Disease Burden and Healthcare Utilization Among Patients with Acute Intermittent Porphyria Experiencing Chronic Neuropathy: Analyses from a National Healthcare Database
Angelika Erwin1, Samuel Silver2, Stephen Meninger3, Joseph Tkacz4, Virginia Noxon4, John J. Ko3
1Cleveland Clinic Cleveland, 2University of Michigan Medical Center, 3Alnylam Pharmaceuticals, 4IBM Watson Health
Objective:
This study aimed to identify AIP patients diagnosed in a nationally representative health care database to estimate healthcare resource utilization among various segments of the AIP patients defined by porphyria attack rates, chronic symptoms, and comorbidities.
Background:

Acute hepatic porphyria (AHP) refers to a family of rare, metabolic diseases that includes four types, acute intermittent porphyria (AIP) being the most common. AHP is characterized by potentially life-threatening acute attacks and, for some patients, chronic debilitating symptoms.

Design/Methods:
This retrospective analysis utilized the IBM® MarketScan® Commercial Claims and Medicare Supplemental Databases. Patients with at least one claim for AIP (ICD-10 diagnosis code E80.21) between October 1, 2015June 30, 2018 were selected for analyses. AIP patients were segmented by frequency of attacks, presence of chronic symptoms and the presence of comorbidities. This analysis focused on the patient segment specific to chronic neuropathy. Means were reported as per patient per year (PPPY).
Results:
56 (24.9%) patients with chronic neuropathy were identified; 80.4% female, mean (SD) age 49.9 years (14.8). Mean observation time of identified diagnosed patients was 2.0 years. Patients had a mean (SD) of 2.7 (3.4) attacks PPPY; 30.4% had 3 attacks/year. The majority had 1 hospitalization (57.1%) and emergency department (ED) visit (75.0%), with a mean (SD) of 1.0 (1.4) admissions and 7.5 (23.2) ED visits PPPY.
Conclusions:
Results from this national representative healthcare claims database demonstrated AIP patients experiencing chronic neuropathy have high disease burden and healthcare utilization.