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Monday, July 21, 2014

In the Spotlight: Myasthenia Gravis

1:50 PM Posted by Stephanie Horgan , ,
I'm proud to say that this is the 160th blog post for Oak Park Behavioral Medicine. This post is also the next part of our "Rare Disease of the Month" blogathon for 2014. This month, we highlight Myasthenia Gravis. This disease is not easy to pronounce (the pronunciation is "my-us-THEE-nee-uh GRAY-vis"). The disease is characterized by weakness and rapid fatigue of any of the muscles under your voluntary control and is caused by a breakdown in the communication between nerves and muscles.

Myasthemia Gravis (MG) has a Latin and Greek origin and literally means "grave muscle weakness." MG is an autoimmune neuromuscular disease that can affect any voluntary muscle. It most frequently affects muscles that control eye and eyelid movement, facial expression, and swallowing. The onset of the disorder may be sudden and symptoms often are not immediately recognized as myasthenia gravis. Without getting too technical, the immune system of a person with MG makes abnormal antibodies against the receptor sites of the neuromuscular junction. These sites require a chemical called acetylcholine to activate them, but with MG patients, this does not happen and thus muscle weakness occurs.

How Common is It?
It is estimated that about 20 out of every 100,000 people in the United States have MG. It most commonly affects women under 40 and men over 60 years old, but it can occur at any age.  MG is not directly inherited nor is it contagious.

What about Diagnosis and Treatment?
Just like other chronic illnesses, the symptoms of MG can vary greatly. Common symptoms include drooping of one or both eyelids, blurred or double vision, an unstable or waddling gait, a change in facial expression, difficulty swallowing, shortness of breath, impaired speech, and weakness is the arms, hands, fingers, legs, and neck.

Muscle weakness is a characteristic of many other disorders. Thus, diagnois includes a review of medical history and a full neurological exam. Blood tests can identify if there are abnormal levels of two antibodies (acetylcholine and anti-MuSK) which MG patients can sometimes experience. EMG (electromyography) studies also can provide support for the diagnosis.

The good news is that, in general, MG can be controlled with medicine. Medications include anticholinesterase agents, which help improve neuromuscular transmission and increase muscle strength. Corticosteroids and immunosuppressive drugs are also used to improve muscle strength by suppressing the production of abnormal antibodies.

Surgery is another option for some patients with MG. A thymectomy (surgical removal of the thymus gland) is needed when there is a tumor of the thymus gland. This occurs in 10-15% of patients with MG. This surgery frequently lessens the severity of the MG weakness after some months. In some people, the weakness may completely disappear and be in remission. The degree to which the thymectomy helps varies with each patient.

Finally, plasma exchange may be useful in the treatment of MG also. This procedure removes the abnormal antibodies from the plasma of the blood. The improvement in muscle strength may be striking, but is usually short-lived, since production of the abnormal antibodies continues. When this treatment is used, it may require repeated exchanges.

What About the Social and Emotional Impact?

Many emotions may arise when dealing with an illness like MG. Feelings of anger and frustration are typical, since as the body can't do what the patient wants it to do. Anxiety about when the next crisis or flare will arise is common as well. Self-esteem can be affected due to the visibility of symptoms of MG (such as drooping eye lids, weight gain, weak facial muscles, and slurred speech). These symptoms can cause patients to avoid social situations and sometimes cause significant life style changes as activities must be limited. A person may feel that his or her identity is changing because they may not be able to do the things that once gave them purpose. It is important to acknowledge the emotions of chronic illness, but also identify thoughts the patient is having about their body that may be unhelpful. Therapy can be a helpful place to do this, as well as meeting with another patient who has MG and who has been on a similar journey.

The Mayo Clinic suggests doing several things to help improve your quality of life if you have MG. Simple things like adjusting your eating routine to when you have good muscle strength or eating smaller meals throughout the day may help. It can be helpful to wear an eye patch if you have double vision, although this can mpact on the person with MG socially and emotionally.

The illness does not only affects the patient. Family members at home may need to help out when MG symptoms are flaring up. A support group can be a place where the patient can process the frustrations of living with MG and the family can gain support from meeting others who are supporting MG patients. It is also important to find ways to relax; stress is thought to worsen the condition. Find ways to slow down and build in time to rest. Continue to do the activities that bring you joy, within your limitations, and remember to listen to your body. Consider finding a walk in your area to raise awareness and funds for those affected by MG.

Helpful Resources:
Myasthenia Gravis Foundation of America, Inc.
National Institute of Neurological Disorders and Stroke
American Autoimmune Related Diseases Association