Muscular Dystrophy

Muscular Dystrophy is a musculoskeletal condition in which muscle fibres are replaced by fat cells, and the muscle gradually wastes (trophy). This causes "a progressive" loss of muscle power.

1.0What is Muscular Dystrophy?

• Muscular Dystrophy is a condition in which muscles gradually become weaker and weaker month by month and year by year. 
• Because the disability gradually gets worse, we say it is progressive.

2.0Muscular Dystrophy Causes 

It mainly affects the body (rarely girls) 

• Often, brothers or male relatives have problems with this. 
• First signs appear around ages 3 to 5. The Child may seem awkward or clumsy, or they begin to walk 'tiptoe' because he cannot put his feet flat. Runs strangely. 
• Falls often. 
• The problem gets steadily worse over the next several years. 
• Muscle weakness first affects the feet, fronts of things, hips, belly, shoulders and elbows. Later, it affects the hands, face and neck muscles. 
• Most children become unable to walk by age 10. 
• May develop a severe curve of the spine. 
• Heart and breathing muscles also get weak. The Child usually dies before age 20 from heart failure or Pneumonia.

3.0Signs of Muscular Dystrophy 

• To get up from the ground, the child 'walks up' his things with his hands. (climbing his own body)––Gower's sign. 
• This is mainly because of weak thigh muscles.

Cause :

• The exact cause of the disease is not fully known. 
• It is observed to be inherited genetically from the mother by mostly the male Child. 
• There are even cases where no positive history is tracked in the family.

Types :

• There are several types. 
• The most common and severe type is Duchenne or Duchenne Muscular Dystrophy.

4.0Duchenne Muscular Dystrophy 

• Characteristics of Duchenne Muscular Dystrophy are
• Progressive weakness of muscle, which starts at the age of 3 years. 
• Weakness progresses from the muscles of the ankle joint to the muscles of the hand, face and neck. 
• The muscles around the pelvic and shoulder girdles are more affected, leading to a typical manner of getting up off the floor (Gower's sign). 
• The muscles show false enlargement (pseudohypertrophy) owing to the deposit of fatty material in place of degenerated muscle tissue. The common site of pseudohypertrophy is the calf muscle. 
• Postures and gait become atypical. [waddling gait] 
• Respiratory and cardiac muscles are eventually affected in the later stage, leading to death.

5.0Associated Problems

• Locomotor Retardation: Locomotor retardation starts appearing in the form of frequent falls and slowness in walking at an early age. Subsequently, climbing, running, and finally, walking become impossible. The person must move around only in a wheelchair in the last stages.
• Skeletal Deformities: Weakness of muscles leads to their shortening, i.e. contractures. This leads to deformities. 

(a) Trunk-Scoliosis 

(b) Neck-flexion deformity 

(c) Lower limb –– (i) ankle - downward and downward bending [plantar flexion] (ii) knee and hip - flexion deformity  

• Obesity: Because of restricted activity and probable compensatory overeating, these children tend to become obese. 
• Slowness in Learning: Slowness in learning, not mental retardation, is present in about 70 per cent of the cases; the intelligence Quotient usually is above 80 but generally below 90.

Educational Implication 

• Progression of the disease does not allow the Child to attend school regularly. 
• This leads to poor performance in school work and eventual scholastic backwardness. 
• Owing to their slower learning speed, a special teacher doing a remedial educational programme at home is most effective. 
• But even such a special educational approach becomes difficult as the age progresses because the Child becomes increasingly tired. 
• The motivation to study becomes less.

Therapeutic Management

• Helping the Child to keep Walking for as long as Possible 

Other aids

• The Child will reach a point where he needs to use crutches, and later (often by age 10), he will not be able to walk. 
• Do not force him when it becomes too hard. Instead, try to obtain or make a wheelchair. 
• Breathing deeply is important, especially when the muscles that move the lungs begin to weaken. 
• Encourage the Child to sing loudly, shout, blow whistles, and blow up balloons.