CF and ageing research workshop
Mapping of current research against the refreshed top 10 CF research priorities, identified that there were several priorities where there were few ongoing studies. One of these was the question “How do we manage an ageing population with cystic fibrosis”.
Cystic Fibrosis Trust held a workshop to explore this topic December 2023. A report summarizing the workshop has now been published.
More information on the background to the refreshed CF research priorities and the context for this workshop are given below.
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Background to refreshed CF research priorities
In 2017 a James Lind Alliance Priority Setting Partnership (PSP) was conducted to identify research priorities in cystic fibrosis. It was a partnership between Cystic Fibrosis Trust, researchers at the University of Nottingham and people with CF and their families. For funders and researchers alike, this list of priorities became one of the ‘guiding principles’ in deciding which areas of CF to focus on. Cystic Fibrosis Trust alone has provided over £10 million of research funding to address these priorities, leveraging an additional £13 million of co-funding from other research funders.
While considerable progress has been made in addressing many of the health priorities identified by the JLA PSP in CF there is work still to be done. Additionally, recent years have seen some significant changes in CF, such as access to the CFTR modulator medicines such as Kaftrio; and changes in the provision of care and delivery of research, due to the COVID pandemic. In light of these changes, last year a refresh of the CF research priorities was conducted. In November 2022 a set of top 10 refreshed CF research priorities were announced.
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Research priorities are ‘unanswered’ research questions
Over 900 questions about CF were submitted as part of the project to refresh the CF research priorities. Similar topics were combined into ‘umbrella’ questions, and checks were done to ensure that these questions were unanswered by research. Where a systematic review of research topic was available (ie a summary of completed research studies), the question was considered answered. See Rowbotham et al 2023 and Question Verification form on James Lind Alliance website for more information.
After they were announced, further analysis was conducted to match ongoing research studies against the refreshed top 10 CF research priorities. Cystic Fibrosis Trust research portfolio, UK CF Registry Research requests, NIHR and UKRI grant databases were used alongside information from other researcher funders within CF Europe and information from individual CF researchers. This analysis showed that there were few ongoing research studies addressing the research priority “How do we manage an ageing population with cystic fibrosis”.
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Knowledge so far about CF and ageing
There is little information in the scientific literature about ageing and CF. Recent reviews ( for example Ticona et al 2023 & Spencer Poore et al 2022 ) discuss higher than the general, age-matched population levels of cardiovascular disease, pulmonary hypertension, liver disease, cancer and CF diabetes. It is unclear whether these effects are a primary cause of CFTR dysfunction or whether they are secondary causes.
While it will be important to consider CF diabetes in the context of ageing in CF, discussions on CF diabetes will be planned for a later, separate research-priority workshop.