Observational studyJournal of cystic fibrosis : official journal of the European Cystic Fibrosis Society2026
Elexacaftor/tezacaftor/ivacaftor is associated with long-term reduction in use of chronic respiratory therapies in cystic fibrosis.
Observational study in Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
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Who cites it
4 citing papers in PubMed.
- The Impact of Cystic Fibrosis-Related Diabetes on Barriers to Self-Management.Pediatric pulmonology · 2026Article
- Real-World Effectiveness of Elexacaftor/Tezacaftor/Ivacaftor in Cystic Fibrosis: A 24-Month Italian National Registry Study.Journal of clinical medicine · 2026Article
- Long-term reductions in inflammation in people with cystic fibrosis treated with elexacaftor/tezacaftor/ivacaftor.Annals of the American Thoracic Society · 2026Observational
- Modulation of immune responses by elexacaftor/tezacaftor/ivacaftor therapy in cystic fibrosis: data from a compassionate use program.Respiratory research · 2026Article
Corrections and comments
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Authors and funding
8 authors.
Funding
Abstract
backgroundLimited data exist on the real-world impact of elexacaftor/tezacaftor/ivacaftor (ETI) on use of chronic respiratory therapies. This post-hoc analysis evaluated long-term changes in use of chronic respiratory therapies following ETI initiation among people with (pw)CF aged ≥6 years in the United States and examined characteristics of those who were taking ≥2 chronic therapies at baseline and post-ETI.
methodsPROMISE is a prospective, multicenter, observational study. Self-reported use of inhaled antibiotics (cycled or continuous), hypertonic saline, dornase alfa, and oral azithromycin were captured before and through 54 months post-ETI initiation in 479 participants ≥12 years and through 36 months post-ETI in 124 participants aged 6-11 years.
resultsETI was associated with reductions in use of four chronic respiratory therapies through 54 months in participants ≥12 years (mean number of therapies decreasing from 2.6 [baseline] to 1.4 [54 months post-ETI]) and 36 months post-ETI in participants aged 6-11 years (mean number of therapies decreasing from 1.9 [baseline] to 1.3 [36 months post-ETI]). Consistent downward trends in use of all four therapies were observed among subgroups based on baseline age strata, sex at birth, prior CFTR modulator use, and ppFEV
conclusionsETI was associated with long-term reductions in use of chronic respiratory therapies (inhaled antibiotics, hypertonic saline, dornase alfa, azithromycin) among pwCF ≥6 years, indicating a sustained decrease in respiratory treatment burden among those on ETI therapy.
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