Protein Supplementation and Exercise in Patients With FSH Muscular Dystrophy- a Randomized Placebo Controlled Study

The Effect of Protein Supplementation Doing Regular Exercise in Patients With Facioscapulohumeral Muscular Dystrophy - a Blinded RCT Study

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.

Inclusion Criteria:

• Verified FSHD
• Age 18-65
• Untrained. Less than two hours cardio-training each week the last 4 month.

Exclusion Criteria:

• Competitive disorders
• Pregnant and breastfeeding
• Unable to walk 200 m within 6 min.