Of Nursing and Health.

The onset of DMD is usually early childhood and is characterized by difficulties with running (Emery 2002). The run will progress into a “waddling” run as the disease progresses (Porter, Hall, & Williams). This “waddling” will eventually be present with walk also (Porter et al., 2002). The child will have difficulty going up steps (Emery 2002). “[This is characterized by having] to first put one foot, then the other foot, on the same step before moving on to the next one” (Porter et al., 2002). Soon he'll have to pull himself up using the handrail (Porter et al., 2002). The progressive weakness of the muscles results in Gower’s maneuver (Emery 2002). Gower’s maneuver is characterized by “climbing up his/her thighs, pushing down on them, to extend the hips and trunk” (Emery 2002). Most patients will have large calves. This enlargement is due to fatty infiltration (Wong 2001).

“Over the next few months, he will walk more and more on his tiptoes, and develop…lordosis. He'll start to fall more often and be able to rise again only by pulling on objects or by placing his hands on the floor while standing and then lifting his upper body by "climbing" up on his legs with his hands. He'll soon have to get out of a chair in the same way. Inevitably, the weakness progresses until the child can no longer walk and requires the use of a wheelchair. The age at which this happens varies, but most children are between 8 and 10 years old and virtually all use a wheelchair by age 12” (Porter et al., 2002).

Although weakness of the arms is present early, it does not advance as rapidly as in the legs, and causes no real problems until after the boy begins using a wheelchair. The child experiences progressive loss of strength in the shoulders and upper arms until it becomes impossible to lift objects of any weight, and eventually impossible to lift his hands to his mouth. Strength in the fingers and hands is retained, but it, too, eventually decreases and fine movements become limited and clumsy.

As the amount of time a child with Duchenne dystrophy uses a wheelchair increases, he develops more curvature of the spine, but now to the side -- "scoliosis" -- as well as to the back. Eventually, his body curves until his ribs may be resting on his hip bones. The affected youngster may also develop tightening of the joints or "contractures" at the hips, knees, ankles, shoulders and elbows, to the point where the legs are bent up against the stomach with the feet pointing down and the arms locked into the sides with elbows bent. With additional weakness of the chest muscles, the youngster will be unable to breathe deeply or cough properly, and even minor colds and chest infections have a high chance of leading to pneumonia. Severe respiratory problems mark the disease's final stages, usually claiming the lives of those affected during their twenties. The heart muscle is affected at times, and some children may die of heart failure.

Some degree of mental impairment is usual, about 20 percent of boys have an IQ of less than 70. Weakness is mainly proximal and progressive. Ultimately, a wheelchair becomes necessary, in most cases by age 12. Pneumonia compounded by cardiac involvement is the most frequent cause of death, which happens in the late teens or early 20’s” (Emery 2002).