Non-invasive ventilation for cystic fibrosis

  • Review
  • Intervention

Authors

  • Fidelma Moran,

    Corresponding author
    1. University of Ulster, Institute of Nursing and Health Research and School of Health Sciences, Newtownabbey, Northern Ireland, UK
    • Fidelma Moran, Institute of Nursing and Health Research and School of Health Sciences, University of Ulster, Shore Road, Newtownabbey, Northern Ireland, BT37 0QB, UK. f.moran@ulster.ac.uk.

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  • Judy M Bradley,

    1. University of Ulster, Centre for Health and Rehabilitation Technologies (CHaRT), Institute of Nursing and Health Research, Newtownabbey, Northern Ireland, UK
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  • Amanda J Piper

    1. Royal Prince Alfred Hospital, Department of Respiratory and Sleep Medicine, Camperdown, NSW, Australia
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Abstract

Background

Non-invasive ventilation may be a means to temporarily reverse or slow the progression of respiratory failure in cystic fibrosis.

Objectives

To compare the effect of non-invasive ventilation versus no non-invasive ventilation in people with cystic fibrosis.

Search methods

We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic database searches, handsearching relevant journals and abstract books of conference proceedings. We searched the reference lists of each trial for additional publications possibly containing other trials.

Most recent search: 22 February 2013.

Selection criteria

Randomised controlled trials comparing a form of pressure preset or volume preset non-invasive ventilation to no non-invasive ventilation in people with acute or chronic respiratory failure in cystic fibrosis.

Data collection and analysis

Three reviewers independently assessed trials for inclusion criteria and methodological quality, and extracted data.

Main results

Fifteen trials were identified; seven trials met the inclusion criteria with a total of 106 participants. Six trials evaluated single treatment sessions and one evaluated a six-week intervention.

Four trials (79 participants) evaluated non-invasive ventilation for airway clearance compared with an alternative chest physiotherapy method and showed that airway clearance may be easier with non-invasive ventilation and people with cystic fibrosis may prefer it. We were unable to find any evidence that NIV increases sputum expectoration, but it did improve some lung function parameters.

Three trials (27 participants) evaluated non-invasive ventilation for overnight ventilatory support, measuring lung function, validated quality of life scores and nocturnal transcutaneous carbon dioxide. Due to the small numbers of participants and statistical issues, there were discrepancies in the results between the RevMan and the original trial analyses. No clear differences were found between non-invasive ventilation compared with oxygen or room air except for exercise performance, which significantly improved with non-invasive ventilation compared to room air over six weeks.

Authors' conclusions

Non-invasive ventilation may be a useful adjunct to other airway clearance techniques, particularly in people with cystic fibrosis who have difficulty expectorating sputum. Non-invasive ventilation, 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 non-invasive ventilation have largely been demonstrated in single treatment sessions with small numbers of participants. The impact of this therapy on pulmonary exacerbations and disease progression remain unclear. There is a need for long-term randomised controlled trials which are adequately powered to determine the clinical effects of non-invasive ventilation in cystic fibrosis airway clearance and exercise.

Plain language summary

Mechanical lung inflation and deflation via face mask to aid breathing and sputum clearance, reduce respiratory failure and 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.