NCLEX Muscular Dystrophy

NCLEX Muscular Dystrophy

Muscular dystrophy (MD) is a group of muscle diseases that weaken the musculoskeletal system and hamper locomotion (walking or moving ).  Muscular dystrophies are characterized by progressive skeletal muscle weakness, defects in muscle proteins, and the death of muscle cells and tissue.

Signs and Symptoms

The main sign of muscular dystrophy is progressive muscle weakness. Specific signs and symptoms begin at different ages and in different muscle groups, depending on the type of muscular dystrophy.

Duchenne muscular dystrophy

About half of people with muscular dystrophy have this variety. Although girls can be carriers and mildly affected, the disease typically affects boys.

Signs and symptoms typically appear between the ages of 2 and 3, and may include:

• Frequent falls
• Difficulty getting up from a lying or sitting position
• Trouble running and jumping
• Waddling gait
• Walking on the toes
• Large calf muscles
• Muscle pain and stiffness
• Learning disabilities

Causes

These conditions are generally inherited, and the different muscular dystrophies follow various inheritance patterns. Muscular dystrophy can be inherited by individuals as an X-linked disorder, a recessive or dominant disorder. Furthermore, it can be a spontaneous mutation which means errors in the replication of DNA and spontaneous lesions. Spontaneous lesions are due to natural damage to DNA, where the most common are depurination and deamination

Risk Factors

Muscular dystrophy occurs in both sexes and in all ages and races. However, the most common variety, Duchenne, usually occurs in young boys. People with a family history of muscular dystrophy are at higher risk of developing the disease or passing it on to their children.

Complications

The complications of progressive muscle weakness include:

• Inability to walk. Some people with muscular dystrophy eventually need to use a wheelchair.
• Shortening of muscles or tendons around joints (contractures). Contractures can further limit mobility.
• Breathing problems. Progressive weakness can affect the muscles associated with breathing. People with muscular dystrophy may eventually need to use a breathing assistance device (ventilator), initially at night but possibly also in the day.
• Curved spine (scoliosis). Weakened muscles may be unable to hold the spine straight.
• Heart problems. Muscular dystrophy can reduce the efficiency of the heart muscle.
• Swallowing problems. If the muscles involved with swallowing are affected, nutritional problems and aspiration pneumonia may develop. Feeding tubes may be an option.

Treatments

Currently, there is no cure for muscular dystrophy. Medications and various therapies help slow the progression of the disease and keep the patient mobile for the longest possible time.

Drugs

The two most commonly prescribed drugs for muscular dystrophy are:

• Corticosteroids: although this type of medication can help increase muscle strength and slow progression, their long-term use can weaken bone and increase weight gain
• Heart medications: if the muscular dystrophy impacts the heart, beta blockers and angiotensin-converting enzyme (ACE) inhibitors may be useful.

Physical therapy

• General exercises: a range of motion and stretching exercises can help combat the inevitable inward movement of the limbs as muscles and tendons shorten. Limbs tend to become fixed in position and these types of activities can help keep them mobile for longer. Standard low-impact aerobic exercises such as walking and swimming can also help slow the disease’s progression
• Breathing assistance: as the muscles charged with breathing become weaker, it may be necessary to use devices to help improve oxygen delivery through the night. In the most severe cases, a patient may need to use a ventilator to breathe on their behalf
• Mobility aids: canes, wheelchairs and walkers
• Braces: these keep muscles and tendons stretched and help slow their shortening. They also give added support to the user when moving.