comparison of two methods of adjusting the mechanical in-exsuflation in neuromuscular adult patients

Conditions: Neuromuscular Diseases

Summary: in the context of neuromuscular diseases, the weakness of the respiratory muscles makes difficult to manage periods of congestion due to a less effective cough. it exposes the patient to bronchial superinfections that may require hospitalization. the use of cough assistance is therefore essential in order to avoid acute situations such as bronchial superinfections. mechanical insuflation-exsufflation (mi / e) is an instrumental technique commonly used by therapists in a hospital or at the patient's home in order to facilitate the airway mucus clearance. the choice of the pressure settings of the im / e is a decisive point because it conditions the effectiveness of the generated cough. it'is important to take into account the presence of any collapse in the use of im / e in order to ensure the most effective cough given by the patient. actually, except the common impression of the physiotherapist and the patient, there is no "simple" way for detecting and objectifying the threshold of collapse in the upper airways. the aim of this study is to compare two modalities for the regulation of the level of the mi-e exsufflation, taking into account the presence of a collapse: a subjective "s" adjustment based on the clinical judgment of the therapist and the patient and an objective "o" adjustment based on the flow-volume curve generated during the cough.

this is a bicentric, prospective crossover, randomized, open label trial. the study will focus on 50 adult patients with stable neuromuscular conditions, outside the congestion period requiring cough help. patients will be recruited from among the neuromuscular disease patients followed for their respiratory management in the home ventilation unit of the raymond poincaré hospital intensive care unit or in the aincourt neurological rehabilitation service attached to the vexin intercommunal hospital group. the cough help performed with the mi / e will be evaluated according to two conditions of adjustment of the exsufflation pressure: a subjective adjustment (modality s) and an objective adjustment (modality o), whose order will be randomized to control a phenomenon of tiredness causing confusion. a respiratory technician and two therapists will participate to the protocol. the therapist "clinician" and "evaluator" role will be defined by a draw. the protocol will take place during the same day of hospitalization in two stages: * t0: validation of the eligibility criteria and inclusion of patients in the study. * t1: evaluation of coughing performance according to the two modes of exsufflation pressure adjustment. adjustment modality "s" according to the clinical impression of the "clinician" therapist and the patient, and the "cough peak expiratory flow" measured by the mi / e. adjustment modality "o": according to the analysis of the flow / volume curve measured using a spirometer and a pneumotachograph.

Eligibility criteria:
inclusion criteria: * aged ≥18 years * neuromuscular pathology * coughing aid information: dept \<180 l / min * in stable condition and without bulk * good understanding and cognition to follow the instructions and to inform the eva * affiliated to a sickness insurance plan or entitled * signed of informed consent exclusion criteria: * pregnant woman * person under guardianship * inability to cooperate and / or communicate * acute decompensated organ * patient with following contraindications to mechanical in-exsufflation (mi-e) devices: 1. pneumothorax 2. pneumomediastinum 3. pulmonary emphysema 4. hemoptysis 5. pleurisy 6. nausea 7. obstructive pulmonary disease or asthma 8. recent lobectomy of the lung 9. intracranial pressure

Keywords: neuromuscular disease