Physical Training Introduction in Lifestyle of Facioscapulohumeral Dystrophy Patients (FSHD1)

Exercise and Myopathies. Physical Training Introduction in Lifestyle of Facioscapulohumeral Dystrophy Patients: Functional, Tissue and Quality of Life Benefits.

It is now accepted that physical activity is not deleterious in myopathies, including muscular dystrophies. In patients suffering from facioscapulohumeral dystrophy (FSHD), aerobic training has been reported to be associated to physiological and functional positive effects without alteration in quality of life. The review papers from Van der Kooi et al. (2005), Cup et al. (2007) and Féasson et al. (2010) suggest that the combination of endurance and strength training is even more relevant. Only a few controlled and randomized studies have been conducted on this topic. The impact of such training programs on the skeletal muscle regenerative capacities has not been yet addressed. Moreover, due to the fact that training programs are mainly performed on short-term supervised periods, there is a lack of knowledge regarding long-term effects, patient's autonomy and whether or not regular exercise practice can be maintained in patient's daily life. Also, only a few experiments have reported an integrative view of the potential benefits of such programs on functional, biological and quality of life.

Study Overview

• Status: Completed
• Conditions: Muscular Dystrophy, Facioscapulohumeral
• Intervention / Treatment: Other: Physical training; Other: Control

Detailed Description

Our research project aims at establishing a program of physical training that will fulfill the two following conditions: (i) being compatible with the daily professional, social and family activity of the patients so it can be integrated in their life habits and (ii) being intensive enough to induce functional benefits. This experimental work will be based on multi-factorial evaluations, i.e. biological, physiological, functional, and quality of life questionnaires. This work will be based on a collaboration between the Universities of Saint Etienne (L. Féasson), Grenoble (B Wuyam) and Örebro (F Kadi) within the Rhône-Alpes Reference Centre for Rare Neuromuscular Diseases (JC Antoine).

It is accepted that exercise therapy can be recommended for patients with myopathies but long-term training load still has to be determined for each pathology. In the specific context of facioscapulohumeral dystrophy, we aim at associating the scientific evaluation of physical activity benefits and a therapeutic education of patients in order to contribute to recommendations for physicians and physiotherapists. The purpose of this study is to combine an integrated approach with a better understanding of biological process implicated in this physiological treatment strategy.

• Study Type: Interventional
• Enrollment (Actual): 15
• Phase: Not Applicable

Contacts and Locations

Study Locations

• France
  • Grenoble, France, 38000
    • CHU de Grenoble
  • Saint-etienne, France, 42055
    • CHU de SAINT-ETIENNE

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

Description

Inclusion Criteria:

• facioscapulohumeral dystrophy
• Being capable of supporting an exercise on ergocycle
• Social Security regimen affiliated
• Consent form signed

Exclusion Criteria:

• Severe cardiac or respiratory insufficiency
• Cardiac pacemaker
• Morbid obesity (BMI upper to 35)
• Anti platelet therapy

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Treatment
• Allocation: Randomized
• Interventional Model: Parallel Assignment
• Masking: None (Open Label)

Number of Arms

2

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
Experimental: Physical training; The training will consist in 3 sessions of 35 min of training per week at home on an ergocycle. As a result, lower limb muscles will be mainly solicited. These muscles are heterogeneous in terms of deficiency, but this latter is compatible with cycling. The training will be divided in (i) 2 sessions of 30 min aerobic exercises at a constant but moderate (60% of maximal aerobic power, MAP) intensity followed with 5 sets of 10 revolutions at near-maximal intensity and (ii) an interval-training session. This latter session will consist in 5 min warm-up at 40% MAP followed by 5 times 1 min at 80% MAP (recovery = 4 min at 40% MAP) followed by 5 min of active recovery. Over a 2 to 4 weeks initial period, the program will be conducted in the laboratory or at home under the supervision of a coach. Then a systematic supervision of the sessions by the coach will be performed by phone, by using the heart rate recordings and values of Analogic Visual Scale for pain and fatigue.	Other: Physical training; Physical training during 24 weeks
Other: control; None intervention	Other: Control; No intervention

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
maximal oxygen uptake (VO2max)	VO2max is meseasured directly by respiratory gas analysis during maximal exercise test on ergocycle	Week 24

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Questionnaire of quality of life	"short form 36 health survey questionnaire" SF36	Day 0, Week 6, Week 12, Week 18 and Week 24
Biopsy	biopsy of the vastus lateralis muscle	Day 0 and Week 24
maximal oxygen uptake (VO2max)	VO2max is meseasured directly by respiratory gas analysis during maximal exercise test on ergocycle	Week 6, Week 12 and Week 18
Questionnaire of subjective fatigue	Fatigue severity scale (FSS)	Day 0, Week 6, Week 12, Week 18 and Week 24
nuclear magnetic resonance imaging	In Paris centre, a total body NMR imaging will be done with standard T2- and T1-weighted images.	Inclusion

Collaborators and Investigators

• Sponsor: Centre Hospitalier Universitaire de Saint Etienne
• Collaborators: Örebro University, Sweden; Association Française contre les Myopathies (AFM), Paris
• Investigators: Principal Investigator: Léonard FEASSON, MD-PhD, CHU de SAINT-ETIENNE; Study Chair: Fawzi KADI, MD-PhD, Örebro University, Sweden

Study record dates

Study Major Dates

• Study Start: October 1, 2010
• Primary Completion (Actual): January 1, 2015
• Study Completion (Actual): January 1, 2015

Study Registration Dates

• First Submitted: April 28, 2010
• First Submitted That Met QC Criteria: May 4, 2010
• First Posted (Estimate): May 5, 2010

Study Record Updates

• Last Update Posted (Estimate): April 1, 2015
• Last Update Submitted That Met QC Criteria: March 31, 2015
• Last Verified: March 1, 2015

More Information

Terms related to this study

• Keywords: Exercise; Muscular Dystrophy; Physical training; Facioscapulohumeral
• Additional Relevant MeSH Terms: Nervous System Diseases; Genetic Diseases, Inborn; Musculoskeletal Diseases; Muscular Diseases; Neuromuscular Diseases; Muscular Disorders, Atrophic; Muscular Dystrophies; Muscular Dystrophy, Facioscapulohumeral
• Other Study ID Numbers: 1001035; 2010-A00288-31 (Other Identifier: AFSSAPS); 2009.1087-14263 (Other Grant/Funding Number: AFM)