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Quality of life in patients with slowly progressive neuromuscular disorders dependent on mechanical ventilation
  1. Mathieu Delorme1,2,
  2. Christian Reveillere3,
  3. Christian Devaux2,
  4. Sandrine Segovia-Kueny2,
  5. Frédéric Lofaso1,4,
  6. Ghilas Boussaid1,2
  1. 1 UVSQ, ERPHAN, Universite Paris-Saclay, Versailles, France
  2. 2 Direction des Actions Médicales, AFM-Téléthon, Evry, France
  3. 3 Département de Psychologie, EE QualiPsy, Université de Tours, Tours, France
  4. 4 Unité des pathologies du sommeil, Service de physiologie - explorations fonctionnelles, Hôpital Raymond Poincaré, AP-HP, Garches, France
  1. Correspondence to Mr Mathieu Delorme, UVSQ, ERPHAN, Universite Paris-Saclay, 78000, Versailles, France;{at}


This cross-sectional study evaluated health-related quality of life (HRQoL) in patients with slowly progressive neuromuscular disorders dependent on mechanical ventilation (MV; ≥16 hours/day). 119 participants, with 9 years (25th–75th percentiles: 4–15 years) of MV dependence, were included. MV was applied via a tracheostomy in 80 participants (67.2%) and non-invasive interfaces in 39 participants (32.8%), including 28 participants (71.8%) with daytime mouthpiece ventilation. HRQoL was rated good or excellent by 81 participants (68.1%), independently from age, diagnosis or respiratory autonomy. On multivariate analysis, time since MV initiation, independence from family member(s), residence in a rural area, ability to go outdoors with MV and tracheostomy were associated with better HRQoL.

  • Non invasive ventilation
  • Assisted Ventilation

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  • Contributors All authors contributed substantially to conceive and design the study, revised the manuscript for important intellectual content, approved the final version, agree to be accountable for all aspects of the work and to ensure that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. MD, GB and FL contributed to acquire, analyse and interpret the data and to draft the manuscript.

  • Funding This research received fundings from the Fondation de France, the AFM-Téléthon and the Fondation Garches which was also the promotor of this study.

  • Competing interests MD reports grants from Fondation de France, AFM Téléthon and Fondation Garches, during the conduct of the study; personal fees from Air Liquide Medical Systems, Breas Medical AB, ResMed SAS and GSK; and non-financial support from L3 Medical, outside the submitted work. Other authors have nothing to disclose.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.

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