Arm Cycling in Facioscapulohumeral Dystrophy (FSHD) Patients

The investigators believe that arm cycling has the potential to be effective in maintaining or improving shoulder muscle functioning. However, there is no evidence for its safety or effectiveness in the upper extremity. The aim of the study is to establish the ability of FSHD sufferers to perform arm cycling to underpin the future design of an arm exercise trial. The study hypothesis is that the limited range of shoulder movement and muscle weakness should not impact the ability of FSHD sufferers to perform arm cycling.

Study Overview

• Status: Completed
• Conditions: Facioscapulohumeral Muscular Dystrophy
• Intervention / Treatment: Other: Arm cycling

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

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years to 60 years (ADULT)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

Description

Inclusion Criteria:

1. Aged 18-60 years
2. Genetically confirmed diagnosis of FSHD
3. Willing to attend the assessment session
4. Able to understand the participant information sheet and provide written informed consent

Exclusion Criteria:

1. Aged <18 years or >60 years
2. Co-morbidity that would affect their ability to perform arm cycling
3. Unwilling to complete the assessments of the study
4. Unable to understand the participant information sheet and provide written informed consent.

Study Plan

How is the study designed?

Design Details

• Primary Purpose: TREATMENT
• Allocation: NA
• Interventional Model: SINGLE_GROUP
• Masking: NONE

Number of Arms

1

Arms and Interventions

Participant Group / Arm

Intervention / Treatment

EXPERIMENTAL: Arm cycling; Participants will perform arm cycling using a table-top arm cycler. Cadence and resistance of the exercise will be determined as per each individual's tolerance. All exercise will be supervised by the neuromuscular specialist physio, to ensure it is completed safely. Participants will be able to have plenty of breaks during the assessments.

Other: Arm cycling; The arm cycle will be placed on table with height adjusted to be the same as the acromion (highest point on shoulder). Participants will start arm cycling at a low intensity and increase as per tolerance. They will exercise for a maximum of two minutes at a time, followed by a rest period for 30 seconds. In this manner 5 cycles of exercise and rest will be performed. The exercise sessions are anticipated to last a maximum of 20 minutes, depending on the patient's abilities.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Oxford Shoulder Score	A 12 item patient-reported shoulder instability questionnaire. Each of the questions presents five possible responses corresponding to a score of 0 (most disability/pain) to 4 (least disability/pain). when the twelve items are summed, the questionnaire then produces a single score with a range from 0 (most disability/pain) to 48 (least disability/pain).	At one-off study visit: Prior to completion of exercise session

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Range of movement	A physiotherapist-assessed score of amount of movement at a joint in degrees, using goniometer. Range of movement is measured for both left and right shoulder joints in abduction, flexion and extension. Range of movement is measured for both left and right elbow joints in flexion and extension.	At one-off study visit: Prior to completion of exercise session
Strength of muscles at shoulder and elbow joints	Assessment of muscle strength at at shoulder and elbow joints, by physiotherapist using a hand held dynamometer device. Using the dynamometer, the physiotherapist pushes against the joint and the patient resists. The dynamometer then provides a strength reading in Newton Metres of Torque. Both left and right shoulders will be assessed in abduction, flexion and extension. Both left and right elbows will be assessed in extension and flexion.	At one-off study visit: Prior to completion of exercise session
Resistance of arm cycling	Patients will be asked to complete the arm cycling exercise at whatever resistance they feel most comfortable. Chosen resistance will be recorded.	At one-off study visit: During exercise session
Cadence of arm cycling	Patients will be asked to complete the arm cycling exercise at whatever speed they feel most comfortable. The chosen cadence (speed) in cycles per minute will be recorded.	At one-off study visit: During exercise session
Borg Rate of Perceived Exertion (RPE)	A patient-reported score of effort during exercise. The score ranges from 6 (no exertion at all) to 20 (maximal exertion). Patients will be asked to exercise at a level that is comfortable to them and indicate which RPE score they feel that they are working to, for each cycle of the arm cycling exercise.	At one-off study visit: During exercise session
Video analysis	Video analysis of shoulder and elbow joint angles during arm cycling. Analysis software will be used to measure patient wrist, elbow and shoulder flexion and extension as well as trunk orientation during the exercise session.	At one-off study visit: During exercise session

Other Outcome Measures

Outcome Measure	Measure Description	Time Frame
Patient suggestions	A free-text questionnaire asking patients to report any suggestions that they may have regarding factors that may limit their performance during this type of exercise, particularly if it were to be completed at home. There is no score for this question.	At one-off study visit: Immediately after completion of exercise session
Patient willingness to continue	A free-text questionnaire asking patients to report whether they would be willing to continue with this sort of exercise at home, should it be proved effective. There is no score for this question.	At one-off study visit: Immediately after completion of exercise session

Collaborators and Investigators

• Sponsor: Robert Jones and Agnes Hunt Orthopaedic and District NHS Trust
• Investigators: Principal Investigator: Richa Kulshrestha, MBBS, MRCPCH, The Robert Jones and Agnes Hunt Orthopaedic Hospital NHS Foundation Trust

Study record dates

Study Major Dates

• Study Start (ACTUAL): December 14, 2016
• Primary Completion (ACTUAL): July 14, 2018
• Study Completion (ACTUAL): July 18, 2018

Study Registration Dates

• First Submitted: February 4, 2020
• First Submitted That Met QC Criteria: February 11, 2020
• First Posted (ACTUAL): February 12, 2020

Study Record Updates

• Last Update Posted (ACTUAL): February 12, 2020
• Last Update Submitted That Met QC Criteria: February 11, 2020
• Last Verified: February 1, 2020

More Information

Terms related to this study

• 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: RL1 627

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No