Effectiveness of Upper Extremity Rehabilitation in pwFSHD (Patient With Facioscapulohumeral Dystrophia)

Facioscapulohumeral Muscular Dystrophy (FSHD) is one of the most common forms of muscular dystrophy, characterized by pronounced skeletal weakness and with a broad spectrum of diseases. It is a hereditary disease seen in 3-5/100,000 of society, usually starting with weakness in the facial and shoulder muscles and progressing to the trunk, pelvis and leg muscles, giving symptoms in the twenties. In FSHD, which shows slow progression and can lead to loss of ambulation ability in about 20% of patients, patients may have difficulty performing activities above shoulder level with the influence of the periscapular area.

The goal of FSHD treatment is to improve muscle strength and/or function. Treatments include medical, conservative and surgical methods. The aim of surgical methods is to improve shoulder function and prevent pain caused by the movements of the scapula. The publications on physiotherapy interventions and aerobic exercise are available as conservative treatment. In patients diagnosed with FSHD, conservative treatment is frequently used to improve muscle strength, regulate function and improve the quality of life of patients.

Patients with FSHD use their affected upper extremities asymmetrically, which leads to the development of restrictive compensation mechanisms in the development of symmetrical postural control. Postural control deficits may occur due to limited use of the affected scapula in individuals with FSHD. Accordingly, in cases with FSHD, there is the use of atypical movements for balance and mobility. It is not yet known whether people with FSHD really have poorer dynamic stability during self-initiated whole-body movements such as walking, and at what stage of the disease these difficulties arise.

Accordingly, the aim of this study was to examine the effects of rehabilitation approaches applied to the upper limb on upper limb function, balance and walking in patients with FSHD.

H1: Within the group of patients with FSHD patients underwent surgery arthrodesis surgery scapulothoracic applied to pre-treatment with the parameters of the rehabilitation program for the evaluation of upper limb functionality after applying the upper extremities, postural control and gait parameters examined, there is statistical difference between the groups.

Voluntary participants who have been diagnosed with FSHD and agreed to voluntary health subjects will be included in the study. Signed voluntary consent will be obtained from participants. Participants will be divided into tree groups. One group will include patients who have undergone scapulothoracic arthrodesis surgery, while the other group will include patients who have not undergone surgery and control group will include participants who have agree to participate the study. A progressive rehabilitation program for the upper extremities will be applied to patients in a group of individuals who have undergone and have not undergone surgery. Both groups will undergo some outcome measures to assess their upper extremity function, balance and walking.

Eligibility Criteria:
Inclusion Criteria:

* Individuals to be included in all groups 18-50 years
* Score between 0.5 and 3.5 according to CSS (Clinical Severity Score) of the cases to be included in the intervention groups.
* Be rated between 3-5 according to FAS (functional ambulation scale) of the cases to be included in intervention groups
* For patients who will be included in the intervention group who have undergone surgery, bilateral scapulothoracic arthrodesis surgery within 3 months at the earliest
* Individuals who will be included in the control group consisting of healthy individuals have not undergone any orthopedic surgery

Exclusion Criteria:

* Having received regular physiotherapy for 1 month, at least 2 days a week in the last 6 months
* Presence of additional neurological problem/problems
* Presence of any other upper limb problem/problems and surgery
* Presence of lower limb problems/problems that can cause balance and walking problems
* Having undergone Spinal fusion surgery
* Having lower back pain described 6 and above according to VAS (Visual Analog Scale) in intervention and control groups
* Having a level of visual and auditory problems that will prevent communication