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Respiratory muscle training (RMT) in late-onset Pompe disease (LOPD): Effects of training and detraining.

Publication ,  Journal Article
Jones, HN; Crisp, KD; Robey, RR; Case, LE; Kravitz, RM; Kishnani, PS
Published in: Mol Genet Metab
February 2016

BACKGROUND: Determine the effects of a 12-week respiratory muscle training (RMT) program in late-onset Pompe disease (LOPD). METHODS: We investigated the effects of 12-weeks of RMT followed by 3-months detraining using a single-subject A-B-A experimental design replicated across 8 adults with LOPD. To assess maximal volitional respiratory strength, our primary outcomes were maximum inspiratory pressure (MIP) and maximum expiratory pressure (MEP). Effect sizes for changes in MIP and MEP were determined using Cohen's d statistic. Exploratory outcomes targeted motor function, and peak cough flow (PCF) was measured in the last 5 subjects. RESULTS: From pretest to posttest, all 8 subjects exhibited increases in MIP, and 7 of 8 showed increases in MEP. Effect size data reveal the magnitude of increases in MIP to be large in 4 (d≥1.0) and very large in 4 (d≥2.0), and effect sizes for increases in MEP were large in 1 (d≥1.0) and very large in 6 (d≥2.0). Across participants, pretest to posttest MIP and MEP increased by a mean of 19.6% (sd=9.9) and 16.1% (sd=17.3), respectively. Respiratory strength increases, particularly for the inspiratory muscles, were generally durable to 3-months detraining. CONCLUSIONS: These data suggest our 12-week RMT program results in large to very large increases in inspiratory and expiratory muscle strength in adults with LOPD. Additionally, increases in respiratory strength appeared to be relatively durable following 3-months detraining. Although additional research is needed, RMT appears to offer promise as an adjunctive treatment for respiratory weakness in LOPD.

Duke Scholars

Published In

Mol Genet Metab

DOI

EISSN

1096-7206

Publication Date

February 2016

Volume

117

Issue

2

Start / End Page

120 / 128

Location

United States

Related Subject Headings

  • Walking
  • Treatment Outcome
  • Respiratory Muscles
  • Respiration
  • Muscle Strength
  • Middle Aged
  • Male
  • Humans
  • Glycogen Storage Disease Type II
  • Genetics & Heredity
 

Citation

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Jones, H. N., Crisp, K. D., Robey, R. R., Case, L. E., Kravitz, R. M., & Kishnani, P. S. (2016). Respiratory muscle training (RMT) in late-onset Pompe disease (LOPD): Effects of training and detraining. Mol Genet Metab, 117(2), 120–128. https://doi.org/10.1016/j.ymgme.2015.09.003
Jones, Harrison N., Kelly D. Crisp, Randall R. Robey, Laura E. Case, Richard M. Kravitz, and Priya S. Kishnani. “Respiratory muscle training (RMT) in late-onset Pompe disease (LOPD): Effects of training and detraining.Mol Genet Metab 117, no. 2 (February 2016): 120–28. https://doi.org/10.1016/j.ymgme.2015.09.003.
Jones HN, Crisp KD, Robey RR, Case LE, Kravitz RM, Kishnani PS. Respiratory muscle training (RMT) in late-onset Pompe disease (LOPD): Effects of training and detraining. Mol Genet Metab. 2016 Feb;117(2):120–8.
Jones, Harrison N., et al. “Respiratory muscle training (RMT) in late-onset Pompe disease (LOPD): Effects of training and detraining.Mol Genet Metab, vol. 117, no. 2, Feb. 2016, pp. 120–28. Pubmed, doi:10.1016/j.ymgme.2015.09.003.
Jones HN, Crisp KD, Robey RR, Case LE, Kravitz RM, Kishnani PS. Respiratory muscle training (RMT) in late-onset Pompe disease (LOPD): Effects of training and detraining. Mol Genet Metab. 2016 Feb;117(2):120–128.
Journal cover image

Published In

Mol Genet Metab

DOI

EISSN

1096-7206

Publication Date

February 2016

Volume

117

Issue

2

Start / End Page

120 / 128

Location

United States

Related Subject Headings

  • Walking
  • Treatment Outcome
  • Respiratory Muscles
  • Respiration
  • Muscle Strength
  • Middle Aged
  • Male
  • Humans
  • Glycogen Storage Disease Type II
  • Genetics & Heredity