Support And Determination

 Make MG Manageable

By Nancy K. Crevier

When a Southbury couple sold their home in 2001 — and everything else they owned in fact, except for some pots and pans, clothes and their cats — to become full-time RVers (recreational vehicle owners), it seemed like a dream come true. Empty nesters, they had taken early retirements, enrolled in a class designed for RVers at the University of Idaho, and said their good-byes to friends and family in Connecticut to embark on the adventure of their lives.

“Joe” and his wife, who prefer to remain anonymous for this article, designed their custom made, $85,000 diesel truck that they picked up in Oregon before heading to Kansas, where their 35-foot-long trailer — 14 tons of truck and home — was being made to their specifications.

Although an eyelid that drooped for no apparent reason periodically had bothered Joe, a brain scan and eye exam found no physiological reason for the odd symptom.

“Go and enjoy life,” his doctor told him, and that was the plan.

But only two months into their new lifestyle, “What was supposed to be a fun time was turning into a not-so-fun time,” Joe said.

Driving down a busy highway in Tennessee, he suddenly found himself experiencing problems with focusing. His eyelid was drooping, obscuring half of the vision in his right eye, and two highways, one on top of the other, appeared in his field of vision. After stopping to rest for a bit, the double vision subsided enough for him to bring them safely to an RV camp for the night.

Continuing their trek to enjoy the New England foliage, though, was a trial. The double vision recurred persistently, as well as more frequently. Taking advantage of the mornings, when he felt better, Joe and his wife finally arrived in Connecticut, where he immediately consulted an ophthalmologist. His ophthalmologist recalled reading in medical school about symptoms Joe exhibited. He set Joe up with a neurologist, who diagnosed him with myasthenia gravis, or MG.

Myasthenia gravis is a neuromuscular transmission disorder, an immunological abnormality at the neuromuscular junction. Muscle end-plate membranes lose their normal folded shape, resulting in a reduction of the concentration of acetylcholine on the post-synaptic membrane. This prohibits normal muscle contraction.

In two-thirds of the approximately 40,000 cases diagnosed in the United States, drooping eyelids (ptosis) and double vision (diplopia) are the initial complaint with which people arrive at the physician’s office. For about 90 percent of those afflicted with MG, the disease will progress to other muscle groups, affecting the person’s ability to chew, swallow, or talk. Respiratory muscle groups can be affected, as can hand, arm, and leg strength.

Affectionately called “the snowflake disease” by the MG afflicted because, like a snowflake, no two cases are alike, symptoms vary in intensity from individual to individual and from day to day.

 “I’m one of the lucky ones,” says Joe. Not only was the MG identified early on, his symptoms have not advanced outside of the ocular form since he was diagnosed in 2001. For Joe, there is a good chance the disease may never progress to other muscle groups.

The diagnosis of MG was only the beginning of a journey unlike any Joe had planned. Treatment is tailored to the individual, but the traditional regimen of Mestonin, a drug that increases the accumulation of acetylcholine at the neuromuscular junction, is not effective for every patient. At increasingly larger doses of the drug, Joe was experiencing no relief from his symptoms; but unpleasant side effects of the Mestonin now interfered with his day-to-day living. Coupled with the ever-present double vision that prevented him from driving at all, severe perspiration and urinary urgency now kept him housebound.

“I felt frustrated, angry, and stressed. I didn’t do much, just hibernated.”

It was clear that life as an RVer was passing him by. Joe and his wife parked the truck and trailer in a secured area at Diamond RV in Hadley, Mass., rented a condo in Connecticut and began pursuing a treatment for the MG.

By February of 2002, Joe had switched to Prednisone, a corticosteroid drug, in hopes of finding relief. Slowly, his vision improved, but at a cost. Diarrhea, weight gain, thin skin, and other side effects took their toll on him. The really scary thing for Joe, though, was the aggravation of his glaucoma.

It was around this time that Joe was introduced to the MG support group at St Vincent’s Hospital in Bridgeport. As Joe sank into a routine of doctors, medicines, and depression, his wife, whom he describes as “extraordinary,” had been funneling her fear into positive research. Through the Internet she found the St Vincent’s support group, which offers hope to MGers and their caregivers.

The group, which meets the third Sunday of each month, is open to MGers, caregivers, and anyone who wants to learn about the disease. Within months of joining the group, Joe found he had a diminished fear of the disease. Information from knowledgeable speakers at the group, the opportunity to exchange ideas and talk with others who understand the frustration of MG, and the courage of others in the program changed Joe’s outlook, and his wife’s, as well. No longer are they plagued by anxiety and depression. They feel in control of their lives again.

Today Joe is taking a combination therapy of Mestinon and an experimental drug, Cell-Cept, which he found out about through the support group. He can drive for several hours, but must be careful not to overdo it.

“I can lead a normal life,” he says, “if I don’t exceed my new life limitations.”

Joe and his wife sold their RV, bought a condo in Connecticut and are building a home in Arizona.

“We may even buy a small RV and travel some,” says Joe. “We lost a lot of money — took a tremendous hit selling the RV and were out of the real estate market. But that’s not important anymore. We live day-to-day.” He chuckles and adds, “We made our plans, but forgot to check with God.”

“Diane” is a 50-year-old woman with MG. Taking a pie from the oven one day in 1980, she dropped it. Everyone has accidents, though, and Diane attributed her clumsiness to weakness in her arms and neck following the birth of her second child two weeks before. But soon she found herself with a peculiar symptom: she could not pucker up and whistle. Swallowing became difficult and she dropped from a double-digit size to a size four. It was not until she visited her eye doctor concerning recurrent double vision that she began to suspect there was more to her array of symptoms than the stresses of life and new motherhood.

Diane was diagnosed with myasthenia gravis when her vision specialist, suspicious of her symptoms, referred her to a neurologist. She is not as lucky as Joe. The MG is throughout her body. She has been hospitalized several times over the years for respiratory problems due to muscle involvement.

Like Joe, she struggled with frustration and fear. The increasing weakness forced her to leave her job in Stamford, where she worked as an administrative assistant. Because her ability to chew and swallow was affected, early on in her diagnosis she was forced to rely on food supplements for sustenance.

Diane has had more success with Mestinon and Prednisone treatment than Joe though, and Cell-Cept is another gun in her arsenal of drugs. She opted for another common procedure for MG patients, as well. A thymectomy, the removal of the thymus gland, was performed. The thymus gland lies beneath the breastbone and processes white blood cells, which govern immunity. Thymus gland abnormalities are associated with MG for unknown reasons. Fortunately, Diane’s thymus gland was cancer-free, and while response to a thymectomy is uncertain, she feels the standard procedure was appropriate to help alleviate her symptoms.

She has also undergone plasma exchange treatment to relieve debilitating fatigue and listlessness.

She knows, after 25 years, that it is important to prioritize her days. Rest is very important for MG patients, and knowing one’s own limitations is crucial. She is able to volunteer three times a week at the food pantry in Bridgeport.

“I can drive now,” says Diane. “I have to plan the day ahead. I know I’m usually [feeling] good in the morning.”

Even though the various treatments helped her remain active as her children grew up, dealing with the stress of doctors, drugs, and frequent hospitalizations was difficult for Diane and her family. Her husband, while supportive, was devastated. She needed to find a balance between the needs of her family and her own illness, and it took two decades to find what she needed.

Two years ago, she discovered the MG support group in Bridgeport. There she found not only information, but also acceptance.

“If I’m feeling down, I can email someone in the group and they can boost me up. They understand better than anyone else. You know they know.”

Like a little family, the members of the support group help each other. Members of the group compare responses to medications, answer questions, and recommend doctors. Speakers are brought in to talk about issues such as insurance, medication, nutrition, and exercise. The caregivers thrive in the support group as much as the MGers.

Attending the MG support group has given Diane strength.

“Stop and smell the roses,” is her advice. “Smell them often.”

The facilitator for the MG support group is a ten-year survivor of MG. “Mary,” who like the others interviewed for this article prefers to remain anonymous, gathers the group for funding meetings, helps plan the calendar, and takes care of the day-to-day duties that keep the group moving.

“It’s an easy group,” she says. “There’s not a lot of pressure.”

Mary believes that she exhibited symptoms of the disease a decade before it was diagnosed. As with many MG patients, she passed off signs of muscle weakness and fatigue on other medical reasons.

“I blamed it on menopause. But by 1993, I couldn’t walk or play golf. I’d be out of breath and fatigued after five holes. I gave up jogging.” She had to leave her job as an RN when she found it too difficult to walk from the parking lot to the building where she worked. She could no longer speak, and she was losing weight due to an inability to comfortably swallow.

Blood tests designed to identify antibodies in the blood from myasthenia gravis tested negative for Mary., as they do for 15 percent of those suffering from MG. It was more than two years before doctors ascertained that she was, indeed, dealing with MG.

Her family, she says, was just relieved to know a diagnosis.

Upon her doctor’s recommendation, she had a thymectomy and has tried several courses of drugs. Steroids gave her bad reactions and stronger, newer drugs left her with pancreaitis. Mestinon is the only drug she now takes, although not even that has given her much measure of relief.

“I changed my lifestyle. I can’t do as much. Some days are okay, some weeks are stable, when I don’t do more than I know I can do.”

Where Mary has found relief is with the MG support group. She discovered the support group through the Myasthenia Gravis Foundation of America, where she had registered with the Connecticut “Nutmeg” Chapter for MG.

Her husband drives her to the meetings, and over the past five years, she has found reassurance in seeing other people with MG. It is there that she has learned tricks to live with the illness.

“Don’t walk when you can drive; don’t stand when you can sit; don’t sit when you can lie down.”

She has other ways of beating back her limitations, too. An avid baker before the disease laid her low, Mary now has learned to spread out the work of baking over two days.

“The first day, I take out the ingredients and the pots and pans. The second day, I put it together, rest, and then finish the recipe.” It is slow work, but she derives great satisfaction out of controlling MG, rather than MG controlling her.

The group’s goal, says Mary, is to share with people [who have MG] that they are not alone. The group is seeing more diagnosed MGers than when she first joined, which she attributes to better diagnostics; or maybe, she muses, more people are becoming involved in support groups.

She says, “Nobody is experiencing the same thing symptomwise, but emotionally they are having the same experiences. In the group we educate ourselves on new information and how to deal with various aspects of the disease.”

Those afflicted with MG realize that the disease exacts a price from family members.

“It’s very hard for families,” Mary goes on. “No matter how much support they give, they don’t have the symptoms, they still aren’t in your shoes.”

Members of the St Vincent’s support group urge those who have been diagnosed with MG, who care for someone with MG, or even those who merely suspect they have MG to join a support group. Myasthenia gravis, says Diane, is not a painful disease, nor is it a death sentence.

“You can and will live.”