Myasthenia Gravis, a Disease Difficult to Manage

Myasthenia Gravis, a Disease Difficult to Manage

Sanjeev Kumar Nath

What Myasthenia Gravis (MG) is : Myasthenia Gravis literally means “grave weakness of muscles”.

It is a rare autoimmune disease involving the neuromuscular junction.

What a myasthenia patient experiences is weakness of muscles, sometimes only of the muscles of the eyes, sometimes of more or less all voluntary muscles of the body. The condition is of various degrees of severity from mild symptoms of fatigue to death caused by what is called myasthenic crisis.

Normally, when we need to move any voluntary muscle of the body, electrical impulses travel through a motor nerve, and the nerve endings release a neurotransmitter (essentially a chemical) called acetylcholine which then binds itself to sites called acetylcholine receptors on the muscle. The binding of acetylcholine to the acetylcholine receptor immediately activates the muscle, and it contracts.

This mechanism of muscle movement occurring in the neuromuscular junction is disrupted in a myasthenic because of an abnormal action of the body’s own immune system. Antibodies produced by the immune system block, destroy or alter the receptors for acetylcholine, preventing the muscle from contracting. In some cases, antibodies to Muscle-Specific Kinase (MuSK) protein impair the transmission at the neuromuscular junction.

In case of some MG patients, the antibodies are produced in the thymus gland, and there may be tumours of the thymus gland. These tumours, called thymomas, are generally harmless but have the potential to become cancerous.

Rarely, a pregnant woman with MG may pass on the antibodies to the foetus. This is called neonatal MG which is usually temporary, and within two or three months of birth the child’s symptoms generally disappear.

Even more rarely, a healthy woman may give birth to a baby with congenital myasthenia.

This is actually not an autoimmune disease, but is caused by defective genes that produce abnormal proteins in the neuromuscular junction, causing symptoms similar to MG.

How rare it is and how dangerous it is :

Medical experts and organisations do not agree on exactly how rare MG is, although everyone agrees that it is a rare condition. Experts say that prevalence rates of MG range from 150 to 200 cases per million, while 3 to 30 persons per million may be diagnosed with MG every year. Earlier, it was considered even rarer simply because some people with MG never knew that they had the disease.

A patient has to go through a series of tests before it can be ascertained that he or she has MG. These tests may involve physical and neurological examination, blood tests, electro-diagnostics, CT scan or MRI, pulmonary function test, etc. Some people actually have the condition, but don’t know because the process of diagnosis is rather complex, and one may suffer from MG for years before it is finally diagnosed.

Often it so happens that a myasthenic patient experiences certain problems with his eyes and sees an ophthalmologist. The ophthalmologist, finding no disease in the eye, might just ignore some of the patient’s complaints and may not suspect that the patient could have MG.

I think that is what happened to me, too. I was diagnosed with MG only some 2 years ago, but I think I have had the condition for years. Now I can see that I have had some of the symptoms of MG without suspecting that I had the disease.

For example, occasionally I have suffered from slurred speech, and my tongue would feel heavy and somewhat sore. Since I am a teacher, I have had to tell the class—on those bad days when my speech was not alright—that I had some trouble with my tongue and the class would have to bear with my imperfect speech. I would speak slowly, repeat certain things, use the blackboard, and somehow manage things.

I also noticed that after sleeping well, my speech improved. Again, sometimes my eyes felt tired while I was driving, and I would just ascribe the condition to the stress of long distance driving, but now I see that the tired, droopy eyes are myasthenic eyes. The glare of the sky would also hurt my eyes and I would have to use a sunglass.

When I saw an ophthalmologist, he performed the routine investigations and said that there was nothing wrong with my eyes. However, I kept experiencing the problem of eye irritation from sunlight and fatigue, especially of the left eye.

Interestingly, it was a general physician  (Dr. Manoj Kumar Hazarika of Dergaon, Assam) who first told me to see a neurologist because he thought I had Myasthenia Gravis.  

Till then I did not even know what myasthenia was. It was after Dr Hazarika advised me to see a neurologist that I went through the required tests, and the neurologist told me that I had MG.

Also, MG can strike a person, stay for years, and then disappear for years, only to resurface again later.

In the west, MG seems to strike women more than men, while in India more MG patients are men. Women usually get it when they are less than 40 years of age, while men are usually diagnosed with MG in their middle age or even old age. However, it is possible for one to get MG at any age. The causes of the disease are uncertain. It doesn’t seem to be inherited, nor is it contagious.

All patients do not have the same experience with MG. Some can have mild symptoms that do not significantly interfere with normal activities, while others may experience much more severe symptoms and may not be able to do their normal work. Difficulty in swallowing, impaired or slurred speech (dysarthria) and weakness in the limbs, fingers, neck, etc., are some other symptoms any one or more of which a patient may experience.

Some patients experience trouble only with their eyes, sometimes with one of the eyes. The eyelids droop (ptosis), and the patient can suffer from double vision (diplopia). In time, however, such cases of ocular myasthenia can aggravate and become generalised myasthenia.

What is called a myasthenic crisis occurs when the patient is unable to breathe, and requires immediate hospitalisation and ventilation support. A myasthenic crisis may be triggered by factors like stress, surgery, infection, and adverse reaction to certain medicines.

Treatment of MG:

There is no cure for MG, but there are several ways in which the disease is treated. As far as I know, there are more treatment options in the western world, and quite a lot of research is also being done to find definitive cures for MG.

In India, the three most common options are (1) to use anticholinesterase medications, (2) to use immunosuppressive drugs, and (3) to surgically remove the thymus gland if thymoma is detected.

Anticholinesterase medicines slow down the breakdown of acetylcholine at the neuromuscular junction, improving neuromuscular transmission and muscle strength to some extent.

Immunosuppressive medicines suppress the production of abnormal antibodies, but taking these medicines involves compromising with the immune system of the body. To minimise the side effects—which can be considerable—the patient has to be in constant touch with the doctor.

Lifestyle changes:

If something extraordinarily good happens to us, we just enjoy the good fortune; we don’t ask why we have been chosen for such good luck. However, if something bad happens, we tend to ask, “Why me?” This is human nature. Being diagnosed with MG can be extremely stressful. Because it is a rare disease, the question “Why me?” can trouble a patient when he is first told that he has MG and what that means.

Occasionally, a myasthenic may even have to put up with unintended jeers and jokes from friends or acquaintances who do not know a thing about what MG is, but make it a point to laugh about it.

I have experienced, for instance, the odd moment when a friend casually made remarks like, “Oh, Myasthenia Gravis! You are in good company! You know that Amitabh Bacchan has Myasthenia Gravis?” or even “Hey! You look quite fine. I’m sure there’s nothing wrong with you…Are you making all this up to avoid work?”

All this can only add to the stress that a myasthenic has to deal with. However, being stressful will only result in aggravation of MG symptoms, so it is important for a patient to accept the fact that he has MG as calmly as possible, and to be particularly careful about certain things.

It is an established fact that a myasthenic feels better after rest or sleep, so it is important for the patient to see that he gets enough rest and sleep.

Taking rest or naps after brief spurts of activity, if possible, is good. Working too hard is certainly bad. Consequently, a myasthenic may have to cut down some of his activities, or at least lessen activities that are not absolutely essential. There is a Sanskrit adage (sarvanashe samutpanne ardham tyjati panditah) which says that faced with total disaster, the wise let go half of what they have.

This is particularly apt for the condition of the MG patient. It is much like throwing some stuff overboard to save yourself if you are in a leaky boat. If you are in a competitive environment at your workplace, for example, and you are diagnosed with MG, you may need to withdraw from the competition; otherwise MG might have the upper hand, and that would be much worse than losing at a competition concerning career.

“Early to bed and early to rise” may be good advice for most people, but a myasthenic can have difficulty in both going to sleep and getting up in the morning.

Many MG patients do not sleep well. Ideally, a myasthenic should not use the alarm at all to wake in the morning. Getting up in the morning and finishing the normal chores is a huge problem for many MG patients, just as they may experience a lot of fatigue in the late afternoon. Consequently, working from home, if possible, is a good option for MG patients.

If you are a person with an exceptionally optimistic outlook (many would call that unrealistic or impractical), you may even find that MG has one or two good things to offer: it teaches you to relax and to engage with things you love doing, as far as possible, rather than working hard chasing impossible dreams. It teaches you to say “no” to engagements that only add to stress and frustration.

It urges you to understand that retirement is alright, and that you do not always have to look for re-employment. How many of your cherished activities have you postponed because of your work schedule? May be MG will enable you to enjoy one or two of them by curtailing your work schedule.

MG as disability:

Although no one seems to have given much thought to this problem in India, in some countries, MG is considered a disability if certain conditions are fulfilled. Severe generalized myasthenia can cause a patient to collapse even in trying to accomplish some routine chores, and a myasthenic crisis requires immediate hospitalization of the patient.

However, in the majority of the cases, MG patients can lead normal or near normal lives provided they take their medication properly and take rest and sleep seriously. Also, it is good to have a house and workplace—if possible—that support MG patients.

The ideal staircases, ramps and wash rooms for the MG patient, for example are the ones that are designed for differently abled persons—with such things as grab bars and railings.

MG and exercise:

Since rest and sleep are good for the myasthenic patient, he may conclude that exercise is bad for him, but that is not so. Exercising the muscles too much will surely lead to fatigue, but not exercising them will also result in further weakening them.

A myasthenic has to use a lot of discretion in choosing what exercises would be good for him and how much to exercise. Incidentally, yogic pranayamas or even normal deep breathing done as exercise, can be of great help. Breathing exercises strengthen the voluntary muscles involved in the process of breathing in and out, and hence can help the myasthenic patient keep myasthenic crisis at bay.

MG support groups :

In the west, there are MG support groups comprising doctors, patients and care-givers. Patients and care-givers can actually benefit from the information that they gather from those groups.

This is particularly important because MG symptoms can vary significantly from patient to patient, and the same person might experience the disease differently at different times.

Since it is a disease that wears many masks, it is difficult to understand what it is, and community discussion can certainly help. Unfortunately, we in India do not seem to have such groups, and many a time the MG patient is bewildered by what he or she has to go through.

Miracle Cure? :

While I was taking my allopathic medicine, I also heard about Tibetan herbal medicine that has the reputation of curing such conditions. I decided to give it a try, and sought out a Tibetan medical clinic in Dharamshala.

After taking the medicines continuously for some 8 months, without discontinuing the allopathic medicine, I observed that my MG symptoms were gradually disappearing.

I went for a check up at CMC, Vellore, where a neurologist confirmed that as of now, I was without MG. Is it the miracle of the Tibetan medicine?

Well, the neurologist says that as per their books, MG is not curable, but it can go away at times. Does it come back? He says yes, it can come back. But I still feel that the Tibetan medicine and my pranayama practice did have a role to play in curing me.

In any case, MG patients cannot come to harm by practising pranayama and also trying Tibetan herbal medicine.

[ Sanjeev Kumar Nath, Department of English, Gauhati Univesity, Email: sanjeevnath21@gmail.com]

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