title: Factors influencing compliance with non-invasive ventilation at long-term in patients with myotonic dystrophy type 1: A prospective cohort.
abstract: This study evaluated compliance with non-invasive ventilation in patients with myotonic dystrophy type 1 and identified predictors of cessation at 5 years in a cohort of patients followed in a specialist center for Neuromuscular Diseases in France. Mechanical ventilation in these patients poses a very strong challenge to caregivers. Factors predicting relative compliance were identified using multivariate linear regressions. Cox proportional-hazards regression was used to estimate hazard ratios associated with risk of cessation. One hundred and twenty-eight patients were included. Compliance during the first year was higher when symptoms of respiratory failure were initially present (+25%, p < 0.003) and lower in the case of acute respiratory failure (-29%, p < 0.003). Long-term compliance was associated with symptoms of respiratory failure (+52%, p < 0.0001) and nocturnal arterial oxygen desaturation (+23%, p < 0.007). Cessation was more frequent in the cases of excessive leaks (HR = 7.81, IC [1.47-41.88], p < 0.01), ventilator dysfunction requiring emergency technical intervention (HR = 12.58, IC [1.22-129.69], p < 0.03) or high body mass index (p < 0.02). Cessation was less frequent for patients with a professional occupation or undergoing professional training (HR = 0.11, IC [0.02-0.77], p < 0.02). Compliance with non-invasive ventilation is poor in patients with no subjective symptoms of respiratory failure. It may be improved with appropriate education and follow-up.
authors.affiliation: CIC 1429, INSERM, AP-HP, Hôpital Raymond Poincaré, 92380 Garches, France; Université de Versailles Saint Quentin en Yvelines, INSERM U1179, France. Electronic address: boussaid.gh@gmail.com.
authors.forename: Ghilas
authors.initials: G
authors.lastname: Boussaïd
authors.affiliation: Université de Versailles Saint Quentin en Yvelines, INSERM U1179, France; Service d'Explorations Fonctionnelles Respiratoires, AP-HP, Hôpital Raymond Poincaré, 92380 Garches, France.
authors.forename: Frédéric
authors.initials: F
authors.lastname: Lofaso
authors.affiliation: Université de Versailles Saint Quentin en Yvelines, INSERM U1179, France; Service d'Explorations Fonctionnelles Respiratoires, AP-HP, Hôpital Raymond Poincaré, 92380 Garches, France.
authors.forename: Dante Brasil
authors.initials: DB
authors.lastname: Santos
authors.affiliation: CIC 1429, INSERM, AP-HP, Hôpital Raymond Poincaré, 92380 Garches, France.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Vaugier
authors.affiliation: CIC 1429, INSERM, AP-HP, Hôpital Raymond Poincaré, 92380 Garches, France.
authors.forename: Sandra
authors.initials: S
authors.lastname: Pottier
authors.affiliation: Université de Versailles Saint Quentin en Yvelines, INSERM U1179, France; Service d'Explorations Fonctionnelles Respiratoires, AP-HP, Hôpital Raymond Poincaré, 92380 Garches, France.
authors.forename: Hélène
authors.initials: H
authors.lastname: Prigent
authors.affiliation: CIC 1429, INSERM, AP-HP, Hôpital Raymond Poincaré, 92380 Garches, France; Université de Versailles Saint Quentin en Yvelines, INSERM U1179, France; Service de Santé Publique, AP-HP, Hôpital Raymond Poincaré, 92380 Garches, France.
authors.forename: David
authors.initials: D
authors.lastname: Orlikowski
authors.affiliation: CIC 1429, INSERM, AP-HP, Hôpital Raymond Poincaré, 92380 Garches, France; Service de Santé Publique, AP-HP, Hôpital Raymond Poincaré, 92380 Garches, France; Université de Versailles Saint Quentin en Yvelines, EA 4047, France; Pôle de Ventilation à Domicile, AP-HP, Hôpital Raymond Poincaré, 92380 Garches, France.
authors.forename: Stéphane
authors.initials: S
authors.lastname: Bahrami
mesh_terms: D000328:Adult; D015992:Body Mass Index; D004868:Equipment Failure; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D053208:Kaplan-Meier Estimate; D016014:Linear Models; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D009223:Myotonic Dystrophy / Q000453:epidemiology* / Q000628:therapy*; D063087:Noninvasive Ventilation; D009790:Occupations; D010349:Patient Compliance; D016016:Proportional Hazards Models; D011446:Prospective Studies; D012131:Respiratory Insufficiency / Q000453:epidemiology / Q000628:therapy; D012306:Risk; D014797:Vital Capacity; D055815:Young Adult
keywords: Compliance; Myotonic dystrophy; Non-invasive ventilation; Prospective cohort; Respiratory care