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Updated: Jan 23, 2026

Design and Development of a Model to Study the Effect of Supplemental Oxygen on the Cystic Fibrosis Airway Microbiome
Published on: August 3, 2021
Non-invasive ventilation versus oxygen therapy in cystic fibrosis: A 12-month randomized trial
Maree A Milross1, Amanda J Piper2,3, Tiffany J Dwyer1,3
1Faculty of Health Sciences, University of Sydney, Sydney, NSW, Australia.
Background And Objective:
No published studies have examined the long-term effects of non-invasive ventilation (NIV) in cystic fibrosis (CF). Our primary aim was to determine if adults with CF and sleep desaturation were less likely to develop hypercapnia with NIV ± O2 compared to low-flow oxygen therapy (LFO2 ) or meet the criteria for failure of therapy over 12 months. We studied event-free survival, hospitalizations, lung function, arterial blood gases (ABG), sleep quality and health-related quality of life.
Methods:
A prospective, randomized, parallel group study in adult patients with CF and sleep desaturation was conducted, comparing 12 months of NIV ± O2 to LFO2 . Event-free survival was defined as participants without events. Events included: failure of therapy with PaCO2 > 60 mm Hg, or increase in PaCO2 > 10 mm Hg from baseline, increases in TcCO2 > 10 mm Hg, lung transplantation or death. Outcomes were measured at baseline, 3, 6 and 12 months, including lung function, ABG, Pittsburgh Sleep Quality Inventory (PSQI), SF36 and hospitalizations.
Results:
A total of 29 patients were randomized to NIV ± O2 (n = 14) or LFO2 (n = 15) therapy for 12 months. Of the 29 patients, 18 met the criteria for event-free survival over 12 months. NIV ± O2 group had 33% (95% CI: 5-58%) and 46% (95% CI: 10-68%) more event-free survival at 3 and 12 months than LFO2 group. No statistically significant differences were seen in spirometry, ABG, questionnaires or hospitalizations.
Conclusion:
NIV ± O2 during sleep increases event-free survival over 12 months in adults with CF. Further studies are required to determine which subgroups benefit the most from NIV.
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