Representation of Sickle Cell Disease Patients in Pediatric and Adult Care: What We Discovered Using Informatics for Integrating Biology and the Bedside (i2b2) Data

Transitioning from pediatric to adult care is a high-risk period for individuals with sickle cell disease (SCD). An initial observation at one academic medical center suggested a sex-based disparity in clinic populations, prompting a national investigation. We aimed to compare the sex distributions among SCD pediatric and adult clinics across the United States. Using the Informatics for Integrating Biology and the Bedside (i2b2) platform, we queried deidentified electronic health record data from 34 geographically diverse academic medical centers. Data on patient sex and age group (pediatric: 0-18 years; adult: ≥19 years) were analyzed from 19 responding centers. The pediatric population (n = 10,400) was balanced by sex (50.7% female, 49.3% male). In contrast, the adult population (n = 20,730) was significantly skewed, with females comprising 59.7% and males 40.3% (p < 0.001). This underrepresentation of males in adult clinics was consistent across 16 of the 19 centers. For one center that maintained balanced sex proportions, a follow-up inquiry revealed flexible clinic scheduling (early mornings, evenings, and weekends) and staffing by advanced practice nurses. These findings demonstrate a significant nationwide disparity in the sex distribution of adults with SCD engaged in specialized care and are hypothesis-generating rather than definitive evidence of transition failure. Future research is needed to elucidate the factors contributing to the underrepresentation of males in adult SCD clinics and to evaluate targeted interventions, including flexible, nurse-led care models, to support engagement and retention in adult care.