Effectiveness of Expiratory Muscle Strength Training for Improving Communication in ALS

Study Overview
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Brief Summary
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A tele-health treatment study for individuals with early stage ALS with the aim to improve communication, cough response, and respiratory strength. All participants complete a respiratory strength training program using an Expiratory Muscle Strength Training (EMST 150) device from the comfort of their homes for 6 weeks.

Detailed Description
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The investigators are looking for people with early stage ALS to participate in a completely tele-health (no in-person visits required) treatment study examining the effects of an Expiratory Muscle Strength Training device (EMST-150) on communication, cough, and respiratory strength. Participants will be required to attend 2 virtual baseline assessment sessions followed by 12 additional virtual training sessions (2 per week for 6 weeks). Participants should plan to be actively enrolled in the study for  10 weeks (3 weeks of baseline monitoring followed by 6 weeks of training). Participants will also be required to fill out a series of questionnaires assessing the EMST's effectiveness via tele-health.

Official Title
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Effectiveness of Expiratory Muscle Strength Training for Improving Communication in ALS

Conditions
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Amyotrophic Lateral Sclerosis

Intervention / Treatment
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* Device: Expiratory Muscle Strength Training (EMST-150)


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Diagnosis of ALS Within the early stages of disease progression (i.e., able to talk, breathe, and eat independently) Speaker of English Have a family member/caregiver willing to assist as needed Have access to an electronic device and internet for tele-health Exclusion Criteria: A history of neurological disease (besides ALS) A history of asthma or respiratory problems (e.g., COPD, emphysema) A history of head, neck, or chest surgery (except mastectomy) A history of smoking within the last 5 years Reliance on mechanical ventilation (including CPAP)

Ages Eligible for Study
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Minimum Age: 40 Years
Maximum Age: 70 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: Treatment
Intervention Model: Single Group Assignment
Interventional Model Description: All participants enrolled will receive the intervention.
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Expiratory Muscle Strength Training<br>All participants will forcefully breathe out into an Expiratory Muscle Strength Training device (EMST-150) 25 times per day for 6 weeks. The EMST device will be set at a moderate intensity level (50% of each participant's maximum expiratory pressure). | Device: Expiratory Muscle Strength Training (EMST-150)<br>* All participants will forcefully breathe out into an Expiratory Muscle Strength Training device (EMST-150) 25 times per day for 6 weeks. The EMST device will be set at a moderate intensity level (50% of each participant's maximum expiratory pressure).<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Change From Baseline to Post-treatment for Maximum Expiratory Pressure | Change in expiratory muscle strength throughout the study duration measured by the participant blowing into a pressure meter | Baseline 2 (week 2), Pre-Training (week 3), and Post-Training (week 10) | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Peak Expiratory Flow Rate | Change in cough strength (as measured by peak expiratory flow rate) throughout the study duration, measured using a flow meter that the participant coughs into - single coughs. Data from baseline 2 and pre-training were averaged for the pre--training value. | Baseline 2 (week 2), Pre-training (week 3), Post-Training (week 10) | 
| Change From Baseline to Post-Treatment in Utterance Length | Change in utterance length (# of syllables produced on one breath) throughout the study duration. Participant read the Rainbow Passage and data were collected from this task. | Baseline 1 (week 1) and Post-Training (week 10) | 
| Change From Baseline to Post-Treatment in Number of Pauses | Change in the total number of pauses that included breathing (breath pauses). Participant read the Rainbow Passage and data were collected from this task. | Baseline 1 (week 1) and Post-Training (week 10) | 
| Change From Baseline to Post-Treatment in Syntactic Location of Pauses | Change in the location of pauses while breathing produced while reading. We determined where participants took breaths while reading the Rainbow Passage and categorized them according to syntax. Categories included at a major boundary (at the end of an independent clause marked by a period) and at a boundary that is not related to syntax (not a major or minor syntactical location). The number of pauses at each category of boundaries were converted to a percent of the total breaths taken while reading the passage. | Baseline 1 (week 1) and Post-Training (week 10) | 
| Telehealth Satisfaction | Participants rated 15 statements with a 5-point Likert scale ranging from strongly disagree (1) to strongly agree (5). Example statements include: I can easily talk to my health-care provider, I can hear my health-care provider clearly, I can see my health-care provider as if we met in person, I think the care provided via telemedicine is consistent, and I receive adequate attention, telemedicine provides for my health care need, and overall, I am satisfied with the quality of service being provided via telemedicine. Total scores range from 0-75 and individual statement scores ranged from 1-5, with higher scores indicating higher satisfaction with the telepractice modality. | Post-Training (week 10) | 
| Change From Pre-Training to Post-Training in Communication Participation Item Bank | Participants were instructed to use a 4-point Likert scale (3: not at all; 2: a little; 1: quite a bit; 0: very much) to rate if/how their ALS impacts ten hypothetical communicative situations. Participants total scores were then added together, ranging from 0-30, and converted to a T-score ranging from 24.2-71.0. High scores are more favorable, indicating that the participant feels that ALS impacts their communicative participation less than those with lower scores. | Pre-Training (week 3) and Post-Training (week 10) | 
| Change From Baseline to Post-Treatment in ALS Quality of Life Scale | The ALS Quality of Life Scale-Revised (ALSQOL-R) (Simmons, 2015) was used to measure how ALS impacts their quality of life in six domains: negative emotion, interaction with people and the environment, intimacy, religiosity, physical symptoms, and bulbar function. Participants rate whether they strongly disagree (0) or strongly agree (10) with 46 statements. Each domain score is given an average value between 0 (worse) and 10 (best). Total scores on the ALSQOL-R range from 0-460, but an average score ranging from 0-10 is computed by dividing the total score by the total number of items completed by the participant (usually 46). Higher scores reflect a higher quality of life. A survey assessing how each participant feels ALS impacts quality of life, and how that changes throughout the study duration. Total scores range from 0-460, with higher scores indicating greater contributions of ALS to quality of life. | Baseline 1 (week 1) and Post-Training (week 10) | 
| ALS Function Rating Scale Revised | A survey that quantifies change in disease progression throughout the study duration. Participants rate how 12 physical functions are impacted by their ALS. For each function, participants are provided with a 4-point Likert scale to rate whether they still have complete control of that function (4) or no ability to perform that function (0). Total scores range from 0 to 48, with lower scores indicating more severe symptoms and higher scores and less severe symptoms. | Baseline 1 (week 1) and Post-Training (week 10) | 
| Psychosocial Impact of Assistive Devices Scale | A survey assessing how the training device/modality impacts patient's psychosocial functions. The PIADS includes 12 items for competence, 6 for adaptability, and 8 for self-esteem. Participants use a 7-point Likert scale to rate the impact of a device on each item: a maximum negative impact (-3), somewhat negative impact (-2 or -1), zero impact (0), somewhat positive impact (1 or 2), maximum positive impact (3). Total scores range from -78 (maximum negative impact) to 78 (maximum positive impact). | Post-treatment (week 10) | 
| Tele-health Session Attendance | % of training sessions scheduled and attended by participants throughout the study duration | Measured at the end of training across the entire training period | 
| Adherence to Training Protocol | Number of of training exhalations completed (25 exhalations into the device, 5 days per week) by participants throughout the study duration | Measured each week of training across the entire training duration |