ArticleChest2026
Effectiveness of Cystic Fibrosis Transmembrane Conductance Regulator Modulator Therapy on Risk of Death for Individuals With Cystic Fibrosis.
Article in Chest, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
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
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
1 citing paper in PubMed.
- Cystic Fibrosis Mortality Trends 1999-2024-A CDC Wonder Study.Advances in respiratory medicine · 2026Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
4 authors.
Funding
Abstract
backgroundTo date, clinical trials of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies have focused on outcomes that can be captured in relatively short follow-up periods. The effectiveness of CFTR modulator therapies on survival has not been described fully. RESEARCH QUESTION: What is the population-level treatment effect of initiating any CFTR modulator therapy compared with no initiation on the risk of death in people with cystic fibrosis (CF)? STUDY DESIGN AND
methodsWe conducted a retrospective cohort study using United States Cystic Fibrosis Foundation Patient Registry data from between 2012 and 2022. We included individuals who met eligibility criteria for a CFTR modulator prescription (based on age and genotype of 2020 regulatory approvals and no prior use). At every clinic or telehealth visit, we assessed eligibility and classified treatment status based on registry records. We included individuals in the analysis multiple times if eligibility was met at multiple visits. We followed individuals from each visit until death (outcome), lung transplantation, loss to follow-up, or administrative censoring. We censored untreated observations if they later initiated CFTR modulator therapy. We estimated 8-year risk curves using Kaplan-Meier and hazard ratios (HRs) from Cox regression models. We used inverse probability weighting to balance baseline covariates and to account for nonrandom censoring.
resultsWe included 25,103 individuals and 178,835 visits; 18,056 individuals initiated therapy. The 8-year risk difference of death resulting from initiating CFTR modulator therapy was -7.2% (95% CI, -9.6 to -4.9). The hazard for death was 66% lower after CFTR modulator initiation than without use (HR, 0.34; 95% CI, 0.28 to 0.41).
interpretationOur results show that CFTR modulator therapies reduce the risk of death among people living with CF over 8 years. This improved understanding will allow individuals living with CF and clinical providers to have reasonable expectations about survival and to allocate care and resources aligned with increased life expectancy appropriately.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.