RT Journal Article SR Electronic T1 Practice of Noninvasive Ventilation for Cystic Fibrosis: A Nationwide Survey in France JF Respiratory Care FD American Association for Respiratory Care SP 1482 OP 1489 VO 53 IS 11 A1 Brigitte Fauroux A1 Pierre-Régis Burgel A1 Pierre-Yves Boelle A1 Claire Cracowski A1 Marléne Murris-Espin A1 Raphaele Nove-Josserand A1 Nathalie Stremler A1 Laurence Derlich A1 Philippe Giovanetti A1 Annick Clément YR 2008 UL http://rc.rcjournal.com/content/53/11/1482.abstract AB BACKGROUND: No guidelines are available for noninvasive ventilation (NIV) for cystic fibrosis (CF). OBJECTIVE: To survey and evaluate the use of NIV for CF in France. METHODS: We surveyed the coordinator physicians of every accredited CF center in France. RESULTS: The respondents represented 36 centers (15 pediatric centers, 13 adult centers, and 8 centers that see both pediatric and adult patients), which had a total of 4,416 patients with CF at the time of the study, 168 (3.8%) of whom were using NIV. NIV was being used more often in the adults centers (7.6% of these patients) than in the pediatric centers (1.2% of these patients) or adult-and-pediatric centers (4.1% of these patients) (P = .01). All the respondent centers use NIV as first-line treatment for severe hypercapnic respiratory exacerbation and for stable diurnal hypercapnia, especially when associated with sleep disturbance. Bi-level pressure-targeted ventilation is the preferred ventilation mode. Settings are adjusted based on arterial blood gas values, noninvasive evaluation of patient-ventilator synchrony, patient comfort, and sometimes a sleep study. The surveyed centers reported a number of expected benefits from NIV, but few of those benefits have been proven. Problems with NIV are common and limit its use. CONCLUSIONS: We found a relative homogeneity in these French centers' stated indications for and use of NIV, which highlights their numerous expectations about the benefits of NIV, which contrasts with the few validated benefits. Studies of the benefits of NIV are needed.