CYSTIC FIBROSIS

Randomised placebo controlled trial of non-invasive ventilation for hypercapnia in cystic fibrosis

A C Young^1,2, J W Wilson^1,2, T C Kotsimbos¹, M T Naughton^1,2

¹ Department of Allergy Immunology and Respiratory Medicine, The Alfred Hospital, Melbourne, Victoria, Australia
² Department of Medicine, Monash University, Melbourne, Victoria, Australia

Dr A C Young, Department of Allergy Immunology and Respiratory Medicine, The Alfred Hospital, Commercial Rd, Prahran, Melbourne, VIC, 3181, Australia; alan.young{at}med.monash.edu.au

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.

Conclusion: 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.

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