DEVELOPMENT OF THE PATIENT-IMPORTANT OUTCOMES NATIONAL DATA REPOSITORY (PIONEER) FOR HEREDITARY ANGIOEDEMA (HAE)

Abstract

<h3>Introduction</h3> Here we describe the development and general characteristics of PIONEER-HAE, an enhanced database combining electronic medical data with insight from chart review, for the purpose of informing future studies. <h3>Methods</h3> Data source: Continuity of care documents (CCD), EMR (labs, procedures, treatment), and visit notes specific to patients in care by the Consortium of Independent Immunology Clinics (CIIC). Data extraction/collection process. [FIGURE] Unstructured data were extracted into electronic forms by clinically-trained scribes and included attack details, on demand treatment, symptoms onset and diagnosis, family history, and comorbidities. Form data were subjected to logical checks, random audit (1-2%), and tertiary data review after combining with CCD and EMR data. Inferred HAE diagnosis, based on treatment, ICD10 D84.1, and/or SNOMED 24743004, was confirmed or corrected by source physicians. <h3>Results</h3> As of Jun 2022, PIONEER-HAE contained data for 480 HAE-confirmed patients in care at 12 practices across 11 US states. Population characteristics: female 68% (327), mean (median) age 43 (42) years with 65% ≤50 years. Scribes reviewed visit notes for 265 patients and 1957 visits representing 1024 patient-years. For this subset, 83% (221/265) and 56% (148/265) patients had a visit in the last 36 or 12 months, respectively. HAE attacks were documented for 93% (246/265) patients at 69% (1358/1957) visits. Attack details included number/frequency (41%; 804/1358 visits), body location (74%; 1008/1358 visits), on-demand medication (72%, 971/1358 visits), and severity/resolution (90%; 1227/1358 visits). <h3>Conclusion</h3> PIONEER-HAE combines visit information with fielded data residing in EMR/CCD to enable better understanding of patient-important outcomes.