The Effect of Aerobic Exercise, on Fitness and Functional Muscle Strength, in Patients With Muscular Dystrophy

The Effect of a Bike Ergometer Training Program in Patients With Bethlem Myopathy

Muscular dystrophy is a group of disorders that are characterized by progressive muscle weakening and loss of muscle mass, caused by defects in muscle proteins. Muscular dystrophy is almost always inherited disorders, and so far, no curative treatments exist.

Previous studies have shown that endurance training significantly improves fitness and self-assessed muscle function in a variety of muscular dystrophies. In this study, we wish to investigate whether patients with Bethlem myopathy (a specific form of muscular dystrophy) also benefit from endurance training.

The study consists of two test days, a 10-week training period and five blood tests.

Patients will be required to train, three times per week, for 10 weeks, on a bike ergometer with a specific training intensity, under pulse rate monitoring.

The training period will be flanked by two test days, where we will determine, and compare the patients' muscle strength and fitness level, from before to after the training program. The patients conditioning level will be determined from a 15 minute cycle ergometer test and patients will undergo three functional tests to determine their functional muscle strength.

We will as a safety measure analyze blood tests before, during and after the training program for the muscle enzyme creatine kinase (an indicator of muscle damage) and through adverse effects as reported by patients during weekly telephone-consultations with the Principal investigator.

We anticipate, that Bethlem myopathy patients will have a similar rise in fitness level and functional muscle strength, as that seen in patients with other forms of muscular dystrophy, who undertake a similar training program.

Study Overview

• Status: Completed
• Conditions: Bethlem Myopathy
• Intervention / Treatment: Behavioral: Moderate-intensity training

• Study Type: Interventional
• Enrollment (Actual): 7
• Phase: Not Applicable

Contacts and Locations

Study Locations

• Denmark
  • Copenhagen, Denmark, 2100
    • Rigshospitalet, Neuromuscular Research Unit, section 3342

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 12 years to 86 years (Child, Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

Description

Inclusion Criteria:

• Patients with Bethlem myopathy

Exclusion Criteria:

• Patients with disorders that would confound the interpretation of the effect from training (cardiomyopathy, pregnant women, etc.)
• Patients who are deemed mentally unfit for cooperation with the training program

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Prevention
• Allocation: N/A
• Interventional Model: Single Group Assignment
• Masking: None (Open Label)

Number of Arms

1

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
Experimental: Moderate-intensity training; Regular supervised moderate-intensity training on a bike ergometer, three times per week, for 30 minutes a total of 10 weeks.	Behavioral: Moderate-intensity training; Training at 70% of VO2max, for 1/2 hour, three times weekly, for 10 weeks, on a bike ergometer.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Maximal oxygen uptake (VO2max)	VO2max will be determined during an incremental bike test to exhaustion. VO2max is a physiological measure of physical fitness. VO2max relies on a persons ability to supply and consume oxygen ("energy") in muscle tissue.	after 10 week bike ergometer training program

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Functional muscle strength	Functional muscle strength will be determined by performance in three functional tests: a five-repetition-sit-to-stand-test, a 6 minute walk test and a 14 step stair test.	After completion of a 10 week training program
Muscle strength determined by dynamometry		After completion of a 10 week training program
Self-assessed improvements in muscle function		After completion of a 10 week training program
Self-assessed improvements in incidence of problems that are related to inactivity (in patients who are confined to a wheel-chair)		After a 10 week training program

Other Outcome Measures

Outcome Measure	Measure Description	Time Frame
Safety	The safety of undertaking a 10-week training program will be determined by analyzing reports of adverse effects faced during the training program, and by interpretation of Creatine Kinase levels measured before, during and after the training program.	During a 10 week training program

Collaborators and Investigators

• Sponsor: Rigshospitalet, Denmark
• Investigators: Principal Investigator: Christoffer R Vissing, BsC, Rigshospitalet, Neuromuscular Research Unit

Study record dates

Study Major Dates

• Study Start: September 1, 2013
• Primary Completion (Actual): December 1, 2014
• Study Completion (Actual): January 1, 2015

Study Registration Dates

• First Submitted: July 3, 2013
• First Submitted That Met QC Criteria: July 3, 2013
• First Posted (Estimate): July 10, 2013

Study Record Updates

• Last Update Posted (Estimate): April 6, 2016
• Last Update Submitted That Met QC Criteria: April 4, 2016
• Last Verified: April 1, 2016

More Information

Terms related to this study

• Keywords: VO2max; Aerobic training; Bethlem myopathy
• Additional Relevant MeSH Terms: Nervous System Diseases; Genetic Diseases, Inborn; Joint Diseases; Musculoskeletal Diseases; Neuromuscular Diseases; Muscular Disorders, Atrophic; Muscular Dystrophies; Muscular Diseases; Contracture
• Other Study ID Numbers: H-2-2013-066