The Disease Burden of Hereditary Angioedema: Insights from a Survey in French-Canadians from Quebec.
J Immunol Res
; 2024: 3028617, 2024.
Article
in En
| MEDLINE
| ID: mdl-38487730
ABSTRACT
Background:
Limited data are available on the clinical profile and disease burden of hereditary angioedema (HAE) in Canadians.Objective:
This study aimed to assess HAE disease characteristics and the burden of disease in Canadians with HAE types I, II, and normal levels of C1 inhibitor (nC1-INH). Materials andMethods:
A 46-item patient survey evaluating clinical characteristics and burden of disease was developed and disseminated by the HAE patient organization Angio-oédeme héréditaire du Québec in Quebec, Canada, from May 2019 to February 2020. The survey received Research Review Board ethics approval.Results:
In the 35 respondents, HAE type I was the most common (46%), followed by nC1-INH (43%). Female participants were significantly younger at first symptom presentation than males (p=0.04). Prior to diagnosis, 69% of participants underwent unnecessary treatments and procedures, with a 10-year delay between first symptoms and diagnosis. Before starting the current treatment, 42% of participants experienced weekly HAE attacks. Most participants identified experiencing attacks in the abdomen (89%), followed by the larynx (66%), feet (66%), hands (63%), and face (63%). Most attacks were severe or moderate, yet almost half of patients waited >1 hr before getting medical attention at their last emergency department (ED) visit. HAE was associated with decreased health-related quality of life, leading to significant functional impairment in personal and professional life. As compared to HAE type I/II, patients with HAE nC1-INH were treated more often with tranexamic acid for long-term prophylaxis, and their condition was less controlled, resulting in more attacks and ED visits.Conclusion:
HAE manifests in this patient population as frequent moderate-to-severe attacks and a high disease burden; the HAE subtype may differentially affect care requirements. There is an urgent need for increased awareness and education on HAE among treating physicians.
Full text:
1
Database:
MEDLINE
Main subject:
Angioedemas, Hereditary
/
North American People
Limits:
Female
/
Humans
/
Male
Country/Region as subject:
America do norte
Language:
En
Journal:
J Immunol Res
Year:
2024
Type:
Article
Affiliation country:
Canada