Alice wrote the following, and my answers are posted after her questions.

hope you don't mind me asking a few questions.
I don't. Ask away.

what do you mean by citrate toxicity, and how did they manage it?
They use citrate as a blood thinner during the procedure to keep your red cells from clotting in the machine so they can be returned. At the same time they also give you calcium because the citrate binds to calcium and depletes it. For some reason, even though they were giving me more calcium than they had ever given anyone before, my ionized calcium would on occasion reach dangerously low levels and I would have a reaction that included a variety of sx, the most serious being a drop in heartrate and blood pressure. They would treat it by giving me fairly massive amounts of calcium and saline, and sometimes oxygen and magnesium. The docs thought after I'd had this three times that perhaps it's the magnesium depletion that's a problem as this also happens, so they started giving me magnesium as well as calcium during the procedure. Since then I've had no reactions.

when you say that they diagnosed your illness clinically, do you mean that you also had a diagnostic EMG?
I did have an EMG, but it was not conclusive. At the time I was mostly having bulbar sx, which are really difficult to test. When I say clinically, I mean that even though I'm seronegative, I have the clinical sx of MG, plus I had the thymic tumor and other thymic abnormalities such as some necrosis, and I responded to the plasma pharesis and had improvement from the surgery, so the conclusion by process of elimination is that I have MG.

the reason I am asking those questions is that I am interested in finding out if there is a subgroup of patients that seem to have their antibodies directed towards other proteins.

if you did not have a thymoma, I am not sure that the antibodies I have mentioned are relevant.

thanks,

alice


Let me know if you have any other questions.

Hi, MK. Welcome.

It doesn't look like from the national MG website (www.myasthenia.org) that there are any chapters in CA, which is weird!!! There are lots of people who live there. Maybe people with MG can't afford to live there.

Interesting discussion about you needing calcium and magnesium. Can't they use something other than citrate? Any vitamin C will do that (bind to calcium so that it isn't absorbed). Have they tried slowing the infusion down as well? Have they tested your magnesium levels (chem panel) in between doing plasmapheresis?

http://www3.interscience.wiley.com/j...28485/abstract
http://cat.inist.fr/?aModele=afficheN&cpsidt=2059781
http://www.pnas.org/content/99/26/16922.full

You don't by any chance have hemochromatosis?

This (citrate issue) also has me thinking about a possible mitochondrial problem. Has anyone thought about doing a muscle biopsy?

I'm sorry you had a tumor. Have they rechecked the EMG with a Single Fiber EMG? They can do that test at Mount Sinai.

I hope you can hook up with some people with MG in CA. I have a couple friends with it and it really helps to be able to talk about it. Then you have a "shorthand" with people and don't have to be explaining it all the time to everyone who asks incessant questions like: Why can't you do anything? Why do your eyelids droop? Why can't you talk long?

I'm glad you found this site. People here are amazing.

Annie

Annie, See answers to your questions below


Interesting discussion about you needing calcium and magnesium. Can't they use something other than citrate? I believe that they use citrate because it's more easily metabolized and doesn't cause the same fibrinogen deficit that heparin and TPA do. Citrate is the standard anticoagulent used in pharesis.

Any vitamin C will do that (bind to calcium so that it isn't absorbed). Have they tried slowing the infusion down as well? Yes, they slow it down if I start to have a reaction. Apparently it is quite unusual to have the reactions I do--the docs told me I was the only person at the pharesis center who had a citrate reaction in the last year, and I had three of them.
Have they tested your magnesium levels (chem panel) in between doing plasmapheresis? Yes, they test all of my electrolytes and my fibrinogen every week. They are often slightly low due to the pharesis treatment itself. My iron is also sometimes low and I have developed anemia when I've done p.pl twice a week and needed IV iron.

You don't by any chance have hemochromatosis? Not that I know of.

This (citrate issue) also has me thinking about a possible mitochondrial problem. Has anyone thought about doing a muscle biopsy? That has never been mentioned.

Have they rechecked the EMG with a Single Fiber EMG? No, although it was discussed. It would be very difficult, and painful, to do on my diaphragm. I don't really have ocular sx.

I hope you can hook up with some people with MG in CA. I have a couple friends with it and it really helps to be able to talk about it. Then you have a "shorthand" with people and don't have to be explaining it all the time to everyone who asks incessant questions like: Why can't you do anything? Why do your eyelids droop? Why can't you talk long? Yes, I do get tired of the questions, and I especially get tired of telling the same people the same things over and over again, because apparently they didn't listen the first or second or third or fourth time I answered their questions.

I'm glad you found this site. People here are amazing.
I'm really glad too, and yes, it does seem that people on here are amazing.

Thanks very much

Hemachromatosis is a buildup of iron! So if you are iron deficient, then probably not.

They actually will NOT do a SFEMG on the diaphragm. It's too dangerous. They could try it on a shoulder (deltoid) which is often positive.

Hemachromatosis is a buildup of iron! So if you are iron deficient, then probably not.
Oh! OK. I didn't know what that was. Glad to know I don't have that at least. I am mildly hypothyroid and I do have asthma. Not sure if either of those things affect the MG or the p.p. reaction. Having asthma along with the MG shortness of breath is kind of hard sometimes.

They actually will NOT do a SFEMG on the diaphragm. It's too dangerous. They could try it on a shoulder (deltoid) which is often positi
The neuro talked about it but then didn't think it was really necessary given all of the other evidence of MG. And I didn't push it because I'm really sick of all of the tests. The EMG was pretty painful and left me bruised and I'm not sure I want to do something similar if I don't absolutely have to.

Thanks very much for your interest and for asking me some interesting questions. It gives me some things to think about and research. I did read the links you sent--fascinating stuff. Thanks for taking the time. I really appreciate it. This thread conversation is the first time I've been able to talk to anyone else who has this disease. As you said, no chapters in CA and I've tried to make contact through some facebook groups to see if anyone in my area might want to talk or meet, but no such luck. I know there are people here with this disease, but I just haven't met any. It's such a relief to have some people who understand. I've really felt so alone . . .

Take care. I hope that your MG is not causing you too many problems.

thanks,

I too am very sensitive to citrate, or rather to calcium level shifts.

and I have a completely normal SFEMG.

the diagnosis of my illness was done by a direct measurement of the diaphragmatic strength. which showed significant weakness and fatiguability.

until then, even after an ICU admission, it was in doubt.

and even after that, quite a few of my "unexplained" and quite unusual symtpoms were atributed to "emotional problems" , and I was advised to seek psychiatric help. (not very helpful as psychiatrists are quite poor in adjusting respirators).

only recently have I found a neurologist, who was ready to think with me and try to figure it out.

alice

How did they test your diaphragm strength? Because I have asthma and MG I have had many, many spirometry and MIP/MEP tests. My MIP is actually close to normal, but my MEP is really not very good. It hovers around 50-60% of normal. But one thing I don't understand is how I get so easily to the point where I feel like I can't get a good breath if my MIP is OK. Do you have any idea how having poor MEP may function with MG--does it have to do with CO2 retention as I saw you wrote about in another thread? I was thinking that maybe I'm not expelling the CO2 and that makes me unable to get a good breath, but when I asked the pulmo didn't seem to think so. And since I'm not a physician I just went with what she said. One good thing is that I'm a singer and so I had diaphragm strength originally that was about 125% of normal, so I've been fortunate in that I've had strength that I could afford to lose without it affecting me too much. I assume that this is why even though I'm often short of breath due to the MG (not the asthma, which is very mild and well controlled and I always have at least 99% saturation), I haven't gone into a full blown crisis. But the MEP has been slowly getting worse, so I'm getting a bit worried.

I'm sorry that your sx were attributed to something psychological. I see that a lot with women. Many physicians are often quite sexist in my experience, and they are also quick to assume if they can't come up with a quick explanation for the problem that it must be psychological. I see a lot of overdiagnosing of depression and anxiety in my work. I'm glad that you have found a good doc who will work with you.

Anyway, thanks for your previous posts and your interest. I appreciate it very much.

MK,

forgive me for not wanting to disucss this too much.

it was a very hard and painful period in my life that I would like to put behind me, as much as I can.

basically, they can insert a transdiaphragmatic balloon and measure the pressure both in voluntary effort and in non-voluntary by stimulating it with a special magnet. this is a fairly invasive procedure and is only done in very few places in the world. when they do it repeatedly they can also check for fatiguability, and also see how much time it takes for the stimulation of the nerve to "translate" into a muscle contraction.

in fact, I have found out, from one of the neurologists I had discussions with, that the initial "objective" diagnosis of MG, was done in a similar way, on other muscles, and it was called the Jolly test. this was later abandoned in favor of the much more easy to perform EMG, with the (in my oppinion wrong) assumption that as long as there is normal conductance of the electrical signal from the nerve to the muscle, it ensured normal muscle contraction. ( which would be like saying that as long as your computer is in the electrical socket, it should work).

interestingly, even the fact that I had clear evidence for myasthenic weakness on direct measurement of muscle contractility, with a completely normal SFEMG, didn't make him even question this assumption.

well, it's not easy to say that the world is round, when everyone thinks it is flat. as obviously if it was really round people in australia would "fall off"", right?

and therefore it is impossible to have MG with a normal SFEMG, just as right.

alice

Hi MK

I would just like to say welcome to the group. Sorry I havn't done this sooner, have been busy reserving energy that seems to be seeping away at the moment.
Look forward to seeing you around
take care
Kate

I am wondering about a low MEP too. My MEP is always lower in predicted value than my MIP. In the hospital I was told by the rt that my MIP was low at first but by the second day had climbed back up to normal (65) but my MEP was still quite low. I would really like to understand better what a low MEP means - what causes that and what effects does that have?

I have low oxysats, well low-ish, down to 90 when I lie flat. Could this have anything to do with a low MEP?

I'm a singer too. Well, I used to be! I can't sing in a choir anymore but I'll still sing any chance I get at home when I'm able. If I could do anything again it would be that, well that and play the violin again. I was a pretty serious singer so I wonder if I also developed a much bigger lung capacity. My spirometry now looks normal but I KNOW I don't have the capacity that I used to have. I feel like half of my lungs haven't even unfolded. I too wonder how this all plays into mg. I seriously think that my prior lung strength/capacity might have contributed to the fact that I never went into crisis and have managed to survive these last eight years even though I feel like I've been on the verge of kicking the bucket many times.

Can anyone explain the MEP to us?

Ally