Myasthenia gravis patients face lifelong battle with autoimmune neuromuscular disorder

Voncile Gissendaner-Wilson has been battling myasthenia gravis since 1979. / AMANDA SOWARDS/ADVERTISER

June is Myasthenia Gravis (MG) Month.

The Muscular Dystrophy Association estimates that MG affects two to seven out of every 10,000 people in Western countries.
The expectation is that an MG patient will live a normal lifespan, but that was not always the case. In the 1930s, and even into the 1950s, before there were ICUs, 50 percent of MG patients died, said Dr. Henry Kaminski, an MG specialist.

CONTACTS

866-749-0844 begin_of_the_skype_highlighting              866-749-0844      end_of_the_skype_highlighting, Alabama MG hotline
800-541-5454 begin_of_the_skype_highlighting              800-541-5454      end_of_the_skype_highlighting, Myasthenia Gravis Foundation of America Inc. (MGFA)
MGFA’s website: www.myasthenia.org
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On a warm and windy day in early April, Voncile Gissendaner-Wilson sits down to talk about the autoimmune disorder that has ruled her life for more than 30 years.
Gissendaner-Wilson has myasthenia gravis, which occurs when the immune system attacks the body's own tissues, interrupting the connection between nerve and muscle. Myasthenia gravis -- abbreviated MG -- literally means "grave muscle weakness," and is characterized by fluctuating weakness of the voluntary muscle groups.
Most MG patients report good days and bad days. Most of them take numerous prescription medications to treat the symptoms of the disease, as well as any other underlying conditions they have.
For Gissendaner-Wilson, today is a good day.
Her initial symptoms back in 1979 were classic -- a droopy eyelid, which still happens occasionally, especially when she's tired, and double vision. A few months later, the disease started affecting her limbs. Her legs collapsed one day while she was climbing stairs at work.
Doctors at Emory University diagnosed her with MG, and soon performed a thymectomy -- a surgical removal of the thymus gland. The thymus plays a role in the development of the body's immune system and in the development of MG. Sometimes a tumor on the gland is to blame, but only about 10 percent of the time. Even without a tumor present, a thymectomy can improve a patient's weakness, but that's generally in the long term.
There is no cure for MG.
Drugs that suppress the immune system (corticosteroids) are often prescribed, as is Mestinon, which decreases muscle weakness. Most medications prescribed come with serious side effects that have to be monitored closely.
Gissendaner-Wilson talks about how it took many years to strike the right balance of medications. Her medication list takes up three-quarters of a page.
The goal of drugs and other treatments is remission -- no symptoms of the disease and no medications for a year. Perhaps a third of all MG patients with proper treatment can achieve remission after five years, said Dr. Henry Kaminski, a professor of neurology and chairman of the Department of Neurology and Psychiatry at Saint Louis University. He's treated MG patients for about 20 years and does research into MG, funded by the National Institutes of Health.
Kaminski said doctors have no understanding of why some patients go into remission while others don't.Gissendaner-Wilson fatigues easily -- "I stay exhausted all the time." And she has other medical problems to deal with, like fibromyalgia and depression.
But today, she said she is in remission to an extent, because her disease is well-controlled by medications. She has learned, in 30-plus years, how to listen to her body.
After a couple of hours of talking, her fatigue begins to emerge -- most people with MG are stronger in the morning, after a night's rest, and become progressively more tired throughout the day. She promises to talk again later, about what she can do to help raise awareness of this rare disease.
She helps run a support group for MG patients, and wants to get the word out about that too.
It's been a good day.

A crisis situation

About a month later, about noon on a Thursday, Gissendaner-Wilson calls from a local hospital. She is in ICU, in full respiratory distress. It is difficult to understand her because she is so short of breath.
That remission that she thought she had been experiencing began to fall away after her initial interview, when she said she started "going downhill." The deaths of two people close to her were very stressful, and stress can be a contributing factor to what MG patients and physicians call a "crisis," which is why she's in the hospital now.
A crisis is an extreme episode of weakness, when the patient can't breathe or swallow adequately. While rare, such crises can be very dangerous. Failure to treat symptoms promptly can result in poor air exchange and respiratory insufficiency or arrest.
About a third of all patients with MG will experience a crisis in their lives, Kaminski said. And there may not be a specific trigger.
"One very common trigger is an infection, which itself seems to trigger a more severe immune reaction," Kaminski said.
But there are certain medications that interfere with how muscle and nerves communicate, and exposure to such drugs can sometimes make the disease manifest, he said.
Gissendaner-Wilson has had many such crises over the years of her disease. There were more of them in the first 10 years or so. But the possibility of a crisis is never far away.The first signs of a crisis for her, after so many years, are easy to detect.
"Your legs are like rubber bands. You can't raise your arms to do normal things, like brushing your hair. Your steps are slower. My voice gets real raspy. It's hard to chew. And simple things like smiling are difficult.
"All your muscles in your body are affected by MG."

Advocates

A must for MG patients is having an advocate -- a friend or family member who can speak for the patient when she is unable to do so. This advocate has to be able to communicate with medical personnel and be assertive about what kind of treatments should or should not be given to the patient. For that reason, the advocate can't be passive, or easily intimidated by doctors.
Gissendaner-Wilson has two cousins who act as her advocates. They know her medical history very well and always have a list of her medications with them. They also carry lists of drugs that may increase her weakness or have possible counteractions.
The main issue, she said, is that because the disease is so rare, most medical professionals -- from paramedics to nurses to physicians -- don't know much about it. They may not have ever encountered an MG patient in their careers.
"Sometimes doctors think they know what's best for MG patients, but they don't (always)," she said.
Gissendaner-Wilson relates a story from her crisis in 2007, which she said was the worst she'd ever had. She was trying to communicate with an emergency staff that was not familiar with the disease.
"You feel like you have an elephant sitting on your chest, and you can't breathe," she said. "They were giving me nitroglycerin under my tongue, and I'm like, I'm having a crisis, I need oxygen!"
Kaminski agrees that any patient with a condition that can be life-threatening needs an advocate.
"Certainly the health-care system is complicated enough for even a healthy individual," he said. "When you have someone who is weak and can't sustain activities, or have significant speaking or vision problems, it's very important for them to have somebody by their side."
During Gissendaner-Wilson's last crisis, she had her advocates with her, as well as Kathy King, a friend and fellow MG patient. The two have worked together to build a local MG support group for years."The nurses had never had a patient with MG," King said. "They didn't know what to do."
How a patient needs to be treated in a crisis varies widely from patient to patient. That's one of the frustrating aspects of MG -- it's very individualized, and very complex.
"What might be best for an older man might not be best for a younger woman," Kaminski said. "Each patient is unique."

Treatment

For Gissendaner-Wilson, the crisis treatment that works for her is called IVIg -- intravenous immunoglobulins. It's also known as gamma globulin, and has been used for decades to treat a variety of infectious or inflammatory diseases.
In patients who are lacking the antibodies necessary to fight infection, IVIg replaces those lost antibodies.
It's infused very slowly, over a period of hours -- Gissendaner-Wilson said hers usually take eight to 12 hours. Like her, many patients receive a series of infusions over several days. Improvement varies widely, but can take a week or two.
King's situation is very different. She was diagnosed in 1982, her senior year of high school. She's had many crises over the years, and has had to be put on a respirator twice.
While Gissendaner-Wilson's crises are usually stress-related, for King, they came about typically when she became immune to the medications she took to control the disease.
She never had the IVIg treatment -- for her, plasmapharesis worked best.
Plasmapharesis, also known as plasma exchange, removes antibodies from the blood.
"It cleansed my blood of all that old medicine," King said. "That was a miracle cure for me. (Then) I would just get back on the medication and start over."
King is now in remission, and has been for about eight years now. Neither she nor her physician knows why.

Misunderstood

Because MG patients appear to be outwardly normal -- aside from the droopy eyelid, which is not always present -- most people don't understand how sick an MG patient really is, whether or not she is in the hospital.
"We're misunderstood," Gissendaner-Wilson said. "There are a number of people who have lots of trouble from their superiors because they don't believe we're as sick as we are."She was forced to retire from her state job much earlier than she wanted to.
"You want to be productive," she said. "You don't want to be labeled as disabled or retired. But physically, you can't keep up the pace. And when you're in a stressful environment, it causes you to be more depleted. Your strength is not what it should be."
King had the same problems when she was sick, too. "That was all the time," she said.

Tough to diagnose

Getting a correct diagnosis can be as frustrating as trying to manage the symptoms.
Kaminski confirms that most general practitioners don't recognize MG, because they just don't see it. Even specialists would not see many such patients, he said.
"A lot of neurologists don't see it -- maybe one or two cases every few years," he said. Neurologists are usually the ones to diagnose MG and help the patient manage it. "Remembering to keep the diagnosis in mind is often an issue."
To add to that, some studies suggest that patients go through three physicians in eight months before a correct diagnosis is achieved, Kaminski said. For some patients, a correct diagnosis can take years.
The symptoms can mimic other autoimmune diseases as well, which can further delay diagnosis.

Spreading the word

More than a month after she left the hospital after her last crisis, Gissendaner-Wilson is still struggling to get better. She is housebound, and will probably remain so for a while.
"It's been a tough go this time," she said from her east Montgomery home. "So many side effects from the medications."
Despite her limitations, she is still eager to have her story shared, so that others who may be suffering will be encouraged to reach out and find support.
And she and King both hope to raise awareness among those who may not know much about MG. They point to the fact that other neuromuscular diseases, like lupus and muscular dystrophy, are much more well-known by the general public than MG.
They both hope to spread the word about the local MG support group. They started it several years ago, but it eventually disbanded. They restarted the group just this year.
Just getting together can be beneficial.
"Just us, talking about what we're doing that helps us, what we use when we're traveling, what we do to help rest," King said. "People say, oh, this has helped me so much."
Additional sources: Muscular Dystrophy Association, Myasthenia Gravis Foundation of America, Inc.