Key Highlights From ECFS 2023: JLA Top 10 Research Priorities

Historically, Cystic Fibrosis has been dominated by repeated cycles of infection and inflammation leading to irreparable structural lung disease.¹

During the last 2 years, 90% of people with CF in the UK have become eligible for treatment with CFTR modulators.² Patients taking these medicines have reported clinical benefits such as improved lung function and a reduction in exacerbations.^3,4

However, modulators are unlikely to repair the already structurally damaged lung, and their effects on the lung microbiome and their potential for reducing inflammation is not yet fully understood.^5,6

In 2022 the James Lind Alliance (JLA) Priority Setting Partnership (PSP) in Cystic Fibrosis published its refreshed list of research priorities.⁷

Priority areas identified in the refresh exercise included managing an aging CF population; managing the effects of CFTR modulators on systems outside the lung; long term safety of CF treatments including CFTR modulators: diagnosing infection in the absence of sputum as well as what the options are for patients unable to take modulators or for whom there are currently no novel therapies available.⁷

There are still many unknowns in this fast-changing CF landscape for both clinicians and patients, as such the need for research remains a high priority in this field.

In this presentation, Dr Freddy Frost summarise some of the data from ECFS 2023 aligned with the JLA Top 10 research priorities for CF.

References:

1. Elborn, J Stuart. Cystic fibrosis. The Lancet 2016; 388(10059): 2519–2531.

2. CF trust registry report 2021. Available at: https://www.cysticfibrosis.org.uk/sites/default/files/2022-10/CFT_2021-Annual-Data-Report-WEB.pdf

3. Middleton PG et al. N Engl J Med 2019; 381(19):1809–19.

4. Heijerman HGM et al. The Lancet 2019; 394(10212):1940–8.

5. Keone et al. Int. J. Mol. Sci. 2020, 21, 6379.

6. Harvey et al. Int. J. Mol. Sci. 2022, 23, 3513.

7. James Lind Alliance CF priorities. Available at: https://www.jla.nihr.ac.uk/priority-setting-partnerships/cystic-fibrosis-refresh/

We hope that you found the content valuable. To ensure that we continue to provide topics and content of interest, we would very much value your feedback by completing a short questionnaire.

• Dr Freddy Frost

  NIHR Academic Clinical Lecturer

  Read biography
  Dr Freddy Frost

  NIHR Academic Clinical Lecturer University of Liverpool and Liverpool Heart and Chest Hospital NHSFT

  Role and Location

  Dr Frost is an NIHR Academic Clinical Lecturer carrying out clinical work at Liverpool Heart & Chest Hospital, and research investigating the long-term health outcomes of people living with chronic lung infection at the University of Liverpool.

  Education

  BMedSci, BMBS, MRCP, MD

  Research

  Dr Frost’s research interests focus on aspects of complex lung infection, including the epidemiology of lung infection, the effects of antibiotics on the lung microbiome and cardiometabolic comorbidities.