Respiratory Strength Training in Persons With Amyotrophic Lateral Sclerosis (ALS)
Condition: Amyotrophic Lateral Sclerosis
MeSH terms: Motor Neuron Disease; Amyotrophic Lateral Sclerosis; Sclerosis
Study type: Interventional
Phase: Early Phase 1

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).
    

Detailed 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.
    

Intervention name(s): PowerLung trainer; Micro Mouth Pressure Meter; Pulmonary Function Testing; Videofluoroscopic swallowing study; Swallowing Quality of Life Questionnaire; Iowa Oral Pressure Instrument; Capsaicin
Intervention type(s): Device; Device; Procedure; Procedure; Other; Device; Drug
Intervention description(s): 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.; 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.; 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.; Videofluoroscopic swallowing study will be performed to measure the oropharyngeal swallowing.; Swallowing Quality of Life Questionnaire (SWAL-QOL) will be used for participant reporting of swallow-related quality of life.; 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.; 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.
Other intervention name(s): digital manometer; Micro Medical device; VFSS; X-ray of swallowing; SWAL-QOL; IOPI; Hot pepper
Intervention MeSH terms: Capsaicin
Allocation: Randomized
Intervention model: Parallel Assignment
Masking: Single (Participant)
Primary purpose: Treatment