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

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).

Conditions

• Amyotrophic Lateral Sclerosis

Interventions

DEVICE

PowerLung trainer

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.

DEVICE

Micro Mouth Pressure Meter

The participant will be seated with the nose occluded using a nose clip. After inhaling to total lung capacity, the participant will place his or her lips around the mouthpiece and blow out as forcefully as possible. A flange rubber mouthpiece will be used to overcome the inability of some individuals to create tight lip seal due to facial muscle weakness. Three trials will be performed and the patients highest Maximum Inspiratory Pressures (MIP) and Maximal Expiratory Pressure (MEP) used.

PROCEDURE

Pulmonary Function Testing

Pulmonary Function testing will be performed using conventional methods and will include the following outcomes: forced vital capacity (FVC), sniff nasal inspiratory pressure (SNIP), peak expiratory flow (PEF), forced expiratory volume (FEV1) expressed as a percentage of predicted values.

PROCEDURE

Videofluoroscopic swallowing study

Videofluoroscopic swallowing study will be performed to measure the oropharyngeal swallowing.

OTHER

Swallowing Quality of Life Questionnaire

Swallowing Quality of Life Questionnaire (SWAL-QOL) will be used for participant reporting of swallow-related quality of life.

DEVICE

Iowa Oral Pressure Instrument

The IOPI is a device that measures the peak pressure performance of lingual strength and endurance via the action of a bulb placed on the hard palate.

DRUG

Capsaicin

A capsaicin challenge with three randomized blocks of 0, 50, 100, 200, and 500 μM capsaicin. The capsaicin will be dissolved in a vehicle solution consisting of 80% physiological saline, and 20% ethanol. Participants will be given the instruction "cough if you need to" prior to capsaicin delivery.The solution will be administered automatically upon detection of an inspired breath and there will be a minimum of one minute between each trial. This is to test the reflexive cough testing for upper airway sensitivity and motor thresholds.

Sponsors & Collaborators

• ALS Association

  collaborator OTHER

• University of Florida

  lead OTHER

Principal Investigators

• Emily K Plowman, Ph.D. · University of Florida

Study Design

Allocation

RANDOMIZED

Purpose

TREATMENT

Masking

SINGLE

Model

PARALLEL

Eligibility

Min Age

21 Years

Max Age

85 Years

Sex

ALL

Healthy Volunteers

No

Timeline & Regulatory

Start

2016-04-30

Primary Completion

2017-11-30

Completion

2019-02-07

FDA Drug

Yes

Countries

• United States

Study Locations