a statistically significant increase in average annual percent change over the study period. Patients with SCD aged 19 to 21 and 22 to 24 were more likely to suffer in-hospital mortality than those aged 16 to 18 (odds ratio = 2.09 and 2.71, respectively); the increased odds in mortality by age were not seen in our non-SCD population. CONCLUSIONS: Transition-aged hospitalizations increase with age, but SCD hospitalizations have disparate age-related mortality rates. Hospital-based comprehensive care models are vital to address the persistent burden of early adulthood mortality in SCD.