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Lead investigator

Collaborators & Co-Investigators

• Lawrence Korngut MD, Msc
• Stephanie Plamondon, MD
• Aneal Khan, MD
• Andrea Loewen, MD
• Karen Rimmer, MD
• Curtis Dumonceaux, RRT
• Ashley Waito, SLP

Research Sites & Affiliations

• University of Calgary, Calgary, Alberta
• University of Toronto, Toronto, Ontario

Budget: $47,837.98

Disorders: Hereditary Myopathies

Research Areas: Enhance Care

Abstract: 

Genetic muscle diseases are a heterogeneous group of disorders, that for the greater part, do not currently have any disease-modifying therapies. Many of these conditions cause abnormal swallowing and/or ventilatory function, which has a major effect on quality of life, morbidity, and mortality. Expiratory muscle strength training (EMST) is a rehabilitative approach that can be performed using a handheld device, that provides a customisable amount of resistance to expiration. It is customisable to individual needs, and has preliminary evidence in other neurodegenerative conditions showing it can result in improved swallowing and respiratory function. It has not yet been studied in patients with hereditary muscle diseases.
The objective is to perform an open-label, interventional study of 20 participants with hereditary muscle disease, who have respiratory and/or swallowing impairment. Obtain preliminary data indicating whether EMST has benefit for respiratory/swallowing function.

Impact: