Lung Volume Recruitment Combined With Expiratory Muscle Strength Training in ALS

Study Overview
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Brief Summary
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The purpose of this study is to investigate the effects of two treatment techniques called Expiratory Muscle Strength Training (EMST) and Lung Volume Recruitment (LVR) on breathing, swallowing, speech, and cough function in persons with mild to moderate ALS. Half of the participants will do EMST alone, and the other half of the participants will do EMST and LVR.

Official Title
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A Pilot Study of Lung Volume Recruitment Combined With Expiratory Muscle Strength Training in ALS

Conditions
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Amyotrophic Lateral Sclerosis

Intervention / Treatment
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* Procedure: Expiratory Muscle Strength Training (EMST)
* Procedure: EMST + Lung Volume Recruitment (LVR)


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: ALS defined as possible, laboratory-supported probable, probable, or definite by El Escorial criteria Reduced Maximal Expiratory Pressure (MEP) compared to norms for age and sex Forced Vital Capacity (FVC) > 65% predicted Exclusion Criteria: Inability to provide informed consent Relative contraindications to LVR, including known pneumothorax, active internal bleeding, unstable hypertension, unstable angina, emphysema, recent barotrauma, or FEV1 (Forced Expiratory Volume, trial 1)/FVC ratio < 0.7. Use of EMST or breath stacking > 3 days/week within 12 weeks of screening Amyotrophic Lateral Sclerosis-Cognitive Behavioral Scale (ALS-CBS) score predictive of dementia (≤ 10) Participation in another clinical trial of an intervention in ALS within 30 days of study enrollment or during study enrollment

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Supportive Care
Allocation: Randomized
Intervention Model: Parallel Assignment
Masking: Single


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Active Comparator: Expiratory Muscle Strength Testing (EMST)<br>EMST is a treatment method that has been used to improve cough function and swallowing in ALS. EMST uses a training device that has a valve set to 50% of a patient's maximum expiratory pressure (MEP). The patient exhales forcefully until the valve releases. Patients perform 5 sets of 5 repetitions a day, 5 days a week. | Procedure: Expiratory Muscle Strength Training (EMST)<br>* EMST uses a training device that has a valve set to 50% of a patient's maximum expiratory pressure (MEP). The patient exhales forcefully until the valve releases. Patients perform 5 sets of 5 repetitions a day, 5 days a week.<br>|
| Active Comparator: EMST + Lung Volume Recruitment (LVR)<br>EMST is a treatment method that has been used to improve cough function and swallowing in ALS. EMST uses a training device that has a valve set to 50% of a patient's maximum expiratory pressure (MEP). The patient exhales forcefully until the valve releases. Patients perform 5 sets of 5 repetitions a day, 5 days a week. LVR is a technique to increase cough function that is performed with a resuscitation bag fitted with a mouthpiece and a one-way valve. The bag is used to expand the lungs, after which the patient makes a voluntary cough. | Procedure: EMST + Lung Volume Recruitment (LVR)<br>* EMST uses a training device that has a valve set to 50% of a patient's maximum expiratory pressure (MEP). The patient exhales forcefully until the valve releases. Patients perform 5 sets of 5 repetitions a day, 5 days a week. LVR is a technique to increase cough function that is performed with a resuscitation bag fitted with a mouthpiece and a one-way valve. The bag is used to expand the lungs, after which the patient makes a voluntary cough. Half of the participants will perform the LVR technique in addition to the ESMT technique.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Peak Cough Flow | Ventilatory measure that assesses the change in coughing ability at Baseline (Week 0), Randomization (Week 6), and End of Study (Week 12) | Baseline (Week 0), Week 6, and Week 12 | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Maximal Expiratory Pressure | Measurement of respiratory muscle strength changes at Baseline (Week 0), Randomization (Week 6), Week 8, Week 10, and End of Study (Week 12) | Week 0, Week 6, Week 8, Week 10, and Week 12 | 
| Forced Vital Capacity | Measurement of changes in how much air is exhaled over a series of forced breaths at Baseline (Week 0), Randomization (Week 6), and End of Study (Week 12) | Baseline (Week 0), Week 6, and Week 12 | 
| Eating Assessment Tool - 10 (EAT-10) | 10-item patient reported outcome assessing changes in swallowing function at Baseline (Week 0), Randomization (Week 6), and End of Study (Week 12) | Baseline (Week 0), Week 6, and Week 12 | 
| Swallowing Related Quality of Life (SWAL-QOL) | 44-item patient reported outcome assessing changes in swallowing impairment at Baseline (Week 0), Randomization (Week 6), and End of Study (Week 12) | Baseline (Week 0), Randomization (Week 6), and End of Study (Week 12) | 
| Speech Intelligibility Test (SIT) | Standardized assessment of speech intelligibility and efficiency in neuromuscular disorders. Measures changes in speech intelligibility at Baseline (Week 0), Randomization (Week 6), and End of Study (Week 12). | Baseline (Week 0), Randomization (Week 6), and End of Study (Week 12) |