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Lung

, Volume 194, Issue 4, pp 555–561 | Cite as

Inspiratory Muscle Training in Late-Onset Pompe Disease: The Effects on Pulmonary Function Tests, Quality of Life, and Sleep Quality

  • Goksen Kuran Aslan
  • Burcu Ersoz Huseyinsinoglu
  • Piraye Oflazer
  • Nilgun Gurses
  • Esen Kiyan
Article

Abstract

Introduction

Late-onset Pompe disease (LOPD) is characterized by progressive skeletal and respiratory muscle weakness. Little is known about the effect of inspiratory muscle training (IMT) on pulmonary function in subjects with LOPD. The aim of the present study was to investigate the effect of an 8-week IMT program on pulmonary function tests, quality of life, and sleep quality in eight patients with LOPD who were receiving enzyme replacement therapy (ERT).

Methods

Before and after the IMT program, spirometric measurements in sitting and supine positions, and measurements of maximum inspiratory and expiratory pressures, peak cough flow, quality of life (assessed using the Nottingham Health Profile), and sleep quality (assessed using the Pittsburgh sleep quality index) were performed.

Results

A significant increase in maximum inspiratory pressure (cmH2O and % predicted) (median [interquartile range]: 30.0 cmH2O [21.5–48] versus 39 cmH2O [31.2–56.5] and 38.3 % [28.1–48.4] versus 50.5 % [37.7–54.9]) was observed after training (p = 0.01). There were no significant changes in the other pulmonary function measurements. With the exception of the social isolation subscore (p = 0.02), quality of life subscores did not change after IMT (p > 0.05). Sleep quality subscores and total scores were similar before and after IMT.

Conclusion

These results suggest that IMT has a positive effect on maximum inspiratory pressure in subjects with LOPD who are under ERT.

Keywords

Pompe diseases Pulmonary functions Inspiratory muscle training Diaphragm weakness Sleep quality Quality of life 

Notes

Compliance with Ethical Standards

Conflict of Interest

None.

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Copyright information

© Springer Science+Business Media New York 2016

Authors and Affiliations

  • Goksen Kuran Aslan
    • 1
  • Burcu Ersoz Huseyinsinoglu
    • 1
  • Piraye Oflazer
    • 2
  • Nilgun Gurses
    • 3
  • Esen Kiyan
    • 4
  1. 1.Division of Physiotherapy and Rehabilitation, Faculty of Health SciencesIstanbul UniversityBakırkoy, IstanbulTurkey
  2. 2.Neurology DepartmentMemorial Sisli HospitalIstanbulTurkey
  3. 3.Division of Physiotherapy and Rehabilitation, Faculty of Health SciencesBezmialem Vakif UniversityIstanbulTurkey
  4. 4.Department of Chest Disease, Istanbul Faculty of MedicineIstanbul UniversityIstanbulTurkey

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