Georgia State Chapter Newsletter Winter 2005

Contents:

TREATMENT FOR SERONEGATIVE MYASTHENIA

COPING WITH FRUSTRATING COMMENTS FROM OTHERS

DRUGS USED IN TREATMENT OF MYASTHENIA GRAVIS

TACROLIMUS MAY BE USEFUL IN MYASTHENIA GRAVIS

REMEMBRANCES

 

 

 

 

TREATMENT FOR SERONEGATIVE MYASTHENIA

Approximately 80% of those patients diagnosed with Myasthenia Gravis test positive for acetylcholine receptor antibodies. Those who test positive on additional tests for myasthenia but who do not have these acetylcholine antibodies have been described as seronegative. For years the treatment has been the same regardless of whether the antibodies were detected.

This is still basically true, but recent findings are modifying the approach to seronegative patients. With a new test released in 2003, it has been found that approximately 35-50% of seronegative patients have antibodies against muscle specific tyrosine kinase (MuSK). Of the patients who have tested positive for the MuSK antibodies, none have had the aceytylcholine antibodies. Only limited data are available for MuSK-positive patients, but they appear to have a variable response to pyridostigmine (Mestinon), an excellent response to plasmapheresis, and an overall good response to immunosuppressant medications. In the small number of patients studied to date, there has not been improvement after thymectomy, but additional studies in the future will be needed to verify this.

AMPS: Upstate NY Jan. 2005






COPING WITH FRUSTRATING COMMENTS FROM OTHERS

If you've been suffering from a medical problem, it's not the easiest thing to deal with, right? Not only can it be difficult, but also it can be even more frustrating to hear foolish comments about the problem from others.

As Ralph Kramden of the Honeymooners would say, "Some people have a B-I-G MOUTH!" You may agree with this when you think of some of the comments you hear from people around you. They may know you have a problem but that doesn't mean they know how to talk to you about it, or what to say. They may say things that they feel are right, witty, intelligent, or even sympathetic. But you may think otherwise! There are times when a certain comment might make you want to implant your knuckles into the speaker's teeth! Or a comment might make you wonder if you're talking to a graduate of the Ignoramus School of Tactlessness.

But why are you reading all this? You cannot change other people. You cannot improve their lack of sensitivity or the way they talk. What you can do is learn how to cope with some of the ridiculous comments that you may hear.

ARE OTHERS BEING CRUEL?

Most people really say things out of sincere concern. They may be trying to make you feel better, show their support, or show an interest in you by questioning how you're feeling. Does that mean you must always be receptive to their questions and respond to all of them seriously? It would be nice. The problem is that hearing the same questions over and over can begin to get on your nerves. Initially, you may try to gently respond to comments or questions, or politely change the subject. However, this does not always work. Some people avoid this by simply not telling anyone about their problem.

For the purpose of this article, let's assume that we're discussing those comments that you can't avoid, from people who haven't yet learned to tune into your feelings. If you haven't experienced this, that's great! But read on anyway. You never know when what you read might come in handy.

THREE WAYS OF RESPONDING!

Many of the things that people say to you may be legitimate comments, but may bug you just the same. Others may not even deserve proper answers. Still others may be said without considering your feelings. But it doesn't matter why the comment is inappropriate. What really matters is how you handle these comments so that you feel comfortable. There are three ways that this can be done.

The first way is by ignoring the comments. This is not always easy, especially if the person is waiting for your response, or seems genuinely insulted by your lack of response. How do you get them to stop asking (besides buying a muzzle!)? Change the subject or walk away - ignore the question. The second way is by trying to answer in a rational and intelligent way, explaining your answer, how you feel, or what you sincerely want to communicate to the other person. But now you may feel like you're banging your head against a wall. What if you just can't convince the other person of what you're trying to say? Such frustration can be painful! There's a limit as to how many times you can try to explain something clearly, and not have it understood or accepted, before you explode. (This isn't good for your physical health, either!)

What if the first two don't do the trick? There's got to be a better way, and there is. The third way is to respond humorously. What does this mean? If someone says something unreasonable to you, or asks you a foolish question that can't really be answered logically, you'll accomplish very little by ignoring it or trying to reasonably explain your feelings. You don't know if your answer will be accepted or if the interrogation will continue. So, in many cases, the third option may be the best. This is called "paradoxical intention". The idea behind it is that the person is asking or saying something that is really unanswerable. So you're going to have a little fun with your response. Let's see how it works.

HANDLING THE "BIG MOUTH" SYNDROME

What might you hear? And how should you handle it? Remember, the best response is one that will educate the "commenter". You'd like to explain your situation nicely, in a non-offensive sincere way. But you're only human. So how can you respond when you get fed up! Read on.......

"BUT YOU LOOK SO GOOD....."

Let's say you've been living with a chronic medical problem that causes you a lot of fatigue. The way you look really doesn't indicate the way you feel. You've been awakened in the morning after a full night's sleep, but you still feel tired. You have a lot of do to get ready for your day's activities, but you don't feel like doing much of anything. Your husband walks into the room and asks you if you are ready to get up. You tell him that you're not ready yet, you'd like to rest some more because you feel really lousy. He looks at you and says, "How can you feel lousy? You look so good."

Wouldn't it be nice if you had enough energy at this point to pop him in the nose? Any time your fatigue makes you feel like your muscles have been drained of energy, it can be very frustrating to be told that you should do more because you look good. This is one of those statements that's hard to ignore, but it's just as hard and impractical to try to answer it rationally. So how can you respond to his statement humorously? You might say "Yes, I know I look good. You can call my plastic surgeon and thank him." Or you can say, "Yes, I look good. Wait until you see me without my mask on." Notice that in both of these cases, you're agreeing with the person first, and then you're saying something humorous. Isn't it better than saying, "How can you say I look good when I feel so awful?"

....OR "YOU LOOK AWFUL!" On the other side of the coin, it can be just as upsetting when somebody says, "Wow, you look lousy!" You may feel lousy but you certainly don't want to be reminded of it. You surely don't want to think that the way you feel is so obvious to others. You'd like to at least believe that you look ok to those around you. Even if it is said sympathetically, being told that you don't look well may be insulting. So what do you say? You might respond, "Thank you, so do you!" or, "Yes, I know. I've worked hard to look that way." Or if you are really in a cynical mood, you might say, "I know I look lousy. That comes from hearing people tell me this!" Of course, you could always say, "That makes sense, since I don't feel so hot, either!"

ENERGY SHORTAGE: Because fatigue related to a medical problem is so common, let's discuss another example. You are quietly sitting in a chair trying to regain some energy because you really feel exhausted. Somebody comes over to you and asks what's wrong. You try to explain that you're feeling very tired and you're trying to gather some energy. In a concerned way, the person says, "You're spending too much time thinking about yourself. Just get up and do something. Soon you won't even remember that you're not feeling well!"

How do you react to that? Do you jump out of your chair? Of course not. If you had the energy to get out of your chair, would you have been slumped there in the first place? Do you sit there and try to explain that you're feeling lousy? No, because it probably won't be believed. So how do you respond humorously? You might say, "I would like to get up, but somebody put fast-drying glue on the chair, and I'm stuck forever!" or you might respond, "I'm trying to set a Guinness World Record for the most time I can spend in this chair." Or you might say, "Do you know how much energy it takes to remain in this chair, when what I really want to do is to get up and knock your block off?" Obviously, the type of response you use depends on how angry or irritated you feel.

Remember: For this approach to work best, you want to respond in a light-hearted way. This will show the person making the comment that you're fine, but you just don't appreciate what he or she is saying.

Amps: Cope Scope and Detroit Chapter





DRUGS USED IN TREATMENT OF MYASTHENIA GRAVIS

By Darrell Childs, Licensed Pharmacist

This article will give a brief summary of the drugs used in the treatment of Myasthenia Gravis. It is by no means complete or can't possibly cover all medications prescribed to patients with this disease state.

Cholinesterase Inhibitors

These drugs include Mestinon (Pyridostigimine bromide) and Prostigmin (Neostigimine bromide.) These medications cause an increase of acetylcholine (the neurotransmitter, which is deficient in Myasthenia Gravis which, may lead to increased muscular strength. All dosages of these medications are highly individualized and titrated carefully for each individual. A dose suitable for one individual may be an overdose or underdose in another individual. Common side effects of these medications include fatigue, nausea, vomiting, and diarrhea. These medications essentially make everything "wetter", thus the patient may experience increased salivation, tearing, and sweating especially in overdose. Difficulty breathing and severe weakness is a sign of severe overdose and immediate medical attention should be sought.

Corticosteroids

These drugs include Prednisone and Methylprednisolone. Seventy-five percent of patients with Myasthenia Gravis have marked improvement of their symptoms with Corticosteroids within eight weeks of initializing treatment. Total remission of the disease sometimes occurs. Dosage of Prednisone usually starts at 1.5 to 2mg/kg per day. Prednisone should usually be taken with breakfast (food decreases stomach upset which the drug may cause) and taking the medication in the morning more closely correlates with Cortisol levels in the body. Diabetics should be aware that Corticosteroids can and will cause increased blood sugar requiring increased dosages of diabetic medications. Corticosteroid therapy should never be stopped suddenly; rather the drug must be tapered down based on current dosages. Side effects of Corticosteroid therapy (especially long-term) include: increased blood sugar, increased susceptibility to infections, redistribution of body fat (including moonface), facial hair on females, and although rare psychiatric disturbances including psychotic behavior have occurred with Corticosteroids. Long-term use may also cause weaker bones especially in females and ulcers in the GI tract.

Immunosuppressant Drugs

These drugs include Azathioprine (Imuran), Cyclosporine, and Cyclophosphamide. Azathioprine reverses symptoms in some patients but the effect may take up to eight months. The drug must be continued or the symptoms usually come back within two months. Azathioprine is usually used in patients where Corticosteroids have failed. In resistant cases both Corticosteroids and Azathioprine are used. Side effects of Azathioprine may include more infections and very rarely some forms of cancer. Some patients are started on both steroids and Azathioprine in the hope of discontinuing the steroid after the Azathioprine starts to become effective. Patients given Cyclosporine may improve in 1 to 2 months and maximum improvement is within 6 months of starting treatment. The dose is reduced to the minimum that maintains the improvement. Kidney damage and high blood pressure (hypertension) are side effects of Cyclosporine. Many drug interactions can also occur with Cyclosporine. Cyclosporine is usually used only in resistant cases of Myasthenia Gravis. Cyclophosphamide (an anti-cancer drug) is also used in resistant cases of Myasthenia Gravis. Side effects are numerous and common and may include: malignancy (cancer), increased infections, hair loss and fatigue.

In conclusion, knowledge is the best weapon in understanding the medications used in the treatment of Myasthenia Gravis. A newly diagnosed patient should demand information about their medications from their physician and pharmacist. The patient should wear a medic alert bracelet and should inform all health care providers of their medications and that they have Myasthenia Gravis as certain medications may exacerbate their conditions. Any new signs or symptoms should be promptly brought to the attention of their physician and pharmacist. Although a very difficult disease, many people with proper treatment, have the fullest and most productive lives as everyone else. The life expectancy of someone with Myasthenia Gravis is equivalent to that of the general population. Future research into this disease will no doubt result in better treatment, better diagnostic tests, and better quality of life for patients with Myasthenia Gravis.

AMPS: Myasthenia Gravis Manitoba




TACROLIMUS MAY BE USEFUL IN MYASTHENIA GRAVIS

A Japanese trial of the relatively new immune system suppressant tacrolimus (Prograf, FK506) in myasthenia gravis (MG) suggests the drug may have promise in this disorder.

In most cases of MG, the immune system attacks the acetylcholine receptor, a docking site on muscle cells that receives signals from the nervous system. The result is fluctuating weakness that can be highly disabling.

Patients are commonly treated with drugs that increase acetylcholine signaling and with corti-costeroids and other medications that dampen the immune response. All these medications have complex and potentially serious side effects when given for long periods.

In this 16-week study of 19 people with MG (16 of whom finished the study), seven showed considerable improvement on a scale of disease activity, and eight improved on measures related to activities of daily living. There were no serious side effects.

The study was open-label, meaning there was no comparison group taking a placebo (inert substance). The investigators say the results suggest that tacrolimus "could safely serve as an adjunct to steroid therapy for MG at low dosage."

MDA Publications, QUEST Volume 11, Number 1, JANUARY/FEBRUARY 2004

 


 

REMEMBRANCES




In Memory of:                                            By:

Tommy Harris                                                                City of East Ridge, TN

                                                                                    John Laramore, MD

                                                                                    Thomas & Sandra Kerfoot

                                                                                    Scherffius, Ballard, Still & Ayres, LLP

                                                                                    C.W. Hayes, MD

 

In Honor of:                                              By:

Elizabeth Kendley                                                         Alison Schemmer