Latest news

We welcome the news that a deal has been reached between the National Health Service in England and Vertex to provide people with CF and relevant genotypes with continued access to modulators, the drugs that are crucial for treating the condition. Similar deals are expected to be announced soon for the devolved nations (Scotland, Wales and Northern Ireland). You can read our 2023 review on the use of these drugs which can correct the most common genetic defect that causes CF here.

Latest podcasts

Macrolide antibiotics (including azithromycin) for cystic fibrosis - this 2024 update included 14 studies, all of which assessed oral azithromycin; 11 studies compared the drug to placebo, while single studies compared high and low dosing, weekly versus daily dosing and oral versus inhaled administration. Evidence showed that oral azithromycin can improve lung function at six months compared to a placebo and may reduce the risk of an exacerbation. There is no clear evidence as to whether high dosing is better than low dosing or whether inhaled azithromycin is better than oral for any of the review's outcomes. While weekly dosing probably leads to less of an improvement in lung function than daily dosing, there is probably a longer time to an exacerbation in the weekly group. Current evidence does not support longer‐term use of azithromycin for all people with CF. Read further details here and listen to the podcast here.*

Nebuliser systems for drug delivery in cystic fibrosis - the 2023 updated review included 13 new studies, but despite this, the reviews's main conclusions have not changed. Authors found insufficient data to establish whether one nebuliser system is better than another overall. There is not evidence for all systems and all types of medication used in CF care, some studies did show that the newer technologies e.g. AAD and VMT have some advantages over conventional systems. The variability in the performance of different nebuliser systems and their compatibility with specific nebulised medication must be considered when choosing the best nebuliser system for each case. Read more details here and listen to the podcast here*.

Antibiotic strategies for eradicating Pseudomonas aeruginosa in people with cystic fibrosis - at the 2023 update four newly included studies the review's conclusions. Evidence shows that nebulised antibiotics, alone or in combination with oral antibiotics, were better than no treatment for early infection with Pseudomonas aeruginosa and eradication may be sustained for up to two years. However, there is still insufficient evidence as to whether any antibiotic strategies decrease mortality or morbidity, improve quality of life, or are linked to adverse effects compared to placebo or standard treatment. The review found no differences in rates of eradication of P aeruginosa between active treatments, but did find evidence that intravenous antibiotic treatment is not superior to oral antibiotics. Read more details here and listen to the podcast here*.

*Podcasts are available directly from Cochrane as well as other platforms such as Apple and Spotify.

Funding news

We have been successful in obtaining funding to the end of March 2025 to be able to continue with a programme of CF reviews and evidence syntheses to support guidelines. We would like to document our gratitude to the CF Foundation and the UK CF Trust for awarding us this grant. More details here.

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.