Emergency Department Management of Hereditary Angioedema Attacks: Patient Perspectives

Iris M Otani; Sandra C Christiansen; Paula Busse; Carlos A Camargo Jr; Bruce L Zuraw; Marc A Riedl; Aleena Banerji

doi:10.1016/j.jaip.2016.06.029

Emergency Department Management of Hereditary Angioedema Attacks: Patient Perspectives

J Allergy Clin Immunol Pract. 2017 Jan-Feb;5(1):128-134.e4. doi: 10.1016/j.jaip.2016.06.029. Epub 2016 Sep 21.

Authors

Iris M Otani¹, Sandra C Christiansen², Paula Busse³, Carlos A Camargo Jr⁴, Bruce L Zuraw², Marc A Riedl², Aleena Banerji⁵

Affiliations

¹ Division of Rheumatology, Allergy, and Immunology, Department of Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, Mass. Electronic address: [email protected].
² Division of Rheumatology, Allergy, and Immunology, Department of Medicine, University of California San Diego, La Jolla, Calif.
³ Division of Clinical Immunology, Department of Medicine, Mount Sinai School of Medicine, New York, NY.
⁴ Division of Rheumatology, Allergy, and Immunology, Department of Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, Mass; Department of Emergency Medicine, Massachusetts General Hospital, Boston, Mass.
⁵ Division of Rheumatology, Allergy, and Immunology, Department of Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, Mass.

PMID: 27665386
DOI: 10.1016/j.jaip.2016.06.029

Abstract

Background: Emergency department (ED) management of hereditary angioedema (HAE) has been hindered by misdiagnosis and limited treatment options. Food and Drug Administration approval of 4 on-demand HAE therapies starting in 2009 and the publication of ED guidelines for angioedema management in 2014 should facilitate improvement of HAE management in the ED.

Objective: The objective of this study was to identify patient-reported areas for improvement in ED management of HAE attacks.

Methods: Patients with self-reported HAE with C1 inhibitor deficiency who attended the 2015 HAE Association Patient Summit were asked to complete an anonymous 30-question survey. Questions addressed patient characteristics and HAE management in the ED.

Results: Patients indicated that understanding of HAE in the ED needed improvement (99%, 104 of 105 patients). Recognition of HAE as a diagnosis (48%, 50 of 105 patients), appreciation of HAE as a serious disease (45%, 47 of 105 patients), and medication management (59%, 62 of 105 patients) were identified as areas needing improvement. Among 39 patients who required ED care within the last year, 6 did not receive any HAE-targeted therapy, and treatment with corticosteroids (n = 3), epinephrine (n = 2), and antihistamines (n = 7) was reported. Among 68 patients whose treatment plan was to receive home on-demand therapy, 26 required ED care because of an inability to receive on-demand therapy at home as outlined in their treatment plan. Having a treatment plan was associated with a greater likelihood of receiving HAE therapy in the ED (99% vs 74%, P = .002).

Conclusion: HAE management in the ED can be improved with a focus on recognition of HAE attacks and administration of effective HAE therapies.

Keywords: Angioedema guidelines; Angioedema management; Disease burden; Emergency department; Hereditary angioedema; Hereditary angioedema attacks; On-demand treatment; Patient-reported outcomes; Quality of life; Treatment plan.

MeSH terms

Adolescent
Angioedemas, Hereditary / diagnostic imaging*
Angioedemas, Hereditary / epidemiology*
Angioedemas, Hereditary / therapy
Child
Child, Preschool
Diagnostic Errors
Disease Management
Emergency Medical Services
Emergency Service, Hospital*
Female
Humans
Infant
Infant, Newborn
Male
Patient Satisfaction*
Practice Guidelines as Topic
Quality Assurance, Health Care
Quality Improvement*
Surveys and Questionnaires
United States
United States Food and Drug Administration