Journal article

Effects of 1-month withdrawal of ventilatory support in hypercapnic myotonic dystrophy type 1

Fergal J O'Donoghue, Jean-Christian Borel, Yves Dauvilliers, Patrick Levy, Renaud Tamisier, Jean-Louis Pepin

RESPIROLOGY | WILEY | Published : 2017

Abstract

BACKGROUND AND OBJECTIVE: The benefits of domiciliary non-invasive ventilation (NIV) in myotonic dystrophy type 1 (DM1) are unclear. We sought to determine the effects of elective discontinuation of ventilatory support for 1 month in DM1 patients receiving NIV for chronic hypercapnic respiratory failure. METHODS: At baseline, 12 patients underwent polysomnography, and assessment of subjective (Epworth Sleepiness Scale) and objective (Oxford Sleep Resistance Test) sleepiness, fatigue (Fatigue Severity Scale), respiratory function including muscle strength, arterial blood gas (ABG), hypercapnic ventilatory response (HCVR), Blood Pressure, peripheral arterial tonometry (PAT) and pulse wave velo..

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Funding Acknowledgements

The authors are grateful to Mlle Marion Perrin for assistance in statistical analysis, and to our colleagues in the Departments of Respiratory and Sleep medicine in the University Hospitals of Grenoble and Lille and Raymond Poincare Hospital, Garches, France. This study was funded by Association Francaise de Lutte contre les Myopathies (French Myopathy Association) and by Fonds de Dotation AGIR pour les Maladies Chroniques (Charitable Foundation of Agir a Dom, a non-profit French Home Care provider).