Non-invasive ventilation is the mechanical inflation and deflation of the lungs via a face mask which may help breathing during sleep, help reduce respiratory failure, help with clearance of sputum and help improve exercise tolerance.

As cystic fibrosis worsens, breathing can become difficult. This indicates the start of respiratory failure, where there is too much carbon dioxide and not enough oxygen in the blood. As respiratory failure progresses people may also have problems clearing sputum. Respiratory failure eventually results in death. This review includes seven trials. Six short-term trials showed that non-invasive ventilation can improve a range of breathing and gas exchange measures and ease sputum clearance. One longer-term trial showed that non-invasive ventilation is effective, safe and acceptable as overnight ventilation. When used with oxygen, non-invasive ventilation may improve gas exchange during sleep more than oxygen therapy alone. We found some evidence to support the use of non-invasive ventilation in addition to other airway clearance methods in people with cystic fibrosis. We were not able to find any evidence that non-invasive ventilation improves life expectancy. Further research needs to show whether non-invasive ventilation should be used in exercise training in severe disease.