Treatment is over! Some confusion

    • November 8, 2007 at 9:28 pm

      sorry no info

    • Anonymous
      November 9, 2007 at 7:47 am

      I don’t know the answer to that Dawn but I suspect I have the same thing. It seems apparent that I have some kind of constant inflammation based on the constant fever but the demylienation seems to have remylienated for the most part. I see the doc next Friday and will ask… if I get an answer I can understand I’ll post it.

    • Anonymous
      November 9, 2007 at 10:35 pm

      I think that is why it is chronic and relapsing – remitting, because of the inflammation. The nerve that is demylinating is not getting the message to the muscle. But the demylination doesn’t always get to the axon which is why the nerve can repair when the inflammation is arrested. That is my understanding. That is also why you need to keep having a cycle of IVIG, to address the remitting inflammation. Does that sound right? Correct me if I am wrong.

    • November 9, 2007 at 10:53 pm

      Kevins nerves are not being affected. For that matter, I don’t even think the myelin is demylienating. If he is able to gain full stregnth in 48 hours of treatment, there is obviously no demylienation. I think the inflamation on top of the myelin is what his and Emily’s problem is.

      Even at his lowest point of weakness last year, when I had to carry him and dress him, with in 48 hours of ivig he was normal again.

      Kind of like an excema flare up, or an arthritis flare up, administer medicine and the flare up subsides. This disease is so confusing!!


    • Anonymous
      November 9, 2007 at 11:24 pm

      I guess I don’t understand. Have Kevies nerves ever been affected? If not, what is the cause of his weakness? Can you have CIDP and not have demylination? I know what you mean about this condition being confusing and now I am even more confused but curious to be educated by your knowledge.

    • November 10, 2007 at 9:10 am

      The ncv/emc shows slowed velocities and conduction blocks in his hand. My question to the doc is going to be, is the block due to the inflamation ON TOP OF THE MYELIN? Since any literature I have read states that nerves regenerate at the rate of a mm a day, I have to assume (you know what they say about the acronym of that word) that there is no damage. Kevin is getting near full recovery in hours, both in his legs and hands. We did not think his legs were that bad this time since he still could run, but after the second day of treatment, he was amazed at how much faster he could run and get up the stairs. Not to mention he had just had the surgery to put the port in 4 days earlier. With these clinical facts I have to assume that there is no nerve damage. If the first line of attack is a flare up, this to me would be the inflamation part of the process. Kevin seems to be able to go 41/2 months before we noticed any physical evidence of weakening. We noticed this the third week of September, we thought it was gbs residuals. Toward the middle of Oct we noticed he was not any better, but not much worse, which brigs us to the fourth day of Nov. when treatment started again. That is a full six months from the last infusion.

      The part I do not understand is IF there is demylienation of the myelin, then an attack on the nerves, how come Kevin’s never demylienates to the point of reaching the nerves after six months? Either we are lucky and he just has a slow attack, or he just has some variant that causes only an inflamation on top of the myelin. This will certainly be a question I have at the symposium next year. The reason I feel I MUST know is because I feel it is imperative for us to space ivig as far apart as we can, maybe even every three months or two months. It is such a huge chunk of his life at once a month four days a week for 6 1/2 hours.

      All of us can live for the hope that in the next few years there will be alternative safe treatments other than steroids or the legnthy and sometimes painful process of ivig. It seems like every day we jomp a new hurdle in the acceptance of Kevin’s disease and the realization that this may be around for a long time. But we still can hope, for everyone!

      Have a great day!
      Dawn Kevies mom

    • Anonymous
      November 10, 2007 at 11:25 am

      [QUOTE=Dawn Kevies mom]
      I am confused about something!!! I know the basics and what ivig does, I know about 1mm/day nerver regeneration, I know about myelin re growth. What I don’t know and cannot explAIn is WHY, IN THREE DAYS IS TOTAL STREGNTH RETURNED? There must be some variant of cidp that only involves inflamation of the myelin and not a demylienation . There is no way that a demylienation could repair and a full recovery happens in 48 hours. benefit. [/QUOTE]
      Dawn, I’ve been wondering for some time about the exact same thing. In the past several people on this forum said they saw almost immediate improvement after they received IVIG. As you mentioned myelin needs time to regrow. I don’t see how myelin inflammation alone can cause the symptoms we are experiencing. Inflammation is just one step in an immune reaction which leads in our case to the destruction of the myelin. I wonder if there could be an inflammation of just the nerves instead of the myelin interfering with nerve impulses.

      The whole thing is quite a puzzle to me. I’m going to ask my neurologist on Monday about this.

    • Anonymous
      November 10, 2007 at 12:29 pm

      These are such good and important questions. I too have been wondering about the part systemic inflammatory process plays in the weakness/fatigue to restoration factor. Please let us know what your doctors say.
      Thanks Norb and Dawn.

    • Anonymous
      November 10, 2007 at 2:47 pm

      Last night I was going through Emily’s medical folder. I found report written about her MRI last November from the MDA dr. The report say that Emily “does not show characteristics of typical CIDP… She may very well be a motor variant of CIDP”.

      It also states “MRI of thoracolumbar spine revealed enhancement of the nerve and cauda equina”.

      Maybe that plays a part in this variant…I don’t know.

      It’ll be interesting to find out though…

      I’m off to Google.


    • Anonymous
      November 10, 2007 at 3:23 pm

      I found some info…

      I went to Google & typed in “MRI of the thoracolumbar spine revealed enhancement of the nerve and cauda equina”. I clicked on one of the links that talked about CIDP.

      Apparently an MRI was done a 3 year old girl with CIDP. Her symptoms & test results were almost exactly like Emily’s.

      Here is the link:

      I find it interesting that she was given Prednisolone for a few months & then the MRI was normal again. I’ve been wondering if an MRI would be normal on Emily by now but I don’t really want to put her through that again anytime soon. I’m also wondering if the few months of Prednisone earlier this really may have been worth it.

      Dawn – If it comes up you should have Kevin get an MRI so we can compare. As if it’s just that easy…


    • Anonymous
      November 10, 2007 at 6:15 pm


      You know that immunology book you have? I bet the section on inflammation is similar in length to the section in the one I have: 21 pages. And my book assumes that I have understood the preceding 330 pages, which I don’t yet. My guess is that the inflammation caused by the demyelination affects the other tissues in the nerve, leading to the clinical presentation.

      When I first started thinking about this problem, I thought of the nerve as a simple cell, with a central body, an axon, and dendrons, wrapped with a myelin sheath. It is actually more complicated than that. First, what I have described as the nerve is actually a nerve fiber. In what doctors call a nerve, there are lots of nerve fibers, most of which are wrapped in myelin (but not all are). Many nerve fibers are bunched into a nerve bundle, which is embedded in endoneurium. Several nerve bundles are then contained in epineurium. Finally, the epineurium is contained within a perineurium sheath, which also contains lymph. See [I]Grey’s Anatomy[/I], 15th ed., page 47, for a good drawing. My guess is that the demyelination not only inflames the axon (without necessarily causing permanent damage) and the Schwann cell, but also the endoneurium and epineurium.

      I think it is like MS patients and heat. I have a friend with MS who can walk, albeit with a cane, for about a quarter-mile without undue exhaustion. On a very hot day, though, walking 100 feet completely exhausts her. Heat slows down nerve conduction in all of us. In her case, though, it is like tipping a scale. The normal slowing caused by heat is just enough to dramatically increase her clinical weakness. Another way to think about it is the pressure paresthesia we all have experienced when we have sat cross-legged too long. This process is, I think, due to an inflammatory response due to a loss of blood supply. Restore the blood, the inflammation goes away, and the paresthesia goes away.

      I will also try to discuss this topic with my neurologist on Friday. We have discussed this topic before, but he admits that he doesn’t really know. This time, we will try to spend some time thinking about it. The inflammatory process is complicated, the anatomy of the nerves is complicated, and the interaction of all the components is almost impossibly complicated. The scientists will figure it out one day, but I bet the inflammation section goes from 21 pages to a book all to itself.


      I am sure that if the NCV tests show demyelination, then Kevin has demyelination. I think though, like I said to Norbert, is that the inflammation caused by the demyelination tips the balance towards weakness.

      Don’t forget that in a normal person, myelin is being replaced continually, just like your hair and nails grow continuously. So in someone with CIDP, there is a continual process of myelination/demyelination. If IVIg is able to suppress the antibody against the myelin, then the myelination should win out. At the end of IVIg’s effectiveness, the antibody production probably ramps up and the demyelination would win out.

      I don’t understand why Kevin is getting the protocol he is getting, and I have forgotten what all you have tried. A common protocol seems to be a loading dose like what he is doing now, followed by one or two days every two weeks for a couple of months, then one or two days every month for a few months, followed by one day every 4 or longer weeks, depending on symptoms. That should be less time overall out of his life, and lower costs to your family, within a few months.

      I do hope things work out well for Kevin. Why on earth could they not access his port, even with some swelling? Seems ridiculous to me.


      We know that what we call CIDP has primarily motor effects in some people, primarily sensory effects in others, and the more usual mix in most. Are they really variants, or just the normal range of variation in response to the same disease? Who knows? As for the enhancement in the MRI, I think just says that there is an inflammation of the nerves near the spine. I think it is a typical process in people with CIDP. In Europe, they typically call CIDP “chronic inflammatory demyelinating poly(radiculo)neuropathy”, where “radiculo” means the nerve roots at the base of the spine. That is, the disease is likely to affect the nerves from the spine out. I would talk to the neurologist about this, but I don’t think the finding is unusual.

      I hope my speculations and guesses are helpful and make sense. Please bear in mind that they could be wildly off the mark, as I am not a medical doctor. If so, it would be good if some doctor would jump in here and correct me.


    • Anonymous
      November 10, 2007 at 7:27 pm

      In lots of reading, I think no one really understands these illnesses–there are just occassional beams of light. It also seems that a lot of the problem is that this is a syndrome meaning a collection of symptoms in different people that appear sometimes to be similar but may have a very different cause. It is hard to separate out the parts to understand the cause when it is as complex as this as Mark so elegantly said. Maybe one relevant feature to why Kevie improved so much is that there seems to be a component that is antibody mediated (in which antibodies made by the person in “error” cause problems) and a compenent of the damage that is due to physical damage to the insulation (myelin) of the nerves. The rapid and severe downturn of GBS is because of the sudden appearance of antibodies. The residuals seems to be more likely from the damage that was done to the nerves and happens long after the abnormal antibodies seem to have settled down. CIDP seems characterized by progressive damage and that much of this damage is of the more permanent physical type, I am guessing from how it shows. To make it even harder, the two processes are likely not separate, but linked. I wish we understood these illnesses and how to better help different people. What I do think is most important for Kevie is that the IV IgG did work so well for him. This means that a lot of the damage is still due to antibodies and that this can be helped. No one knows exactly how IgG infusions work, but one thing they do is flood the body with immunoglobulin. If there is so much immunoglobulin to a lot of things, there is less specific binding. This is a fast process to change because antibodies bind and release and bind and release. [B][/B] If there is less specific binding, there is less damage accumulated. The other way to look at his fast response is not that he does not need the immunoglobulin, but that he does. It works for him; it makes a difference and a dramatic one. This may mean/makes sense that reducing the amount of production of the abnormal immunoglobulin he makes (by shutting down his total immunoglobulin production by flooding the system with given immunoglobulin) could really help him because his body is really sensitive to this abnormal immunoglobulin. Let me know if this does not make sense and I will try again.

      I also know that illnesses in children as often not the same as in adults. This is a special challenge for very rare illnesses like GBS or CIDP. I also know that children are especially amazing and that they go and go and go until they cannot go anymore. Adults tend to gripe a lot and quit earlier and tolerate much less. Very often I see a child suddenly get worse and rapidly get better because they are so strong. It is a straw that breaks their camels back and even removing a small part of the problem (a straw) allows them to courageously march on in life.

      Even though I would not want to be a child again in this age, I wish I had a child’s body!!
      With Hope for cure of these illnesses (and understanding).

    • November 10, 2007 at 9:21 pm

      Thanks With Hope and Mark Ens!

      I agree, the ivig works for Kevin! Now we just have to figure out a protocol. Two weeks ago the plan was to wait 30 days and do another loading dose. The doc wanted to do this a couple of times to get things in check so to speak. Then we were going to discuss maintenance. It sounded like he was thinking every 6 weeks, the full 42 day cyle of ivig.

      I was wondering, how do you count the days? Is the first day of infussion the first day in the 30 day cycle, or do you start day one upon completion(the day after his fourth day of treatment) When you add four days up over the course of the year thats 48 days, one extra treatment.

      Thanks for the thought out, long replies. Knowing that you took the time to compose that well thought out explanation really makes me see the sincerity of concearn. It is also good to know that I am not alone in my thoughts, concearns and confusion.

      Dawn Kevies mom

    • Anonymous
      November 11, 2007 at 8:49 am

      Mark –

      I noted the MRI results because Emily & Kevin have a very quick response to IVIG. I’m just wondering if they have the same variant of CIDP or if they just respond quickly because they are children or because they have antibodies that aren’t causing as much damage to the nerves as they do in adults. OR is something completely different.

      I had stopped thinking about it researching it because, honestly, it made me crazy. Then Dawn had to go & call me & get me thinking again (THANKS DAWN, LOL!).

      I think it’s great that we can have this kind of dialogue going on this forum. I think it helps us all to try to gain some sort of understanding of this disease.


    • Anonymous
      November 13, 2007 at 12:35 pm

      Just a quick post. Our granddaughter (2 1/2) is visiting for a few days and she’s complaining that I won’t share the microphone with her.

      I did ask the neurologist yesterday. She said they don’t really understand how IVIG works and why some of her patients see improvements very quickly and others don’t. I was hoping she would have a better answer for us.

      Mark: I read your post but I need some quiet time to think about it which is probably not going to happen until after Thanksgiving. We will be leaving in two days for a family reunion in Battle Creek, Michigan.

    • Anonymous
      November 14, 2007 at 1:16 pm

      [QUOTE=MarkEns]Norbert,You know that immunology book you have? I bet the section on inflammation is similar in length to the section in the one I have: 21 pages. [/QUOTE]

      I have a few quiet minutes while my wife and our granddaughter are out shopping. My textbook only has two pages on inflammation, not very helpful considering the $80 or so I paid for it 10 years ago.

      [QUOTE]My guess is that the demyelination not only inflames the axon [/QUOTE]

      I wonder about the sequence: does the myelin get inflamed first followed by its destruction? After that does inflammation spread to the axon and beyond because of cell damage to the myelin? My guess is that the autoimmune reaction in CIDP is directed only against myelin and not the axon since the molecular surface structure of both would be different. Damage to the axon probably is secondary. Of course, we don’t know enough about the exact mechanism involved in CIDP.

    • November 14, 2007 at 7:47 pm

      That is exactly what I think, the inflamation on top is the start and maybe the end for some. Perhaps inflamation on top is one variant (Kevin Emily, others who see quick response tio ivig) Next comes another variant, the demylienation and then axonal damage. There is no other way to explain why some have axon damage and others don’t.

      Also, I was wondering, either you or Mark ens or someone mentioned that ALL people regenerate myelin all the time (those without cidp/gbs) , so why is it a big deal if those with cidp regenerate myelin. Does it grow back in a different form if it is attacked as opposed to just regenerating? Is the regeneration of myelin likened to constant cell regeneration in skin cells (in an otherwise healthy person?) Oh well, just some more thoughts.

    • Anonymous
      November 21, 2007 at 3:10 am

      Howdy ya’ll, sorry it’s been so long since I’ve posted… to make a long story short, Dawn, I did ask my Neuro about the possibility of “just inflammation” vs. “demyelination” and at first he said it’s not possible to have inflammation without demyelination and still call it GBS/CIDP but then in the next breath he said “it’s a controversial question”. Apparently there is still some debate about this but it hasn’t been thrown out completely. IMHO, I still think that’s what is happening to me – on Friday I had no reflexes, Saturday, Sunday and Monday I had IVIg, Tuesday I had NCV tests again and they showed definate improvement. That’s awfully quick to remyelinate… I’ll keep asking him about it as time goes by but I wouldn’t expect any startling revelations for the immediate future. :rolleyes: