Muscular Dystrophy Treatment

Muscular Dystrophy Treatment

Muscular dystrophy (MD) is a group of genetic diseases described by progressive dysfunction and/or weakness of skeletal muscles. They are caused by genetic mutations that affect the synthesis and regulation of these proteins leading to disruption in the normal structure and function of the muscle tissue. The ensuing muscle tissue damage causes weakness and sometimes loss of muscle bulk or muscle scarring. There are many different types of muscular dystrophy. They include Becker MD, Duchenne MD, Emery-Dreifuss MD, Facioscapulohumeral MD, Limb-girdle MD, Oculopharyngeal MD, Myotonic MD.

 

Symptoms of MD vary among its different types and the muscles affected. There is a slow progression of muscle weakness. Other symptoms include delayed development of muscle motor skills, difficulty using one or more muscle groups, drooling, eyelid drooping (ptosis), frequent falls, loss of strength in a muscle or group of muscles as an adult, loss in muscle size, problems walking, and in some cases, intellectual disability.

 

There is no known cure for MD, but there are treatments available that improve the quality of life of these patients. The options range from steroids to surgery and more.

Occupational therapy/Physical therapy (OT/PT): is one such treatment option for MD, which is helpful to maintain range of motion and to prevent or delay development of contractures. PT also helps to strengthen other muscles to compensate for weakness in affected muscles. Regular exercise is helpful but is best guided by PT to prevent overzealous exercises that may damage muscles further. Customized wheelchairs, braces, handicap-friendly residential, or work environments may also be considered as needed. These also include modifications at home, dressing aids, wheelchair accessories, and communication aids.

Surgery: If the contractures from MD become severe, surgery, which includes procedures in which the tendon of the contracted muscle may be severed to relieve the tension and then bracing it in a normal resting position while it heals. If there is associated scoliosis, surgery is needed to repair that.  A common surgical treatment for MD is Achilles tendon and iliopsoas tenotomies for ease of fit into braces, tibialis posterior tendon transfers or tenotomies for more rigid deformities of the foot, as well as segmental spinal stabilization for rapidly developing scoliosis. Some relative contraindications for surgery in MD include obesity, rapidly progressive muscle weakness, poor cardiopulmonary status, etc.

Nutrition: While nutrition has no direct effect on MD progression or symptoms, a healthy diet is important in MD patients who are malnutritioned due to MD-related swallowing difficulty or fatigue, with special attention paid to alternative methods of feeding.

Cardiac care: MD is associated with dysrhythmias or cardiomyopathies (such as seen with Duchenne and Becker). Sometimes, these are serious enough to require placement of cardiac pacemakers and other therapies.

Respiratory care: MD can affect the muscles of respiration, such as the diaphragm, which can lead to severe respiratory compromise. These patients may require a ventilator device to continue breathing adequately. Cough-assist devices might also be required to help clear respiratory secretions (pulmonary toilet) and prevent infections.

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