Characterizing Rare Disease Healthcare Utilization Using a Real-World Data Platform

Author(s)

Nichols C1, Pace ND1, Nysetvold E1, Cotter K1, Cutillo CM2, Tisdale A2
1AllStripes Research, San Francisco, CA, USA, 2National Center for Advancing Translational Sciences (NCATS), Bethesda, MD, USA

Objectives: The cumulative economic burden of rare diseases surpasses that of common conditions, yet patterns of healthcare utilization (HCU) across rare diseases remain poorly characterized. This study leverages data collected during clinical care to provide an in-depth evaluation of patients’ HCU across the disease journey.

Methods: Patients with confirmed genetic diagnosis of two rare pediatric conditions were recruited (SLC6A1 epileptic encephalopathy = 30; Kleefstra syndrome (KS) = 40). Structured and unstructured data were abstracted from patients' medical records. Encounters per patient-year of follow-up were calculated.

Results: Most patients were female (SLC6A1 = 67%, KS = 60%) and diagnosed at <6 years of age (SLC6A1 median = 5; KS = 2.7). Records were available for a median of 6.1 years from a median of 4.5 healthcare facilities (268 unique facilities). Patients on average experienced the following number of encounters: outpatient (SLC6A1 = 87.0; KS = 78.3), procedure (SLC6A1 = 0.5; KS = 2.6), ER (SLC6A1 = 1.4; KS = 1.5), and direct inpatient admissions (SLC6A1 = 1.3; KS = 0.6). Records documented 7.5 and 4.3 total medications on average for SLC6A1 and KS patients, respectively, over the study period.

The numbers of outpatient, ER, and procedure encounters were not significantly different pre- and post-diagnosis for either condition. Direct admissions per year of follow-up in SLC6A1 increased post-diagnosis (average: 0.11 vs. 0.30, p=0.02); all post-diagnosis direct admissions during the study period were seizure/EEG-related, a significant increase compared to pre-diagnosis (p=0.01).

Conclusion: This study provides real-world evidence (RWE) on the healthcare burdens experienced by families living with rare pediatric conditions, particularly during the diagnostic journey. Patients with SLC6A1 experienced more direct admissions post-diagnosis, likely due to increased seizure monitoring. Continued examination of HCU patterns using RWE can further elucidate the social and economic costs of rare disease.

Conference/Value in Health Info

2022-05, ISPOR 2022, Washington, DC, USA

Value in Health, Volume 25, Issue 6, S1 (June 2022)

Code

SA36

Topic

Study Approaches

Topic Subcategory

Electronic Medical & Health Records, Prospective Observational Studies, Registries

Disease

Neurological Disorders, Pediatrics

Your browser is out-of-date

ISPOR recommends that you update your browser for more security, speed and the best experience on ispor.org. Update my browser now

×