Freddy J Frost, Daniel G Peckham, Imogen C Felton, Joanna E Snowball, Robert D Gray, Andrew M Jones, Nicholas J Simmonds, Robert W Lord, Gregory Y H Lip, Hannah Chandler, Kevin Murphy, Damian G Downey, David N Sheppard, Jane C Davies, Jane Bull, Paula Sommer, Belinda Cupid, Lucy Allen, Jamie Duckers
{"title":"管理老龄化囊性纤维化人群:挑战和优先事项。","authors":"Freddy J Frost, Daniel G Peckham, Imogen C Felton, Joanna E Snowball, Robert D Gray, Andrew M Jones, Nicholas J Simmonds, Robert W Lord, Gregory Y H Lip, Hannah Chandler, Kevin Murphy, Damian G Downey, David N Sheppard, Jane C Davies, Jane Bull, Paula Sommer, Belinda Cupid, Lucy Allen, Jamie Duckers","doi":"10.1183/16000617.0261-2024","DOIUrl":null,"url":null,"abstract":"<p><p>The increasing life expectancy of people with cystic fibrosis (pwCF), largely driven by advancements in early diagnosis, multidisciplinary care and the recent introduction of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies, is likely to herald a shift in the focus of care toward managing the complexities of ageing. This review highlights key challenges and research priorities for addressing the health needs of an ageing CF population. A growing body of evidence underscores the heightened risks of cancers, cardiovascular diseases and changing nutritional and metabolic profiles as pwCF age. CFTR modulators have improved clinical outcomes, but their effects on inflammation, immunity and long-term disease trajectories remain incompletely understood. Nutritional management, particularly the implications of obesity and body composition, poses new challenges, as does the potential accelerated ageing of immune and pulmonary systems in CF. Emerging issues such as menopause in females with CF, lifetime antimicrobial resistance and the interplay between chronic inflammation and ageing further complicate the care landscape. The review emphasises the urgent need for multidisciplinary research programmes that integrate clinical, patient and community perspectives. Leveraging established CF registries, clinical trial networks and collaborations with ageing research frameworks is critical to addressing these challenges. Ultimately, the goal is to ensure that pwCF not only live longer but also experience improved quality of life and holistic wellbeing as they realise the full benefits of therapeutic advances.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"34 176","pages":""},"PeriodicalIF":9.0000,"publicationDate":"2025-05-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12076158/pdf/","citationCount":"0","resultStr":"{\"title\":\"Managing an ageing cystic fibrosis population: challenges and priorities.\",\"authors\":\"Freddy J Frost, Daniel G Peckham, Imogen C Felton, Joanna E Snowball, Robert D Gray, Andrew M Jones, Nicholas J Simmonds, Robert W Lord, Gregory Y H Lip, Hannah Chandler, Kevin Murphy, Damian G Downey, David N Sheppard, Jane C Davies, Jane Bull, Paula Sommer, Belinda Cupid, Lucy Allen, Jamie Duckers\",\"doi\":\"10.1183/16000617.0261-2024\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>The increasing life expectancy of people with cystic fibrosis (pwCF), largely driven by advancements in early diagnosis, multidisciplinary care and the recent introduction of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies, is likely to herald a shift in the focus of care toward managing the complexities of ageing. This review highlights key challenges and research priorities for addressing the health needs of an ageing CF population. A growing body of evidence underscores the heightened risks of cancers, cardiovascular diseases and changing nutritional and metabolic profiles as pwCF age. CFTR modulators have improved clinical outcomes, but their effects on inflammation, immunity and long-term disease trajectories remain incompletely understood. Nutritional management, particularly the implications of obesity and body composition, poses new challenges, as does the potential accelerated ageing of immune and pulmonary systems in CF. Emerging issues such as menopause in females with CF, lifetime antimicrobial resistance and the interplay between chronic inflammation and ageing further complicate the care landscape. The review emphasises the urgent need for multidisciplinary research programmes that integrate clinical, patient and community perspectives. Leveraging established CF registries, clinical trial networks and collaborations with ageing research frameworks is critical to addressing these challenges. Ultimately, the goal is to ensure that pwCF not only live longer but also experience improved quality of life and holistic wellbeing as they realise the full benefits of therapeutic advances.</p>\",\"PeriodicalId\":12166,\"journal\":{\"name\":\"European Respiratory Review\",\"volume\":\"34 176\",\"pages\":\"\"},\"PeriodicalIF\":9.0000,\"publicationDate\":\"2025-05-14\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12076158/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"European Respiratory Review\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1183/16000617.0261-2024\",\"RegionNum\":1,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2025/4/1 0:00:00\",\"PubModel\":\"Print\",\"JCR\":\"Q1\",\"JCRName\":\"RESPIRATORY SYSTEM\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"European Respiratory Review","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1183/16000617.0261-2024","RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2025/4/1 0:00:00","PubModel":"Print","JCR":"Q1","JCRName":"RESPIRATORY SYSTEM","Score":null,"Total":0}
Managing an ageing cystic fibrosis population: challenges and priorities.
The increasing life expectancy of people with cystic fibrosis (pwCF), largely driven by advancements in early diagnosis, multidisciplinary care and the recent introduction of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies, is likely to herald a shift in the focus of care toward managing the complexities of ageing. This review highlights key challenges and research priorities for addressing the health needs of an ageing CF population. A growing body of evidence underscores the heightened risks of cancers, cardiovascular diseases and changing nutritional and metabolic profiles as pwCF age. CFTR modulators have improved clinical outcomes, but their effects on inflammation, immunity and long-term disease trajectories remain incompletely understood. Nutritional management, particularly the implications of obesity and body composition, poses new challenges, as does the potential accelerated ageing of immune and pulmonary systems in CF. Emerging issues such as menopause in females with CF, lifetime antimicrobial resistance and the interplay between chronic inflammation and ageing further complicate the care landscape. The review emphasises the urgent need for multidisciplinary research programmes that integrate clinical, patient and community perspectives. Leveraging established CF registries, clinical trial networks and collaborations with ageing research frameworks is critical to addressing these challenges. Ultimately, the goal is to ensure that pwCF not only live longer but also experience improved quality of life and holistic wellbeing as they realise the full benefits of therapeutic advances.
期刊介绍:
The European Respiratory Review (ERR) is an open-access journal published by the European Respiratory Society (ERS), serving as a vital resource for respiratory professionals by delivering updates on medicine, science, and surgery in the field. ERR features state-of-the-art review articles, editorials, correspondence, and summaries of recent research findings and studies covering a wide range of topics including COPD, asthma, pulmonary hypertension, interstitial lung disease, lung cancer, tuberculosis, and pulmonary infections. Articles are published continuously and compiled into quarterly issues within a single annual volume.