Non‐invasive ventilation versus oxygen therapy in cystic fibrosis: A 12‐month randomized trial

ABSTRACT 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. A prospective, randomized, parallel group study in adult patients with cystic fibrosis (CF) and sleep desaturation to compare 12 months of non‐invasive ventilation (NIV) ± O2 to low‐flow oxygen therapy (LFO2) during sleep was conducted. NIV ± O2 increases event‐free survival over 12 months in adults with CF. No differences were seen in arterial blood gases (ABG), lung function, health‐related quality of life (HRQoL) or hospitalizations. Watch the video https://youtu.be/bw8SEDzE8EM See related Editorial