Duchenne muscular dystrophy

Definition:it is one of genetic determined myopathy that are characterized by steady progressive degeneration and weakness of the muscle.it is x linked disease and is insidious in its onset.

clinical presentation:

1-Delayed motor development.

2-Walking is clumsy and there is frequent falling.

3-Walking with wide BOS with waddling and lurching gait.

4-Walking on tips of foot.and relactance of walking and running.

5-As weakness increase , gower sign appear: as child climb him self for raising due to weakness of the back and abdominal muscle, calf, iliopsoas,iliotibial band and knee flexors.

6-Using wheel chair for ambulation.

7- Affection of respiration.

8-Positional deformity as scoliosis.

7-Obesity due to lack of activity and mobility and sitting on wheel chair.

By progression:

1- there is shortening in specific muscles.

2- Weakness and deposition of fibrous tissue.

Shortening of specific muscle help sitting before weakness but he can not maintain the upright posture.

Management: 

Goal: maintainance of child functional and ambulant as much as possible.

Evaluation:

1- Functional ability assessment:what he can do from his ADL activity according to his age:

At 6-7 years : he can walk and run.

At 9-10 years:child can walk with assisstance due to deterioration of the muscles.

Examples for functional assessment.

For upper limb: 1- bilateral abduction and elevation of both arms or one.

                            2- raise hand just above head.

                            3- mouthing only.

For lower limb:1-walking and ascending stairs without assistance.

                          2 -walking and ascending stairs mild assistance.

                          3-walking and ascending stairs with moderate assistance.

                          4-use wheel chair for mobility.

                          5-just getting off or raising from wheel chair.

2-Muscle power assessment: manual muscle test,we do group muscle test to avoid exhuation,

it is applied for the following muscles:1-upper and lower fibers of trapezius.

                                                                2- serratus anterior.

                                                                3- pectoralis .

                                                                4-abdominal.

                                                                5-gluteus maximus and medius.

                                                                6- rhomboids.

                                                                7-triceps.

                                                                8-iliopsoas.

                                                                9-quadriceps.

                                                               10-tibialis anterior.

                                                                11-latissimus dorsi. 

3- ROM assessment: active and passive range of motion.

limited active range of motion refers to muscle weakness.but if there is limitation in both active and passive range this refers to muscle tightness.

Prolonged sitting on wheel chair leads to limitation of range of hip extension ,knee extension,ankle dorsiflexion and elbow extension.

4- Flexability assessment: is used for test for tight muscles as

a- Iliopsoas muscle: thomas test.

b-Ilio tibial band: obar test.

c- Hamstring and calf muscles.

5- Muscle tone assesment:we can use gower test :patient sitting and therapist stand behind him and hold child shoulder from under axilla then elevate shoulder. there is increase in the upward displacement with sudden release , the shoulder drop sudden not gradually.

 6-Gait and wheel chair assessment:

a- By observation: we can see pattern of walking as walking on tip of toes with wide BOS.

b-wheel chair: if he can do his activities as transfere and the distance that patient can travel and the amount of energy expenditure.
7-Pulmonary function assessment: to evaluate vital capacity and oxygen consumption.
Respiratory failure is the common cuase of deathdue to weakness of the respiratory muscles and accumulation of secretions which lead to infection.
treatment:
1- To prevent deformity:a- ROM exercise for all jointsin the early stage     

                                           b- Stretching exercise for hamstring and calf muscles.

                                           c- Application of braces as AFO and KAFO.

                                            d- Strengthening exercise .

2- To maintain functional ability: this can be achieved through proper exercises  which must be:

                                                    a- functional exercise.

                                                    b- aerobic exercise.

                                                    c- exercise for large group of muscles.

                                                    d- child must talk rest to avoid fatigue.

3- Family support: parents are depressed and feel guilt and furstration so we must help them to accept this problem and avoid giving false hope for them .

4- Pain control: proper exercise help reducing pain through prevention of deformity and delay appearance of complication.

For child who sits on wheel chair must change his position periodically and change  position of the propelling hand every 6months.

For child who is bed ridden ,we must use air matress to avoid development of pressure ulcers also we must do respiratory care in the form of breathing exercise and posture drainage.

5- To control obesity: it develops as child in put is more than the out put and decrease his activity level .so we advice for proper diet and exercise program and avoiding food with high caloric value.                                        

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