RECRUITINGINTERVENTIONAL

Lung Insufflation Capacity Training and Respiratory Function in Amyotrophic Lateral Sclerosis

Exploratory Study on the Effects of Lung Insufflation Capacity Training Using the LIC Trainer in Patients With Amyotrophic Lateral Sclerosis: A Single-Center Prospective Interventional Study

About This Trial

The goal of this clinical trial is to learn whether lung insufflation capacity (LIC) training can help maintain respiratory function and prolong survival in people with amyotrophic lateral sclerosis (ALS). The main questions it aims to answer are: * Does early and continuous LIC training slow the decline in forced vital capacity (FVC)? * Does LIC training delay the need for tracheostomy or noninvasive ventilation (NIV)? This single-center study at the National Center of Neurology and Psychiatry (NCNP) in Japan will enroll 15 adults with ALS, diagnosed according to the El Escorial or Awaji criteria. Participants will: * Use the LIC Trainer device to perform lung insufflation training twice daily at home * Visit the clinic every 3 months for respiratory and functional assessments * Have lung tests, including FVC, LIC, maximum insufflation capacity (MIC), cough peak flow (CPF), and complete the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) There is no control group within this trial; researchers will compare results with matched historical controls from the Pooled Resource Open-Access ALS Clinical Trials (PRO-ACT) database.

Who May Be Eligible (Plain English)

Who May Qualify: - Age 20 years or older - Diagnosis of amyotrophic lateral sclerosis (ALS) according to the El Escorial or Awaji criteria - Not using noninvasive ventilation (NIV) and without tracheostomy at the start of LIC training - Able to perform study assessments and provide written willing to sign a consent form (or assisted signature with communication aid) Who Should NOT Join This Trial: - Chronic pulmonary disease other than ALS (e.g., COPD, interstitial lung disease) - Severe cognitive or communication impairment preventing study participation - Uncontrolled cardiovascular disease, including unstable angina, recent myocardial infarction, decompensated heart failure, serious arrhythmia, severe aortic stenosis, or active myocarditis/endocarditis - Uncontrolled hypertension - Acute systemic illness or fever - Recent pulmonary embolism, acute cor pulmonale, or severe pulmonary hypertension - Severe hepatic or renal dysfunction - Any condition judged inappropriate by the investigator Always talk to your doctor about whether this trial is right for you.

Original Eligibility Criteria

View original clinical language

Inclusion Criteria: * Age 20 years or older * Diagnosis of amyotrophic lateral sclerosis (ALS) according to the El Escorial or Awaji criteria * Not using noninvasive ventilation (NIV) and without tracheostomy at the start of LIC training * Able to perform study assessments and provide written informed consent (or assisted signature with communication aid) Exclusion Criteria: * Chronic pulmonary disease other than ALS (e.g., COPD, interstitial lung disease) * Severe cognitive or communication impairment preventing study participation * Uncontrolled cardiovascular disease, including unstable angina, recent myocardial infarction, decompensated heart failure, serious arrhythmia, severe aortic stenosis, or active myocarditis/endocarditis * Uncontrolled hypertension * Acute systemic illness or fever * Recent pulmonary embolism, acute cor pulmonale, or severe pulmonary hypertension * Severe hepatic or renal dysfunction * Any condition judged inappropriate by the investigator

Treatments Being Tested

DEVICE

LIC Trainer (Lung Insufflation Capacity Trainer)

The LIC Trainer is a medical device designed to enable lung insufflation through a one-way valve system, allowing passive air stacking without voluntary glottic closure. The device includes a safety valve that automatically releases pressure above 60 cmH₂O and a manual relief valve to ensure patient safety. It is used with a bag-valve-mask to deliver multiple assisted insufflations, approximately 10 per session, twice daily. Participants with amyotrophic lateral sclerosis (ALS) perform LIC training at home after initial instruction by physical therapists and are re-evaluated every 3 months for up to 36 months.

Locations (1)

National Center of Neurology and Psychiatry (NCNP)

Kodaira, Tokyo, Japan