title: Exercise training in adults with Pompe disease: the effects on pain, fatigue, and functioning.
abstract: OBJECTIVE
  To assess if a 12-week exercise intervention to improve aerobic fitness, muscle strength, and core stability also had an impact on fatigue, pain, activity, and participation in adults with Pompe disease, an inherited neuromuscular disorder.
  
  
  METHODS
  Open-label trial. Change was assessed by the chi-square test and Wilcoxon signed-rank test.
  
  
  METHODS
  Physiotherapy practices.
  
  
  METHODS
  Mildly affected adult patients with Pompe disease who were not dependent on ventilators and/or walking devices and were receiving enzyme replacement therapy.
  
  
  METHODS
  Patients participated in a 12-week exercise program, which included 36 sessions of standardized aerobic, resistance, and core stability exercises.
  
  
  METHODS
  Before and after the training program we evaluated fatigue (Fatigue Severity Scale), pain (yes/no), motor function (Quantitative Muscle Function Test, Rasch-built Pompe-specific Activity Scale), amount of physical activity (activity monitor), and health status (Medical Outcomes Study 36-Item Short-Form Health Survey).
  
  
  RESULTS
  Of the 25 patients enrolled, 23 completed the program. At the end of the program, levels of fatigue (median, 5.33 to 4.78, P=.01) and pain (56.5% to 21.7%, P=.04) improved. The quality of motor function and the amount of physical activity patients engaged in did not change. Changes in pain and fatigue were not related to improvements in aerobic fitness or muscle strength.
  
  
  CONCLUSIONS
  This study in mildly affected adult patients with Pompe disease suggests that a combined training program aiming to increase aerobic fitness, muscle strength, and core stability also leads to improvements in fatigue and pain.
authors.affiliation: Department of Pediatrics, Center for Lysosomal and Metabolic Diseases, Erasmus MC University Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands; Department of Rehabilitation Medicine and Physical Therapy, Erasmus MC University Medical Center, Rotterdam, The Netherlands.
authors.forename: Marein M
authors.initials: MM
authors.lastname: Favejee
authors.affiliation: Department of Pediatrics, Center for Lysosomal and Metabolic Diseases, Erasmus MC University Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands.
authors.forename: Linda E M
authors.initials: LE
authors.lastname: van den Berg
authors.affiliation: Department of Pediatrics, Center for Lysosomal and Metabolic Diseases, Erasmus MC University Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands.
authors.forename: Michelle E
authors.initials: ME
authors.lastname: Kruijshaar
authors.affiliation: Department of Neurology, Center for Lysosomal and Metabolic Diseases, Erasmus MC University Medical Center, Rotterdam, The Netherlands.
authors.forename: Stephan C A
authors.initials: SC
authors.lastname: Wens
authors.affiliation: Department of Rehabilitation Medicine and Physical Therapy, Erasmus MC University Medical Center, Rotterdam, The Netherlands.
authors.forename: Stephan F E
authors.initials: SF
authors.lastname: Praet
authors.affiliation: Department of Clinical Genetics, Center for Lysosomal and Metabolic Diseases, Erasmus MC University Medical Center, Rotterdam, The Netherlands.
authors.forename: W W M
authors.initials: WW
authors.lastname: Pim Pijnappel
authors.affiliation: Department of Neurology, Center for Lysosomal and Metabolic Diseases, Erasmus MC University Medical Center, Rotterdam, The Netherlands.
authors.forename: Pieter A
authors.initials: PA
authors.lastname: van Doorn
authors.affiliation: Department of Rehabilitation Medicine and Physical Therapy, Erasmus MC University Medical Center, Rotterdam, The Netherlands.
authors.forename: Johannes B J
authors.initials: JB
authors.lastname: Bussmann
authors.affiliation: Department of Pediatrics, Center for Lysosomal and Metabolic Diseases, Erasmus MC University Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands. Electronic address: a.vanderploeg@erasmusmc.nl.
authors.forename: Ans T
authors.initials: AT
authors.lastname: van der Ploeg
mesh_terms: D000328:Adult; D000368:Aged; D005081:Exercise Therapy / Q000379:methods*; D005221:Fatigue / Q000503:physiopathology*; D005260:Female; D006009:Glycogen Storage Disease Type II / Q000534:rehabilitation*; D006304:Health Status; D006801:Humans; D008297:Male; D008875:Middle Aged; D053580:Muscle Strength; D010146:Pain / Q000503:physiopathology*; D010809:Physical Fitness / Q000502:physiology*; D011788:Quality of Life; D012720:Severity of Illness Index
keywords: Exercise; Fatigue; Glycogen storage disease type II; Pain; Rehabilitation