(148) The burden of Prader-Willi Syndrome on patients and the healthcare system: a cross-sectional examination of emergency department visits and inpatient stays in US claims

Background: : Prader-Willi syndrome (PWS) is a rare genetic disorder with a median age of death of 23 years (McCandless et al., 2020). Butler et al. (2017) concluded that individuals with PWS show heightened hyperphagia-related mortality (e.g. high rates of choking, accidents, and gastrointestinal perforation), contribute to one third of all US reported deaths.

Objective: : This analysis examined the utilization of emergency department (ED) visits and inpatient (IP) stays to inform the burden of PWS on patients and healthcare systems.

Methods: : Observational retrospective analysis was performed on a de-identified US closed-claims dataset (2021-2023). Patients (pts) with ≥2 claims, and a PWS diagnosis (Q87.11) >2 weeks apart were included. In this cross-sectional analysis, pts were assigned to one of five age groups ( < 12, 12-23, 24-35, 36-47, 48-59 years-old). All statistics were descriptive and annualized. All diagnostic/service codes from this three-year period were included.

Results: : This study included 3685 pts ( < 12: 33.19%; 12-23: 30.53%; 24-35: 17.20%; 36-47: 9.50%; 48-59: 7.06%). The average annual ED visit rate was 0.93 visits/pt and ranged from 0.71 in the < 12 group, to 1.35 in the 24-35 group. Pts with ≥1 visit averaged 1.25 ( < 12 group) to 1.92 visits (24-35 group) annually. For inpatient stays, the average annual rate was 3.75 stays/pt, ranging from 3.00 (24-35 group) to 4.63 (36-47 group).

Conclusions: : These results validate previous studies examining healthcare resource utilization of PWS patients. The high utilization of ED and IP services were observed for all age groups, despite a dwindling population size as a function of age due to mortality. Utilization of ED and IP services were >4 times higher in pediatric PWS patients vs the general pediatric population (2019 CDC data). These real-world analyses illustrate the considerable burden of PWS on patients, and the US healthcare system, highlighting the considerable need for treatment of hyperphagia in PWS.

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