Development of the Hereditary Angioedema Rapid Triage (HAE-RT) Tool

Abstract Background HAE patients present to the emergency department (ED), where their symptoms are often incorrectly attributed to common allergic and gastrointestinal conditions resulting in major delays in diagnosis and treatment. Objective The goal of this study was to develop a rapid triage HAE tool for ED settings. Methods A mixed methods approach was conducted in 3 phases: Phase 1 : A literature review on the current management of HAE patients in the ED. Phase 2 : A Delphi study with HAE specialists (N=9) and Patient Advocacy Group Members (N=3) to reach consensus on the predictor variables (PV) to be included in the HAE-RT tool. Phase 3 : A retrospective chart review to assess the performance of the PV for HAE. Results The literature review informed the final list of PVs included in the HAE-RT prototype. Nine experts participated in the Delphi study. Of 8 identified HAE-specific PVs identified, 3 reached consensus: (1) absence of urticaria; (2) recurrent abdominal pain/swelling; and (3) lack of response to allergic directed therapy. The retrospective study included 107 patients (N=66 with HAE; N=41 non-HAE). HAE patients were more likely to have a family history of HAE (71%; P Conclusion Expert consensus led to the identification and prioritization of variables that when incorporated into an HAE-RT tool, were associated with a high level of sensitivity and specificity when applied to known patients.