遗传性血管性水肿(HAE)是一种与随机且通常无法预测的疼痛性肿胀发作有关的疾病,通常会影响四肢。肠粘膜,生殖器,面部和上呼吸道。攻击与严重的功能障碍有关,与健康相关的生活质量下降,和死亡率在喉部发作的情况下。由于这种疾病的复杂性,照顾患有HAE的患者可能是具有挑战性的。加拿大对HAE患者的护理既不是最佳的,也不是全国统一的。它落后于其他国家,那里有更多的HAE管理模式,和额外的治疗选择被许可和可供使用。本指南的目的是为加拿大HAE患者的管理提供分级建议。这包括治疗攻击,短期预防,长期预防,以及自我管理的建议,个体化治疗,生活质量,全面的护理。预计通过向护理人员提供这一指南,政策制定者,患者和他们的倡导者,我们将更好地理解当前关于HAE管理的建议,以及在为个体患者选择疗法和治疗计划时需要考虑的因素.本指南的主要目标用户是管理HAE患者的医疗保健提供者。其他可能使用本指南的医疗保健提供者是急诊医生,胃肠病学家,牙医和耳鼻喉科医生,谁会遇到HAE患者,需要意识到这种情况。医院管理员,保险公司和政策制定者也可能会发现这一指导方针很有帮助。
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.