The hypotheses is that regular post exercise supplementation increase fitness and daily activity level in patients with Fascioscapulohumeral (FSH) muscular dystrophy. All patients are tested before and after 12 weeks of cycle-ergometer exercise. Maximal oxygen consumption and 6MWT is used as primarily effect goals. Secondary effect goals are risk of falls and daily activity level.
The purpose of this study is to investigate the response to protein-carbohydrate supplementation doing regular exercise in patients with FSHD. All patients are tested before and after 12 weeks of cycle-ergometer exercise. Maximal oxygen consumption and a functional 6 min walk test(6MWT) is used as primarily effect goals. Secondary effect goals are risk of falls and daily activity level. We measure manuel muscle strength, a balance test, a 5-time-up-and-down-chair-test, a 14 steps-stair-test, accelerometer measuring, daily activity level questionnaire and SP36. We use a blinded randomized controlled trail. The interventions are 12 weeks regular exercise and consumption of a post-exercise drink. The exercise consists of 30 session of 30 minutes moderate exercise on a cycle-ergometer. After each exercise session patients consume a protein or a non-caloric placebo drink.
Study Type
INTERVENTIONAL
Allocation
RANDOMIZED
Purpose
SUPPORTIVE_CARE
Masking
QUADRUPLE
Enrollment
42
Duration: 3 session each week, in 12 week. Intensity: 70 % of VO2max. Type: cycle-ergometer exercise.
Dosage: 45g powder mixed in 0.5 L water After each exercise session, 3 times each week, in 12 weeks.
Neuromuscular Research Unit, Department of Neurology, Rigshospitalet
Copenhagen, Denmark
Maximal oxygen consumption test
All participant are tested with a standardized maximal oxygen consumption test. Maximal oxygen uptake pr. minute pr. kg body (VO2max) weight and maximal work load (Wmax) is measured. The effect of intervention is found in different between groups in relative and absolute improvement in VO2max and Wmax.
Time frame: up to Week 12
6MWT
6 min walk test. Outcome in meter.
Time frame: up to Week 12
Risk of falls
Measuring of: Muscle strength, balance, 5-times-up-and-down-chair-test, 14-steps-stair-test.
Time frame: week: 0 and 12
Daily activity level
assessment of daily-activity level by accelerometer daily-activity level questionary SP36 life quality questionary
Time frame: week: 0 and 12
Blood samples
Creatine Kinase and myoglobin levels (safe parameter) Inflammatory level
Time frame: week: 0, 4, 7, 10, 12
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