Ally, Thank you so much for posting that information!

I would bet that Rach's docs are withholding valuable information from her as well. It makes me so sick that doctors are now doing this. It's a "trend" to keep themselves from being sued when they dismiss a patient's concerns or say they're fine.

My neuro from now eight years ago withheld a positive AChR antibody test from me. He knew about it when we had an appt. And when I requested all labs, they didn't send me that one. Coincidence? No way.

Ally, those tests are HUGE clues. It makes me sick. I don't know what your state statutes say but the ones in my state are VERY clear that they cannot withhold your records. Nor can they not tell you about them!!!! You can call your local representative or state senator about it or look it up on your state's website. Consulting a lawyer might not be a bad idea either, since they already tried to get you to sign that waiver.

Allen, What a generous offer. Where are the billionaires when patients need them? Or the ethics.

I hope you're feeling a bit better, Rach.

Annie

Ally, You snuck in more info before I could post! I agree with you that not doing a biopsy was ridiculous. Mitochondrial disorders are not rare. Neither is CMS in Northern Europe.

I just want to say one more thing, Rach:

When looking up some of these disease, particularly the mitochondrial diseases, a lot of the descriptions found on the internet sound horrific and hopeless. This is NOT true. What happened is that they used to think that these diseases only occurred in children and, sometimes, young adults. The diseases were considered fatal or very, very disabiling. Now, they're realizing that a lot of adults and elderly people have the mito diseases and they can have disease courses which are mild or asymptomatic or moderate. These cases outnumber the other ones by a long shot.

My terrible neuro sent me home after telling me that I might have a mito disease. Well, I thought I was dead meat I thought my children would be horribly affected. That's NOT true. My neuro was an idiot. And a lot of the internet descriptions haven't caught up. Please be forewarned: they are talking about the worst possible cases. The more up to date sources make this clear. In fact, the UK site is very clear about this. For instance, a horrific sounding mito disease, MELAS, when googled seems hopeless. In contrast, here's the description from the UK site:

This is one of the most common causes of mitochondrial disease. Patients with this mutation have variable disease manifestations ranging from no symptoms at all, to being quite severely affected with the syndrome called MELAS , this is the short name for a collection of symptoms called mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes.

- and -

In other words if you have a lot of good mitochondrial DNA you are unlikely to develop severe symptoms. If you have a lot of bad mitochondrial DNA then you do tend to develop more symptoms and the disease might be more serious. However, it is only a guide and it has been stressed throughout this website there is an enormous amount of variation between different individuals even with the same level of mutation and even within families.

- and -

The clinical features associated with this mutation can, as stated above, be very variable. We have a number of individuals who clearly carry the mutation who are completely asymptomatic. Other patients have very, very mild symptoms perhaps with a tendency to have diabetes or very mild deafness requiring no treatment. These patients might not be aware that they had the mutation apart from the fact that they were family members of somebody who had more serious disease. Some people with the 3243A>G mutation, also develop diabetes and deafness ultimately requiring the use of a hearing aid or requiring insulin to control their diabetes. Other patients have more severe involvement with muscle weakness sometimes affecting the peripheral muscles and sometimes affecting the muscles around the eyes.

PLEASE take this to heart before you start googling (if you choose to do that). I DON'T want you to go through the h*** that I went through. And I want to assure you that the mito forums are loaded with people, well into old age and not completely or even at all disabled who have some health problems and definitely have some of these mutations but are OKAY.

I just wanted to make this very, very clear!!!!!!!!

Ally

Thank you so much I have been overwhelmed by your kindness - thank you Allen for your offer. Thank you Ally, Jana, nicky and Annie.

Ok I have the report and its far too long for me to type here. However there is something new to throw into the mix. My response to the tensilon test was negative. I reacted to the atropine. I will give you the paragraph

"Although there was a response to atropine I am not aware of any disorder that responds specifically to this drug but I suppose one might speculate on some curious form of movement disorder that might do so although I have been able to find no reference to such a phenomenon in literature. This sort of problem is in any case outside my area of expertise."

The good thing about the letter is he hasn't said its all in my head. The bad news is he's given up trying to find out.

So if anybody knows of a disorder that responds to atropine..........

Rach, I wasn't sure I could respond to that without swearing profusely and getting kicked off the forum!!!! OMG. I'll say it's outside of his expertise . . . like the expertise it takes to look a drug up on his computer.

Well, the congenital slow channel myasthenic syndrome (SCCMS) certainly does respond to Atropine. Those people get too much acetylcholine.

Listen up, UK doctors, time for a lesson in pharmacology. Chime in here anytime, Mrs. D.

Atropine: Does the opposite of Mestinon and has longer acting effects than it does. Like those eye drops they use to numb your eyes. It "paralyzes" the muscles. DUH. It reduces the amount of acetylcholine in the neuromuscular junction.

Mestinon: Gives you more acetylcholine for longer, due to it's effects on the enzyme acetylcholinesterase.

http://neuromuscular.wustl.edu/synmg.html

So when did they give you the Atropine? Its effects tend to last longer than Tensilon. Did they give that too? In what order. I imagine they would all be injected.

SCCMS shows what is called a Repetitive CMAP or R-CMAP after Tensilon is injected while they are doing a repetitive nerve stimulation. This is taken directly from the book "Neuromuscular Junction Disorders: Diagnosis and Treatment" by Doctors Matthew N. Meriggioli (University of IL), James F. Howard, Jr. (UNC) and C. Michel Harper (Mayo Clinic).

http://www3.interscience.wiley.com/j...TRY=1&SRETRY=0

Just Google "repetitive CMAP" for more articles. Do you think your doctor could've taken a couple of minutes to look up Atropine?

So, if you have a slow channel syndrome, you have basically been given the opposite of what you should have. I am most definitely NOT recommending this but quinine is in tonic water. It's sort of like Atropine. People with MG should not have it.

Who knows what you have, however. If you responded to the Atropine, did you respond due to the drug or due to the drug wearing off? Atropine and Tensilon can "cancel" each other out, making you look fine. Although no amount of Mestinon after an eye appointment with those Atropine drops makes my MG better. Atropine makes me so unbelievably bad for the next couple of days.

I cannot believe this insanity. Theirs, Rach, not yours!!!

I have to go take a nap now. Wish I could drink. I wish you could drink.

Annie

Okay, I tried to think of an example equally as crazy as giving someone Atropine during a Tensilon test. Which, BTW, is NOT done over here in the US. They either give saline or Tensilon. Too dangerous to give an MGer Atropine.

EXAMPLE: If an allergist wanted to know if you had an allergy to peanuts, would they give you Benadryl before testing you for it? NO. You have to be off of all antihistamines two days before testing. TWO DAYS. Histamine and an antihistamine cancel each other out. Well, depending upon the amount of antihistamine and the severity of the reaction.

Attached Files

AtropineInjection.pdf (25.1 KB, 94 views)

Ok, Annie, so let me get this straight. When WE (the "regular" MGers) take too much Mestinon, they can give us Atropine to counteract the effect.

So, Rach's body may be naturally producing too much of its OWN "Mesty" and when she got the Atropine -- she returned to a state of "normal"??

Where do these UK docs get their training??

Too funny, Jana. Yup, they train at "R. U. Kidding University."

It's a little more complicated than "producing too much acetylcholine." It's more like the doorway to the neuromuscular junction is left open too long so that too much gets to the muscle. Make sense? That's why they call it SLOW channel! The channel gets flooded with too much.

Yeah, Atropine is what they give us when we have a "cholinergic crisis," from having too much acetylcholine.

And not to go on and on but this is important. Doctors in the US who do the Tensilon test keep Atropine on hand in case they overdose a patient. It is not part of the protocol. Just like allergists keep antihistamines on hand if a patient reacts to a skin prick or intradermal allergy test. What do we patients need to keep on hand to counteract doctors like this? Knowledge.

It's so simple even a UK doctor could understand it.

Its a bit concerning as this guy is supposed to be the UK expert on CMS and MG, if he doesn't know what disorders react positively to atropine.

The tensilon test went as follows Saline, Atropine, Tensilon. Given at 5 minute intervals. All strictly timed.

I'm struggling to get my head around it at the moment. I will just may take 24 hours or so!

Thank you!

Love
Rach

they didn't do any emg or sfemg whilst doing the test. I forgot to say that. They did those the day before, no abnormalities shown.

I almost can't look up any more about this topic, Rach. It's very upsetting.

I have found out that the reason some doctors give Atropine is as a "premedication" so that someone with MG does not go into any kind of exacerbation or crisis when given the Tensilon.

I have not read anywhere that it is a "part of" the Tensilon test. It would be illogical to use it in that way or use it as a "diagnostic tool" to see if someone has MG. Even when they inject something for the sodium channel CMS, it is Tensilon before the repetitive nerve stimulation.

Atropine, apparently, has a much longer "half life" or life than Tensilon. We're talking HOURS.

http://dailymed.nlm.nih.gov/dailymed...nfo.cfm?id=649

Here is the protocol of the Tensilon test from WUSTL:

http://neuromuscular.wustl.edu/mtime/mgdx.html

Yes, it is indeed concerning that a leading expert would not know what Atropine does to the sodium channel CMS. And it is also concerning that he would give you a drug, as part of a protocol, that could've tanked you if you had MG, would obviously counteract any effect of the Tensilon and would result in you not getting the results you so desperately need. Not to mention the financial aspects of this.

I find this highly unethical. The neuro who did my Tensilon test was so careful. He only gave me 2 mg. of Tensilon at a time, which I did not know until after the test. I responded immediately and dramatically. They can give UP TO 10 mg. I wonder how much you had. And I have to wonder how much Atropine they gave you.

I know there are good doctors out there. I have good doctors. But how are we supposed to trust doctors in general when they do this kind of thing? When they keep test results from you, as in Ally and my case?

Seriously, didn't these guys go to medical school? And if they don't know something, they can readily look it up. And I have to ask, how many other of this doctor's patients have been given this protocol and not been properly diagnosed?

This is what is really upsetting. If this guy is smart, then he WOULD know what Atropine was all about. Was he giving you the Atropine to avoid a potential lawsuit due to a cholinergic crisis? I know that sounds absurd but how can this guy not know what the drug does when he is using it? It is completely nonsensical.

Rach, I am so sorry to have even brought this up but I believe the truth is so important. I am sorry you were treated in this manner. I honestly believe, if you did respond to the Atropine, that you need to be tested for the CMS-related genetic disorders. Did they videotape this test? Send a DVD copy off to Dr. Bruno Eymard in Paris and ask him what he thinks.

I'm really sorry for all you have to deal with right now.

Annie

I seriously don't even know what to say about this...It's absurd, seriously...

Okay, if he has doubts that you have MG, and he knows that you've had tensilon in the past, and haven't had a crisis, why would he give you atropine first (before tensilon), especially when its effects last for so long, that it'd render the results of the tensilon useless (because they'd be inaccurate)? I think that this is why a lot of docs. don't use tensilon anymore- because of this ridiculous situation that might materialize...

If you reacted to the Atropine, does that mean that you got better or worse? I'm assuming better? Didn't they used to give something to people with MG to see if they'd get worse? I remember reading something like that, but they had to stop it because people would sometimes almost die...

I don't know...This seems really weird...

Imagine how many other people are going through the same problems because the doctors don't know what they're doing...How are you supposed to even know what to do/where to turn when you have to tell the docs. how to do their job? This is so weird...



Nicky