Brief Summary

Dysphagia (swallow impairment), dystussia (cough impairment) and respiratory impairment are hallmark features of amyotrophic lateral sclerosis (ALS). These symptoms are the cause of fatal aspiration, malnutrition and respiratory insufficiency that together account for 91.4% of ALS mortality. Unfortunately, treatments to prolong and maintain these vital functions are currently lacking. Although the use of exercise in ALS is controversial, recent evidence suggests that mild to moderate intensity exercise applied early in the disease slows disease progression, improves motor function, preserves motor neuron number, reduces muscle hypoplasia, atrophy astrogliosis, and prolongs survival in animal models of ALS and human clinical trials. This research study is designed to determine the impact of respiratory strength training on breathing, airway protection and swallowing in persons with Amyotrophic Lateral Sclerosis (ALS).

Trial Health

On Track

Trial Health Score

Automated assessment based on enrollment pace, timeline, and geographic reach

Enrollment

participants targeted

Target at P50-P75 for early_phase_1

Timeline

Completed

Started Apr 2016

Typical duration for early_phase_1

Geographic Reach

1 country

1 active site

Status

completed

Health score is calculated from publicly available data and should be used for screening purposes only.

Trial Relationships

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Study Timeline

Key milestones and dates

2.9 years study duration

First Submitted

Initial submission to the registry

February 26, 2016

Completed

19 days until next milestone

First Posted

Study publicly available on registry

March 16, 2016

Completed

16 days until next milestone

Study Start

First participant enrolled

April 1, 2016

Completed

1.7 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

November 30, 2017

Completed

1.2 years until next milestone

Study Completion

Last participant's last visit for all outcomes

February 7, 2019

Completed

Last Updated

January 27, 2020

Status Verified

January 1, 2020

Enrollment Period

1.7 years

First QC Date

February 26, 2016

Last Update Submit

January 23, 2020

Conditions

Amyotrophic Lateral Sclerosis

Outcome Measures

Primary Outcomes (6)

Maximum Expiratory Pressure will be measured between the groups for a change from baseline to month 3
Maximum expiratory pressure is a measure of force generating capabilities as assessed by having subjects quickly inhale then rapidly blow out air into a handheld manometer (Micro Mouth Pressure Meter). Three trails will be performed and the highest will be used.
Changes from baseline to month 3
Maximum Inspiratory Pressure will be measured between the groups for a change from baseline to month 3
Maximum inspiratory pressure is a measure of force generating capabilities as assessed by having subjects quickly inhale then rapidly blow out air into a handheld manometer (Micro Mouth Pressure Meter). Three trails will be performed and the highest will be used.
Changes from baseline to month 3
Pulmonary function to measure the forced vital capacity (FVC) between the groups for a change from baseline to month 3
Pulmonary Function testing will be performed using a micro pressure meter. The participant will perform a maximum inhalation and then slowly blow out air into the mouthpiece of the meter. The following measurement will be displayed on the meter after the exhalation is complete forced vital capacity (FVC) expressed as a percentage of predicted values.
Changes from baseline to month 3
Sniff Nasal Inspiratory Pressure will be measured between the groups for a change from baseline to month 3
Changes from baseline to month 3
Pulmonary function to measure the peak expiratory flow (PEF) will be measured between the groups for a change from baseline to month 3
Pulmonary Function testing will be performed using a micro pressure meter. The participant will perform a maximum inhalation and then slowly blow out air into the mouthpiece of the meter. The following measurement will be displayed on the meter after the exhalation is complete peak expiratory flow (PEF) expressed as a percentage of predicted values.
Changes from baseline to month 3
Pulmonary function to measure the forced expiratory volume (FEV1) will be measured between the groups for a change from baseline to month 3
Pulmonary Function testing will be performed using a micro pressure meter. The participant will perform a maximum inhalation and then slowly blow out air into the mouthpiece of the meter. The following measurement will be displayed on the meter after the exhalation is complete forced expiratory volume (FEV1) expressed as a percentage of predicted values.
Changes from baseline to month 3

Secondary Outcomes (5)

The Penetration-aspiration scale will be used to measure swallowing function
Changes from baseline to month 3
Lingual strength will be measured between the groups for a change from baseline to month 3
Changes from baseline to month 3
Lingual endurance will be measured between the groups for a change from baseline to month 3
Changes from baseline to month 3
Voluntary cough function will be measured between the groups for a change from baseline to month 3
Changes from baseline to month 3
Reflexive cough will be measured between the groups for a change from baseline to month 3
Changes from baseline to month 3

Study Arms (2)

Active Respiratory Trainer

EXPERIMENTAL

Participants enrolled in this arm will be given the PowerLung trainer. The adjustable spring allows for discrete and calibrated changes to the valve, which in turn blocks air until sufficient inspiratory or expiratory pressure is applied by an individual. In addition, the Micro Mouth Pressure Meter, Pulmonary Function testing, videofluoroscopic swallowing study (VFSS), Swallowing Quality of Life Questionnaire (SWAL-QOL), Iowa Oral Pressure Instrument (IOPI), and capsaicin for use in the reflexive cough test.

Device: PowerLung trainerDevice: Micro Mouth Pressure MeterProcedure: Pulmonary Function TestingProcedure: Videofluoroscopic swallowing studyOther: Swallowing Quality of Life QuestionnaireDevice: Iowa Oral Pressure InstrumentDrug: Capsaicin

Sham Trainer

SHAM COMPARATOR

Participants enrolled in this arm will be given the PowerLung trainer; however, the adjustable spring allows for discrete and calibrated changes to the valve and these will not have any resistance. In addition, the Micro Mouth Pressure Meter, Pulmonary Function testing, videofluoroscopic swallowing study (VFSS), Swallowing Quality of Life Questionnaire (SWAL-QOL), Iowa Oral Pressure Instrument (IOPI), and capsaicin for use in the reflexive cough test.

Interventions

PowerLung trainerDEVICE

PowerLung trainer will be utilized which has both an inspiratory and expiratory loading capacity for training. A single daily training session will consist of three sets of 10 repetitions for a total of 30 inspiratory repetitions and 30 expiratory repetitions (i.e. 60 repetitions) for 5 days a week, for 3 months.