Arm Cycling in Facioscapulohumeral Dystrophy (FSHD) Patients

Study Overview
=================
Brief Summary
-----------------
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.

Official Title
-----------------
Arm Cycling in Facioscapulohumeral Dystrophy (FSHD) Patients

Conditions
-----------------
Facioscapulohumeral Muscular Dystrophy

Intervention / Treatment
-----------------
* Other: Arm cycling


Participation Criteria
=================
Eligibility Criteria
-----------------
Inclusion Criteria: Aged 18-60 years Genetically confirmed diagnosis of FSHD Willing to attend the assessment session Able to understand the participant information sheet and provide written informed consent Exclusion Criteria: Aged <18 years or >60 years Co-morbidity that would affect their ability to perform arm cycling Unwilling to complete the assessments of the study Unable to understand the participant information sheet and provide written informed consent.

Ages Eligible for Study
-----------------
Minimum Age: 18 Years
Maximum Age: 60 Years

Sexes Eligible for Study
-----------------
All

Accepts Healthy Volunteers
-----------------
No

Study Plan
=================
How is the study designed?
-----------------

Design Details

Primary Purpose: Treatment
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Arm cycling<br>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<br>* 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.<br>|

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 |