Inspiratory Muscle Training in Late-Onset Pompe Disease: The Effects on Pulmonary Function Tests, Quality of Life, and Sleep Quality.
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.
Division of Physiotherapy and Rehabilitation, Faculty of Health Sciences, Istanbul University, Demirkapi Cad. Karabal Sk, 34147, Bakırkoy, Istanbul, Turkey. goksenkuran@yahoo.com.
Goksen Kuran
GK
Aslan
Division of Physiotherapy and Rehabilitation, Faculty of Health Sciences, Istanbul University, Demirkapi Cad. Karabal Sk, 34147, Bakırkoy, Istanbul, Turkey.
Burcu Ersoz
BE
Huseyinsinoglu
Neurology Department, Memorial Sisli Hospital, Istanbul, Turkey.
Piraye
P
Oflazer
Division of Physiotherapy and Rehabilitation, Faculty of Health Sciences, Bezmialem Vakif University, Istanbul, Turkey.
Nilgun
N
Gurses
Department of Chest Disease, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.
Esen
E
Kiyan
D000328:Adult; D001945:Breathing Exercises; D003371:Cough / Q000503:physiopathology; D056947:Enzyme Replacement Therapy; D005260:Female; D006009:Glycogen Storage Disease Type II / Q000503:physiopathology / Q000628:therapy*; D006801:Humans; D001239:Inhalation / Q000502:physiology*; D000067562:Late Onset Disorders / Q000503:physiopathology / Q000628:therapy; D008297:Male; D000072277:Maximal Respiratory Pressures; D008875:Middle Aged; D011788:Quality of Life; D012890:Sleep; D013147:Spirometry; D055815:Young Adult
Diaphragm weakness; Inspiratory muscle training; Pompe diseases; Pulmonary functions; Quality of life; Sleep quality