June named myasthenia gravis awareness month

Educational efforts targeting emergency workers

It is important that emergency workers, emergency department (ED) personnel, and school nurses understand the signs and symptoms of myasthenia gravis (MG) and what constitutes a medical crisis. Family and friends of people who have this disorder of neuromuscular transmission, which produces fatigue and muscle weakness, need to understand these signs and symptoms as well.

"MG is not fatal unless people go into a crisis and get the wrong treatment, so emergency workers, emergency room personnel and even family members need to be aware of what the symptoms are when someone goes into crisis so that they can respond accordingly," says Debora K. Boelz, MS, chief executive officer of the Myasthenia Gravis Foundation of America (MGFA) in Minneapolis.

When a person suffering from MG goes into medical crisis, the first response of emergency medical personnel is to administer oxygen. Yet the person could need to take medications as well. The oxygen doesn’t help if the person can’t pull air into his or her lungs, says Marian Forschler, RN, BSN, president of the Pacific Northwest Chapter MGFA in Renton, WA.

A medical crisis can be caused by too much medication as well and the symptoms are almost identical, says Forschler. Therefore, in the ED, the attending physician must determine if the patient has too much or too little of the medications in his or her system. "We walk a tightrope between not enough medication and too much medication," says Forschler, who has had MG for 45 years.

There are other incidents when the symptoms of MG can cause difficulties for those who suffer with the disease. For example, in school settings, children will have a difficult time sitting in their seats and holding their heads up or their eyes will begin to droop, so teachers interpret that as laziness or assume the child is falling asleep in class. Children often are expected to play sports such as soccer, yet they feel they can’t because of weak muscles. The symptoms are often misinterpreted and children are disciplined for their lack of attention or participation in sports, says Boelz.

Because MG affects all the voluntary muscles in the body when someone who suffers from the disease takes the wrong medication dosage, they may begin to walk from side to side and their speech becomes slurred, and they start to drool because they are having difficulty swallowing. Often, they are thought to be intoxicated and are taken to a police station to sober up, says Boelz.

These are some of the reasons the MGFA has named June Myasthenia Gravis Awareness Month. An increased understanding of this autoimmune, neuromuscular disease would benefit those who exhibit symptoms.

It’s important for family members and close friends to understand what people with MG can and cannot do, says Boelz. "We have a difficult time working with spouses because it is a whole loss of lifestyle when their loved one gets diagnosed with MG," she says. People with MG need more rest and therefore don’t have the energy to do as much as they used to. It’s also more difficult for them to function normally in the morning until their medications have gotten into their system.

Often they have difficulty chewing and must eat soft foods. "Corn on the cob and steak are things of the past," says Boelz. Also, they have difficulty getting out of a chair because their arms and legs are like dead weight.

MG sufferers should take along a companion when doing errands, such as grocery shopping, so that there is someone to intercede should the person with MG go into crisis and not be able to speak. MG sufferers need to wear a Medic Alert bracelet or carry a card in their wallet as well, says Boelz. Depending on the setting, it is sometimes wise for them to use a wheelchair or at least walk with a cane.

The onset of MG is most common for women in their twenties and thirties and for men who are in their seventies or eighties. However, this disease strikes people of all ages.

When babies have MG, they don’t have the sucking instinct, so it is difficult for them to eat, says Boelz. Because the disease impacts muscles they don’t hold their head up, roll over, or start to crawl. Therefore, it is important that children, especially infants, be properly diagnosed early and prescribed medications.

Parents must help the child do special exercises to develop muscle tone. "When an adult is diagnosed with MG, they have good muscle tone; but with a baby, they never had the opportunity to develop the muscle tone, so it is a different approach," says Boelz.

Adults must keep their muscle tone by regular exercise as well. Walking regimens are good but best done in a shopping mall with a friend or on a treadmill at home rather than outdoors where it is more difficult to get help in case of an emergency, says Boelz.

There are two forms of myasthenia gravis. About 15% have the ocular form with signs and symptoms consisting of a drooping eyelid and double vision. The generalized form affects all the major voluntary muscles in the body, so there is a lack of facial expression.

The disease is treated with prescription drugs and in some cases the thymus gland is removed. To remove the gland, patients must undergo major surgery. About 50% have good results and lead a more normal life with minimum prescription drugs. About 30% do well, and for the remaining 20% there is no difference following surgery. The prevalence of myasthenia gravis in the United States is estimated at 14 per 100,000 people.

Sources

For more information about Myasthenia Gravis Awareness Month scheduled for June, or for other information about the disease, contact:

• Debora K. Boelz, MS, Chief Executive Office, Myasthenia Gravis Foundation of America, 5841 Cedar Lake Road, Suite 204, Minneapolis, MN 55416. Telephone: (952) 543-5350. E-mail: deborakboelz@msn.com.

• Marian Forschler, RN, BSN, President, Pacific Northwest Chapter MGFA, 18115-116th Ave. S.E., Renton, WA 98058-6562. Telephone: (425) 235-1435. E-mail: NWMG@cs.com.