ArticleJournal of cystic fibrosis : official journal of the European Cystic Fibrosis Society2020
Answering the call to address cystic fibrosis treatment burden in the era of highly effective CFTR modulator therapy.
Article in Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT05392855 (Symptom Based Performance of Airway Clearance Therapy After Starting Highly Effective CFTR Modulator Therapy for Cystic Fibrosis), which is not on this map. Cited by 21 papers.
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.
Symptom Based Performance of Airway Clearance Therapy After Starting Highly Effective CFTR Modulator Therapy for Cystic Fibrosis
Who cites it
21 citing papers in PubMed.
- Trial
- Exploring Exercise as Airway Clearance in Cystic Fibrosis: A Qualitative Study From the ExACT-CF Feasibility Trial.Pediatric pulmonology · 2026Trial
- Impact of Discontinuing Both Hypertonic Saline and Dornase Alfa after Elexacaftor-Tezacaftor-Ivacaftor in Cystic Fibrosis.Annals of the American Thoracic Society · 2024Trial
- The effect of discontinuing hypertonic saline or dornase alfa on mucociliary clearance in elexacaftor/tezacaftor/ivacaftor treated people with cystic fibrosis: The SIMPLIFY-MCC Study.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2024Trial
- Discontinuation versus continuation of hypertonic saline or dornase alfa in modulator treated people with cystic fibrosis (SIMPLIFY): results from two parallel, multicentre, open-label, randomised, controlled, non-inferiority trials.The Lancet. Respiratory medicine · 2023Trial
- Trial
- Medication discontinuation in the era of elexacaftor/tezacaftor/ivacaftor: an Australian survey and qualitative interview.Internal medicine journal · 2026Article
- Elexacaftor/tezacaftor/ivacaftor is associated with long-term reduction in use of chronic respiratory therapies in cystic fibrosis.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2026Observational
- Cystic fibrosis transmembrane conductance regulator modulator therapy and lived experiences in South Africa: A mixed-methods study.The South African journal of physiotherapy · 2026Article
- The clinical, psychosocial, and economic burden of cystic fibrosis lung disease in the era of CFTR modulator therapy.Annals of the American Thoracic Society · 2026Review
- Reducing Treatment Burden in Cystic Fibrosis: Is There a Risk to OverSIMPLIFYing Therapies?Annals of the American Thoracic Society · 2024Article
- Assessing Safety of Discontinuing Hypertonic Saline in Those with Lower Forced Expiratory Volume in 1 Second after Elexacaftor/Tezacaftor/Ivacaftor.Annals of the American Thoracic Society · 2024Article
- Real-world effectiveness of airway clearance techniques in children with cystic fibrosis.The European respiratory journal · 2023Observational
- Patient and Caregiver Perceptions of Airway Clearance Methods Used for Cystic Fibrosis.Canadian respiratory journal · 2023Article
- Exploring the impact of elexacaftor-tezacaftor-ivacaftor treatment on opinions regarding airway clearance techniques and nebulisers: TEMPO a qualitative study in children with cystic fibrosis, their families and healthcare professionals.BMJ open respiratory research · 2022Article
- Treatment patterns in people with cystic fibrosis: have they changed since the introduction of ivacaftor?Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2022Article
- Clinical Effectiveness of Elexacaftor/Tezacaftor/Ivacaftor in People with Cystic Fibrosis: A Clinical Trial.American journal of respiratory and critical care medicine · 2022Observational
- A new era for people with cystic fibrosis.European journal of pediatrics · 2021Review
- Evaluating the Impact of Stopping Chronic Therapies after Modulator Drug Therapy in Cystic Fibrosis: The SIMPLIFY Clinical Trial Study Design.Annals of the American Thoracic Society · 2021Article
- Building global development strategies for cf therapeutics during a transitional cftr modulator era.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2020Review
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
backgroundWe recognize an unprecedented opportunity to study the effects of withdrawing one or more chronic treatments in people with CF (PwCF) who benefit greatly from CFTR modulator therapy, but feasibility and acceptance of such a study within the community is unknown.
methodsWe surveyed PwCF, their families, and their acquaintances between November 16, 2018, and December 2, 2018, and CF clinicians between December 19, 2018, and January 2, 2019, about treatment withdrawal research. We sought feedback from these groups about their level of interest in this research, the consistency with which they were taking modulator and non-modulator treatments, the ways in which they conceptualized health changes, and what chronic non-modulator treatments they were most interested in stopping. We also asked for stakeholder perspectives on the design of a treatment withdrawal trial, but we intend to report these perspectives elsewhere.
resultsEighty percent (541/675) of CF community respondents and 95% (206/218) of CF clinicians said that a trial of treatment simplification should be performed in the context of highly effective modulator therapy. Most current CFTR modulator users (292/359, 81%) have not stopped another chronic treatment. Worsening lung function by spirometry or increased daily symptoms were important health indicators. PwCF, their families, and/or their acquaintances ranked airway clearance techniques and inhaled antibiotics as the most burdensome treatments.
conclusionsThere is considerable support among the CF community and CF clinicians in the U.S. for controlled trials to assess the safety and impact of treatment simplification in patients taking highly effective modulator therapy.
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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.