Authors
Cynthia D Brown, Kevin J Psoter, Kathryn A Sabadosa, Joshua S Ostrenga, Bradley H Rosen, Georgene Hergenroeder, Lisa Bendy, Connie Zhang, Nell Meosky Luo, Clement L Ren
Published in
Annals of the American Thoracic Society. Aug 05, 2026. Epub Aug 05, 2026.
Abstract
Given improvement in symptoms and lung function in people with cystic fibrosis (CF) taking elexacaftor/tezacaftor/ivacaftor (ETI), there is increasing interest in de-escalation of care although the effect on outcomes remains unknown.
To determine if self-reported discontinuation of chronic daily therapies (CDT) is associated with changes in pulmonary exacerbations and lung function in individuals taking ETI.
The Home Reported Outcomes in Cystic Fibrosis 2 study was a 12-month prospective observational study in individuals age 12 and older taking ETI. This remote study collected patient-reported use and prior discontinuation of CDT at study entry via the Folia Health application. Primary outcomes were lung function and intravenous-antibiotic treated pulmonary exacerbations (PEx) as reported in the CF Foundation Patient Registry. Inverse probability weighting was used to compare outcomes between those that did and did not discontinue a CDT.
A total of 860 individuals consented, and 709 were included in these analyses. At baseline, 41.5% self-reported discontinuation of 1 or more CDTs. Over 12 months, those who discontinued CDT did not have any significant difference in the probability of PEx (mean difference -2.0%, 95% CI -6.7, 2.8%) or rate of change in lung function (mean difference 0.41%, 95% CI -1.40, 2.22).
In individuals taking ETI, self-reported discontinuation of CDT was not associated with an increased risk of PEx or decline in lung function.
PMID:
42554296
Bibliographic data and abstract were imported from PubMed on 05 Aug 2026.
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