Non-invasive ventilation for cystic fibrosis

Area of Science:

• Respiratory Medicine
• Pulmonology
• Critical Care

Background:

• Cystic fibrosis (CF) patients often experience mucus retention, breathlessness, and reduced respiratory muscle strength.
• Non-invasive positive pressure ventilation (NIPPV) is a treatment for acute and chronic respiratory symptoms in individuals with CF.
• Previous research has explored NIPPV's role in airway clearance during sleep and exercise for CF management.

Purpose of the Study:

• To evaluate the effects, acceptability, and individual preferences of NIPPV in people with cystic fibrosis (pwCF).
• To synthesize current evidence on NIPPV's efficacy for both acute exacerbations and stable conditions in CF patients.

Main Methods:

• Conducted systematic searches of clinical trial registries and reference lists up to April 2, 2025.
• Included randomized controlled trials (RCTs) of parallel and cross-over designs comparing NIPPV with no NIPPV or other treatments in pwCF.
• Assessed outcomes including mortality, quality of life, lung function, respiratory symptoms, and adverse events using GRADE and RoB 2.0 criteria.

Main Results:

• A total of 14 RCTs with 261 participants were included, with most studies having short durations (max 3 months).
• Evidence regarding the acute effects of NIPPV (specifically BiPAP) on sputum expectoration, hospital stay, and patient comfort was very uncertain.
• Evidence for NIPPV's effects on lung function (FEV1, FVC), sleep quality, and acceptability in stable CF patients was also very uncertain due to limited data and methodological limitations.

Conclusions:

• Current evidence on the effectiveness of NIPPV for pwCF, during both pulmonary exacerbations and stable states, remains uncertain.
• Included studies had limitations, including small sample sizes, insufficient assessment of critical outcomes, and methodological weaknesses, leading to significant uncertainty.
• High-quality, longer-term studies are necessary to accurately determine the benefits of NIPPV for airway clearance, nocturnal ventilation, and exercise in CF patients.