Emergency Department Management of Hereditary Angioedema Attacks: Patient Perspectives.
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.



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



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.



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).



HAE management in the ED can be improved with a focus on recognition of HAE attacks and administration of effective HAE therapies.
Division of Rheumatology, Allergy, and Immunology, Department of Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, Mass. Electronic address: iris.otani@ucsf.edu.
Iris M
IM
Otani
Division of Rheumatology, Allergy, and Immunology, Department of Medicine, University of California San Diego, La Jolla, Calif.
Sandra C
SC
Christiansen
Division of Clinical Immunology, Department of Medicine, Mount Sinai School of Medicine, New York, NY.
Paula
P
Busse
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.
Carlos A
CA
Camargo
Division of Rheumatology, Allergy, and Immunology, Department of Medicine, University of California San Diego, La Jolla, Calif.
Bruce L
BL
Zuraw
Division of Rheumatology, Allergy, and Immunology, Department of Medicine, University of California San Diego, La Jolla, Calif.
Marc A
MA
Riedl
Division of Rheumatology, Allergy, and Immunology, Department of Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, Mass.
Aleena
A
Banerji
D000293:Adolescent; D054179:Angioedemas, Hereditary / Q000000981:diagnostic imaging* / Q000453:epidemiology* / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D003951:Diagnostic Errors; D019468:Disease Management; D004632:Emergency Medical Services; D004636:Emergency Service, Hospital; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D017060:Patient Satisfaction; D017410:Practice Guidelines as Topic; D011785:Quality Assurance, Health Care; D058996:Quality Improvement; D011795:Surveys and Questionnaires; D014481:United States; D014486:United States Food and Drug Administration
Angioedema guidelines; Angioedema management; Disease burden; Emergency department; Hereditary angioedema; Hereditary angioedema attacks; On-demand treatment; Patient-reported outcomes; Quality of life; Treatment plan