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. The in-person workshop took place in December 2023
A report of the discussions will be produced and shared on our website.
Agenda - CF and Ageing workshop
The aim of the workshop was to understand more about some of the topics/challenges raised in the refreshed research priority “How do we manage an ageing population with CF?”.
Session 1 CF and ageing - is 80 the new 40?
9.30-10.00am Refreshments and Registration
10.00 – 11.00 Session 1 CF and ageing – is 80 the new 40?, Chair Dr Jamie Duckers
10.00-10.05 Welcome from Dr Lucy Allen, Director of Research and Healthcare Data, Cystic Fibrosis Trust
10.05 – 10.15 Introduction from Dr Jamie Duckers and Dr Freddy Frost
10.15 – 10.25 Getting older with CF, Jane Bull
10.25-10.40 Overview of CF, Professor Nick Simmonds
10.40-10.50 Funders perspective 1: Dr Katherine Tuggle, Cystic Fibrosis Foundation
10.50-11.00 Funders perspective 2: Dr Jess Boname, Medical Research Council
11.00 – 11.15 Coffee break and table introductions
Session 2 Biomedical basis of ageing
Chair - Professor Andy Jones
11.15-11.25 Effects of modulators on lung inflammation, Dr Rob Lord
11.25-11.35 Inflammation and ageing, Professor Robert Gray
11.35 – 11.45 Ageing in CF: a basic science perspective, Professor David Sheppard
11.45 – 12.00 Q&A with speakers
12.00 – 12.15 Coffee and round table discussions
Session 3 Complexities of an ageing population with CF
Chair Joanna Snowball
12.15 – 12.25 CF and diet, Joanna Snowball
12.25 – 12.35 Cancer and CF, Professor Daniel Peckham
12.35 – 12.45 Q&A with speakers
12.45 – 1.15 Round table discussions
1.15 - 2.15 Lunch
Session 4 Getting to the heart of it
Chair Dr Freddy Frost
2.15 – 2.25 Funder perspective 3: Dr Antoniya Kalphov, British Heart Foundation
2.25 – 2.35 Cardiovascular disease and CF, Professor Damian Downey
2.35 - 2.45 Integrated approaches to cardiovascular comorbidities: What can we learn from other diseases?, Professor Greg Lip
2.45 – 2.55 Imaging brain vascular function in patients with cystic fibrosis, Dr Kevin Murphy & Dr Hannah Chandler
2.55 – 3.10 Q&A with speakers
3.10 – 3.40 Panel discussion with session chairs
3.40 – 4.00 Round up and reflections
More information on the background to the refreshed CF research priorities and the context for this workshop are given below.
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.
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”.
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.