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Intervention Review

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Non-invasive ventilation for cystic fibrosis

  1. Fidelma Moran1,*,
  2. Judy M Bradley2,
  3. Amanda J Piper3

Editorial Group: Cochrane Cystic Fibrosis and Genetic Disorders Group

Published Online: 30 APR 2013

Assessed as up-to-date: 14 MAR 2013

DOI: 10.1002/14651858.CD002769.pub4


How to Cite

Moran F, Bradley JM, Piper AJ. Non-invasive ventilation for cystic fibrosis. Cochrane Database of Systematic Reviews 2013, Issue 4. Art. No.: CD002769. DOI: 10.1002/14651858.CD002769.pub4.

Author Information

  1. 1

    University of Ulster, Institute of Nursing and Health Research and School of Health Sciences, Newtownabbey, Northern Ireland, UK

  2. 2

    University of Ulster, Centre for Health and Rehabilitation Technologies (CHaRT), Institute of Nursing and Health Research, Newtownabbey, Northern Ireland, UK

  3. 3

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

*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.

Publication History

  1. Publication Status: New search for studies and content updated (no change to conclusions)
  2. Published Online: 30 APR 2013

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This is not the most recent version of the article. View current version (20 FEB 2017)

 
Characteristics of included studies [ordered by study ID]
Fauroux 1999

MethodsRandomised, cross-over trial.


Participants16 participants with CF. Stable participants.
Mean (SD) age 13 (4) years.


InterventionsOrder of intervention was randomised.
Session 1: CPT (10 to 15 forced expiration manoeuvres separated by rest periods) and inspiratory PSV via nasal mask using pressure support generator.
Session 2: CPT with no PSV.

Sessions 20 minutes. Time between sessions unclear - paper states sessions were conducted on two different days at the same time of day by same physiotherapist.


OutcomesFVC; FEV1; PEF; FEF 25%; FEF50%; FEF25-75%; airway resistance; SpO2; RR; PI max; PE max; FEF25-75; sputum weight; subjective participants impressions of fatigue, ease sputum clearance; participant preference (1 = worse to 3 = marked preference).


Notes


Risk of bias

BiasAuthors' judgementSupport for judgement

Random sequence generation (selection bias)Unclear riskStates order of intervention was randomised, but no details given.

Allocation concealment (selection bias)Unclear riskNot discussed.

Blinding (performance bias and detection bias)
All outcomes
Unclear riskIn one trial, participants' subjective impressions were evaluated by individuals who were not involved in the trial and were unaware of the treatment regimen; but it was not reported who was responsible for collecting and weighing secretions and lung function testing.

Incomplete outcome data (attrition bias)
All outcomes
Low riskAll participants were accounted for.

Selective reporting (reporting bias)Low riskAll outcome measures were reported.

Other biasLow riskMethods of statistical analysis were described.

Gozal 1997

MethodsRandomised, cross-over trial.


ParticipantsSix participants with CF and moderate to severe lung disease and significant gas exchange abnormalities during sleep. Stable participants.
Mean (SD) age 22.3 (4.7) years (range 13 - 28 years). Mean (SD) FEV1% predicted 29.4 (3.4).


InterventionsOrder of intervention was randomised.
Three nights within a 15-day period.
Session 1: Room air Session 2: Night-time low flow O2.
Session 2: Night-time bilevel NIPPV with supplemental O2.


OutcomesTST; Sleep latency; NREM; NREM %TST; REM min; REM %TST; undetermined % TST; total arousals; arousal index; SaO2; TcCO2.


Notes


Risk of bias

BiasAuthors' judgementSupport for judgement

Random sequence generation (selection bias)Unclear riskStated as randomised, methods not discussed.

Allocation concealment (selection bias)Unclear riskNot discussed.

Blinding (performance bias and detection bias)
All outcomes
Unclear riskParticipants were obviously aware of the intervention being administered; however, all were reportedly unaware of the purpose of the trial with participants and sleep technicians blinded to the results until completion of the third night.

Incomplete outcome data (attrition bias)
All outcomes
Unclear risk2 participants dropped out due to non-tolerance of NIV; it was not explicitly stated that intention-to-treat was not used, but results are based on the remaining 6 participants who completed the trial.

Selective reporting (reporting bias)Low riskAll outcome measures were reported.

Other biasUnclear riskMethods of statistical analysis were described.

Holland 2003

MethodsRandomised cross-over trial.


Participants26 participants with CF and moderate to severe disease. Acute participants.
Mean (SD) age 27.04 (6.42) years.
Mean (SD) FEV1% predicted 33.85 (11.85).


InterventionsOrder of intervention was randomised on days 3 and 4 of hospital admission.
Session 1: CPT by ACBT i.e. (thoracic expansion x6, breathing control) x2, forced expiration technique and cough as required.

Session 2: ACBT as above with NIV - EPAP 4 - 5 cmH2O, IPAP 10 - 12 cmH20 with heated humidification entrained.


OutcomesFVC; FEV1; FEF25-75; PiMax; PeMax; SpO2; sputum weight; Borg breathlessness score; participant preference.


Notes


Risk of bias

BiasAuthors' judgementSupport for judgement

Random sequence generation (selection bias)Unclear riskStates order of intervention was randomised, but no details given.

Allocation concealment (selection bias)Unclear riskNot discussed.

Blinding (performance bias and detection bias)
All outcomes
Low riskAn independent data collector who was blinded to the treatment order obtained all measurements.

Incomplete outcome data (attrition bias)
All outcomes
Low riskInformation provided about one drop out at the start of testing.

Selective reporting (reporting bias)Low riskAll outcome measures were reported.

Other biasUnclear riskMethods of statistical analysis were described.

Kofler 1998

MethodsRandomised, cross-over trial.


Participants20 participants with CF. No detail on whether participants are in acute or stable state, but participants have mean (SD) S-K score of 80.8 (15.3) indicating that they have mild disease.
Mean age 15.25 years (range 6 - 23) years.


InterventionsOrder of intervention was randomised.
Session 1: PEP treatment (no details of PEP treatment).
Session 2: bilevel positive airway pressure (BiPaP) treatment.

Treatment sessions on 2 successive days. Time between 2 sessions is 1 day.


OutcomesFEV1; FVC; SaO2; FEF25-75; MEF50; FEF25-75; participant preference.


Notes


Risk of bias

BiasAuthors' judgementSupport for judgement

Random sequence generation (selection bias)Unclear riskStates order of intervention was randomised, but no details given.

Allocation concealment (selection bias)Unclear riskNot discussed.

Blinding (performance bias and detection bias)
All outcomes
Unclear riskData collection was not described.

Incomplete outcome data (attrition bias)
All outcomes
Low riskAll participants were accounted for.

Selective reporting (reporting bias)Unclear riskNot clear whether all outcomes measured were reported in this abstract.

Other biasUnclear riskMethods of statistical analysis were not described.

Milross 2001

MethodsRandomised, cross-over trial.


Participants13 participants with CF with severe lung disease.
Mean (SD) age 26 (5.9) years.
Mean (SD) FEV1 % predicted, 31.7(10.6); awake PaO2 range 53-77 mmHg; PaCO2 ≥ 45 mmHg; mean (SD) BMI 20 (3) kgm2.


InterventionsOrder of intervention randomised.
Night 1: Room air and low-level CPAP (4 - 5 cm H2O).
Night 2: Oxygen (1.4 +/- 0.9L/min to maintain SaO2 ≥ 90%) and low-level CPAP (4 - 5 cm H2O).
Night 3: BVS +/- oxygen (0.7+/-0.9 L/min to maintain SaO2 ≥ 90%).

3 nights within a 1-week period. Time between nights unclear.


OutcomesVI, VT; RR; respiratory disturbance indices; SaO2 TcCO2.


Notes


Risk of bias

BiasAuthors' judgementSupport for judgement

Random sequence generation (selection bias)Unclear riskStated as randomised, methods not discussed.

Allocation concealment (selection bias)Unclear riskNot discussed.

Blinding (performance bias and detection bias)
All outcomes
High riskNo evidence of blinding of assessors, investigators or participants.

Incomplete outcome data (attrition bias)
All outcomes
Low riskNo dropouts.

Selective reporting (reporting bias)Low riskAll outcome measures were reported.

Other biasUnclear riskMethods of statistical analysis were described.

Placidi 2006

MethodsRandomised, cross-over trial.


Participants17 participants with CF. Severe lung disease. Acute participants.
Mean (SD) age 27 (7) years; mean (SD) FEV1% predicted 25 (6); mean (SD) BMI 18 (3) kg/m2; mean (SD) MIP% predicted 87 (17); mean (SD) wet weight sputum 5 (5) g.


InterventionsOrder of intervention randomised. Treatment twice daily for 70 mins for 2 days per intervention.
Intervention 1 directed cough;
Intervention 2 mask PEP;
Intervention 3 mask CPAP;
Intervention for NIV with IPAP 8 - 12 cmH20; EPAP 4 cmH20.


OutcomesSputum wet and dry weight; number spontaneous coughs; FEV1; mean SpO2; participants subjective impression of the effectiveness and fatigue induced by each treatment.


Notes


Risk of bias

BiasAuthors' judgementSupport for judgement

Random sequence generation (selection bias)Low riskRandomisation of treatment order was done according to the Latin square design which provided a balanced assignment to each treatment and a balance in the sequence of treatments.

Allocation concealment (selection bias)Unclear riskNot discussed.

Blinding (performance bias and detection bias)
All outcomes
Unclear riskReported data collection but not who was responsible for weighing sputum or collating subjective impressions induced by the treatment.

Incomplete outcome data (attrition bias)
All outcomes
Unclear riskAll participants were accounted for.

Selective reporting (reporting bias)Low riskAll outcome measures were reported.

Other biasUnclear riskMethods of statistical analysis were described.

Young 2008

MethodsRandomised, cross-over trial.


Participants8 participants with CF. Moderate and severe lung disease. No details on whether participants are in acute or stable state.
Mean (SD) age 37 (8) years. Mean (SD) FEV1% predicted 35 (8). Mean (SD) BMI 21.1 (2.6) kg/m2. Mean (SD) PaCO2 52 (4) mmHg.


InterventionsOrder of intervention was randomised with a 2-week washout period; 6 weeks of nocturnal air (placebo), oxygen and NIV.


OutcomesCF specific QoL questionnaire; daytime sleepiness; exertional dyspnoea; awake and asleep gas exchange; sleep architecture; lung function; peak exercise capacity. Neurocognitive function (PVT :mean; error; lapse); stroop color & word test; trail making test; controlled oral word association; digital span test were reported in the online supplement. They are not reported in this review as they were not relevant to the aims of this review.

Post treatment assessments were carried out during a period of clinical stability i.e. no current need for hospitalisation or intravenous antibiotics.


Notes


Risk of bias

BiasAuthors' judgementSupport for judgement

Random sequence generation (selection bias)Low riskComputer-generated Latin square design.

Allocation concealment (selection bias)Low riskSealed in sequentially numbered opaque envelopes by a person not involved in the trial and opened as each participant was enrolled.

Blinding (performance bias and detection bias)
All outcomes
Unclear riskParticipants remained blinded as to whether they were receiving oxygen or room air only.

Incomplete outcome data (attrition bias)
All outcomes
Low riskOne withdrawal after randomisation due to a pneumothorax. One withdrawal from NIV group due to mask discomfort (NIV n=7; O2 n=8).

Selective reporting (reporting bias)Low riskAll outcome measures were reported.

Other biasLow riskMethods of statistical analysis were described.

 
Characteristics of excluded studies [ordered by study ID]

StudyReason for exclusion

Elkins 2004This trial did not compare NIV to increase minute ventilation and is not linked to the outcome measures in this review.

Falk 2006This trial did not use NIV.

Fauroux 2000This trial did not compare NIV with other management for acute or chronic respiratory failure.

Fauroux 2001This is not a randomised controlled trial of NIV versus no NIV.

Fauroux 2004This trial did not compare NIV with other management for acute or chronic respiratory failure.

Greenough 2004This trial did not use NIV.

Piper 1992This is not a randomised controlled trial.

Regnis 1994This is not a randomised controlled trial.

Riethmueller 2006This trial did not use NIV.

Serra 2000This is not a randomised controlled trial of NIV versus no NIV.

Serra 2002This is not a randomised controlled trial of NIV versus no NIV.

 
Comparison 1. NIV versus other methods of airway clearance (chest physiotherapy including directed cough or PEP)

Outcome or subgroup titleNo. of studiesNo. of participantsStatistical methodEffect size

 1 Lung function - chest physiotherapy including directed cough3Std. Mean Difference (IV, Fixed, 95% CI)Subtotals only

    1.1 FEV1
3118Std. Mean Difference (IV, Fixed, 95% CI)-0.05 [-0.41, 0.31]

    1.2 FVC
3118Std. Mean Difference (IV, Fixed, 95% CI)0.02 [-0.35, 0.38]

    1.3 FEF25-75
3118Std. Mean Difference (IV, Fixed, 95% CI)-0.03 [-0.39, 0.33]

 2 Lung function - chest physiotherapy including PEP3Std. Mean Difference (IV, Fixed, 95% CI)Subtotals only

    2.1 FEV1
3118Std. Mean Difference (IV, Fixed, 95% CI)-0.06 [-0.43, 0.30]

    2.2 FVC
3118Std. Mean Difference (IV, Fixed, 95% CI)-0.01 [-0.37, 0.35]

    2.3 FEF25-75
3118Std. Mean Difference (IV, Fixed, 95% CI)-0.00 [-0.36, 0.36]

 3 Respiratory muscle strength (cmH20)1Mean Difference (IV, Fixed, 95% CI)Totals not selected

    3.1 PImax
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

    3.2 PEmax
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 4 Airway resistance % predicted1Mean Difference (IV, Fixed, 95% CI)Subtotals only

 5 Oxygen saturation during airway clearance (%)1Mean Difference (IV, Fixed, 95% CI)Subtotals only

 6 Oxygen saturation during airway clearance (change in SpO2 % during treatment)1Mean Difference (IV, Fixed, 95% CI)Subtotals only

 7 Oxygen saturation after airway clearance (SpO2) - chest physiotherapy including directed cough1Mean Difference (IV, Fixed, 95% CI)Subtotals only

 8 Oxygen saturation after airway clearance (SpO2) - chest physiotherapy including PEP1Mean Difference (IV, Fixed, 95% CI)Subtotals only

 9 Sputum wet weight (g) - chest physiotherapy including directed cough3118Mean Difference (IV, Fixed, 95% CI)-0.69 [-3.06, 1.67]

 10 Sputum wet weight (g)- chest physiotherapy including PEP3118Mean Difference (IV, Fixed, 95% CI)-1.54 [-3.96, 0.89]

 11 Sputum dry weight (g) - chest physiotherapy including directed cough1Mean Difference (IV, Fixed, 95% CI)Subtotals only

 12 Sputum dry weight (g)- chest physiotherapy including PEP1Mean Difference (IV, Fixed, 95% CI)Subtotals only

 13 Breathlessness1Mean Difference (IV, Fixed, 95% CI)Subtotals only

 
Comparison 2. NIV in overnight ventilation compared to oxygen

Outcome or subgroup titleNo. of studiesNo. of participantsStatistical methodEffect size

 1 CFQoL chest symptom score1Mean Difference (IV, Fixed, 95% CI)Totals not selected

    1.1 Up to 3 months
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 2 CFQoL transitional dyspnoea index1Mean Difference (IV, Fixed, 95% CI)Totals not selected

    2.1 Up to 3 months
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 3 Symptoms of Sleep Disordered Breathing1Mean Difference (IV, Fixed, 95% CI)Totals not selected

    3.1 Epworth Sleepiness Scale (up to 3 months)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

    3.2 Global PSQI (up to 3 months)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 4 Lung function during sleep1Mean Difference (Fixed, 95% CI)Totals not selected

    4.1 VI (L/m) while awake (single night)
1Mean Difference (Fixed, 95% CI)0.0 [0.0, 0.0]

    4.2 VI (L/m) during REM (single night)
1Mean Difference (Fixed, 95% CI)0.0 [0.0, 0.0]

    4.3 VI (L/m) during NREM (single night)
1Mean Difference (Fixed, 95% CI)0.0 [0.0, 0.0]

    4.4 VT (L) while awake (single night)
1Mean Difference (Fixed, 95% CI)0.0 [0.0, 0.0]

    4.5 VT (L) during REM (single night)
1Mean Difference (Fixed, 95% CI)0.0 [0.0, 0.0]

    4.6 VT (L) during NREM (single night)
1Mean Difference (Fixed, 95% CI)0.0 [0.0, 0.0]

 5 Lung function while awake1Mean Difference (IV, Fixed, 95% CI)Totals not selected

    5.1 FEV1% predicted (up to 3 months)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

    5.2 FVC % predicted (up to 3 months)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 6 Respiratory rate (breaths/min)1Mean Difference (Fixed, 95% CI)Totals not selected

    6.1 RR while awake (single night)
1Mean Difference (Fixed, 95% CI)0.0 [0.0, 0.0]

    6.2 RR during REM (single night)
1Mean Difference (Fixed, 95% CI)0.0 [0.0, 0.0]

    6.3 RR during NREM (single night)
1Mean Difference (Fixed, 95% CI)0.0 [0.0, 0.0]

 7 Mean Respiratory Rate (breaths/min)1Mean Difference (IV, Fixed, 95% CI)Totals not selected

    7.1 Up to 3 months
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 8 ABG: pH1Mean Difference (IV, Fixed, 95% CI)Totals not selected

    8.1 Up to 3 months
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 9 ABG: PaO2 (mmHg)1Mean Difference (IV, Fixed, 95% CI)Totals not selected

    9.1 Up to 3 months
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 10 ABG: PaCO2 (mmHg)1Mean Difference (IV, Fixed, 95% CI)Totals not selected

    10.1 Up to 3 months
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 11 ABG: HCO3 (mmol/L)1Mean Difference (IV, Fixed, 95% CI)Totals not selected

    11.1 Up to 3 months
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 12 ABG: SaO2 (%)1Mean Difference (IV, Fixed, 95% CI)Totals not selected

    12.1 Up to 3 months
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 13 Exercise performance (MSWT) (metres)1Mean Difference (IV, Fixed, 95% CI)Totals not selected

    13.1 Up to 3 months
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 14 Total sleep time (min)2Mean Difference (IV, Fixed, 95% CI)Totals not selected

    14.1 Single night
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

    14.2 Up to 3 months
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 15 REM sleep architecture2Mean Difference (IV, Fixed, 95% CI)Totals not selected

    15.1 REM (single night)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

    15.2 REM %TST (single night)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

    15.3 REM % TST (up to 3 months)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 16 Sleep latency (min)3Mean Difference (IV, Fixed, 95% CI)Subtotals only

    16.1 Single night
238Mean Difference (IV, Fixed, 95% CI)2.93 [-0.32, 6.19]

    16.2 Up to 3 months
115Mean Difference (IV, Fixed, 95% CI)-5.0 [-19.17, 9.17]

 17 Nocturnal oxygen saturation (%)2Mean Difference (IV, Fixed, 95% CI)Totals not selected

    17.1 Mean SpO2 REM (single night)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

    17.2 Mean SpO2 NREM (single night)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

    17.3 Mean SpO2 for TST (up to 3 months)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

    17.4 TST with SpO2 < 90% (up to 3 months)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 18 Nocturnal oxygen saturation (%)1Mean Difference (Fixed, 95% CI)Totals not selected

    18.1 Percentage TST SpO2>90%
1Mean Difference (Fixed, 95% CI)0.0 [0.0, 0.0]

    18.2 Percentage REM SpO2>90%
1Mean Difference (Fixed, 95% CI)0.0 [0.0, 0.0]

    18.3 Percentage NREM SpO2>90%
1Mean Difference (Fixed, 95% CI)0.0 [0.0, 0.0]

 19 Nocturnal TcCO2 (mmHg2Mean Difference (IV, Fixed, 95% CI)Totals not selected

    19.1 TcCO2 during REM (single night)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

    19.2 TcCO2 during NREM (single night)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

    19.3 Mean change PtCO2 (mmHg) (up to 3 months)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

    19.4 Mean change PaCO2 (mmHg) (up to 3 months)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 20 Nocturnal TcCO2 (mmHg)1Mean Difference (Fixed, 95% CI)Totals not selected

    20.1 Mean change TcCO2 NREM to REM (single night)
1Mean Difference (Fixed, 95% CI)0.0 [0.0, 0.0]

    20.2 Maximum TcCO2 (single night)
1Mean Difference (Fixed, 95% CI)0.0 [0.0, 0.0]

 21 Nocturnal TcCO2 TST (mmHg)1Mean Difference (IV, Fixed, 95% CI)Totals not selected

    21.1 Mean PtCO2 TST (up to 3 months)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

    21.2 Maximum PtCO2 TST (up to 3 months)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 22 Hypopneas1Relative rate (Fixed, 95% CI)Totals not selected

    22.1 Single night
1Relative rate (Fixed, 95% CI)0.0 [0.0, 0.0]

 
Comparison 3. NIV in overnight ventilation compared to room air

Outcome or subgroup titleNo. of studiesNo. of participantsStatistical methodEffect size

 1 CF QoL chest symptom score1Mean Difference (IV, Fixed, 95% CI)Totals not selected

    1.1 Up to 3 months
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 2 CF QoL traditional dyspnoea index score1Mean Difference (IV, Fixed, 95% CI)Totals not selected

    2.1 Up to 3 months
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 3 Symptoms of sleep disordered breathing1Mean Difference (IV, Fixed, 95% CI)Totals not selected

    3.1 Epworth sleepiness scale (up to 3 months)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

    3.2 Global PSQI (up to 3 months)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 4 Lung function during sleep1Mean Difference (Fixed, 95% CI)Totals not selected

    4.1 VI while awake (single night)
1Mean Difference (Fixed, 95% CI)0.0 [0.0, 0.0]

    4.2 VI during REM (single night)
1Mean Difference (Fixed, 95% CI)0.0 [0.0, 0.0]

    4.3 VI during NREM (single night)
1Mean Difference (Fixed, 95% CI)0.0 [0.0, 0.0]

    4.4 VT while awake (single night)
1Mean Difference (Fixed, 95% CI)0.0 [0.0, 0.0]

    4.5 VT during REM (single night)
1Mean Difference (Fixed, 95% CI)0.0 [0.0, 0.0]

    4.6 VT during NREM (single night)
1Mean Difference (Fixed, 95% CI)0.0 [0.0, 0.0]

 5 Lung function while awake1Mean Difference (IV, Fixed, 95% CI)Totals not selected

    5.1 FEV1% predicted (up to 3 months)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

    5.2 FVC % predicted (up to 3 months)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 6 Respiratory rate(breaths/min) during sleep1Mean Difference (Fixed, 95% CI)Totals not selected

    6.1 RR while awake (single night)
1Mean Difference (Fixed, 95% CI)0.0 [0.0, 0.0]

    6.2 RR during REM (single night)
1Mean Difference (Fixed, 95% CI)0.0 [0.0, 0.0]

    6.3 RR during NREM (single night)
1Mean Difference (Fixed, 95% CI)0.0 [0.0, 0.0]

 7 Mean respiratory rate1Mean Difference (IV, Fixed, 95% CI)Totals not selected

    7.1 Up to 3 months
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 8 ABG: pH1Mean Difference (IV, Fixed, 95% CI)Totals not selected

    8.1 Up to 3 months
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 9 ABG: PaO2 (mmHg)1Mean Difference (IV, Fixed, 95% CI)Totals not selected

    9.1 Up to 3 months
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 10 ABG: PaCO2 (mmHg)1Mean Difference (IV, Fixed, 95% CI)Totals not selected

    10.1 Up to 3 months
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 11 ABG: HCO3 (mmol/L)1Mean Difference (IV, Fixed, 95% CI)Totals not selected

    11.1 Up to 3 months
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 12 ABG: SaO2 (%)1Mean Difference (IV, Fixed, 95% CI)Totals not selected

    12.1 Up to 3 months
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 13 Exercise performance (metres)1Mean Difference (IV, Fixed, 95% CI)Totals not selected

    13.1 Up to 3 months
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 14 Total sleep time (min)2Mean Difference (IV, Fixed, 95% CI)Totals not selected

    14.1 Single night
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

    14.2 Up to 3 months
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 15 REM sleep architecture2Mean Difference (IV, Fixed, 95% CI)Totals not selected

    15.1 REM (single night)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

    15.2 REM %TST (single night)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

    15.3 REM % TST (up to 3 months)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 16 Sleep latency3Mean Difference (IV, Fixed, 95% CI)Subtotals only

    16.1 Single night
238Mean Difference (IV, Fixed, 95% CI)-2.63 [-7.37, 2.11]

    16.2 At 6 weeks
115Mean Difference (IV, Fixed, 95% CI)-3.00 [-19.88, 13.88]

 17 Nocturnal oxygen saturation (%)2Mean Difference (IV, Fixed, 95% CI)Totals not selected

    17.1 Mean SpO2 REM (single night)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

    17.2 Mean SpO2 NREM (single night)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

    17.3 Mean SpO2 for TST (up to 3 months)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

    17.4 TST for SpO2 < 90% (up to 3 months)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 18 Nocturnal oxygen saturation (%)1Mean Difference (Fixed, 95% CI)Totals not selected

    18.1 Percentage TST SpO2 > 90% (single night)
1Mean Difference (Fixed, 95% CI)0.0 [0.0, 0.0]

    18.2 Percentage REM SpO2 > 90% (single night)
1Mean Difference (Fixed, 95% CI)0.0 [0.0, 0.0]

    18.3 Percentage NREM SpO2 > 90% (single night)
1Mean Difference (Fixed, 95% CI)0.0 [0.0, 0.0]

 19 Nocturnal TcCO2 (mmHg)2Mean Difference (IV, Fixed, 95% CI)Totals not selected

    19.1 TcCO2 during REM (single night)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

    19.2 TcCO2 during NREM (single night)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

    19.3 Mean change PtCO2 (up to 3 months)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

    19.4 Mean change PaCO2 (up to 3 months)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 20 Nocturnal TcCO2(mmHg)1Mean Difference (Fixed, 95% CI)Totals not selected

    20.1 Mean change TcCO2 NREM to REM (single night)
1Mean Difference (Fixed, 95% CI)0.0 [0.0, 0.0]

    20.2 Maximum TcCO2 (single night)
1Mean Difference (Fixed, 95% CI)0.0 [0.0, 0.0]

 21 Nocturnal TcCO2 TST (mmHg)1Mean Difference (IV, Fixed, 95% CI)Totals not selected

    21.1 Mean PtCO2 TST (up to 3 months)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

    21.2 Max PtCO2 TST (up to 3 months)
1Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]

 22 Hypopneas1Relative rate (Fixed, 95% CI)Totals not selected

    22.1 Single night
1Relative rate (Fixed, 95% CI)0.0 [0.0, 0.0]

 
Table 1. List of abbreviations

AbbreviationDefinitionExplanation

ABGanalysis of blood gases

CFcystic fibrosis

COPDchronic obstructive pulmonary disease

CPAPcontinuous positive airway pressurea system that maintains a positive pressure in the circuitry and airways throughout inspiration and expiration

CPTchest physiotherapy

CSSChest symptom scoreValidated CF Quality of Life Measurement.

Scale: 0 = worst; 100 = best.

ESSEpworth sleepiness scaleScale: 0 = best; 24 = worst.

FEF25-75flow rate between 25 and 75% of maximal expiration

FEV1forced expiratory volume in 1 second

FRCfunctional residual capacityresting volume of the lungs

FVCforced vital capacitytotal volume of air expired during a forced expiration following a full inspiration

Global PSQIGlobal score Pittsburgh sleep quality indexScale: 0 = best; 21 = worst.

MEF50maximal expiratory flow with 50% of vital capacity remaining in the lung

mmHgmillimetres of mercury

mSpO2mean oxygen saturation

MSWTmodified shuttle walk testIncremental exercise tolerance test with minimum clinically important difference = 40m.

nadirSpO2the largest fall expressed in the absolute value of SpO2

NIPPVnon-invasive positive pressure ventilation

NIVnon-invasive ventilation

NREMnon-rapid eye movementa phase during sleep

PaCO2partial pressure of carbon dioxide in arterial blood

PaO2partial pressure of oxygen in arterial blood

PEPpositive expiratory pressurean airway clearance technique

PSVpressure support ventilation

QoLquality of life

RDIrespiratory disturbance index

REMrapid eye movementa phase during sleep

RRrespiratory rate

SaO2saturation of haemoglobin with oxygen in arterial blood

SDstandard deviation

SpO2saturation of haemoglobin with oxygen using pulse oximetry

*SpO2 maxthe largest fall expressed as the difference with the SpO2 just before the manoeuvre

*SpO2 meanthe mean of *SpO2 max during the whole chest physiotherapy period

TcCO2transcutaneous carbon dioxide

TDItransitional dyspnoea indexCF QoL measurement.

Score: -9 = worst; +9 = best.

MCID = 1 unit.

TLCtotal lung capacitytotal volume of air in lungs following a maximum inspiration

TSTtotal sleep time

VIminute ventilation

VTtidal volumevolume air inspired or expired during normal breathing

IPAPinspiratory positive airway pressure

PIMaxinspiratory respiratory muscle strength

PEMaxexpiratory respiratory muscle strength