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Meta-Analysis
. 2017 Feb 20;2(2):CD002769.
doi: 10.1002/14651858.CD002769.pub5.

Non-invasive ventilation for cystic fibrosis

Fidelma Moran  1 , Judy M Bradley  2 , Amanda J Piper  3
Affiliations
Meta-Analysis

Non-invasive ventilation for cystic fibrosis

Fidelma Moran et al. Cochrane Database Syst Rev. .

Abstract

Background: Non-invasive ventilation may be a means to temporarily reverse or slow the progression of respiratory failure in cystic fibrosis by providing ventilatory support and avoiding tracheal intubation. Using non-invasive ventilation, in the appropriate situation or individuals, can improve lung mechanics through increasing airflow and gas exchange and decreasing the work of breathing. Non-invasive ventilation thus acts as an external respiratory muscle. This is an update of a previously published review.

Objectives: To compare the effect of non-invasive ventilation versus no non-invasive ventilation in people with cystic fibrosis for airway clearance, during sleep and during exercise.

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: 08 August 2016.

Selection criteria: Randomised controlled trials comparing a form of pressure preset or volume preset non-invasive ventilation to no non-invasive ventilation used for airway clearance or during sleep or exercise 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: Ten trials met the inclusion criteria with a total of 191 participants. Seven trials evaluated single treatment sessions, one evaluated a two-week intervention, one evaluated a six-week intervention and one a three-month intervention. It is only possible to blind trials of airway clearance and overnight ventilatory support to the outcome assessors. In most of the trials we judged there was an unclear risk of bias with regards to blinding due to inadequate descriptions. The six-week trial was the only one judged to have a low risk of bias for all other domains. One single intervention trial had a low risk of bias for the randomisation procedure with the remaining trials judged to have an unclear risk of bias. Most trials had a low risk of bias with regard to incomplete outcome data and selective reporting.Six trials (151 participants) evaluated non-invasive ventilation for airway clearance compared with an alternative chest physiotherapy method such as the active cycle of breathing techniques or positive expiratory pressure. Three trials used nasal masks, one used a nasal mask or mouthpiece and one trial used a face mask and in one trial it is unclear. Three of the trials reported on one of the review's primary outcome measures (quality of life). Results for the reviews secondary outcomes 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 non-invasive ventilation increases sputum expectoration, but it did improve some lung function parameters.Three trials (27 participants) evaluated non-invasive ventilation for overnight ventilatory support compared to oxygen or room air using nasal masks (two trials) and nasal masks or full face masks (one trial). Trials reported on two of the review's primary outcomes (quality of life and symptoms of sleep-disordered breathing). Results for the reviews secondary outcome measures showed that they measured lung function, gas exchange, adherence to treatment and preference, 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.One trial (13 participants) evaluated non-invasive ventilation on exercise capacity (interface used was unclear) and did not reported on any of the review's primary outcomes. The trial found no clear differences between non-invasive ventilation compared to no non-invasive ventilation for any of our outcomes.Three trials reported on adverse effects. One trial, evaluating non-invasive ventilation for airway clearance, reported that a participant withdrew at the start of the trial due to pain on respiratory muscle testing. One trial evaluating non-invasive ventilation for overnight support reported that one participant could not tolerate an increase in inspiratory positive airway pressure. A second trial evaluating non-invasive ventilation in this setting reported that one participant did not tolerate the non-invasive ventilation mask, one participant developed a pneumothorax when breathing room air and two participants experienced aerophagia which resolved when inspiratory positive airway pressure was decreased.

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. The effect of NIV on exercise is unclear. 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.

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Conflict of interest statement

Amanda Piper was a clinical consultant to ResMed Australia until 2004. She has also been involved in: educational activities sponsored by manufacturers of bilevel devices (Mayo Healthcare, Australia; Weinmann, Germany; Air Liquide, Australia; ResMed, Australia; Philps Respironics, Australia) and transcutaneous carbon dioxide devices (Sentec, Switzwerland; industry‐sponsored research (ResMed, Australia) and has received equipment for research projects from distributors of bilevel equipment (Air Liquide, Australia; Mayo Healthcare, Australia) as well as a competitive research grant from the ResMed Foundation.

Fidelma Moran and Judy Bradley received a NIPPY3 ventilator for a research clinical trial from Respicare.

Figures

1.1
1.1. Analysis
Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 1 CFQ scores.
1.2
1.2. Analysis
Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 2 Fatigue.
1.3
1.3. Analysis
Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 3 FEV1 (L).
1.4
1.4. Analysis
Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 4 FEV1 (% predicted).
1.5
1.5. Analysis
Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 5 FVC (L).
1.6
1.6. Analysis
Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 6 FVC (% predicted).
1.7
1.7. Analysis
Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 7 FEF25‐75 (L).
1.8
1.8. Analysis
Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 8 FEF25‐75 (% predicted).
1.9
1.9. Analysis
Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 9 Respiratory muscle strength PImax (cmH20).
1.10
1.10. Analysis
Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 10 Respiratory muscle strength PEmax (cmH20).
1.11
1.11. Analysis
Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 11 Airway resistance % predicted.
1.12
1.12. Analysis
Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 12 LCI.
1.13
1.13. Analysis
Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 13 Oxygen saturation during airway clearance (%).
1.14
1.14. Analysis
Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 14 Oxygen saturation during airway clearance (change in SpO2 % during treatment).
1.15
1.15. Analysis
Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 15 Oxygen saturation after airway clearance (SpO2).
1.16
1.16. Analysis
Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 16 PaCO2.
1.17
1.17. Analysis
Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 17 Breathlessness.
1.18
1.18. Analysis
Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 18 Sputum wet weight (g).
1.19
1.19. Analysis
Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 19 Sputum dry weight (g).
1.20
1.20. Analysis
Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 20 Sputum volume per day (VAS score).
1.21
1.21. Analysis
Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 21 Pseudomonas density (log CFU/g).
1.22
1.22. Analysis
Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 22 Exercise capacity: 25 level modified shuttle test (m).
1.23
1.23. Analysis
Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 23 Energy (VAS score).
1.24
1.24. Analysis
Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 24 6MWT.
1.25
1.25. Analysis
Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 25 Length of hospital stay (days).
1.26
1.26. Analysis
Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 26 Time to next admission (days).
2.1
2.1. Analysis
Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 1 CFQoL chest symptom score.
2.2
2.2. Analysis
Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 2 CFQoL transitional dyspnoea index.
2.3
2.3. Analysis
Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 3 Symptoms of sleep‐disordered breathing.
2.4
2.4. Analysis
Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 4 Lung function during sleep.
2.5
2.5. Analysis
Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 5 Respiratory rate (breaths/min).
2.6
2.6. Analysis
Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 6 Lung function while awake.
2.7
2.7. Analysis
Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 7 Arterial blood gases: pH.
2.8
2.8. Analysis
Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 8 Arterial blood gases: PaO2 (mmHg).
2.9
2.9. Analysis
Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 9 Arterial blood gases: PaCO2 (mmHg).
2.10
2.10. Analysis
Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 10 Arterial blood gases: HCO3 (mmol/L).
2.11
2.11. Analysis
Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 11 Arterial blood gases: SaO2 (%).
2.12
2.12. Analysis
Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 12 Exercise performance (MSWT) (metres).
2.13
2.13. Analysis
Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 13 Total sleep time (min).
2.14
2.14. Analysis
Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 14 REM sleep architecture.
2.15
2.15. Analysis
Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 15 Sleep latency (min).
2.16
2.16. Analysis
Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 16 Nocturnal oxygen saturation (%).
2.17
2.17. Analysis
Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 17 Nocturnal oxygen saturation (%).
2.18
2.18. Analysis
Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 18 Nocturnal TcCO2 (mmHg.
2.19
2.19. Analysis
Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 19 Nocturnal TcCO2 (mmHg).
2.20
2.20. Analysis
Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 20 Nocturnal TcCO2 TST (mmHg).
2.21
2.21. Analysis
Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 21 Hypopneas.
2.22
2.22. Analysis
Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 22 Mean heart rate (beats/min).
2.23
2.23. Analysis
Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 23 Respiratory rate.
3.1
3.1. Analysis
Comparison 3 NIV in overnight ventilation compared to room air, Outcome 1 CF QoL chest symptom score.
3.2
3.2. Analysis
Comparison 3 NIV in overnight ventilation compared to room air, Outcome 2 CF QoL traditional dyspnoea index score.
3.3
3.3. Analysis
Comparison 3 NIV in overnight ventilation compared to room air, Outcome 3 Symptoms of sleep‐disordered breathing.
3.4
3.4. Analysis
Comparison 3 NIV in overnight ventilation compared to room air, Outcome 4 Lung function during sleep.
3.5
3.5. Analysis
Comparison 3 NIV in overnight ventilation compared to room air, Outcome 5 Respiratory rate(breaths/min) during sleep.
3.6
3.6. Analysis
Comparison 3 NIV in overnight ventilation compared to room air, Outcome 6 Mean Respiratory Rate.
3.7
3.7. Analysis
Comparison 3 NIV in overnight ventilation compared to room air, Outcome 7 Mean Heart Rate (beats/min).
3.8
3.8. Analysis
Comparison 3 NIV in overnight ventilation compared to room air, Outcome 8 Lung function while awake.
3.9
3.9. Analysis
Comparison 3 NIV in overnight ventilation compared to room air, Outcome 9 Arterial blood gases: pH.
3.10
3.10. Analysis
Comparison 3 NIV in overnight ventilation compared to room air, Outcome 10 Arterial blood gases: PaO2 (mmHg).
3.11
3.11. Analysis
Comparison 3 NIV in overnight ventilation compared to room air, Outcome 11 Arterial blood gases: PaCO2 (mmHg).
3.12
3.12. Analysis
Comparison 3 NIV in overnight ventilation compared to room air, Outcome 12 Arterial blood gases: HCO3 (mmol/L).
3.13
3.13. Analysis
Comparison 3 NIV in overnight ventilation compared to room air, Outcome 13 Arterial blood gases: SaO2 (%).
3.14
3.14. Analysis
Comparison 3 NIV in overnight ventilation compared to room air, Outcome 14 Exercise performance (metres).
3.15
3.15. Analysis
Comparison 3 NIV in overnight ventilation compared to room air, Outcome 15 Total sleep time (min).
3.16
3.16. Analysis
Comparison 3 NIV in overnight ventilation compared to room air, Outcome 16 REM sleep architecture.
3.17
3.17. Analysis
Comparison 3 NIV in overnight ventilation compared to room air, Outcome 17 Sleep latency.
3.18
3.18. Analysis
Comparison 3 NIV in overnight ventilation compared to room air, Outcome 18 Nocturnal oxygen saturation (%).
3.19
3.19. Analysis
Comparison 3 NIV in overnight ventilation compared to room air, Outcome 19 Nocturnal oxygen saturation (%).
3.20
3.20. Analysis
Comparison 3 NIV in overnight ventilation compared to room air, Outcome 20 Nocturnal TcCO2 (mmHg).
3.21
3.21. Analysis
Comparison 3 NIV in overnight ventilation compared to room air, Outcome 21 Nocturnal TcCO2(mmHg).
3.22
3.22. Analysis
Comparison 3 NIV in overnight ventilation compared to room air, Outcome 22 Nocturnal TcCO2 TST (mmHg).
3.23
3.23. Analysis
Comparison 3 NIV in overnight ventilation compared to room air, Outcome 23 Hypopneas.
4.1
4.1. Analysis
Comparison 4 NIV versus no NIV during exercise testing, Outcome 1 6 minute walk test.
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References

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