Increased inspiratory and expiratory muscle strength following respiratory muscle strength training (RMST) in two patients with late-onset Pompe disease.

Journal Article

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.

Full Text

Duke Authors

Cited Authors

  • Jones, HN; Moss, T; Edwards, L; Kishnani, PS

Published Date

  • November 2011

Published In

Volume / Issue

  • 104 / 3

Start / End Page

  • 417 - 420

PubMed ID

  • 21641843

Pubmed Central ID

  • 21641843

Electronic International Standard Serial Number (EISSN)

  • 1096-7206

International Standard Serial Number (ISSN)

  • 1096-7192

Digital Object Identifier (DOI)

  • 10.1016/j.ymgme.2011.05.006


  • eng