Respiratory Strength Training in Persons With Amyotrophic Lateral Sclerosis (ALS)

University of Florida

Status and phase: Completed; Early Phase 1

Conditions: Amyotrophic Lateral Sclerosis

Treatments: Other: Swallowing Quality of Life Questionnaire; Drug: Capsaicin; Procedure: Videofluoroscopic swallowing study; Device: PowerLung trainer; Device: Iowa Oral Pressure Instrument; Device: Micro Mouth Pressure Meter; Procedure: Pulmonary Function Testing

Study type: Interventional

Funder types: Other

Identifiers

NCT02710110

IRB201501172

Details and patient eligibility

About

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).

Full description

This research study will measure the maximum inspiratory and expiratory pressure with secondary measures of respiration, swallow, cough, quality of life and global disease progression following twelve-weeks of treatment compared to the sham group.

As a participant two evaluations at the University of Florida Swallowing Systems Core laboratory located at Shands Hospital, Gainesville will take place. Each will take approximately two-hours and these will be spaced exactly three-months apart. During each evaluation tests will be performed to look at breathing, swallowing, and ability to cough doing standard clinical exams. In addition, some surveys about eating, speaking and quality of life will be taken.

Breathing Exercises: a home research therapist will train the participants how to use the hand-held respiratory trainer. Training for both treatment groups will be very similar, except the PowerLung trainer device will have an additional spring load valve inside it that will place a force on the respiratory muscles during training. The other breathing trainer will not have this spring so that no resistance will be placed on the respiratory muscles during training and this will represent an aerobic respiratory treatment. The breathing exercises will be completed five days a week for a total of three months (a total of 60 therapy sessions). During each session, a total of three sets of ten repetitions of breathing exercises will be performed. Once a week the home research therapist will visit and guide participants through the breathing exercise.

Enrollment: 50 patients

Sex: All

Ages: 21 to 85 years old

Volunteers: No Healthy Volunteers

Inclusion criteria

• diagnosis of probable or definite Amyotrophic Lateral Sclerosis (ALS),
• Amyotrophic Lateral Sclerosis Rating Scale Revised score greater than 34,
• forced vital capacity greater than 70%,
• cognition within normal limits as determined by Montreal assessment of cognition score >25

Exclusion criteria

• allergies to barium,
• tracheotomy or mechanical ventilation,
• diaphragmatic pacer,
• concurrent respiratory disease (e.g. COPD),
• pregnant at the time of the study due to radiation exposure

Trial design

Primary purpose: Treatment

Allocation: Randomized

Interventional model: Parallel Assignment

Masking: Single Blind

---

50 participants in 2 patient groups

Active Respiratory Trainer

Experimental group

Description:

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.

Treatment:

Procedure: Pulmonary Function Testing

Device: Micro Mouth Pressure Meter

Device: Iowa Oral Pressure Instrument

Device: PowerLung trainer

Drug: Capsaicin

Procedure: Videofluoroscopic swallowing study

Other: Swallowing Quality of Life Questionnaire

Sham Trainer

Sham Comparator group

Description:

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.

Treatment:

Procedure: Pulmonary Function Testing

Device: Micro Mouth Pressure Meter

Device: Iowa Oral Pressure Instrument

Device: PowerLung trainer

Drug: Capsaicin

Procedure: Videofluoroscopic swallowing study

Other: Swallowing Quality of Life Questionnaire