Prevalence and Impact of Autism Spectrum Disorder and Selected Neurodevelopmental Diagnoses in Hospitalized Youth With Sickle Cell Disease.

Abstract

Background: Sickle cell disease (SCD) is a chronic illness accounting for 134,000 hospital admissions annually. Youth with SCD and youth with autism spectrum disorder (ASD) experience health disparities, yet the hospitalization outcomes of these youth have not been assessed. This study aimed to (i) determine the prevalence of ASD and selected neurodevelopmental disabilities (NDD) among hospitalized youth with SCD and (ii) compare hospitalization outcomes in youth with selected NDD and SCD to youth with SCD.

Procedure: ICD-10 diagnosis codes were used to identify admitted youth (2-18 year olds) with SCD, ASD, and selected NDD (i.e., developmental delay) in the Pediatric Health Information System (October 2015 to April 2024). Demographic (age, race) and clinical variables (length of stay, intensive care unit [ICU] admission, 30-day readmissions, hydroxyurea use, genotype) were assessed.

Results: Among 16,369 unique inpatients diagnosed with SCD (54.7% hemoglobin SS, median age = 11.9 years, and 86.4% non-Hispanic Black), 2.6% were diagnosed with a selected NDD; 1.7% were diagnosed with ASD. Hydroxyurea use during hospitalization did not differ between groups (3.3% vs. 3.5%; p = 0.19). Individuals with selected NDD had significantly higher annualized rates of hospitalization (0.88 vs. 0.65; p < .0001), ICU admission (0.12 vs. 0.05; p < .0001), and 30-day readmissions (20.2% vs. 17.4%; p = 0.0004) compared to youth without these neurodevelopmental diagnoses. The median length of stay in both groups was 3 days.

Conclusions: Youth with selected NDD and SCD are at an increased risk of frequent and complicated hospitalizations. Additional research should investigate how inpatient and outpatient care delivery can be tailored and optimized to reduce the frequency and severity of hospitalizations.