Hydrotherapy In Child With Progressive Muscular Dystrophy (Case Study)

P M D (Progressive Muscular Dystrophy) ▶ Description : The term muscular dystrophy refers to a group of disorders characterized by progress muscle weakness and loss of muscle tissue.

All forms of muscular dystrophy cause progressive weakness and degeneration of the muscle that control movement. Some also affect the heart muscle or other organs. Age of onset is between infant to late teens.

Muscular dystrophy includes many inherited disorders such as ; 1. Becker ’ s muscular dystrophy - Slowly progressive form of MD ; affects legs and pelvis most severely 2. Duchenne ’ s musclar dystrophy - Similar to Becker ’ s, but progresses more rapidly

3. Facioscapulaohumeral muscular dystrophy - Mild, slowly progressive form ; face, shoulder, and upper arms affected 4. Limb-girdle muscular dystrophy

▶ Symptom Initial Symptoms (progressive weakening of muscles) - Waddling manner of walking - Difficulty climbing stairs or rising from a sitting position - Repeated falling

Later symptom - Abnormal enlargement of the calves (Pseudohypertrophy) - Abnormal curved spine - Abnormal fixation of certain joint (Joint contracture - equinovarus) - Distortion of the body - Muscle contracture and stiffening

▶ Diagnosis - Physical examination & history - A serum CPK(muscle enzyme) is highly elevated. - NCV study & EMG - Blood enzyme tests - Muscle tissue biopsy confirms the diagnosis

Duchenne Muscular Dystrophy - A rapidly progressive form of muscular dystrophy - Occurs in approximately 2 out of 10,000 people - Symptom usually appear in boys aged By 10 age, brace may be required for walking, and by age 12, most patients are confined to a wheelchair.

- Inherited in an X-linked recessive pattern

Case Study - Name : Kwon ● ● - Sex : Male - Date of birth : Age : 10 - Onset : P/I : 평소 건강하던 아이로 1 yr ago(2000) L/E 의 Weakness 있었으나 observation 했고, 2001 년 초 Up stair 시 난간을 잡고 가고 Sit to stand 가 어려운 Sx. 로 연세 세브란스 병원 visit

Examination - CPK : OT / PT : 210 / 385 NCV : H. reflex (bilateral) EMG : Mild fibrillation, positive sharp wave, amplitude polyphasic MUAPs Waddling gate (+) Gower sign (+) Psedohypertrophy (±/±) Ankle contracture (-/-) DTR : Knee jerk (-/-)

Clinical Examination - Supine position control on the water Chair sitting control Upright position control in the water Breathing control Check vital capacity Examination on the mat (GMFM)

Problem list 1. Muscle weakness - Posture change ( trunk, L/E ) 2. Rt. Ankle contracture (equinovarus) 3. Scoliosis 4. Decrease vital capacity

Goal 1. Decreasing muscle weakness. 2. Decreasing deformities. 3. Maintain respiratory muscles and vital capacity 4. Decreasing the risk of respaitory infections 5. The child ’ s personal attitude to the disease

Gower sign

Pseudohypertrophy & Ankle contracture Rt. Ankle contracture Pseudohypertrophy of both calves

The advantages of activity in water for children with PMD 1. Water mobility is encouraged and can be maintained long after activity on land has become extremely difficult 2. Stamina and endurance can be encouraged 3. Respiratory function is enhanced 4. A social and recreational activity is established that can be enjoyed with others, enabling a happier and more interasting active life to be maintained

Sitting activity on the land

Balance control (Water horse)

Supine position control (Halliwick swimming)

Erect posture control (walking in the water)