Effectiveness of Upper Extremity Rehabilitation in pwFSHD (Patient With Facioscapulohumeral Dystrophia)
Condition: Facioscapulohumeral Muscular Dystrophy
MeSH terms: Muscular Dystrophies; Muscular Dystrophy, Facioscapulohumeral
Study type: Observational

Summary: 
      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.
    

Detailed description: 
      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.
    

Intervention name(s): upper extremity rehabilitation program; Results of assessment
Intervention type(s): Other; Diagnostic Test
Intervention description(s): Patients with FSHD who have undergone and have not undergone surgery will be given activity training by the same physiotherapist after they agree to participate in the study. Both groups will be given the same exercises, the same number of repetitions and the same frequency. A two-phase exercise protocol will be established and progression will be performed after 4 weeks. The exercises will be based on the upper extremity and will include various types of exercises and the primary affected November muscles in the upper extremity. It is planned that the exercises will be accompanied by a physiotherapist 2 days a week, with a total of 16 sessions for 8 weeks. It is planned that there will be a total of 8-10 exercises in 10 repetitions in the exercise program. The total length of the exercise session will be 30 minutes. It is planned that the same physiotherapist will be one-on-one with the patient from the beginning to the end of the exercises.; The total follow-up period of participants included in intervention programs will be 8 weeks. Evaluations will be evaluated by physiotherapist before and at the end of treatment.
Observational model: Case-Control
Time perspective: Prospective
Sampling method: Probability Sample
Study population: 
        Patients who have been diagnosed with FSHD, patients who have undergone scapulothoracic
        arthrodesis, healthy people for control group
      
Keywords: Neuromuscular Disease; Scapulothoracic Arthrodesis; Exercise Therapy; Kinetic Chain