Lisa Fu1, Amin Kanani2, Gina Lacuesta3, Susan Waserman4, Stephen Betschel5. 1. Department of Medicine, Division of Clinical Immunology and Allergy, University of Toronto, Toronto, Ontario, Canada. Electronic address: lisa.wy.fu@gmail.com. 2. Division of Allergy and Immunology, Department of Medicine, University of British Columbia, Vancouver, British Columbia, Canada. 3. Department of Medicine, Dalhousie University, Halifax, Nova Scotia, Canada. 4. Division of Clinical Immunology and Allergy, McMaster University, Hamilton, Ontario, Canada. 5. Department of Medicine, Division of Clinical Immunology and Allergy, University of Toronto, Toronto, Ontario, Canada.
Abstract
BACKGROUND: Hereditary angioedema (HAE) is a rare disease that has significant morbidity and may be potentially fatal because of airway obstruction. OBJECTIVE: To determine practice patterns in physicians treating HAE. METHODS: A survey was designed to determine HAE practice patterns among Canadian physicians. These physicians were identified by sending the survey to members of 3 physician organizations (Canadian Hereditary Angioedema Network, Canadian Society of Clinical Immunology and Allergy, and Canadian Hematology Society). RESULTS: Thirty-six physicians responded to the survey. Thirty-four physicians were included in the analysis. Most referrals to HAE-treating physicians were from family and emergency department physicians. The most common sites of swelling reported by patients to physicians were facial, peripheral, and abdominal. A mean of 53.9% of patients with type 1 and 2 HAE and 53.4% of patients with HAE with normal C1 esterase inhibitor were undergoing long-term prophylaxis. A mean of 41.9%, 19.4%, and 93.5% of respondents had some patients taking danazol, tranexamic acid, and C1-esterase inhibitor, respectively. Most physicians believed that severity and frequency of attacks were the most important determinants in deciding when to use prophylaxis. A mean of 88.2% of physicians used C1-esterase inhibitor to treat acute attacks and 79.4% used icatibant. All respondents were aware of HAE guidelines. CONCLUSION: Physicians are using guidelines to support their practice and using agents suggested by guidelines with confidence. C1 inhibitor is being used widely for prophylaxis and treatment of acute attacks along with icatibant. However, certain special patient populations may require additional focus in future guidelines.
BACKGROUND:Hereditary angioedema (HAE) is a rare disease that has significant morbidity and may be potentially fatal because of airway obstruction. OBJECTIVE: To determine practice patterns in physicians treating HAE. METHODS: A survey was designed to determine HAE practice patterns among Canadian physicians. These physicians were identified by sending the survey to members of 3 physician organizations (Canadian Hereditary Angioedema Network, Canadian Society of Clinical Immunology and Allergy, and Canadian Hematology Society). RESULTS: Thirty-six physicians responded to the survey. Thirty-four physicians were included in the analysis. Most referrals to HAE-treating physicians were from family and emergency department physicians. The most common sites of swelling reported by patients to physicians were facial, peripheral, and abdominal. A mean of 53.9% of patients with type 1 and 2 HAE and 53.4% of patients with HAE with normal C1 esterase inhibitor were undergoing long-term prophylaxis. A mean of 41.9%, 19.4%, and 93.5% of respondents had some patients taking danazol, tranexamic acid, and C1-esterase inhibitor, respectively. Most physicians believed that severity and frequency of attacks were the most important determinants in deciding when to use prophylaxis. A mean of 88.2% of physicians used C1-esterase inhibitor to treat acute attacks and 79.4% used icatibant. All respondents were aware of HAE guidelines. CONCLUSION: Physicians are using guidelines to support their practice and using agents suggested by guidelines with confidence. C1 inhibitor is being used widely for prophylaxis and treatment of acute attacks along with icatibant. However, certain special patient populations may require additional focus in future guidelines.
Authors: Katarzyna Piotrowicz-Wójcik; Małgorzata Bulanda; Aldona Juchacz; Joanna Jamróz-Brzeska; Jacek Gocki; Krzysztof Kuziemski; Robert Pawłowicz; Grzegorz Porebski Journal: J Clin Med Date: 2021-11-29 Impact factor: 4.241