Sullivan Lauren J, Mingora Christina M, Flume Patrick A
Medical University of South Carolina, 96 Jonathan Lucas Street, Room 816-CSB, Charleston, SC, 29425, USA.
Drugs Aging. 2025 Apr 24. doi: 10.1007/s40266-025-01207-3.
Cystic fibrosis (CF) is an inherited condition that leads to multiorgan dysfunction, especially in the respiratory, gastrointestinal, and reproductive tracts, with associated conditions including persistent pulmonary infection, liver disease, pancreatic insufficiency, and infertility. Historically, people with CF (pwCF) suffered a shortened lifespan due to complications of the condition, namely respiratory. The emphasis on center-based, multidisciplinary care and the widespread introduction of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy has resulted in pwCF living longer and healthier lives. Now they may encounter some of the health and social issues associated with growing older, which previously were not a typical experience for this population. In this article, we review relevant health issues for the aging CF population, including complications that arise from the condition itself, issues encountered due to treatment, and general conditions associated with aging that may manifest earlier or differently in pwCF. We discuss the recommendations for screening and treatment of relevant conditions, and considerations for the integration of healthcare professionals across disciplines into the care of this population.
囊性纤维化(CF)是一种遗传性疾病,可导致多器官功能障碍,尤其是在呼吸道、胃肠道和生殖道,相关病症包括持续性肺部感染、肝脏疾病、胰腺功能不全和不孕不育。从历史上看,囊性纤维化患者(pwCF)由于该病症的并发症,即呼吸系统并发症,寿命缩短。对以中心为基础的多学科护理的重视以及囊性纤维化跨膜传导调节因子(CFTR)调节剂疗法的广泛应用,使得pwCF能够活得更长、更健康。现在,他们可能会遇到一些与衰老相关的健康和社会问题,而这些问题以前在这一人群中并不常见。在本文中,我们回顾了老年CF人群的相关健康问题,包括该病症本身引发的并发症、治疗过程中遇到的问题,以及与衰老相关的一般病症,这些病症在pwCF中可能会更早出现或表现不同。我们讨论了相关病症的筛查和治疗建议,以及跨学科医疗专业人员融入该人群护理工作的注意事项。