title: Canadian hereditary angioedema guideline.
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.
authors.affiliation: University of Toronto, Toronto, Ontario Canada.
authors.forename: Stephen
authors.initials: S
authors.lastname: Betschel
authors.affiliation: HAE Canada, Notre Dame des Lourdes, Manitoba, Canada.
authors.forename: Jacquie
authors.initials: J
authors.lastname: Badiou
authors.affiliation: University of Toronto, Toronto, Ontario Canada.
authors.forename: Karen
authors.initials: K
authors.lastname: Binkley
authors.affiliation: Department of Medicine, Laval University, Quebec City, Quebec Canada.
authors.forename: Jacques
authors.initials: J
authors.lastname: Hébert
authors.affiliation: Department of Medicine, University of British Columbia, Vancouver, British Columbia Canada.
authors.forename: Amin
authors.initials: A
authors.lastname: Kanani
authors.affiliation: Department of Medicine, McMaster University, Hamilton, Ontario Canada.
authors.forename: Paul
authors.initials: P
authors.lastname: Keith
authors.affiliation: Department of Medicine, Dalhousie University, Halifax, Nova Scotia Canada.
authors.forename: Gina
authors.initials: G
authors.lastname: Lacuesta
authors.affiliation: University of Ottawa Medical School, Ottawa, Ontario Canada.
authors.forename: Bill
authors.initials: B
authors.lastname: Yang
authors.affiliation: Goethe-Universität Frankfurt am Main, Frankfurt am Main, Germany.
authors.forename: Emel
authors.initials: E
authors.lastname: Aygören-Pürsün
authors.affiliation: Department of Internal Medicine, University of Cincinnati, Cincinnati, Ohio USA.
authors.forename: Jonathan
authors.initials: J
authors.lastname: Bernstein
authors.affiliation: Department of Dermatology, University Hospital of the Johannes Gutenberg-University of Mainz, Mainz, Germany.
authors.forename: Konrad
authors.initials: K
authors.lastname: Bork
authors.affiliation: Hospital La Paz Health Research Institute, Madrid, Spain.
authors.forename: Teresa
authors.initials: T
authors.lastname: Caballero
authors.affiliation: Department of Internal Medicine, UniversitadegliStudi di Milano, Ospedale L. Sacco, Milan, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Cicardi
authors.affiliation: Departments of Medicine and Pediatrics, Penn State University, Hershey, Pennsylvania USA.
authors.forename: Timothy
authors.initials: T
authors.lastname: Craig
authors.affiliation: 3rd Department of Internal Medicine, Faculty of Medicine, Semmelweis University, Budapest, Hungary.
authors.forename: Henriette
authors.initials: H
authors.lastname: Farkas
authors.affiliation: Department of Immunology, Barts and the London NHS Trust, London, England, UK.
authors.forename: Hilary
authors.initials: H
authors.lastname: Longhurst
authors.affiliation: University of California, San Diego, San Diego, California USA.
authors.forename: Bruce
authors.initials: B
authors.lastname: Zuraw
authors.affiliation: HAE International, Skanderborg, Denmark.
authors.forename: Henrik
authors.initials: H
authors.lastname: Boysen
authors.affiliation: Department of Medicine, Queen's University, Kingston, Ontario Canada.
authors.forename: Rozita
authors.initials: R
authors.lastname: Borici-Mazi
authors.affiliation: Departments of Medicine and Paediatrics, University of Calgary, Calgary, Alberta Canada.
authors.forename: Tom
authors.initials: T
authors.lastname: Bowen
authors.affiliation: Saskatoon Health Region, Saskatoon, Saskatchewan Canada.
authors.forename: Karen
authors.initials: K
authors.lastname: Dallas
authors.affiliation: BC Children's Hospital, Vancouver, British Columbia Canada.
authors.forename: John
authors.initials: J
authors.lastname: Dean
authors.affiliation: University of Toronto, Toronto, Ontario Canada.
authors.forename: Kelly
authors.initials: K
authors.lastname: Lang-Robertson
authors.affiliation: Centre hospitalier de l'université de Montréal, Montréal, Quebec Canada.
authors.forename: Benoît
authors.initials: B
authors.lastname: Laramée
authors.affiliation: Department of Medicine, University of Toronto, Oakville, Ontario Canada.
authors.forename: Eric
authors.initials: E
authors.lastname: Leith
authors.affiliation: University of Toronto, Toronto, Ontario Canada.
authors.forename: Sean
authors.initials: S
authors.lastname: Mace
authors.affiliation: Department of Immunology, McGill University Health Centre, Montreal, Quebec Canada.
authors.forename: Christine
authors.initials: C
authors.lastname: McCusker
authors.affiliation: Department of Medicine, Western University, London, Ontario Canada.
authors.forename: Bill
authors.initials: B
authors.lastname: Moote
authors.affiliation: Southern Alberta Rare Blood and Bleeding Disorders Comprehensive Care Program, Calgary, Alberta Canada.
authors.forename: Man-Chiu
authors.initials: MC
authors.lastname: Poon
authors.affiliation: Departments of Medicine and Medical Oncology, University of Alberta, Edmonton, Alberta Canada.
authors.forename: Bruce
authors.initials: B
authors.lastname: Ritchie
authors.affiliation: Department of Medicine, University of British Columbia, Vancouver, British Columbia Canada.
authors.forename: Donald
authors.initials: D
authors.lastname: Stark
authors.affiliation: University of Toronto, Toronto, Ontario Canada.
authors.forename: Gordon
authors.initials: G
authors.lastname: Sussman
authors.affiliation: Department of Medicine, McMaster University, Hamilton, Ontario Canada.
authors.forename: Susan
authors.initials: S
authors.lastname: Waserman
keywords: Acute attacks; Comprehensive care; GRADE; Guideline; Hereditary angioedema; Individualized therapy; Long-term prophylaxis; Quality of life; Recommendations; Self-administration; Short-term prophylaxis