Division of Palliative Medicine, Department of Family and Preventive Medicine, Emory University, Atlanta, Georgia, USA.
Department of Psychiatry, Massachusetts General Hospital, Boston, Massachusetts, USA.
J Palliat Med. 2021 Jan;24(1):18-30. doi: 10.1089/jpm.2020.0311. Epub 2020 Sep 16.
Cystic fibrosis (CF) affects more than 70,000 individuals and their families worldwide. Although outcomes for individuals with CF continue to improve, it remains a life-limiting condition with no cure. Individuals with CF manage extensive symptom and treatment burdens and face complex medical decisions throughout the illness course. Although palliative care has been shown to reduce suffering by alleviating illness-related burdens for people with serious illness and their families, little is known regarding the components and structure of various delivery models of palliative care needed to improve outcomes for people affected by CF. The Cystic Fibrosis Foundation (CFF) assembled an expert panel of clinicians, researchers, individuals with CF, and family caregivers, to develop consensus recommendations for models of best practices for palliative care in CF. Eleven statements were developed based on a systematic literature review and expert opinion, and address primary palliative care, specialty palliative care, and screening for palliative needs. These recommendations are intended to comprehensively address palliative care needs and improve quality of life for individuals with CF at all stages of illness and development, and their caregivers.
囊性纤维化（CF）影响着全球超过 70000 个人及其家庭。尽管 CF 患者的预后仍在不断改善，但该病仍是一种致命性疾病，尚无治愈方法。CF 患者需要应对广泛的症状和治疗负担，并在整个病程中面临复杂的医疗决策。虽然姑息治疗已被证明可以通过减轻严重疾病患者及其家属的疾病相关负担来减轻痛苦，但对于改善 CF 患者预后所需的姑息治疗的各种提供模式的组成部分和结构知之甚少。囊性纤维化基金会（CFF）召集了一组临床医生、研究人员、CF 患者和家庭照顾者的专家小组，为 CF 中的姑息治疗最佳实践模式制定了共识建议。根据系统文献回顾和专家意见制定了 11 项声明，涉及主要姑息治疗、专业姑息治疗和姑息治疗需求筛查。这些建议旨在全面满足 CF 患者及其照顾者在疾病各个阶段的姑息治疗需求，并提高他们的生活质量。
