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Non‐invasive ventilation for cystic fibrosis

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Abstract

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Background

Non‐invasive ventilation (NIV) may be a means to temporarily reverse or slow the progression of respiratory failure in cystic fibrosis (CF).

Objectives

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

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: October 2006.

Selection criteria

Randomised controlled trials comparing a form of pressure preset or volume preset NIV to no NIV in people with acute or chronic respiratory failure in CF.

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 only and one evaluated a six‐week intervention.

Four trials (79 participants) evaluated NIV for airway clearance compared with an alternative chest physiotherapy method and showed that airway clearance may be easier with NIV and people with CF 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 NIV for overnight ventilatory support. Lung function and nocturnal transcutaneous carbon dioxide were evaluated within two trials. 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 NIV compared with oxygen or room air except for exercise performance, which significantly improved with NIV 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 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 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 CF airway clearance and exercise.

PICOs

Population
Intervention
Comparison
Outcome

The PICO model is widely used and taught in evidence-based health care as a strategy for formulating questions and search strategies and for characterizing clinical studies or meta-analyses. PICO stands for four different potential components of a clinical question: Patient, Population or Problem; Intervention; Comparison; Outcome.

See more on using PICO in the Cochrane Handbook.

Plain language summary

available in

Non‐invasive ventilation may help breathing during sleep, help reduce respiratory failure, help with clearance of sputum and help improve exercise tolerance in people with moderate to severe disease

As cystic fibrosis worsens, breathing can become difficult, usually indicating the start of respiratory failure (too much carbon dioxide and not enough oxygen). As respiratory failure worsens people may also have difficulty clearing sputum. Respiratory failure eventually results in death. Evidence from a small number of short‐term trials shows that non‐invasive ventilation can improve a range of breathing and gas exchange variables and ease sputum clearance. The longer term efficacy, safety and acceptability of non‐invasive ventilation, as a method of airway clearance for overnight ventilation, has not been established. Further research needs to establish if there is a role for non‐invasive ventilation in exercise training in severe disease. We were unable to find any evidence that non‐invasive ventilation improves life expectancy.