Randomised placebo controlled trial of non-invasive ventilation for hypercapnia in cystic fibrosis.
BACKGROUND
The clinical benefits of domiciliary non-invasive positive pressure ventilation (NIV) have not been established in cystic fibrosis (CF). We studied the effects of nocturnal NIV on quality of life (QoL), functional and physiological outcomes in CF subjects with awake hypercapnia (arterial carbon dioxide pressure PaCO2>45 mm Hg).


METHODS
In a randomised, placebo controlled, crossover study, eight subjects with CF, mean (SD) age 37 (8) years, body mass index 21.1 (2.6) kg/m2, forced expiratory volume in 1 s 35 (8)% predicted and PaCO2 52 (4) mm Hg received 6 weeks of nocturnal (1) air (placebo), (2) oxygen and (3) NIV. The primary outcome measures were CF specific QoL, daytime sleepiness and exertional dyspnoea. Secondary outcome measures were awake and asleep gas exchange, sleep architecture, lung function and peak exercise capacity.


RESULTS
Compared with air, NIV improved the chest symptom score in the CF QoL Questionnaire (mean difference 10; 95% CI 5 to 16; p = 0.002) and the transitional dyspnoea index score (mean difference 3.1; 95% CI 1.2-5.0; p = 0.01). It reduced maximum nocturnal pressure of transcutaneous CO2 (PtcCO2 mean difference -17 mm Hg; 95% CI -7 to -28 mm Hg; p = 0.005) and increased exercise performance on the Modified Shuttle Test (mean difference 83 m; 95% CI 21 to 144 m; p = 0.02). NIV did not improve sleep architecture, lung function or awake PaCO2.


CONCLUSIONS
6 weeks of nocturnal NIV improves chest symptoms, exertional dyspnoea, nocturnal hypoventilation and peak exercise capacity in adult patients with stable CF with awake hypercapnia. Further studies are required to determine whether or not NIV can improve survival.
Department of Allergy Immunology and Respiratory Medicine, The Alfred Hospital, Commercial Rd, Prahran, Melbourne, VIC, 3181, Australia. alan.young@med.monash.edu.au
A C
AC
Young
J W
JW
Wilson
T C
TC
Kotsimbos
M T
MT
Naughton
D000328:Adult; D002245:Carbon Dioxide / Q000097:blood; D003072:Cognition Disorders / Q000628:therapy; D018592:Cross-Over Studies; D003550:Cystic Fibrosis / Q000150:complications*; D015444:Exercise / Q000502:physiology; D005080:Exercise Test; D005260:Female; D005541:Forced Expiratory Volume / Q000502:physiology; D006801:Humans; D006935:Hypercapnia / Q000150:complications / Q000628:therapy*; D008297:Male; D010100:Oxygen / Q000008:administration & dosage / Q000009:adverse effects; D010313:Partial Pressure; D010349:Patient Compliance; D017286:Polysomnography; D011175:Positive-Pressure Respiration / Q000009:adverse effects / Q000379:methods*; D011788:Quality of Life; D012893:Sleep Wake Disorders / Q000150:complications / Q000628:therapy; D016896:Treatment Outcome
D002245:Carbon Dioxide; D010100:Oxygen