Breathing With Amyotrophic Lateral Sclerosis

Amyotrophic Lateral Sclerosis (ALS) is a rare neurodegenerative pathology without curative treatment at present, the outcome is fatal after 3 to 5 years of evolution on average. The disease results in progressive paralysis of the muscles involved in voluntary motricity (progressive and intricate impairment of locomotor, phonation and swallowing capacities as well as ventilatory function). In France, the management of ALS is based on a diversified multidisciplinary follow-up (physiotherapy, speech therapy, occupational therapy, neuropsychology) in the ALS Reference Center in conjunction with the city care networks. It is centered on the evaluation, and the early compensation of the various symptoms, especially the respiratory problems.

Thus, this study aims to evaluate the effect of mechanical insufflator-exsufflator on the respiratory functions of these patients, evaluated via cough peak expiratory flow measurements (CPEF).

The study is conducted in collaboration with the physiotherapists of each patient enrolled, to evaluate the respiratory function by peak expiratory flow on cough (PEFC) after 4 weeks of treatment with Mechanical In-Exsufflator (MIE) (3 weekly sessions for two weeks, then one daily session for 2 weeks) in ALS patients presenting with a poorly effective cough (PEFC between 160 and 255 L/min) and having an indication of punctual MIE.

Inclusion criteria

• Confirmed ALS categorized as probable or certain according to the revised Awaji-Shima criteria [27].
• ALS of spinal, bulbar or respiratory forms
• Peak Flow measure between 160 and 255 L/min (recommendation of MIE in case of congestion, with the possibility of daily sessions)
• Patient who has not benefited from treatment by MIE in the past year (less than 10 sessions in total)
• Patient able to use the MIE and perform the collection, or having a caregiver able to help
• Patient receiving physiotherapist treatment greater than or equal to twice a week

Exclusion criteria

• Psychiatric (DSM-5) or cognitive disorders incompatible with participation in the study
• Contraindication to the use of an MIE (pneumothorax for example)
• Insufficient level in French for understanding the study and completing the questionnaires
• Current or past participation in another innovative research or care program relating to respiratory functions
• Emergency situation for which the MIE must be set up within less than a week

Exclusion during the study :

• Appearance of a serious contraindication to the practice of MIE during the study (for example rib fracture during a fall)
• Patient's physiotherapist refusing to do the study