Inspiratory Muscle Training in Late-Onset Pompe Disease: The Effects on Pulmonary Function Tests, Quality of Life, and Sleep Quality
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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).
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.
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.
These results suggest that IMT has a positive effect on maximum inspiratory pressure in subjects with LOPD who are under ERT.
KeywordsPompe diseases Pulmonary functions Inspiratory muscle training Diaphragm weakness Sleep quality Quality of life
Compliance with Ethical Standards
Conflict of Interest
- 34.Agargun MYKH, Anlar O (1996) Pittsburgh Uyku Kalitesi İndeksinin geçerliliği ve güvenilirliği. Türk Psikiyatri Dergisi 7:107–115Google Scholar
- 43.Smith BK, Collins SW, Conlon TJ et al (2013) Phase I/II trial of adeno-associated virus-mediated alpha-glucosidase gene therapy to the diaphragm for chronic respiratory failure in Pompe disease: initial safety and ventilatory outcomes. Hum Gene Ther 24(6):630–640. doi: 10.1089/hum.2012.250 CrossRefPubMedPubMedCentralGoogle Scholar
- 44.Boentert M, Karabul N, Wenninger S et al (2015) Sleep-related symptoms and sleep-disordered breathing in adult Pompe disease. Eur J Neurol 22 (2):369–376, e327. doi: 10.1111/ene.12582