Non-invasive ventilation for cystic fibrosis

The use of non-invasive ventilation (NIV) has been proposed as a means to temporarily reverse or slow the progression of worsening respiratory failure in cystic fibrosis (CF).

To compare the effect of NIV versus no NIV in people with CF.

We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group trials register which comprises references identified from comprehensive electronic database searches, handsearching relevant journals and abstract books of conference proceedings. The reference lists of each trial were searched for additional publications that may contain other trials. Date of most recent search: January 2002.

Relevant randomised controlled trials in which a form of pressure preset or volume preset NIV versus no NIV was used in people with acute or chronic respiratory failure in CF.

Two reviewers independently assessed trials for inclusion criteria, methodological quality and data extraction.

Six trials were identified and four trials met the inclusion criteria with a total of 55 participants. All of these trials only evaluated single treatment sessions. Two trials (36 participants) evaluated the use of NIV for airway clearance compared with an alternative physiotherapy method. These trials showed that airway clearance may be easier and people with CF may prefer to use NIV for airway clearance. There was no evidence that NIV increases sputum expectoration or improves lung function. Two trials (19 participants) evaluated the use of NIV for overnight ventilatory support. Of the primary outcomes examined in this review, only lung function was evaluated within the two trials. Due to the small numbers of participants and the differing statistical techniques used within the review and the primary trials, there were discrepancies in the results obtained by the RevMan analysis and the original trial analysis. No clear differences were found between NIV compared with either oxygen or room air.

Non-invasive ventilation may be a useful adjunct to other airway clearance techniques, particularly in people with CF who have difficulty expectorating sputum. Non-invasive ventilation when used in addition to oxygen may improve gas exchange during sleep to a greater extent than oxygen therapy alone in moderate to severe disease. These benefits of NIV have only been demonstrated in single treatment sessions and its efficacy, safety and acceptability in the longer term are unknown. There is a need for long-term randomised controlled trials to determine the clinical effects of non-invasive ventilation in CF.