June is Myasthenia Gravis Month

June has been set aside as Myasthenia Gravis Month.

From the MGFA – Myasthenia Gravis Foundation of America:

Myasthenia Gravis comes from the Greek and Latin words meaning “grave muscular weakness.” The most common form of MG is a chronic autoimmune neuromuscular disorder that is characterized by fluctuating weakness of the voluntary muscle groups. The prevalence of MG in the United States is estimated to be about 20/100,000 population. However, MG is probably under diagnosed and the prevalence may be higher. Myasthenia Gravis occurs in all races, both genders, and at any age. MG is not thought to be directly inherited nor is it contagious. It does occasionally occur in more than one member of the same family.

The voluntary muscles of the entire body are controlled by nerve impulses that arise in the brain. These nerve impulses travel down the nerves to the place where the nerves meet the muscle fibers. Nerve fibers do not actually connect with muscle fibers. There is a space between the nerve ending and muscle fiber; this space is called the neuromuscular junction.

When the nerve impulse originating in the brain arrives at the nerve ending, it releases a chemical called acetylcholine. Acetylcholine travels across the space to the muscle fiber side of the neuromuscular junction where it attaches to many receptor sites. The muscle contracts when enough of the receptor sites have been activated by the acetylcholine. In MG, there is as much as an 80% reduction in the number of these receptor sites. The reduction in the number of receptor sites is caused by an antibody that destroys or blocks the receptor site.

Antibodies are proteins that play an important role in the immune system. They are normally directed at foreign proteins called antigens that attack the body. Such foreign proteins include bacteria and viruses. Antibodies help the body to protect itself from these foreign proteins. For reasons not well understood, the immune system of the person with MG makes antibodies against the receptor sites of the neuromuscular junction. Abnormal antibodies can be measured in the blood of many people with MG. The antibodies destroy the receptor sites more rapidly than the body can replace them. Muscle weakness occurs when acetylcholine cannot activate enough receptor sites at the neuromuscular junction.

Common symptoms can include:

* A drooping eyelid
* Blurred or double vision
* Slurred speech
* Difficulty chewing and swallowing
* Weakness in the arms and legs
* Chronic muscle fatigue
* Difficulty breathing

I was diagnosed with this at the age of 40. I had a thymectomy about 5 months after my dx. Even though I had a CT scan that showed a normal Thymus gland, it was actually enlarged. From the time of my diagnosis I was started on a treatment of Mestinon. I was taking up to 720 mg a day at times when I was in full blown disease. I also take Cellcept which is an immunosuppressant.

Now my dose of Mestinon has dropped a great deal. I usually take 60 mg every 3 hours. If I feel like I can hold out for four hours I try it, but don’t wait for weakness to set in. I have been very blessed that my thymectomy has achieved it’s goal.

I am considered to be in drug induced remission by some doctors. This means that as long as I take my meds on time I am OK. No more ER visits or swallowing or breathing crises. And the drop in my dose of meds is incredible.

My neurologist is the best. He specializes in MG and is the Clinic Director of the two MDA clinics near me. I feel like I owe my life to his expertise. His name is Dr. Allan Weiss. The MGFA and MDA are so important and have been my life lines. Their information and support is outstanding.

Getting a diagnosis and the proper treatment is often a hurdle. Please contact me about any of these issues and please visit the websites of the MGFA and MDA.