Canadian hereditary angioedema guideline

Position article and guidelines

Stephen Betschel^1*, Jacquie Badiou², Karen Binkley¹, Jacques Hébert³, Amin Kanani⁴,Paul Keith⁵, Gina Lacuesta⁶, Bill Yang⁷, Emel Aygören-Pürsün⁸, Jonathan Bernstein⁹,Konrad Bork¹⁰, Teresa Caballero¹¹, Marco Cicardi¹², Timothy Craig¹³, Henriette Farkas¹⁴,Hilary Longhurst¹⁵, Bruce Zuraw¹⁶, Henrik Boysen¹⁷, Rozita Borici-Mazi¹⁸, Tom Bowen¹⁹,Karen Dallas²⁰, John Dean²¹, Kelly Lang-Robertson¹, Benoît Laramée²², Eric Leith²³, Sean Mace¹, Christine McCusker²⁴, Bill Moote²⁵, Man-Chiu Poon²⁶, Bruce Ritchie²⁷, Donald Stark⁴, Gordon Sussman¹ and Susan Waserman⁵

• *Corresponding author: Stephen Betschel betschels@smh.ca

Author Affiliations

For all author emails, please log on.

Allergy, Asthma & Clinical Immunology 2014, 10:50  doi:10.1186/1710-1492-10-50

Published: 22 October 2014

Abstract

Hereditary angioedema (HAE) is a disease which is associated with random and often unpredictable attacks of painful swelling typically affecting the extremities, bowel mucosa, genitals, face and upper airway. Attacks are associated with significant functional impairment, decreased Health Related Quality of Life, and mortality in the case of laryngeal attacks. Caring for patients with HAE can be challenging due to the complexity of this disease. The care of patients with HAE in Canada is neither optimal nor uniform across the country. It lags behind other countries where there are more organized models for HAE management, and where additional therapeutic options are licensed and available for use. The objective of this guideline is to provide graded recommendations for the management of patients in Canada with HAE. This includes the treatment of attacks, short-term prophylaxis, long-term prophylaxis, and recommendations for self-administration, individualized therapy, quality of life, and comprehensive care. It is anticipated that by providing this guideline to caregivers, policy makers, patients and their advocates, that there will be an improved understanding of the current recommendations regarding management of HAE and the factors that need to be considered when choosing therapies and treatment plans for individual patients. The primary target users of this guideline are healthcare providers who are managing patients with HAE. Other healthcare providers who may use this guideline are emergency physicians, gastroenterologists, dentists and otolaryngologists, who will encounter patients with HAE and need to be aware of this condition. Hospital administrators, insurers and policy makers may also find this guideline helpful.

Keywords: 

Hereditary angioedema; Guideline; Recommendations; Acute attacks; Short-term prophylaxis; Long-term prophylaxis; Self-administration; Individualized therapy; Quality of life; Comprehensive care; GRADE

Viewing options

• Full text
• PDF(376KB)
• Additional files