Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Respiratory Unit; Respiratory Unit and Adult Cystic Fibrosis Center, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milano, Italy.
All Wales Adult Cystic Fibrosis Centre, University Hospital Llandough, Cardiff and Vale University Health Board, Cardiff, UK.
J Cyst Fibros. 2024 May;23(3):375-387. doi: 10.1016/j.jcf.2024.05.007. Epub 2024 May 24.
This is the final of four papers updating standards for the care of people with CF. That this paper "Planning a longer life" was considered necessary, highlights how much CF care has progressed over the past decade. Several factors underpin this progress, notably increased numbers of people with CF with access to CFTR modulator therapy. As the landscape for CF changes, so do the hopes and aspirations of people with CF and their families. This paper reflects the need to consider people with CF not as a "problem" to be solved, but as a success, a potential and a voice to be heard. People with CF and the wider CF community have driven this approach, reflecting many of the topics in this paper. This exercise involved wide stakeholder engagement. People with CF are keen to contribute to research priorities and be involved in all stages of research. People with CF want healthcare professionals to respect them as individuals and consider the impact of our actions on the world around us. Navigating life presents challenges to all, but for people with CF these challenges are heightened and complex. In this paper we highlight the concerns and life moments that impact people with CF, and events that the CF team should aim to support, including the challenges around having a family. People with CF and their care teams must embrace the updated standards outlined in these four papers to enjoy the full potential for a healthier life.
这是更新 CF 患者护理标准的四篇论文中的最后一篇。之所以认为有必要编写这篇题为“规划更长久的生命”的论文,突显了 CF 护理在过去十年中取得了多大的进展。有几个因素为这一进展提供了支撑,其中显著的是有更多 CF 患者可以获得 CFTR 调节剂治疗。随着 CF 领域的发展,CF 患者及其家属的希望和愿望也在发生变化。本文反映了需要将 CF 患者视为一种成功、一种潜力和一个需要被听到的声音,而不是一个有待解决的“问题”。CF 患者及其更广泛的 CF 社区推动了这种方法,反映了本文中的许多主题。这项工作涉及广泛的利益相关者参与。CF 患者渴望为研究重点做出贡献,并参与研究的所有阶段。CF 患者希望医疗保健专业人员尊重他们作为个体的身份,并考虑我们的行动对周围世界的影响。对所有人来说,生活都充满了挑战,但对 CF 患者来说,这些挑战更加严峻和复杂。在本文中,我们强调了影响 CF 患者的关注点和生活时刻,以及 CF 团队应该努力支持的事件,包括组建家庭所面临的挑战。CF 患者及其护理团队必须接受这四篇论文中概述的更新标准,才能充分发挥更健康生活的潜力。