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Neuromuscul Disord. 2016 Oct;26(10):666-674. doi: 10.1016/j.nmd.2016.07.014. Epub 2016 Aug 1.

Factors influencing compliance with non-invasive ventilation at long-term in patients with myotonic dystrophy type 1: A prospective cohort.

Author information

1
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.
2
Université de Versailles Saint Quentin en Yvelines, INSERM U1179, France; Service d'Explorations Fonctionnelles Respiratoires, AP-HP, Hôpital Raymond Poincaré, 92380 Garches, France.
3
CIC 1429, INSERM, AP-HP, Hôpital Raymond Poincaré, 92380 Garches, France.
4
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.
5
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.

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.

KEYWORDS:

Compliance; Myotonic dystrophy; Non-invasive ventilation; Prospective cohort; Respiratory care

PMID:
27542439
DOI:
10.1016/j.nmd.2016.07.014
[Indexed for MEDLINE]

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