Department of Women's and Children's Health, University of Liverpool, Liverpool, UK.
All Wales Adult Cystic Fibrosis Centre, University Hospital Llandough, Cardiff and Vale University Health Board, Cardiff, UK.
J Cyst Fibros. 2024 Jan;23(1):12-28. doi: 10.1016/j.jcf.2023.12.002. Epub 2023 Dec 21.
This is the second in a series of four papers updating the European Cystic Fibrosis Society (ECFS) standards for the care of people with CF. This paper focuses on establishing and maintaining health. The guidance is produced using an evidence-based framework and with wide stakeholder engagement, including people from the CF community. Authors provided a narrative description of their topic and statements, which were more directive. These statements were reviewed by a Delphi exercise, achieving good levels of agreement from a wide group for all statements. This guidance reinforces the importance of a multi-disciplinary CF team, but also describes developing models of care including virtual consultations. The framework for health is reinforced, including the need for a physically active lifestyle and the strict avoidance of all recreational inhalations, including e-cigarettes. Progress with cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy is reviewed, including emerging adverse events and advice for dose reduction and interruption. This paper contains guidance that is pertinent to all people with CF regardless of age and eligibility for and access to modulator therapy.
这是更新欧洲囊性纤维化学会（ECFS）囊性纤维化护理标准的系列论文中的第二篇。本文侧重于建立和维持健康。本指南是使用循证框架并广泛征求利益相关者意见制定的，包括囊性纤维化社区的人员。作者对其主题和陈述进行了叙述性描述，这些陈述更具指令性。这些陈述通过德尔菲法进行了审查，所有陈述都得到了广泛群体的高度一致。本指南强调了多学科囊性纤维化团队的重要性，但也描述了正在开发的护理模式，包括虚拟咨询。健康框架得到了加强，包括需要积极的生活方式和严格避免所有娱乐性吸入，包括电子烟。对囊性纤维化跨膜电导调节剂（CFTR）调节剂治疗的进展进行了审查，包括新出现的不良反应以及关于减少和中断剂量的建议。本文中的指导意见适用于所有囊性纤维化患者，无论其年龄以及是否有资格获得和使用调节剂治疗。
