Increased inspiratory and expiratory muscle strength following respiratory muscle strength training (RMST) in two patients with late-onset Pompe disease |
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| Authors: | Jones Harrison N Moss Tronda Edwards Laurie Kishnani Priya S |
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| Affiliation: | aDepartment of Surgery, Division of Speech Pathology & Audiology, Duke University, Durham, NC, USA;bDepartment of Speech Pathology & Audiology, Duke University, Durham, NC, USA;cDivision of Medical Genetics, Department of Pediatrics, Duke University, Durham, NC, USA |
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| Abstract: | Respiratory muscle strength training (RMST) is an exercise-based intervention which targets respiratory muscle weakness. We implemented RMST in two patients with late-onset Pompe disease (LOPD), both who had received long-term enzyme replacement therapy and had severe respiratory weakness. Over 16–32 weeks, inspiratory muscle strength increased by 73–74%. Expiratory muscle strength increased 31–48% over 12–22 weeks. These findings suggest that RMST may increase respiratory muscle strength, even in the setting of LOPD and severe baseline weakness. |
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| Keywords: | Respiratory muscle strength Training Pompe disease Late-onset Pompe disease Treatment Respiratory weakness |
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