Latest news

Dr Andrew Prayle, one of our editors, has been developing evidence maps. These visual tools demonstrate which studies and papers contribute to specific outcomes in a review. He has developed maps for several of our recently published reviews which to support guidelines currently being updated by the UK CF Trust and the CF Foundation. You can access the current evidence maps and more will be added soon.

Latest publications

Over recent years, CFTR modulator therapies have transformed the lives of many people with CF, specifically those with class II genetic variants (most commonly the F508del variant). In people with these variants, there is no meaningful salt transport at the cell membrane. This means that airway surface liquid volume in the lungs is reduced, leading to infection and progressive lung damage. CFTR modulators can correct this defect in people who have at least one copy of the F508del variant. Research into new agents and their effectiveness for people with different genotypes has been accelerating and increasing in volume, so we took the editorial decision to split our combined review of these therapies into separate monotherapy, dual therapy and triple therapy reviews. The monotherapy review has now been published on the Cochrane Library and the other two reviews will follow later in 2026.

Another new review considers the evidence base for lung transplantation in people with CF who have nontuberculous mycobacteria (NTM) pulmonary disease. People with CF may be considered for lung transplantation as their lung function declines. While infection with NTM may offer a poor prognosis for in lung transplantation, guidelines recommend NTM infection should not automatically preclude the surgery.

Funding news

We have been successful in obtaining further funding to the end of March 2027 from the CF Foundation to continue producing systematic reviews and supporting guideline development. We will also be working with Queen's University Belfast and the UK CF Trust to support colleagues producing a series of systematic reviews of interventions for the diagnosis and treatment of infection in children with CF and vulnerable newborn infants .

Learn about our contributions to the "Standards of care for CFTR variant-specific therapy for people with CF" here.

Learn about our collaboration with the JLA here.