Expiratory Muscle Strength Training (EMST) in Neuromuscular Disorders

University of Calgary20 enrolled

Overview

The purpose of this study is to investigate the impact of expiratory muscle strength training (EMST) on the swallowing, breathing, oral intake, quality of life and cough function of people with oculopharyngeal muscular dystrophy (OPMD).

Outline: Twenty participants with OPMD, with dysphagia, will be recruited from Neuromuscular clinics within Calgary. The investigators will enrol patients in a parallel group, sham-controlled, randomized clinical trial, with 10 participants in each group (active EMST and sham EMST). Participants will have baseline measurements of: (i) global swallowing function via modified barium swallow study, (ii) maximum expiratory pressure, (iii) voluntary cough spirometry, (iv) forced vital capacity, (v) functional oral intake, (vi) patient report of self-perceived swallowing impairment (EAT-10 Questionnaire), and (vii) biomarker analyses. Participants will undergo 5-weeks of EMST (active or sham). All baseline measurements will be repeated after 5-weeks of EMST and 10-weeks post-EMST to measure durability of effect. Outcomes: The end-goal of the current research is to obtain preliminary data for the benefit of EMST in a new study population, and direct future studies that may provide evidence for a new standard of care in treating neuromuscular diagnoses.

Study Type

INTERVENTIONAL

Allocation

RANDOMIZED

Purpose

TREATMENT

Masking

DOUBLE

Enrollment

Conditions

Oculopharyngeal Muscular Dystrophy Muscular Dystrophies Myopathy; Hereditary

Interventions

Expiratory muscle strength therapy (EMST150, Aspire LLC)DEVICE

Active therapy calibrated to the participant's maximum expiratory pressure

Eligibility

Sex: ALLMin age: 18 Years

Medical Language ↔ Plain English

Inclusion Criteria: * Diagnosis of oculopharyngeal muscular dystrophy (OPMD) * 18 years of age or older * Must be capable of providing informed consent * Must be able to undergo respiratory function testing and swallowing studies * Must have a forced vital capacity (FVC) greater than 60% * A score of 3 or greater on the Eating Assessment Tool-10 (EAT-10; self-administered, symptom-specific outcome instrument for dysphagia. A score of 3 or greater indicates increased stress around eating) * A score of 26 or greater on the Montreal Cognitive Assessment (MoCA; 30-point screening assessment used for detecting cognitive impairment. A score of 26 or greater is considered to be within functional limits.) Exclusion Criteria: * Severe coronary artery disease * Acute myocardial infarction * Moderate to severe hypovolemia * Acute neurological events * Unstable cardiac status * Recent hernia * Severe chronic obstructive pulmonary disease (COPD) * Uncontrolled reflux issues * Women who are pregnant, or who suspect they may be pregnant * Cognitive impairment that would prevent comprehension of instructions and adherence to intervention guidelines (a score of less than 26 points on the MoCA)

Locations (1)

Neuromuscular Clinic, South Health Campus

Calgary, Alberta, Canada

Outcomes

Primary Outcomes

Global Swallowing Function

Global swallowing function is rated from videofluoroscopy swallowing studies (VFSS), using the Dynamic Imaging Grade of Swallowing Toxicity (DIGEST), a validated 5-point scale. Global swallowing function is rated from 0-4: 0 = no pharyngeal dysphagia; 1 = mild; 2 = moderate; 3 = severe; 4 = life-threatening. A lower score is a better outcome.

Time frame: Change in score from week 0 to week 5

Secondary Outcomes

Global Swallowing Function

Time frame: Change in score from week 0 to week 15; change in score from week 5 to week 15.

Maximum expiratory pressure (MEP)

MEP is a measure of respiratory muscle strength and is assessed with a handheld manometer, measured in centimetres of water (cmH2O). A higher score is a better outcome.

Time frame: Change in score from week 0 to week 5; change in score from week 0 to week 15; change in score from week 5 to week 15.

Volitional cough strength (peak cough flow)

Measure of cough strength that is assessed using a spirometer, measured in litres per minute (L/min). A higher score is a better outcome.

Time frame: Change in score from week 0 to week 5; change in score from week 0 to week 15; change in score from week 5 to week 15.

Forced vital capacity (FVC)

Measure of how much air is exhaled during forced exhalation and is assessed with a spirometer, measured in litres. A higher score is a better outcome.

Time frame: Change in score from week 0 to week 5; change in score from week 0 to week 15; change in score from week 5 to week 15.

Data from ClinicalTrials.gov

This platform is for informational purposes only and does not constitute medical advice. Always consult a qualified healthcare professional.