Duchenne Muscular Dystrophy Life Expectancy

Muscular dystrophy is a genetic disorder, classified under 20 categories of which, Duchenne muscular dystrophy is the most common one and affects only males, and in only extreme cases, does it impinge females. It causes progressive tendon weakness leading to disruption of muscle cells, targeting a single protein called dystrophin, found in the muscle fibers. The malady is named after Dr. Duchenne de Boulogne who pursued the subject during the 19th century. The intensity of respiratory and cardiac complications govern the life expectancy of the patient.

What is Duchenne Muscular Dystrophy
This is a eugenic condition that is observed in early childhood itself inflicting weakness of fibrous tissues which affects the proximal muscles of the trunk, hips and shoulders, and gradually leading to loss of ability to walk and death. It is not observed at birth but as the child grows, the weakness becomes more and more apparent.

Causes
It is a sex-linked disorder, affecting only boys after they inherit an X-chromosome with mutated dystrophin gene, passed on by the mother. This disorder hampers the production of dystrophin, the important muscle protein, eventually causing dissolution of the cells.

Symptoms
The prominent symptom is tendon debility, predominantly affecting the hips, pelvic region, thighs, calf muscles and the shoulders. These appear at around 1 to 3 years of age and the child may face difficulty while walking, running, jumping or climbing stairs, thus, walking in a typical waddling style. Some boys may even begin to walk very late, and as the shoulder tendons become weak, they tend to slip when held up in the arms.

The child's calf muscles begin to look very bulky yet are forceless. As the child grows, he may have problems getting up and begins using his hands which is referred to as Grower's sign, indicating that he needs to support the legs whilst getting up causing him to fall more often. The fat and connective tissues commence replacing the muscle tissue in the tongue, inducing speech problems due to an enlarged tongue. By the age of 12, the child completely loses his ability to walk and is confined to a wheelchair.

Treatment
This ailment is not curable. Aimed at improving the quality of life, treatments like physical therapy, braces to improve flexibility and muscle strength, medications like corticosteroids, etc. are employed.

Lifespan
The average lifespan is late teens to mid 30s. In very rare cases, patients in the age group of 40s and 50s have survived with the help of ventilator support, heart medications, proper positioning on the bed, etc.

This is a progressive weakness, that gradually affects the functioning of all voluntary muscles; thus, leading to respiratory and cardiac complications and in turn leading to death.