Practice of Noninvasive Ventilation for Cystic Fibrosis: A Nationwide Survey in France

Authors: Fauroux, Brigitte1; Burgel, Pierre-Régis2; Boelle, Pierre-Yves3; Cracowski, Claire4; Murris-Espin, Marlène5; Nove-Josserand, Raphaele6; Stremler, Nathalie7; Derlich, Laurence8; Giovanetti, Philippe9; Clément, Annick10

Source: Respiratory Care, Volume 53, Number 11, November 2008 , pp. 1482-1489(8)

Publisher: The Journal Respiratory Care Company

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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.

Keywords: CF; CYSTIC FIBROSIS; GUIDELINES; HOME CARE; HYPERCAPNIA; NONINVASIVE VENTILATION; PRESCRIPTION; RESPIRATORY FAILURE

Document Type: Research Article

Affiliations: 1: Pediatric Pulmonary Department, Hôpital Armand Trousseau, Centre de Ressources et de Compétences pour la Mucoviscidose (CRCM), Université Pierre et Marie Curie - Paris 6, Institut National de la Santé et de la Recherche Médicale, INSERM UMR S-893 Equipe 12, 28 Avenue du Docteur Arnold Netter, Paris, France F-75012 France;, Email: brigitte.fauroux@trs.aphp.fr 2: Pulmonary Department, Hôpital Cochin, Assistance Publique Hôpitaux de Paris (AP-HP), University of Paris 5, Paris, France 3: Biostatistics Department, Hôpital Saint Antoine, AP-HP, Université Pierre et Marie Curie - Paris 6, INSERM UMR S-707, Paris, France 4: Pulmonary Department, Centre Hospitalier Universitaire (CHU) de Grenoble, Grenoble, France 5: Pôle des Voies Respiratoires, CRCM Adulte, Hôpital Larrey, Hôpitaux de Toulouse, France 6: CRCM Adulte, Centre Hospitalier (CH) Lyon Sud, Lyon, PierreBénite, France 7: Pediatrics Department, CRCM Timone-Enfants, Hôpital La Timone, Marseille, France 8: CRCM Adulte, CHU de Bordeaux, Groupe Hospitalier Sud, Bordeaux, France 9: Fédération des Maladies Respiratoires, CRCM Adulte, Hôpital Sainte Marguerite, Marseille, France 10: Pediatric Pulmonary Department, Hôpital Armand Trousseau, Centre de Ressources et de Compétences pour la Mucoviscidose (CRCM), Université Pierre et Marie Curie - Paris 6, Institut National de la Santé et de la Recherche Médicale (INSERM), Unité Mixte de Recherche (UMR) S-893 Equipe 12, Paris, France

Publication date: November 1, 2008

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