Latest news

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

Latest publications

CFTR modulator therapies have transformed the lives of many people with CF over recent years, specifically those with class II genetic variants (most commonly the F508del variant). These variants result in no meaningful salt transport at the cell membrane and in the lungs of people with CF reduce airway surface liquid volume leading to infection and progressive lung damage. CFTR modulators can correct this defect in people with at least one copy of the F508del variant. As research into new agents and their effectiveness with different genotypes has been accelerating and increasing in volume, we took the editorial decision to split our review of these therapies into separate monotherapy, dual therapy and triple therapy reviews. The review of monotherapies has now been published on the Cochrane Library (access here) and the other two reviews will follow later in the year.

People with CF may be considered for lung transplantation as their lung function declines. Infection with nontuberculous mycobacteria (NTM) is a poor prognostic factor in lung transplantation, but guidelines recommend NTM infection should not automatically preclude the surgery. You can read our evaluation of the evidence base for lung transplantation in people with CF who have NTM pulmonary disease here.

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.