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

Bethlem Myopathy Clinical Trial

Official title:

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

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT01895283
• Other study ID #: H-2-2013-066
• Status: Completed
• Phase: N/A
• First received: July 3, 2013
• Last updated: April 4, 2016
• Start date: September 2013
• Est. completion date: January 2015

Study information

• Verified date: April 2016
• Source: Rigshospitalet, Denmark
• Contact: n/a
• Is FDA regulated: No
• Health authority: Denmark: Danish Dataprotection AgencyDenmark: The Regional Committee on Biomedical Research Ethics
• Study type: Interventional

Clinical Trial Summary

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.

Recruitment information / eligibility

• Status: Completed
• Enrollment: 7
• Est. completion date: January 2015
• Est. primary completion date: December 2014
• Accepts healthy volunteers: Accepts Healthy Volunteers
• Gender: Both
• Age group: 16 Years to 90 Years

Eligibility

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 Design

Endpoint Classification: Safety/Efficacy Study, Intervention Model: Single Group Assignment, Masking: Open Label, Primary Purpose: Prevention

Related Conditions & MeSH terms

• Bethlem Myopathy
• Contracture
• Muscular Diseases
• Muscular Dystrophies

Intervention

Behavioral:
• Moderate-intensity training
Training at 70% of VO2max, for 1/2 hour, three times weekly, for 10 weeks, on a bike ergometer.

Locations

Country	Name	City	State
Denmark	Rigshospitalet, Neuromuscular Research Unit, section 3342	Copenhagen

Sponsors (1)

Lead Sponsor	Collaborator
Rigshospitalet, Denmark

Country where clinical trial is conducted

Denmark, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Other	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	Yes
Primary	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	No
Secondary	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	No
Secondary	Muscle strength determined by dynamometry		After completion of a 10 week training program	No
Secondary	Self-assessed improvements in muscle function		After completion of a 10 week training program	No
Secondary	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	No