The Importance of Positive Expiratory Pressure Associated with the In-exsufflator in Patients with Amyotrophic Lateral Sclerosis on the Effectiveness of Therapy
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
• Patient followed by the ALS mobile team of the Groupe Hospitalier du Havre or the CHU of Dijon
• Patient whose disease progression kinetics is medically deemed compatible with inclusion in the study
• Patient willing to participate in the research after receiving adequate information and the information letter.
• Patient affiliated with social security or a beneficiary of such a scheme.