Alice, Have you read this yet? At least someone is looking at MuSK MG patients. Hope you're doing okay.

http://www.ncbi.nlm.nih.gov/pubmed/21674519

And now some think that thymectomy in MuSK shouldn't be done?

http://www.ncbi.nlm.nih.gov/pubmed/21595175

Also, it sounds like India is doing research on MuSK patients too. I just thought you might want to know, since you have access to publications.


And I want to vomit when I read articles like this.

http://www.ncbi.nlm.nih.gov/pubmed/21650005

Sure, if we take massive amounts of harmful drugs, our lives are magically all better. Not.

Annie

Thanks Annie,

The first paper is authored by 2 people who still think that it is "impossible" to have a normal SFEMG.
Quite likely all the patients they describe had a dianogstic SFEMG, and were diagnosed relatively early. Therefore their long term outcome was good.
It is quite different in patients with a normal SFEMG.

It just makes me more determined to do what I can to change this. Because, quite likely the time of diagnosis and treatment is crucial just like in any other disease.

There are quite a few studies that show that thymectomy has no place in seronegative/MuSK MG. And quite a few neurologists that are unaware of that.

In fact the most open-minded and innovative studies come from the far east (not india). But, are not accepted by most of the narrow minded western neurology community. Sometimes I think that the SFEMG was planted in their mind, to the extent that they become deaf and blind to anything else. But, I think and hope that this will eventually change.

Many patients who are diagnosed and treated early, can do very well without enormous amounts of drugs. If you wait for years until you start giving treatment, chances are that you will have a relatively resistant disease, that even with enormous amounts of medications with all their side effects will not respond very well.

Again, those studies and surveys are based on patients who were easy to diagnose and treat, not on people like Chloe Atkins, you or me. And we are only the edge of the ice-berg, because we can talk and write about what we have been through, but I am sure there are many others who silently accept the verdict, when being told that it is all in their head.

My son said to me a while ago, that if I wasn't a physician, most likely I would either be in a psychiatric ward or dead by now. Probably he is right.
When I read the book by Chloe Atkins I realized how "fortunate" I am. (compared to her). And I couldn't help thinking how many others are there we don't even know about.

20% of MG patients will not have antibodies. Out of those about 50% will have a normal SFEMG. so we are talking roughly of 10% of the MG patients (patients with clinically evident MG who have no other alternative diagnosis other then various ill-defiined psychiatric problems), who are walking around undiagnosed. Some who may have a severe and life-threatening illness.

World-wide this is a significant number of patients no one in the medical community knows or cares about. But, I do.

Thanks for this thread. It's been a while since I've been here...I'm still sick and probably still in a bit of denial. I am going to have the MUSK test done as soon as I can make it into the lab.

My PCP is the one treating me and he said that he is 100% sure I have MG and he felt that there were probably undiscovered antibodies, variants of MG that could explain my situation. If only I could find a neuro willing to treat me. He prescribes my Mestinon and said he could order IVIG, plasmapheresis if needed.

Anyway, thanks for all that you are doing Alice to try and help those of us who "don't fit the mold."

Annie - Thanks for sharing that article. Seems to me that it's a good thing - connecting international clinical databases and doing population studies / reviews / whatever you want to call it to develop scientific results that can be used for further research or to improve clinical treatment. I know patient recruitment is one of the major obstacles for MG research, due to the rarity of the disease, so (in my opinion, not that its worth much) it's good to see them working internationally and using a database that can help overcome the patient recruitment issues.

It's interesting that PEX was so much more effective than IVIg in the MuSK+ patients reviewed - I saw results from another study comparing the effectiveness of IVIg and PEX, but not limited to just MuSK patients, and there wasn't a significant difference in their effectiveness. That's also interesting that most of the MuSK+ patients were female.

Alice - can you explain what you mean by saying that 2 of the doctors who wrote that article think it's impossible to have a normal SFEMG? I don't know your full story, but I remember reading that you had a hard time with diagnosis and treatment, so maybe is that what you're referring to? I also don't understand the connection between SFEMG and this article, since it seems this article was focused on the limited purpose of describing characteristics of MuSK-antibody-positive patients in these 2 databases, not the accuracy or use of SFEMG as a diagnostic tool. Maybe I'm missing something, since I can only see the abstract and I gather from Annie's comment you can see the full article?

First, I agree with you that gathering international data is very helpful in relatively rare diseases. MuSK is a relatively new entity and much is not yet known and understood about it, so this kind of data can be very helpful. This is also the first paper I have seen that clearly states that MuSK can be atypical in its clinical presentation and misleading in diangostic tests (such as the tensilon test), and therefore hard to diagnose.

The point I was referring to, is that the vast majority of the patients (97%) in this series had a diagnostic SFEMG. It also seems (even though it is not clearly mentioned) that the diagnosis was relatively fast. Therefore, the outcome which is overall favorable can not be extrapolated to patients in which it took much longer for the diagnosis of their illness.

And you are right that I was referring to my own experience. The two authors I was referring to are the ones that lead the approach of "a normal SFEMG rules out MG". Although they seem a bit less amendment about it in this paper and also do mention that it is not clear how MuSK affects the NMJ. This is why I mentioned that I think and hope that this will change.

Alice, I knew you'd have the background on these articles. I had questions but just wanted to get the input from you. That's what I liked about the first article too, that it FINALLY said MuSK can be atypical. MG is atypical.

What I find sort of funny is that PubMed articles often are portrayed as "the truth" when the truth can be anywhere from A to Z.

I think that a big reason so many doctors cling to these ideas of what the truth is (i.e., you can't have MG without a positive SFEMG) is because if they or other "experts" proclaim what the truth is, then saying later that it's not entirely accurate is either calling them a liar or calling them into question. It's as if no one in the medical profession can handle being wrong. If only they'd see science as something that evolves; that's not static but plastic. It doesn't mean they are less as doctors or humans are less valid. The ironic part of all this is if they would be more open to variability from the beginning, they would look more valid, more reliable.

When they first began this "MG hypothesis," they proclaimed the only antibody was the AChR one. Oops, they were wrong. So then they padded their theories by saying that 80% of people with MG have the AChR antibody, 15% have the MuSK and others are "seronegative." Oops, they're probably still wrong. There are indications of a number of other areas in the NMJ that are being attacked. But until the "mammoth institutions" proclaim this fact, no one dares to go against what they say.

It's a very closed-minded way to look at the world.

This sort of sums up how I feel about the way neurologists "see" MG. The human body is so complex and I simply think it makes THEM feel better if they have an MG algorithm to guide them. MG will never pay attention to an algorithm.

"Saying a patient’s MG is mild, or nonexistent, is mildly amusing. MG is like a noble gas, also once mistakenly thought to be “inert.” Add movement and heat and you have an MG explosion. It’s not easy to see how a patient’s MG is truly doing while they’ve been sitting in an exam room for over an hour, early in the day, resting their muscles. That’s perhaps the most ridiculous part of “examining” a patient with MG and declaring they must be fine. They should put us in a sauna for one minute, watch us plod up some stairs and then examine us, since heat and activity make MG worse. MG patients can get weak on a speed spectrum from uphill slowly to downhill fast. Like doctors, you have to see MG in action to truly know how dangerous it is to a patient."

I'm sure your son is correct. And that's so sad. I hate that there are others out there, suffering for the inability of the medical world to think creatively. And to have faith in what they are seeing, not in the imperfect testing.

I found it interesting that there's such a high number of people with MG in China (especially childhood onset). Why? Toxins? That was in other articles I read. Their society is so relatively secretive that we may not know why for centuries.

Shalynn, I'm still not convinced you don't have CMS. Or an odd variant of MG. Have they tried to see if plasmapheresis or IVIG helps? Some doctors will do that to try to determine if it's genetic or autoimmune.

Jenn, Sorry, I only had abstract access while Alice has the ability to read the entire articles. We peasants, I mean patients, don't deserve to read full scientific information! As Alice knows, as much as they talk about sharing info, so many of them don't want to because that would mean someone else would take credit for their "discoveries." Because of this attitude, so many drugs or information are slow to come out. We would probably solve problems a lot more quickly if people weren't so greedy.

When I'm exhausted by doctoring, as I am now, I like to do more research. Learning helps me to feel more empowered and believe that there is "hope" out there for patients of all diseases. Maybe if doctors (no offense meant, Alice) and researchers stopped getting in their own way, we might have half a chance at better lives. I think they need to take courses in creativity, so that their brains remain wide open.

Annie

Hi Annie,

I know you have had very bad experiences with doctors. I did too, but as a physician I can possibly see it a bit differently. And therefore be more hopeful about it.

Overall physicians are people, and most people find it hard to change what they think (be it political, religious or any other thoughts or beliefs they have, even in arts and music ). And obviously some more then others.

I think that the advantage of modern medicine (as opposed to other healing approaches) is that it constantly questions the "truth". Nothing is sacred in our textbooks or journal articles. We constantly write "letters to the editors" that question the validity of the conclusions of certain articles, or raise new questions etc. Yet, in medicine and also in science you do have to work hard to convince people to accept what doesn't fully fit with what they know and understand. Many times it takes more then one person or group to gradually gather the evidence. But, more times then not it will happen eventually.

An excellent teacher I had in medical school told us that half of what is written in our textbook is going to be incorrect 20 years from now. The problem is that he can't tell us which half. And he was quite right.
There are numerous examples I can give you-medications that were contraindicated in heart failure are now used to treat heart failure. the approach to peptic ulcer has changed 180 degrees, etc. etc. my field of practice-hematology, is one of the fields that evolved tremendously (and still is). On the other side of the spectrum is neurology, which has changed much less. And I think the main reason for that is that it is much less accessible for research. I can take my patients samples to the lab at the end of the day. they can't take a "slice" of the brain, or the nerve or the muscle.

Yet, as biological systems are very similar, what was learned in my field of practice, can be extrapolated to neurology. And in fact I am finding a few neurologists who are willing to understand that (albeit quite a few others that do not). My understanding of receptors and down-stream signaling comes from my own research. Why should muscle cells be different in their biological behavior from lymphocytes? The understanding of the complexity of antibodies and their interactions with their targets is my field of practice, so obviously I have a better understanding of that then they do.

To me (if I put aside my own personal emotions regarding the personal consequences of this for me) the "SFEMG axiom" is just another such misconception, that is gradually being realized as such. I can tell you that I have personally managed to convince 2 neurologists (one who was my treating neurologist for 3 years, and I nicknamed-my SFEMG neuro, because he put so much emphasis on this test), that this has to be questioned.

Angela Vincent recently presented in a conference, a few patients with MuSK MG and non-diagnostic SFEMGs. (diplomatically she didn't use the word "normal"). And you are right that people tend to accept such results more readily when they come from a place like Oxford. In fact the person who sent this abstract to me was my SFEMG neuro. who told me two years before that it is impossible to have myasthenia with a normal SFEMG in a weak muscle.

I recently sent a letter the editor of a neurology journal, in response to a paper that classified MG as either AchR positive, MuSK positive or SFEMG positive. (eg- if there are no antibodies and non-diagnostic EMG the patient does not have MG). This letter was accepted and published, and I even received a request for reprints. I recently gave it to my neurologist, who told me about two patients he saw in his clinic with non-questionable MG and a completely normal SFEMG.

So, I think there is hope.

You are perfectly right that a one time neurological examination in the office, gives you very little information regarding the patient's condition or the severity of his/her MG. And most neurologists know that. In fact my neurologist never examines me, because he thinks it will add nothing. He just asks me how I am doing and have been doing since he last saw me. And lets me use what ever is convenient for me to document it.
Yet, they have to cling to something, mostly if the patient can't describe his/her symptoms in a clear way . I think most patients in the early days of their illness can't, because it is so overwhelming and confusing. I know I couldn't. It didn't resemble anything that I knew as a person or as a physician, except for maybe "alice in wonderland", (but this is not a very useful description to give your physician).

Having or not having access to full text articles is only a matter of money. In fact many people are now ready to pay so that their paper will be on open-access, and many journals will give open access, because they know that if more people will read, more people will cite and this will increase their impact factor. ( a very important number that says how good the journal is, or how good the specific researcher is). Most students in universities (not only medical school) will have full access to most journals. Hospital or university libraries buy packages, that a single user can't afford.
the way papers are reviewed and accepted (or rejected) is another topic. Pharmaceutical companies have too much influence and power in this.
It is a very complex issue with many ethical, political etc. ramifications.
And you are right that sometimes excellent papers, that could add to our knowledge base and understanding are rejected, while others that add very little are readily accepted.

The good news is that more and more physicians realize the importance of internet forums and interactions with patients. They realize that when physician and patients join their knowledge and experience 1+1 becomes much more then 2. I do think that within the next decade we will witness a significant change in the way knowledge is disseminated. I think that joined physician-patient discussion groups will be the norm, and the peer review process will be done on-line. There is one such experimental journal like that, in which you can publish what ever you want (similar to this forum, but in a paper format) and then whoever wants to review it, and write comments does so. So far, there are only esoteric publications there, but possibly with time it will change.

So, yes there is hope.

Alice

Sure, I know there is hope. But at this moment in time, things have not gone far enough.

And when, after all my doctoring nightmares, my neurologist - whom I thought I could trust - looked at me last year and proclaimed that my MG is "mainly ocular anyway," I could hardly manage a comeback let alone have hope. I suppose I can hope that when I wear myself out for him in two weeks, I'll have the strength to help him see the truth. That plus making O2 saturation videos and taking photos. He is not an MG expert, so that doesn't help either. He was the one neuro left here who hadn't jumped on the "keep all of us from being sued" bandwagon by minimizing her disease. Geez, they're so paranoid. After 52 years of having this disease, having s-loads of proof, positive antibodies, an MG crisis, breathing tests that fluctuate highly according to how I'm doing and so much other proof, I'm still having to "fight" to be heard. I'm tired of fighting. I find it very hard to stand up to someone who has ultimate power over my health and is the only one left to care for my MG. Sort of like ticking off your jailer.

There's a distinct difference between ignorance of knowledge which causes a doctor to harm a patient and intentional dismissal of knowledge by a doctor to keep themselves from harm, which still harms the patient. I do not forgive the latter and don't believe there are any excuses good enough for such behavior, especially by those who supposedly have sworn to "Do No Harm." It's far more easy to forgive a "mistake" than an intentional act of harm.

Critical thinking, combining left and right sides of the brain through creativity, may be the only way to crack open people's minds. So many doctors have controlling personalities though and they are near to impossible to change because they cannot admit they're wrong without falling apart. They are so insecure - which I know sounds strange but is true - that they HAVE TO make everyone else appear wrong to feel good. And many "CP's" are attracted to positions of power, like doctoring, governing, policing, etc.

I digress. The bottom line for so many patients is that they need help and need it NOW. I'm grateful that there are doctors willing to rethink outdated facts. I know some of them who do. I'm glad you are doing your best to change what you can. We can all "try" to inch our doctors in a better direction. But don't expect me to have hope right now. My MG is bad and I have no hope for more treatment.

Annie

We both know that unfortunately you do not have "occular MG". We also both know that many times neurologists (even those who want to help and care) get totally confused by this illness.

I had a patient with "occular MG" who was referred to me by his neurologist, who I know is a fairly good and caring physician. The reason for the referral was symptomatic anemia, as he was having increasing episodes of shortness of breath. It didn't take me long to understand that he has significant respiratory muscle involvement, and send him for the right tests. I then sent him to my pulmonologist (who learned about MG from all the mistakes he did with me) who gave him proper NIV support. Only then was his neurologist ready to "consider" the possibility of generalized MG.

It doesn't sound as if your current neurologist is intentionally harming you, it is more likely that he just lacks the knowledge and understanding required. Possibly a clear letter from your pulmonologist could help.( I am saying a clear letter, because many times pulmonologists write letters that neurologists are incapable of understanding. they then interpret them any way that suits them).

As you know, I too had the kind of experiences you are describing, but don't want to elaborate about them here. Don't let them make you lose hope.

Alice - thanks for clarifying this and providing a little more info since this was not in the abstract. I'll have to get a copy so I can read the full article. As for the idea that delayed treatment can make MG harder to treat and achieve optimal results.... I've kinda heard that sentiment all along the way, from various doctors and patients, so I'm a bit surprised that it's such a point of contention.

Hadn't heard this one before... I will definitely have to look into it.

Annie - no need to apologize for only having access to the abstracts! I totally understand that - my problem was connecting the discussion of the SFEMG without the clarification that Alice got that part from the full article, not just the abstract. But I got it now :-)