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Effects of respiratory muscle training (RMT) in children with infantile-onset Pompe disease and respiratory muscle weakness.

Publication ,  Journal Article
Jones, HN; Crisp, KD; Moss, T; Strollo, K; Robey, R; Sank, J; Canfield, M; Case, LE; Mahler, L; Kravitz, RM; Kishnani, PS
Published in: J Pediatr Rehabil Med
2014

PURPOSE: Respiratory muscle weakness is a primary therapeutic challenge for patients with infantile Pompe disease. We previously described the clinical implementation of a respiratory muscle training (RMT) regimen in two adults with late-onset Pompe disease; both demonstrated marked increases in inspiratory and expiratory muscle strength in response to RMT. However, the use of RMT in pediatric survivors of infantile Pompe disease has not been previously reported. METHOD: We report the effects of an intensive RMT program on maximum inspiratory pressure (MIP) and maximum expiratory pressure (MEP) using A-B-A (baseline-treatment-posttest) single subject experimental design in two pediatric survivors of infantile Pompe disease. Both subjects had persistent respiratory muscle weakness despite long-term treatment with alglucosidase alfa. RESULTS: Subject 1 demonstrated negligible to modest increases in MIP/MEP (6% increase in MIP, d=0.25; 19% increase in MEP, d=0.87), while Subject 2 demonstrated very large increases in MIP/MEP (45% increase in MIP, d=2.38; 81% increase in MEP, d=4.31). Following three-month RMT withdrawal, both subjects maintained these strength increases and demonstrated maximal MIP and MEP values at follow-up. CONCLUSION: Intensive RMT may be a beneficial treatment for respiratory muscle weakness in pediatric survivors of infantile Pompe disease.

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Published In

J Pediatr Rehabil Med

DOI

EISSN

1875-8894

Publication Date

2014

Volume

7

Issue

3

Start / End Page

255 / 265

Location

Netherlands

Related Subject Headings

  • Respiratory Muscles
  • Muscle Contraction
  • Lung
  • Inhalation
  • Humans
  • Glycogen Storage Disease Type II
  • Exhalation
  • Child
  • Breathing Exercises
  • Airway Resistance
 

Citation

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Jones, H. N., Crisp, K. D., Moss, T., Strollo, K., Robey, R., Sank, J., … Kishnani, P. S. (2014). Effects of respiratory muscle training (RMT) in children with infantile-onset Pompe disease and respiratory muscle weakness. J Pediatr Rehabil Med, 7(3), 255–265. https://doi.org/10.3233/PRM-140294
Jones, Harrison N., Kelly D. Crisp, Tronda Moss, Katherine Strollo, Randy Robey, Jeffrey Sank, Michelle Canfield, et al. “Effects of respiratory muscle training (RMT) in children with infantile-onset Pompe disease and respiratory muscle weakness.J Pediatr Rehabil Med 7, no. 3 (2014): 255–65. https://doi.org/10.3233/PRM-140294.
Jones HN, Crisp KD, Moss T, Strollo K, Robey R, Sank J, et al. Effects of respiratory muscle training (RMT) in children with infantile-onset Pompe disease and respiratory muscle weakness. J Pediatr Rehabil Med. 2014;7(3):255–65.
Jones, Harrison N., et al. “Effects of respiratory muscle training (RMT) in children with infantile-onset Pompe disease and respiratory muscle weakness.J Pediatr Rehabil Med, vol. 7, no. 3, 2014, pp. 255–65. Pubmed, doi:10.3233/PRM-140294.
Jones HN, Crisp KD, Moss T, Strollo K, Robey R, Sank J, Canfield M, Case LE, Mahler L, Kravitz RM, Kishnani PS. Effects of respiratory muscle training (RMT) in children with infantile-onset Pompe disease and respiratory muscle weakness. J Pediatr Rehabil Med. 2014;7(3):255–265.

Published In

J Pediatr Rehabil Med

DOI

EISSN

1875-8894

Publication Date

2014

Volume

7

Issue

3

Start / End Page

255 / 265

Location

Netherlands

Related Subject Headings

  • Respiratory Muscles
  • Muscle Contraction
  • Lung
  • Inhalation
  • Humans
  • Glycogen Storage Disease Type II
  • Exhalation
  • Child
  • Breathing Exercises
  • Airway Resistance