Arm Cycling in Facioscapulohumeral Dystrophy (FSHD) Patients

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).

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.

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.

Patients will be asked to complete the arm cycling exercise at whatever resistance they feel most comfortable. Chosen resistance will be recorded.

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.

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.

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.

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.

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.