What is Muscular Dystrophy?
Muscular dystrophy is a group of disorders that affect voluntary muscles. People who experience progressive weakening of the skeletal muscles also the loss of muscle mass who are suffering from muscular dystrophy. The disease is caused due to the intrusion of the abnormal genes in the formation of proteins which is needed for muscle formation.
Muscular dystrophy can be seen in infancy up to the middle age or even later and its form of severity is determined in part by the age at which it occurs. Some types of dystrophy affect males only. Some people with muscular dystrophy enjoy a normal life span and also with mild symptoms that progress very slowly whereas the other experience severe muscle weakness and wasting who are dying in their late teens to early ’20s.
Through advanced treatment, children with muscular dystrophy are living longer than ever before.
There are different types of muscular dystrophy which include the given points:
Duchenne muscular dystrophy
It is the most common form of illness where the symptoms normally start before the child’s third birthday. They are wheelchair-bound by 12 years and die of respiratory failure by their early to mid-twenties.
Becker muscular dystrophy
Its symptoms are similar to the Duchenne but with a later onset and due to alow progression death occurs in the mid-forties.
The myotonic form is also one of the most common adult-onset forms that are usually characterized by an inability to relax a muscle once it has contracted. The muscle of the face and neck are often affected first in which the symptoms include cataracts, sleepiness.
This type can be obvious from birth or before the age of 2 years as it affects both genders. Some forms progress slowly whereas the others can move swiftly which causes the impairment.
Onset can be at any age but it is commonly seen in the teenage years. The muscular weakness begins in the face and also in the shoulders. People with this may sleep with their eyes slightly open and have trouble fully closing their eyelids. When an individual with facioscapulohumeral raises arms then their shoulder blades protrude like wings.
This begins in childhood years or also in teenage years and first affects the shoulder and hip muscles. Individuals with limb-girdle muscular dystrophy have trouble raising the front part of the foot which makes a common problem.
Oculopharyngeal muscular dystrophy
Onset is between the ages of 40 and 70 years whereas the eyelids, throat, face are firstly affected that is followed by the pelvis as well as the shoulder.
Muscular dystrophy is caused by defects in genes. most of the muscular dystrophies are forms of the inherited disease which is known as the genetic disease that mothers can transmit to their sons even though the mothers themselves are unaffected by the disease.
Each version of muscular dystrophy is due to a set of mutations but all prevent the body from producing dystrophin whereas dystrophin is a protein essential for repairing muscles. Duchenne muscular dystrophy is caused by the mutations in the gene that encodes the cytoskeletal protein dystrophin.
In Duchenne muscular dystrophy, dystrophy is totally absent whereas the less dystrophin is produced the worse the symptoms and etiology of the disease. There is a reduction in the amount of size of the dystrophin protein in becker muscular.
The symptoms of muscular dystrophy generally manifest themselves in children through certain forms of the disease which do not surface till adulthood. The general symptoms include poor balance, inability to run, waddling gait, trouble in getting up from lying positions, and inability to learn from others.
Currently, there is no cure for muscular dystrophy while medication and various therapies help to slow the progression of the disease. It keeps the patient mobile for the longest time.
The two most commonly prescribed drug for muscular dystrophy is corticosteroid and heart medications. A corticosteroid helps to increase muscle strength whereas heart medications inhibit the beta-blockers and angiotensin-converting enzyme.
- General exercise– A range of motion helps to combat the inevitable inward movement of the limbs and the tendons shorten. Standard low-impact aerobic exercise such as walking and swimming helps to slow the progression of muscular dystrophy.
- Breathing exercise– As the muscle used for breathing becomes weaker then it may be necessary to use devices to help oxygen delivery through the nighttime.
- Mobility aids– Walkers and wheelchairs can help the person to stay mobile.
- Braces– These keep muscles and tendons stretched and also help to slow their shortening which also gives added support to the user when moving.