Abstract
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.
- noninvasive ventilation
- cystic fibrosis
- CF
- respiratory failure
- hypercapnia
- home care
- prescription
- guidelines
Footnotes
- Correspondence: Brigitte Fauroux MD PhD, AP-HP, Hôpital Armand Trousseau, Pediatric Pulmonary Department, Université Pierre et Marie Curie - Paris 6, INSERM UMR S-893 Equipe 12, 28 Avenue du Docteur Arnold Netter, Paris, France F-75012 France. E-mail: brigitte.fauroux{at}trs.aphp.fr.
Dr Fauroux's research is supported by the Association Française Contre les Myopathies, Vaincre la Mucoviscidose, Assistance Publique - Hôpitaux de Paris, INSERM, Legpoix, and Université Pierre et Marie Curie - Paris 6. The authors report no other conflicts of interest related to the content of this paper.
- Copyright © 2008 by Daedalus Enterprises Inc.