Abstract

IntroductionHome mechanical ventilation has significantly improved survival in patients with slowly progressive neuromuscular diseases and chronic respiratory failure. Specifically, the development of non-invasive ventilation (NIV) has provided a genuine alternative to tracheostomy ventilation for patients requiring long-term mechanical ventilation. MethodsPhD thesis from the university Paris Saclay prepared in the ERPHAN research unit (université Paris-Saclay, UVSQ) under the supervision of Frédéric Lofaso, professeur des universités. ResultsIn this thesis, we have evidenced that during the initiation of NIV in patients with slowly progressive NMD, the intensity of ventilatory parameters does not significantly affect daytime carbon dioxide level, as opposed to adherence, which is its main determining factor. We have also shown that the algorithms for automatic adjustment of the main ventilatory parameters are currently very heterogeneous and all raise important concerns. Independently of the ventilatory parameters, we have also highlighted the major impact of the configuration of the ventilation circuits on the efficiency of NIV. Finally, in the most severely affected and ventilator-dependent patients, we identified that quality of life, the main target of long-term ventilation, is more likely to be explained by the social and material environment than by the ventilation technique itself (invasive or non-invasive). DiscussionTechnological innovations in the field of home ventilation are constantly opening up new possibilities, making their evaluation and optimization an ongoing and sustainable clinical challenge.

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