Abstract
Background: The clinical benefits of home non-invasive positive pressure ventilation (HNIV) have not been well established in adult patients with cystic fibrosis (CF).
Objective: The purpose of this matched case-control study was to compare the effects of HNIV and long-term oxygen therapy (LTOT) on survival of adult CF patients with chronic hypercapnic respiratory failure (CHRF).
Methods: Twelve patients receiving HNIV were matched with 12 patients receiving LTOT regarding age, FEV1, PaCO2 and BMI.
Results: There were no differences between groups of patients in demographics, clinical and functional characteristics (HNIV patients: 24.9±4.8 years, BMI 16.1±3.3 kg/m2, FEV1 15.3±3.4%, PaCO2 59.8±7.7 mmHg; and LTOT patients: 23.9±4.6 years, BMI 15.8±4.2 kg/m2, FEV1 16.1±4.4%, PaCO2 61.4±7.9 mm Hg). HNIV settings were: ST mode, IPAP 20.1±4.2 cm H2O, EPAP 4.6±0.7 cm H2O (nasal mask – 4, oronasal mask – 8). Compared with LTOT, HNIV significantly reduced breathing rate (p=0.036), PaCO2 (p=0.038) and number of exacerbations (p=0.028). Survival was significantly better in HNIV group in comparison with LTOT group (survival time 12.5 [95%CI 0.6-16.4] months vs 6.0 [95%CI 0.7-7.3] months; log-rank test, p=0.024).
Conclusions: The survival of adult CF patients receiving HNIV was better than that of patients treated with LTOT alone. We suggest HNIV is a first-line treatment for adult CF patients with CHRF.
- © 2012 ERS