Myasthenia Gravis

Originally my main association with this condition was that I remembered Jackie Kennedy's second husband, Aristotle Onassis, had it. I had a vague recollection of reading that he had to have his eyes taped open so that he could see, so I knew it had something to do with your eyelids drooping, but I had never really studied it.

About three years ago I started to notice that in pictures my right eye seemed smaller than my left eye. And I started to realize that my right eyelid had started to droop. Now I always had rather heavy eyelids - "bedroom eyes" as my mother used to tell me they were called, when she was trying to make me feel better about them. But this was something new. I looked at old pictures, trying to decide if it had always been that way. I didn't see evidence of it going back to my younger days.

I knew it was an actual problem when a friend of mine at work pointed it out, asking if I realized my eyelid was drooping. This was serious; not only was it drooping, and I didn't know why, but it was also - horrors - a cosmetic issue! People could notice it! It wasn't just me being paranoid!

I started Googling myasthenia gravis. Naturally I found it could be a serious illness; it wasn't necessarily just droopy eyelids. It could lead to generalized weakness and could even be fatal. Ah, a perfect disease for a hypochondriac. Hypochondriacs need to not only fear that they have a disease, but that that disease is serious, and preferably life-threatening. (Actually, nowadays treatments are very effective, so MG is not all that dangerous anymore).

Some people would have gone to the eye doctor first. Not me. I called up the neurologist that we'd taken my father to when he started to develop dementia. I made an appointment and went to see him. He agreed my right eyelid was kind of droopy and scheduled me for a special test that they do in the hospital (on an outpatient basis). Basically MG is caused by a lack of a certain chemical, and the way the test works, they give you an infusion of something else that increases the level of this chemical and see if your eyelid springs open suddenly. I sat there waiting as they injected the IV. I felt a slight twitch of my eyelid but no change was seen. Apparently I didn't have Myasthenia Gravis.

So then I went to the eye doctor, who told me that many times as people age (I hate that, why is it everything going wrong with me is from "aging"?) the muscles holding up the eyelids often loosen up and need to be tightened up surgically. Since it wasn't bothering me that much I didn't pursue it, but now three years later I'm noticing that it's harder to see out of the right eye and I think I may address the problem this year.

In the meantime, I will share with you all the knowledge I have learned about myasthenia gravis in the course of my experience with the possibility of having it.

What is Myasthenia Gravis?

Literally, taken from both Greek and Latin, it means "grave muscle weakness" - a pretty descriptive title.

Myasthenia gravis is a "chronic autoimmune neuromuscular disease characterized by varying degrees of weakness of the skeletal (voluntary) muscles of the body." (taken from this website:
http://www.ninds.nih.gov/disorders/myasthenia_gravis/detail_myasthenia_gravis.htm

What this means in plain English is, the body actually starts attacking its own cells and causes muscle weakness. This weakness increases during activity and improves after rest. The muscles that control eye and eyelid movement, facial expression, chewing, talking, and swallowing are often affected. The muscles that control breathing and neck and movements of the arms and legs can also be involved.

The way the muscles usually work is, nerve impulses stimulate the muscles by releasing a chemical called acetylcholine, a "neurotransmitter." Acetylcholine then binds to receptors that then cause the muscle to contract. In myasthenia gravis, the body's own immune system starts making antibodies that attack the receptor cells and disrupt the signal from the nerves to the muscles. As a result, the muscles aren't able to contract as they usually would.

It is believed that the thymus gland plays a role in myasthenia gravis. This gland, which is found in the upper chest area, is part of the body's immune system. The gland is larger when you are a baby; it shrinks when you become an adult. But, in some adults with myasthenia gravis, the thymus is abnormally large and has an overgrowth of certain cells. Some people also have tumors of the thymus, called "thymomas." Usually, thymus gland tumors are noncancerous. Scientists are still unsure of the exact role that the thymus plays in the condition.

Some factors can make myasthenia gravis worse, including fatigue, illness, stress, extreme heat, and some medications, such as beta blockers, calcium channel blockers, quinine and some antibiotics.

What are the symptoms?

In many cases, the first sign of the disease is, as I correctly remembered, weakness of the eyelids, making them droop (called "ptosis"). In some cases, the condition is limited to the eyelids alone, which is called "ocular myasthenia gravis." However, in other cases, MG causes a general effect over the body, and other symptoms can include:

-Facial muscle weakness causing change in facial expression;
-Double vision;
-Difficulty in breathing, talking, chewing or swallowing;
-Muscle weakness in your arms or legs;
-Fatigue brought on by repetitive motions (the more times you use a muscle the weaker it gets).

Not all days will be the same; some days a myasthenia gravis patient will feel better, other days he or she will feel worse. Temporary remissions may occur.

How is it diagnosed?

There are a number of ways to find out whether or not you have myasthenia gravis. The first would be a simple neurological examination, where the doctor tests your reflexes, muscle strength, ability to walk, your balance and coordination.

Another test is a blood test that looks for special antibodies that are the culprits attacking the acetylcholine receptors. However, not all cases can be diagnosed this way since these antibodies don't alway show up in the ocular type of myasthenia gravis.

Another test is called the "edrophonium test." Although I had also had the neurological exam and the blood test, this was the test that my doctor decided ruled out MG in my case. As I mentioned above, the doctor gives an intravenous dose of a chemical called edrophonium chloride or Tensilon(r), which temporarily increases the levels of acetylcholine at the junction between the nerves and the muscles. If the person has myasthenia gravis affecting the eye muscles, the Tensilon(r) will briefly relieve weakness and cause the eyelids to raise.

Doctors also do a study which tests for specific muscle "fatigue" by repetitive nerve stimulation.

Another common test for myasthenia gravis is single fiber electromyography (called EMG), in which single muscle fibers are stimulated by electrical impulses. Muscle fibers in myasthenia gravis do not respond as well to repeated electrical stimulation compared to muscles of normal individuals.

The doctor may also do a CT scan to see whether the patient has an abnormal thymus gland or a tumor of the thymus.

Pulmonary function testing may also be used to test the breathing strength.

How is it treated?

The good news is, there are a number of effective treatments for myasthenia gravis.

Medications include the cholinesterase inhibitors, such as pyridostigmine (Mestinon) and neostigmine (Prostigmin. These drugs help communication between the nerves and muscles, improving muscle contraction and strength.

Drugs such as prednisone, cyclosporine, and azathioprine, which suppress the immune system, can also help. These medications improve muscle strength by suppressing the production of abnormal antibodies. They may cause major side effects such as bone thinning, weight gain, diabetes, increased risk of some infections and a redistribution of body fat. Anyone who has ever temporarily taken prednisone for a bad case of poison ivy or some more serious ailment knows the "moon face" look that they get from these drugs. Longer term, fat can be deposited in other places as well.

In some cases, the doctor may surgically remove the thymus gland. Removal of the gland reduces symptoms in many cases and can be curative. This treatment is usually recommended for younger patients under 60.

Some patients who are severely affected by MG are given a treatment called plasmapheresis, where the blood is filtered and the antibodies affecting the acetylcholine receptors are removed. This is not a permanent cure since antibodies re-form, but it can help temporarily.

Intravenous immune globulin, which temporarily provides the body with normal antibodies from donated blood, is sometimes given during periods of weakness.

Sometimes a life-threatening situation, called a myasthenic crisis, occurs when the muscles that control breathing weaken severely, requiring a respirator for assisted breathing. These crises may be triggered by infection, fever, or a reaction to medication.

Generally the prognosis is good for myasthenia gravis sufferers. With all of the treatments available, they can expect to live a normal lifespan with good quality of life.

Research continues and it is likely that more new treatments will be developed as understanding of this condition continues to increase.

Will you get it?

Probably not; lifetime risk is 500 in a million, or about 0.1%, if my math serves me. Annually, between 2.5 and 20 people per million get this disease.

Myasthenia gravis more commonly affects young adult women (under 40) and older men (over 60), but it can occur at any age. Any ethnic group can get it.

In neonatal myasthenia, the fetus may acquire antibodies from a mother affected with myasthenia gravis. Generally, cases of neonatal myasthenia gravis are temporary, and the child's symptoms usually disappear within 2-3 months after birth. Other children develop myasthenia gravis indistinguishable from adults.

Myasthenia gravis is not directly inherited and is not contagious, so you don't have to worry about catching it from anybody. However, the disease may occasionally occur in more than one member of the same family.

This post is based on information from the website posted above, plus the following links:
http://www.neuro.wustl.edu/neuromuscular/mtime/mghxpe.html
http://www.mayoclinic.com/health/myasthenia-gravis/DS00375/DSECTION=3

High Blood Pressure

High blood pressure is often called "the silent killer" because most people have no symptoms of this condition. But untreated, it can cause a myriad of conditions that can lead to an earlier death than would have otherwise been scheduled! High blood pressure is implicated in all kinds of heart disease, stroke, and kidney failure.

Both my husband and I have high blood pressure. In his case, he found out he had it over ten years ago when he was only about 42. He hadn't been to the doctor for a checkup since he went off to college, and after incessant nagging on my part, he finally went for a checkup. (My theory as to why married men live longer than single men is that they have wives nagging them to go to the doctor).

We sat in the waiting room, him sulking and giving me smoldering looks because I had dragged him there. When the nurse checked his blood pressure, it was sky-high - about 180 over 110. (More on what is normal vs. high to follow). Even after he'd sat there for awhile and gotten over his annoyance at being there, it was still well above normal.

Of course he didn't want to go on medication - who does? So the doctor gave him three months to lose weight and exercise, and see if it would go down to normal.

So, he began carrying a salad with plain tunafish and a yogurt to work for lunch every day, walking to and from the train, and cutting back on fattening foods in the evening. When he went back to the doctor, he had lost 25 pounds and his blood pressure was down to just under the high-normal range. He was able to keep it that way for about three years.

Unfortunately, over time it crept back up again, and finally he had to go on medication. He now takes three different types of blood pressure medicine. In his case, it was probably inevitable - his father has high blood pressure, and so did his father's mother.

In my case, it seemed to be directly caused by stress. It doesn't really run in my family (my father didn't have it until he was about 80 and my mother still has no high blood pressure despite smoking a pack of cigarettes a day her entire adult life - she is now 88). And I never had particularly high blood pressure either, even as I gained weight in mid-life. But in 2005 I had a bad year...my father passed away in a nursing home at age 92, then I was diagnosed with oral cancer (a posting to follow on that) and then in July of the same year, our dog died unexpectedly.

That was the last straw - I happened to go to the doctor not long after that and my blood pressure was up. It kept being up, and my doctor put me on a simple diuretic. That didn't seem to do the trick so she added another drug, which was eventually replaced with a different drug due to side effects. Now my blood pressure is normal--as long as I am medicated. However, I know if I lost weight and exercised, I probably could bring it down naturally. Some day...

So, after this rather lengthy story, here is the scoop on high blood pressure.

What is high blood pressure?

High blood pressure is measured by two numbers: the systolic and diastolic pressures. The systolic is the pressure in your arteries that exists when the heart pumps; the diastolic is the pressure when your heart is at rest. At one time they used to think only the diastolic number was significant; experts now know both numbers are important.

High blood pressure (also called hypertension) is officially defined as blood pressure that is at or above 140 (systolic) "over" 90 (diastolic), commonly shown as 140/90.

High normal (or "pre-hypertension) is 120-139 over 80-89, and anything under 120/80 is normal. Lower is even better.

Similar to other conditions, the experts keep moving the goal posts. A generation ago, a blood pressure of 140/90 was seen as only "borderline" and not a big concern. Now it is considered time for medication.

There are two major types of high blood pressure: primary (or "essential") hypertension and secondary. Secondary hypertension is called that because it is not the primary problem you have; it is caused by something else. In this case, it is usually caused by the narrowing of an artery, a defect in the aorta (the main artery bringing fresh blood out of the heart), or certain kidney conditions. If the original problem can be fixed, then the high blood pressure goes away.

Most high blood pressure is the "essential" kind - there is no good reason for it. In fact, 85-90% of high blood pressure is this type.

The way blood pressure changes is due to the widening or narrowing of your arteries; nerve impulses play a part in this process, causing the arteries to dilate or contract. When the vessels are open enough, the blood gets through easily. If not, then the blood pressure increases as the heart works harder to get the blood through so it can carry oxygen to your cells.

When this happens, your heart becomes strained and blood vessels become damaged. Changes in the vessels that supply blood to your kidneys and brain then may cause these organs to be affected. You can have a heart attack, a stroke, develop kidney failure (since the kidneys are also very dependent on the blood vessels to function) or even go blind. This is why it is so important to control high blood pressure as soon as it is diagnosed. There are various factors that can cause the arteries to narrow or become less elastic, all of which can lead to hypertension.

You may wonder whether there is such a thing as blood pressure that is TOO low. In general, the lower the better. However, there are a few underlying conditions that may cause unusually low blood pressure, such as nerve disorders, endocrine diseases (such as thyroid problems), or even internal bleeding. Therefore if you discover your blood pressure has dropped unexpectedly, further investigation is warranted.

How is it diagnosed?

It is often discovered at a routine doctor visit or at screenings that are held at work places or other locations. You can even check it yourself on one of those machines in the drug store. If you do this a few times and your blood pressure is above normal consistently, then a trip to the doctor is in order.

The way both the machines and the doctor (or nurse or other technician) measure blood pressure is by attaching a cuff around your upper arm. When the cuff is inflated, it compresses a large artery in your arm, momentarily stopping the blood flow. As the air in the cuff is released, the doctor listens with a stethoscope. When the blood starts to pulse through the artery, it makes a sound. Sounds continue to be heard until pressure in the artery exceeds the pressure in the cuff.

The doctor listens and watches the gauge, then records the pressure when the heart beats (the systolic pressure, when the first sound is heard). He or she then records the pressure when the last sound is heard -the diastolic pressure. The measurements are in millimeters of mercury, which is abbreviated mm Hg. Of course, there are now new digital machines, even machines you can have at home, that still use the same measuring system but record the readings digitally rather than by measuring a column of mercury.

The 120/80 criterion is based on the "resting" blood pressure. So a person should sit quietly for a few minutes before having the pressure checked. It is also important to check more than once. The key thing to find out is whether the blood pressure is consistently high.

Often a person has high blood pressure from being at the doctor's office in the first place - this is called "white coat hypertension." If you think this is what happens to you, you can buy a blood pressure monitor at your local drug store and test your blood pressure at home. The best thing to do is test it periodically and take an average over several readings. If you see it is still elevated, then tell your doctor.

Are you sure there are no symptoms?

High blood pressure itself has no symptoms; however, because prolonged high blood pressure can lead to heart problems and other issues, symptoms of those conditions could be a warning sign of high blood pressure as well (such as arrhythmias, palpitations or chest pain). Naturally if you are having any of these symptoms, you should see a doctor right away. Sometimes a headache develops if an individual has particularly high blood pressure or it has gone up suddenly.

Severe or long-standing high blood pressure that is untreated (especially malignant hypertension,which is a very severe kind of high blood pressure where the pressure is over 210/120) can produce symptoms because it can damage the brain, eyes, heart, and kidneys. Symptoms include headache, fatigue, nausea, vomiting, shortness of breath, restlessness, and blurred vision. It can even cause the brain to swell, and cause a patient to fall into a coma. This would be a medical emergency.

How is it treated?

The first step is lifestyle changes - diet and exercise. Studies have been done that show that eating a certain diet, known as the "DASH" diet (Dietary Approaches to Stop Hypertension), can lower blood pressure. When that diet also is lower in salt, it works even better. Salt works in your body to regulate the amount of water that is retained; less salt results in less water, and lower blood pressure.

The DASH diet calls for more whole grains, low fat dairy, fruits and vegetables, and healthy oils like olive oil, and less saturated fat, red meat, and all the other usual culprits. Here is a useful site that explains it all: http://www.nhlbi.nih.gov/health/public/heart/hbp/dash/how_make_dash.html

If you are going to start exercising for the first time in years, please do consult your doctor first and work up to it slowly.

Practicing meditation and yoga may also help reduce stress and help your general frame of mind which can help your blood pressure - or at least help you accept your condition!

If you are overweight, lose weight until you get back to the proper weight range for your height. Being on the DASH diet should help you accomplish this. If you still struggle with your weight, join a group such as Weight Watchers that helps keep you on track by group support and weight loss tips. If you're not into the group thing, some people's insurance will cover visits to a nutritionist who can help you tame your weight problems.

If you smoke, quit - smoking is another strain on the heart and blood vessels. Excessive alcohol consumption can also affect the flexibility of the blood vessel walls over time, so keep your drinking to the "moderate" level - one drink a day for a woman, or two for a man.

Say you try all that - faithfully - and it doesn't do the job. The next step is medication.

There are literally dozens of medications out there with various effects on blood pressure. Never despair - if you try several and they still don't work, your doctor can try a lot of different combinations until he/she finds one that does. If you have side effects from a drug even if it's working beautifully, don't keep it to yourself. Let your doctor know and you can try something different. There is no reason you should have to put up with any discomfort.

One of the oldest, and still one of the most effective type of drug, is the group of drugs known as diuretics (or as some people call them, "water pills"). These are just pills that help take water and salt out of your body and lower the amount of blood volume, which lowers the pressure. They also cause the blood vessels to dilate, another helpful effect. If your doctor tries to start you on something else first, ask to try a diuretic. They have the fewest side effects and the least hit on your medical insurance.

The one thing to be cautious about is that some diuretics cause potassium to be excreted in the urine, so potassium supplements sometimes must be taken with a thiazide diuretic.

Thiazide diuretics go under names like "Aquatensin," "Diucardin," "Diuril," "Naqua," and others. Many people just get the generic form of these drugs. The one my husband and I take is just called hydrochlorothiazide.

Another common class of diuretics include drugs such as "Lasix" (furosemide), which increase the kidneys' output of urine. There are also potassium-sparing kinds of diuretics, which include "Aldactone" (spironolactone), "Dyrenium" (triamterene) or "Midamor" (amiloride).

Another common type of blood pressure medication is the "adrenergic blockers," which block the effects of the sympathetic division, the part of the nervous system that can rapidly respond to stress by increasing blood pressure. They include beta-blockers, such as "Tenormin" (atenolol), "Lopressor" or "Toprol XL" (metoprolol) and others. Beta-Blockers are very effective for people who have had a previous heart attack and are also helpful for certain arrhythmias. They work by blocking adrenalin and thus slowing down the heart rate. (They can also make a person feel "slowed down" as well, which bothers some people). Another type, Alpha-blockers, affect the artery walls by reducing nerve impulses that cause constriction. A common medication of this type is "Cardura" (doxazosin).

Alpha-agonists are centrally-acting drugs that keep the arteries from constricting, similar to alpha-blockers. "Catapres" (clonidine) is an example of this type of drug.

ACE inhibitors (angiotensin-converting enzyme inhibitors) work by preventing the formation of an enzyme that constricts the smaller arterial blood vessels. "Lotensin" (benazapril) and "Vasotec" (enalapril) are two examples. (My husband is on enalapril as one of his medications). ACE inhibitors work very well but a very common side effect is an annoying dry cough. My husband never got this but I tried the same drug and after several months of being driven crazy by the cough, switched to another drug. The cough sneaks up on you...at first you just think it's an allergy or an irritation. But it just won't go away. Within a few days of switching drugs it disappeared completely for me.

Angiotensin II receptor blockers (ARBs) are similar to ACE inhibitors but instead of preventing an enzyme from forming that eventually results in the creation of Angiotensin II, the substance that actually causes constriction of arteries, this drug blocks the receptors for the Angiotensin II. Because they work in a different way from ACE inhibitors, they have fewer side effects. I am now taking one of these drugs, "Diovan" (valsartan), and have no problems with it. Other brands include "Avapro" (irbesartan) and "Benicar" (olmesartan).

Calcium-channel blockers are another blood pressure medication type. As the name suggests, they regulate calcium uptake into your cells, which prevents the walls of your arteries from constricting. "Norvasc" (amlodipine), "Cardizem" (diltiazem) and "Procardia" (nifedipine) are common brand names. My husband takes Norvasc as part of his regimen. If you use one of these drugs, be sure it is the "long-acting" version - recent studies have shown a slightly higher risk of sudden death with the short-acting varieties of this type of drug.

One other type of drug used to treat high blood pressure is the group known as direct vasodilators. This type of drug is only used in conjunction with other drugs, not alone. Examples are "Lonitin" (minoxidil) and "Apresoline" (hydralazine).

All drugs have side effects for some people. The drugs mentioned here can cause anything from dizziness to weakness or fatigue, or various other symptoms. When your doctor prescribes the medication be sure to ask him or her to explain what the common side effects may be. If you read the literature you get with the drug, you may never want to take it at all, because the drug companies are required to list every possible side effect ever reported. Please remember that the most important thing is to get your blood pressure down; you have to remind yourself that most people do not get these side effects, and have a positive attitude toward the drug. Don't expect the worst!

Also ask your doctor about drug interactions. Be sure to mention if you are taking any herbal supplements as well, since these too can interfere with blood pressure medications. Grapefruit juice (or grapefruit) also can affect how certain drugs are absorbed in your body; be sure to find out whether the one your doctor is prescribing is one of them. If you don't know, then avoid grapefruit juice or fruit until you find out.

Will you get it?

Very possibly. About 1 in 4 people in the United States has high blood pressure. It is even more prevalent among African-Americans (about 40%). For more information, see this link: http://www.cdc.gov/MMWR/preview/mmwrhtml/mm5401a3.htm

If you're over 65, even if you have normal blood pressure, your overall risk of developing hypertension before the end of your life is 90%. See article:

http://www.medscape.com/viewarticle/457696_1

For more information on blood pressure medication, please see this website: http://familydoctor.org/797.xml

For a fuller explanation of high blood pressure, please see the following links, where this information was obtained:

http://www.merck.com/mmhe/sec03/ch022/ch022a.html

http://www.americanheart.org/presenter.jhtml?identifier=2114

http://www.nhlbi.nih.gov/hbp/