Amyotrophic Lateral Sclerosis (ALS) is a fatal neurodegenerative disorder that impairs motor neurons, with a life expectancy of 2 to 7 years after diagnosis. ALS manifests as 'spinal' when it primarily affects limbs, or 'bulbar' when it impairs speech and swallowing. The disease progressively weakens all skeletal muscles, causing respiratory issues and increased risk of lung infections due to ineffective coughing. Mechanical cough assistance via In-exsufflation therapy/ mechanical in-exsufflator devie (INEX/MI-E) applies positive and negative airway pressures non-invasively to improve coughing. However, MI-E may fail in some ALS patients due to airway collapse, often related to brainstem muscle dysfunction.Research by Andersen et al. in 2017 highlighted that during MI-E, ALS patients often experience adverse laryngeal movements, which can obstruct airways and reduce the therapy's effectiveness. To combat this, they suggested individualized MI-E settings to minimize airway collapse. Modern MI-E devices, such as the EOVE-70, offer adjustable positive expiratory pressure (PEP) between cycles to potentially enhance airway stability and coughing efficiency. The current study focuses on the impact of PEP during therapy pauses on the peak expiratory flow rate in ALS patients, which could lead to improved therapeutic outcomes.
Male or female patient aged over 18 years
Patient with Amyotrophic Lateral Sclerosis (ALS)
Patients with established or beginning bulbar disorders, identified by the healthcare team (speech impairment, hypersalivation, or swallowing difficulties)
Patient naive to INEX therapy but prescribed for its installation or patient already treated by an INEX device
Mental illness interfering with the proper use of the device
History of laryngospasm
Inability to come for consultation with the ALS team of the Groupe Hospitalier du Havre or the CHU of Dijon
Pregnancy
Crossover
MI-E with PEP function activated during the pause
DevicePEP function will be activated during the pause when using the EOVE-70. The PEP function in cmH2O will start at 8 cmH2O; the PEP setting can be decreased or increased at the discretion of the practitioner to optimize. The other therapeutic settings (positive pressure, negative pressure, inspiratory time, expiratory time and pause time) will be set individually for each patient by an experienced physiotherapist in order to reach the best clinical efficacy (usual clinical care)
MI-E without PEP function activated during the pause
DeviceThe PEP function will not be activated during the pause when using the EOVE-70. The other therapeutic settings (positive pressure, negative pressure, inspiratory time, expiratory time and pause time) will be set individually for each patient by an experienced physiotherapist in order to reach the best clinical efficacy (usual clinical care)
The average difference in PEF (Peak Expiratory Flow) in L/min with the use of the PEP function and without the use of this function.
We aim to study the effect of using PEP during the pause on cough peak expiratory flow (PEF) in patients with ALS during MI-E therapy. The PEF from all treatment cycles measured by an external pneumotachograph connected to the circuit between the machine and the patient will be averaged and compared between the arms: with and without PEP during the pause
The average difference in inspiratory volume in mililiters with the use of the PEP function and without the use of this function.
We aim to study the effect of using PEP during the pause on inspiratory volume delivered in patients with ALS during MI-E therapy. The inspiratory volume from all treatment cycles measured by an external pneumotachograph connected to the circuit between the machine and the patient will be averaged and compared between the arms: with and without PEP during the pause
The perceived effect of PEP during pause on the effectiveness of MI-E therapy by the patient as well as their perceived comfort
We wish to measure the average difference on the VAS (Visual Analogue Scale) with the use of the PEP function and without the use of this function, for the sensation of effectiveness and comfort reported by the patient
Qualitative analysis of cough using flow/time graphs of patient coughs with the use of the PEP function and without the use of this function.
We wish to measure qualitatively (visually in post-analysis) the flow/time data recorded by the external pneumotachograph with the use of the PEP function and without the use of this function.
Comparison of PEF (Peak Expiratory Flow) reported by the MI-E machine versus PEF measured by the external pneumotachograph in L/min
We wish to compare the data reported by the device versus the data externaly recorded for a post-analysis comparison.
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Groupe Hospitalier du Havre
Lead sponsor
French Physiotherapy Society / Société Français de Physiothérapie
Collaborator
