Anybody with CIDP AND Myastenia Gravis?
AnonymousMarch 20, 2009 at 2:08 pm
We’re currently testing our 13 year old daughter, Julie Ann, for Myastenia Gravis (MG). Our neuro has never heard of a patient experiencing both CIDP and MG, but the MG explains new symptoms she’s experienced over the last year (difficulty chewing/swallowing, double vision, asymmetric weakness, much more frequent weakness and a lower total response to IVIG). There is also a chance that instead of MG it’s Lambert-Eaton Myastenic Syndrom (LEMS), but MG is a better fit.
Has anybody else experienced MG co-existing with CIDP?
AnonymousMarch 20, 2009 at 4:50 pm
I have CIDP with cranial nerve involvement (also autonomic) not MG but I can remember when I had my first bad attack years ago, I was tested for MG as I had ptosis, double vision, difficulty with chewing, swallowing, upward gaze (very uncomfortable) plus the usual weakness, difficulty walking, sensory loss/sensations, absent reflexes, etc.
My tests came back negative for MG. It took another few years to be diagnosed with CIDP – so as a result never had treatment for it, so I cannot say how I would have responded to treatment. Apparently, in my case it was hard to diagnose (because of the unusual cranial nerve symptoms).
In my case, the above symptoms started at the same time (with an attack) and still play up years later if I overdo things. I would never be able to chew a steak even now (too tiring for my jaw) – it’s now mashed potato and sausages. Interestingly, I have a tremor in my jaw 24/7 since my attack – I was told it was from weakness.
I’m so sorry your daughter has been experiencing these new symptoms – the old symptoms are bad enough! I also hope you will be able to find out what’s happening very soon.
All the best,
AnonymousMarch 20, 2009 at 11:38 pm
My daughter was tested for MG at 5 years old. She was dx’d with CIDP at 4 & needed quite a bit of IVIG to sustain normal function. She tested negative for the MG though.
Emily had cranial nerve involvement, difficulty swallowing & chewing, eye involvement (her right eye turned inward & couldn’t move to the side), & double vision.
The ONLY thing that helped her was very frequent (2-3 times a week) IVIG treatments.
We also tried steroids for a bit in conjunction with the IVIG but they only made her worse.
Have they done any other genetic tests?
I remember reading, when we were going through the test, that it’s extremely uncommon to have both CIDP & MG but it is possible as both are auto-immune & it’s common to have more than 1 auto-immune disease.
I did a quick Google search for Myasthenia Gravis & CIDP. Here is a link to the results page:
Hope that helps.
AnonymousMarch 21, 2009 at 8:32 pm
First, thank you for the support and the Google link. It seems like the combination of MG and CIDP is very rare, but it happens (as if getting CIDP at 5 years old isn’t rare enough on its own). Julie Ann has been taking Mestinon for the MG for a week now and she seems to show improving strength. The hope is that we’ll we find that it’s been the MG and not the CIDP that’s been causing the more frequent weakness and be able to spread out the IVIG treatments again. It’s too early to tell. We are planing another broad round of testing in a few weeks to validate the dx and see how her nerves are doing.
If all this wasn’t enough we discovered this afternoon that she had asymmetric facial paralysis and couldn’t close her left eye. A trip to meet her pedio-neuro at the emergency room resulted in a dx of Bell’s Palsy. I guess all things considered, Bell’s Palsy is a good choice if you have to have a third auto-immune, but it scare me with a third A-I to think about what’s next for her. We’re lucky that she’s such a trooper, but she shouldn’t have to deal with this on top of everything else (especially in junior high).
I will say that I’m so thankful that we have a good neuro that listens to Mom and a hospital that will work with parents. Dr. Kerr and Dell Children’s have been God sends. I could write an essay on how much Dr. Kerr has helped us manage her treatments protocals; and the nurses on the 4th floor at Dell’s Children are so fantastic. While I wish we didn’t [I]have [/I]to know them all so well, I’m so thankful they’re there. As much as I’m stressing over this latest diagnosis, I read about so many others out there that struggle to find a good doctor that will take the time to listen enough to diagnosis them and I realize how lucky we are.
AnonymousMarch 28, 2009 at 1:55 am
I hope you will let us know how your daughter’s MG tests went.
I also had facial weakness on one side of my face after my first attack.
Has her voice been affected? Mine went down to a whisper for a few weeks after my attack – and is still weak to this day.
March 28, 2009 at 12:09 pm
I understand the frustration you guys are feeling. It is getting harder to survive every day, for myself and Kevin. I suspect he may have asthma now and very bad allergies. Migranes plague him daily. He just feels like crap every day. It breaks my heart that these children have to suffer so much. We go to the immunol. soon and the neuro again in April. We are trying to r/o things to figure out the origin of others?????/
AnonymousMarch 28, 2009 at 5:17 pm
There are two things here that I want to mention, as you mention two different problems, and I can give you some answers as an actual experience that I have.
First, I have full CIDP with full autonomic involvement. About two years ago, I, too, had trouble chewing/swallowing, and they also did tests on me for MG. They also did a swallow test on me, and saw the problems there, (they recorded it, and was able to show me the results, pointing out the problems. They later determined I did not have MG, but because of the autonomic involvement, they then added to my many diagnosises from the CIDP hitting the different autonomic organs/processes, “Dysautonomia with dysphagia and gastroparesis and esophageal dysmotility”. They did put me on Mestinon, later increasing it, and am still on it today. Though, as time goes on, swallowing is now getting worse, and I have to stop eating cookie-like textures, (crumb-like foods), and cut everything up small…I probably will soon have to go on a soft diet. They told me a duodonal feeding tube, (a feeding tube that bypassed the stomach, since the nerves controlling the stomach are not working), might be in my future
Second, you mention the asymmetric weakness.
When I was finally diagnosed a few years ago, after two years of going through what I did NOT have, the end result and diagnosis was, “CIDP with Lewis-Sumner variant”. If you google that, you can read about it. The CIDP in the beginning, only hit the left side of my body, the leg and arm with great weakness, no reflexes, and the normal CIDP tests, (i.e. ooo, the brain fog is making me forget the name of those electronic tests with the needle in the muscles, but you know what I am talking about), all proving CIDP in the left side only. That is the Lewis-Sumner variant, which is rare. Though, later after a couple of years, the CIDP affected both sides of the body.
I hope this gives you some input of a possibility with your daughter–perhaps mentioning to your doctor. I do hope she does not have MG, as, when I was on plasmaphereses a couple of years ago, I became friends with a guy who was also getting PE, (plasmaphereses), because he had MG. He had it bad–would have to get PE once or twice WEEKLY. IVIG did not help him–only the PE would work for him. He was also a patient of my neuro., who is head of neurology at my hospital.
AnonymousApril 24, 2009 at 7:32 pm
New wrinkle in Julie Ann’s treatment journey.
Our neuro had more comprehensive testing conducted a few weeks ago that changes his MG diagnosis. Following the test they’re now telling us that Julie Ann is [U]negative [/U]for MG, Myostenic syndrome and the few other variants of CIDP (even though she had some MG markers). Given that, the doctors don’t have an answer for the double vision, week jaw or slight incontinence.
We’re pleased that there’s not a second AI disorder to deal with, but at least when we thought she had MG, there was another treatment option to deal with her ongoing symptoms…now we’re still stuck trying to decide if we need to do something more than just IVIG. Currently we’re doing in-patient half treatments every 1-2 weeks, and it wearing everybody down.
We’re told that the fact she has “f-waves” in her arms meant there is no autonomic (sp?) nerve involvement, but we’re not sure how conclusively we can rely on that fact. Our neuro had suggested that we go to the Mayo clinic in Minnesota for a second opinion. At first we were looking forward to another expert’s advice, but it seems the Mayo clinic wanted to repeat the same tests that have been performed several times over the past 8 years, including this past year. What we really want is a review of the existing tests, explanations for the less common symptoms and suggestions on treatment options, so we’re still trying to decide next steps.
On a positive note – Julie Ann bounced back very quickly from the Bell’s Palsy paralysis in her face. Within two weeks I’d say she was 80% recovered and the paralysis was barely perceptible two weeks after that.
AnonymousApril 27, 2009 at 5:16 pm
I’m getting deja vue over and over again reading your posts. This is almost exactly what happened to me too – first diagnosed with CIDP then Myasthenia Gravis when it hit my face and one side drooped. All tests for MG were negative and I was eventually taken off Mestinon. It seems like different neuros want to call it different things – rather than Bells Palsy my neuro just said “cranial nerve involvement”. I’ve never heard of F-waves being present as diagnostic for not having autonomic involvement… I have had F-waves come and go but my neuro has never put them in conjunction with autonomic involvement which I definately have. I also started off on one side (my right) but CIDP eventually paralyzed the left side as well. Now I am not paralyzed, just numb and weak – the left side is doing pretty well but I still have more weakness in the right.
The eyes opening up can be a real pain for several reasons – the likely open up at night and she might not be aware of it – her eyes will be really dry in the morning if so and dry eyes can get scratched easier. After three years of sleeping with my eyes open I finally got my routine down to keep it from happening – I use Refresh Liquigel eye drops and then put an eye mask on to make sure they stay shut. The mask doesn’t always stay on at night but it does help some.
You are in the hardest part right now – figuring out what the whole picture looks like. Hang in there… be patient… unfortunately sometimes it just takes more time for things to show up and make a diagnosis definate. Try to focus on real life with her – Twilight is, I’m sure, much more fascinating to her than EMG tests.
AnonymousApril 28, 2009 at 5:20 am
I’m glad you are keeping us up to date with your daughter’s progress.
Yes, I am getting deja vue too – just like Julie! My facial weakness: eg: eye probs, chewing probs, weak voice, etc have been put down to ‘cranial nerve’ involvement just like Julie’s has.
My nerve conduction tests in my lower limbs over the past few years always have absent F waves. Though, I think the F waves in my arms are okay.
My Neuro did say I had a very unusual presentation of CIDP – actually the technical name for my form is sensory ganglionopathy with autonomic and cranial nerve involvement -that’s why it took so long to diagnose me. He never gave up though and eventually worked it out!
Just remember you guys are not alone – there are others who know what you are going through (’cause we are going through it too).
April 29, 2009 at 12:25 am
I was diagnosed with CIDP in 2001 and was just diagnosed with MG 2 months ago. My Neuro also said she had not seen this before either. Lucky us, huh? This really does explain a lot. I was hospitalized last year with all the symptoms of MG in full bloom but they did not get it because they kept thinking it was CIDP. I got a new Neuro and she detected double vision, swallowing, etc. and did some blood work that came back with MG. She then put me on Mestinon and I have felt better than I have in years.
It is always nice know that you’re not alone and that is why I have always been appreciative of all the people who post here with the problems and solutions that you share here.
AnonymousApril 29, 2009 at 4:45 am
I should be clear – I do NOT have Myasthenia Gravis – according blood tests and the repetetive stimulation and single fiber tests. Mestinon worked for me briefly and then started having the opposite effect which they also said pointed to me not having MG. I suspect Mestinon worked briefly because nothing was working at all and having what little neurotransmitters I was producing stay around longer brought me out of total paralysis.
NOW, however, I find that I am extremely sensitive to bug spray (not DEET, Raid stuff) to the point that it makes my heart race, my muscles spasm and I pass out because I can’t breathe. I was told when taking Mestinon that the compound is similar to bug spray and that I needed to be careful about it. I did a little research at the time which I confess I’ve forgotten now but enough to understand that they work similarly. I kind of forgot about it until last weekend when my husband sprayed the downstairs bathroom for ants. A short time later I was passed out on the floor unable to breathe or walk outside to get fresh air. I was never like that until I got CIDP.
My neuro says the MG like symptoms are “a version” of CIDP that is rare but apparently not that rare as several of us on here have been that route. Of course it’s possible that your daughter has both, there is so little research into this disease and it’s variants practicing doctors frequently work off of decades old information, which is not a slam on them at all, just an acknowledgement that we do not have a sexy disease worth funding research. Although now that it’s public that William “The Fridge” Perry has CIDP perhaps it will be a bit more attractive and we can get to the bottom of our variants and so on.
AnonymousApril 30, 2009 at 9:15 am
I so appreciate everybody’s feedback and information. It is a rough journey for all of us so I’m grateful to have your insights and support.
Julie – how long have you dealt with CIDP and how has the cranial nerve involvement progressed? We didn’t have the weak chewing or double vision for most of the first 6 years that Julie Ann has been dealing with CIDP, so we’re wondering now if this is something that we should anticipate getting worse over time. In fact, that’s part of the reason we’re trying to decide where to go to consult with a CIDP specialist about additional treatment options.
Kazza – I’m also be interested in progression of symptoms with your cranial nerve involvement. Did that happen right away, over time? How has it responded to treatment?
With her Bell’s Palsy under control, Julie Ann isn’t having to wear the eye mask at night, but it’s helpful to know that we’ll still need to watch out for symptoms of her eyes not staying closed at night with her high potential for cranial nerve involvement. Thanks for that advice Julie.
Julie Ann also improved temporarily on the Mestinon (before the re-diagnosis) but we didn’t get to the point where it made things worse. It’s good to know about the potential for bug spray interacting with her the same way as Mestinon (though my wife hasn’t allowed bug spray in our house since JA was diagnosed as a precaution). It means however that we’ll need to ask the school for notification before they fumigate or spray for bugs.
April 30, 2009 at 12:17 pm
I should start out w/a brief hist. on Kev. GBS, CIDP,GBS, now cidp. We did loads (2g/kg) for 8 months straight after the last cidp dx. He was back to normal. In the begining, he had autonomic involvement, constipation, inability to empty his bladder, difficulty breathing, intense headaches, inability to sleep and of course the typical weakness. Once we were on the 8 month load, things improved dramatically. This past June, we started tapering down w/ the ivig to reach a 1g/kg dose. By Oct. he was tired/sluggish but still passed the subjective neuro assesments. From Oct-to 2nd week of April, we had several virus’s go through our schools and several of the kids including Kevin got them. Personally, I think we had the swine flu the week of April 12th. Anyway, the illness’e seemed to be using up the ivig faster and we were back to constipation bouts, difficulty breathing, not able to ride bike again, shoot hoops etc. We went to the neuro 2 weeks ago and I begged for some loads again to boost the antibody production again and to keep the autoantibodies under control. He has noticed that his pain level has decreased and he does not feel as tight and he has not complained about the breathing. Yesterday he was playing football and on the trampoline again. He is not back to scratch but there is improvement. We too have the awful aseptic meningitis reactions sometimes and ALWAYS have the nausea, migranes, bodyaches. I thought we found a solution by switching to Octagam, as last month when it was not a load, things were not too bad. This month now that it is a load, it was like it was the first time in the hospital, he said he would rather be paralysed again, that it is not worth it for how he felt then. 2 days past and yesterday he was playing with his friends happy again. I am hoping it is just going to take a little time to just get reacclimated to that amount again.
I HONESTLY think your daughter needs to get loading doses for a while. A maint. dose of 1g/kg is not going to cut it especially if you are spreading it to 4 weeks sometimes and not being consistent. The dose she is getting is allowing a progression to happen, just maybe at a slower rate had she not been getting any ivig. She may even need loads eveyr 2-3 weeks for a while. A friend of mines daughter just returned from Mayo w/the same situation as you, after all the testing, they agreed she needed loads for a year or so. I had been begging her to talk her doc into that for the last few months. I had to beg our doc too and my reasoning was much like yours, I did not want to go to Mayo to repeat tests and cut into life time benefit. I asked if we could use the ivig as a tool to gauge if there was improvement. He agreed as long as I promised to get another ncv/emg and a pt assesment. I told him I agreed with that and we hugged on it. I am at a standstill now though because of this swine flu thing. Not that I think getting the flu is a big deal AND I think we had it anyway, its just that it is in our county and at the hospital down the way from our hospital, so I just don’t want to take the chance and get sick again while we are finally feeling better. I hope when I fax him a letter stating that and promising to go when things blow over he will understand!!
Bottom line, I think you have to beg for ivig, her body is telling you it needs more, you know it is not MG, so it has to be the lack of ivig. Good luck and I hope I was not too forward, it just kills me seeing Kevin in a bad way and I hate seeing any kids suffer.
Dawn Kevies mom
AnonymousApril 30, 2009 at 5:37 pm
[QUOTE=steveh]Julie – how long have you dealt with CIDP and how has the cranial nerve involvement progressed? We didn’t have the weak chewing or double vision for most of the first 6 years that Julie Ann has been dealing with CIDP, so we’re wondering now if this is something that we should anticipate getting worse over time. In fact, that’s part of the reason we’re trying to decide where to go to consult with a CIDP specialist about additional treatment options. [/QUOTE]
I have had CIDP since 2005 but did not get cranial nerve involvement until August 2006. From there it progressed very rapidly – a matter of hours to a day – from one side of my face to the other until my entire face was paralyzed. Within six months I got most of the feeling back and could smile without looking haunted. However I still have quite a lot of damage in my cranial nerves leading to difficulties with my eyes and chewing. My swallowing has improved and I hardly choke anymore but I cannot chew food for the life of me. You don’t ever want to eat dinner with me – it takes forever and I end up spitting out the things I just can’t chew – gross!!! 😮
My face looks different too though if you hadn’t known me before you would just say I had cheekbones (as per my nurse) but I have lost all those muscles between your cheekbones and jaw. When I have a CIDP flare my face is the first thing that goes numb and droops. I get double vision pretty easily when trying to read small print and sometimes the computer too. It comes and goes like all my symptoms do now but I can say for certain it has not gotten worse.
From what I understand cranial and autonomic nerves heal differently and at a slower rate than peripheral nerves. In addition they are more responsive to IgA which is only found in small amounts in IVIg. My neuro tells me to be patient that they are watching to see that it doesn’t get worse but can’t really do much to make it heal faster. 🙁
I’m glad Julie Ann is doing pretty well and some of the things are getting better. I hope she continues to improve. I don’t have any advice for you on whether to pursue further specialization with a neuro – I am going to Hopkins but ended up there after my local neuro was baffled.
Take care and good luck – Julie
AnonymousMay 1, 2009 at 4:32 pm
I forgot to mention this to you. There is one DEFINITE test to show dysautonomia, (to show that the CIDP is hitting the autonomic system).
It is a Skin Biopsy. A very simple test done in the doctor’s office.
If I remember right, they numbed the area on my thigh, then just stuck in and out, what looked like a thick needle in one area. Simple. It just left a little hole, that was covered with a bandaid.
If the result shows: “small fiber atrophy”, then you know your CIDP is hitting your autonomic system. Google “autonomic system”, and you will see what areas of the body can become affected.
As my CIDP progresses, of which treatments have been unable to stop the progression of it–just slow it down some, my diagnoseses keep getting longer as it hits the different areas of the autonomic system. Mind you, I was healthy as can be prior to all of this junk! I always ate healthy, when flu came around, it always seemed to skip me, and never saw a doctor for years.
Now, because I have a team of 7 doctors, I seem to have a doctor’s appointment or treatment, (chemo at the hospital), every couple of weeks or so. My current diagnosises are: C.I.D.P., (Chronic Inflammatory Demylenating Polyneuropathy), with Lewis-Sumner variant; with small fiber atrophy, (autonomic neuropathy); Dysautonomia with dysphagia and gastroparesis and esophageal dysmotility, tachycardia, syncope, neurogenic bladder, and phrenic nerve dysfunction with hypoxemia; and with Horner’s Syndrome with photophobia; Benign autoimmune thyroid disease; Anemia; Hypothyroidism; Left subclavian vein Mediport, (single lumen); Shingles reported 10JUL08.
(I have to keep a current sheet with current diagnosises and current meds handy, for when an ambulance is called, every now and then).
It all started with eye involvement, with my Primary Care Doctor thinking I had “Horner’s Syndrome”, (which you can google), of which I went to him complaining of light hurting my eyes, and causing me bad headaches/migraines, (photophobia). He then sent me to a neuro, to get to the root of what was causing it, and after two years of further symptoms happening, it was explained as CIDP.
By the way, my Neuro-Ophthalmologist put these new plugs into my eye ducts. He said he saw my eyes were dry, and that the plugs were to help stop dry eyes which lead to migraines. Between that, and a pill called “Indocin” that I take 2 times daily, I have not had a migraine for years now.
So, I hope this helps– a good suggestion to ask for that test to see if the CIDP has hit your daughter’s autonomic system for sure.
May 1, 2009 at 5:04 pm
There is a Dr. Sladky at Emory in Georgia. However, if you are going to Mayo, they work with your doc, basically, they give your doc instructions and recomendations. So if you like the demeanor of your doc you might be able to stay with him/her.
Dawn Kevies mom
AnonymousMay 2, 2009 at 6:02 pm
My cranial nerve problems developed (along with the other CIDP stuff) during my first ‘biggee’ attack. I did have smaller attacks for several months preceeding this – with weakness, band pain (tight squeezing sensation) around abdomen and blurred/double vision. But these attacks didn’t impact on my face as much as the later attack. As it took years to get a diagnosis – I didn’t have treatment for my CIDP – so I had to get better on my own (which wasn’t complete) – can you believe that!
The old symptoms (not an attack though) come back if I overdo things, so I have to have frequent rests in between chores, etc.
AnonymousMay 3, 2009 at 3:01 pm
Our goal is to stay with our neuro here, but find someone who sees more children and who can help us with the meds. Up until this point we have been doing the research and letting our neuro know what we want to do.
I will check out the Dr. in GA. We did see a neuro in Houston 8 1/2 years ago but we didn’t agree with his approach. His idea was to use sterroids and we couldn’t justify that for a 5 yr old child.
Has anyone heard of Vivaglobin? It has been used in Europe since 1994 for people with PI. It is administered subcutaneously over 2 or so hours. It is supposed to has less side effects than IVIG. We are looking into this for Julie Ann as she is getting tired of going to the hospital every weekend and would like to have more time to spend with her friends.
Thank you for the info on the autonomic system. I will also look into this and the test.
AnonymousMay 3, 2009 at 4:28 pm
My nurse has successfully transitioned two of his IVIg patients to Vivaglobin and they no longer have any side effects at all. I am considering asking my neuro about this next time I go as I still get meningitis headaches and other side effects. My nurse says it’s easy to do once they teach you how and you can schedule it at your convenience once a week or whatever.
May 4, 2009 at 9:31 am
My son suffers greatly from the reactions, but the amount of ivig needed for a cidp patient vs a pi patient is enormous. It would not be practical to give that much sub q. My biggest concern would be how much is wasted by being absorbed by the body as opposed to going directly into the blood. Try switching brands, sometimes that helps. I am going to private message you something.
Dawn Kevies mom
AnonymousMay 4, 2009 at 2:37 pm
[QUOTE=Dawn Kevies mom] My son suffers greatly from the reactions, but the amount of ivig needed for a cidp patient vs a pi patient is enormous. It would not be practical to give that much sub q. [/QUOTE]
Dawn I had the same concern but Mike (my nurse) says it’s more concentrated so you use less. I went to the website and read some stuff on it and it seems a possibility – our only concern is whether I’ve got the dexterity in my fingers to be able to stick myself. As for the absorption – granted it takes more time but on the other hand I think that’s part of the problem – too much in too short a time and my body freaks out… it needs more time to adjust so maybe the dermal route would work better for me. It looks kinda weird – check out the website and see the little tubes and so on… I think I’d feel like I have leeches on me but if I can get rid of the headaches I will make it work. 🙂
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