12 year old son with atypical GBS/CIDP

    • May 26, 2013 at 2:55 pm

      Hi- I am writing from my 12 year old son’s hospital room at Seattle Children’s Hospital. After many weeks (7 weeks until diagnosis) he has been diagnosed with GBS that has been changed to “sub acute”. Because his timeline and progression of symptoms are not typical of GBS the word Chronic has been thrown into the pot also, although no one seems ready to make that leap yet. We have had 2 doses of IVIG and after progressing weakness we are now about to complete our 5th time of a 5 treatment plan of Plasmapheresis. The only change he has noticed is a “jumping of his nerves” like if a door closes or a loud noise happens he is not startled but his nerves are. We are hoping this may be a good sign?? The Dr’s don’t seem to know or won’t say.
      Chad’s symptoms started in Mid March with pain in the back of his thighs and calves which led to him not being able to bend over or extend his legs out when sitting. The pain didn’t stop him he just couldn’t do it. A couple of weeks in the pain and lack of extension went into his biceps and he couldn’t reach over his head. He was admitted here for an MRI and testing around week 3 and they said “Not neurological maybe viral go back to Physical therapy” Then 10 days after that he had a follow up at our PCP’s insistence (He had told them he suspected GBS) where once again they said not neurological…how is school and your friendships?? We all know what that means…
      Finally around week seven he was unable to climb stairs, rise from the floor or up onto his toes, jogging was impossible and walking was getting more difficult. I had finally gotten him into Rheumatology with a weak ANA result, since I felt it might be Juvenile Polymyositis or Dermatomyositis, but prior to our 3 o’clock appointment our PCP once again INSISTED neurology take another look. This time they admitted him for a NCS which showed to everyones surprise slowing nerves. He had his 2 dose treatment of IVIG and was admitted to Rehab. During his week there I never felt he was getting better only worse and although I told them he wasn’t supporting himself at all they felt with their muscle testing that his weakness had stabilized and we could go home. We did…for 3 days and after I went back to my PCP and begged for an MRI with contrast of his legs to see if there was possibly an overlapping muscle condition, we were readmitted and the plan changed to Plasmapheresis…so here we sit. They say definitely no muscle involvement but they seemed so focused on the pain as the reason he cannot stand or walk. He can’t roll over unassisted, touch the top of his head or even push a button on his TV remote anymore without Herculean effort. He says it is not the pain his body is so weak he can’t do it.
      Does this story sound familiar to any other parent with this syndrome? I am coming to terms with the fact that his case and timeline lend themselves more to CIDP but since the Dr’s won’t say we have no idea what to expect…should he at least see some recovery before he relapses? How long can it take to see results after plasmapheresis? I know the nerves have to regenerate but should we see ANY evidence that this process has at least stopped (or gone into a temporary remission)?
      Sorry this is so long but we are desperate for answers, help or advice…Any ideas??
      Thank you!

    • GH
      May 26, 2013 at 10:10 pm

      It isn’t unusual to take some time to arrive at a diagnosis, particularly when the presentation is atypical. CIDP is an unusual disease, and even more so in children. Even if the diagnosos of CIDP is correct, there is so much individual variation that you cannot know this early how it will play out.

      When you write “they,” who do you mean? Your son should have a single neurologist overseeing treatment for his neuropathy, not the hospitalist team. And preferably one with experience with CIDP. Pain is not the reason a person with a peripheral neuropathy can’t move, as you know. If “they” are unclear about this, “they” must not be neurologists.

      I have CIDP, but I am an adult. There are others who have contributed here in the past, and who have children with CIDP, so hopefully one or more will see this.

    • GH
      May 26, 2013 at 10:22 pm

      It is not unusual to change treatment from IvIg to plasma exchange. PE is a good treatment when IvIg is ineffective. I was switched to PE when I continued to decline after IvIg. I had nine PE treatments. Five is more or less conventional, but in my view more is better. I didn’t start to recover until about my sixth or seventh treatment.

      The pattern of relapse cannot be predicted. I have had no relapse after getting through a very difficult period. Others have milder cases, but may struggle with relapse. It depends on the individual case and the particular treatment. Your neurologist must try to find the treatment plan that works best. It is not cut-and-dried.

    • May 30, 2013 at 5:33 pm

      Hi, we had similar symptoms and our first dx was gbs and then several months later cidp after a relapse. I was wondering if you could explain the “two ivig doses” Do you mean 2 days of treatments or 2 loading doses which consist of (typically) 4-5 days in a row of ivig, wait 4-6 weeks and then you did it again? If it was just 2 days and that is all, that could explain why you did not see a difference, that would not have been a full loading dose. Regarding the pp, many times when people are not responding to ivig (if you did the 4-5 days for 2 months consecutively with no difference, it would be fair to say there was little to no response to ivig) they will do pp and immediately follow up with ivig and then go for 2-6 weeks with no other treatment. Unfortunately the variance in how people respond to treatment and the frequency of relapse varies greatly. No to people are really the same. It just takes a while to figure out the plan that works best for you.

      We DID see an immediate difference after our 5 day load. On the 4Th day of the 5 day load he was able to run down the hospital halls and do push ups and a day early he could not walk, use the bath room, difficulty breathing, could not even lift the remote. It has been six years and we are going to try to wean again after summer. There is hope, things do get better. We could talk if you like. Be persistent and don’t give up. We too were thrown into the “psychological” category as well as the muscular dystrophy (CMT) category finally I said what about GBS? After the third doc we finally received the care we needed. Leave an email if you like and maybe we could talk

    • May 30, 2013 at 9:44 pm

      Thanks for the replies! I am glad I finally have a moment to respond because I do have many more concerns and questions. First of all to answer you GH, Seattle Children’s is a teaching hospital so while he has a neuromuscular Dr. that diagnosed him and is “generally” overseeing the treatment, he has had several attending neurologists, several “Fellows” and a couple of “residents”. Along with a Pain mangaement team, a Rehab team, a phsyciatric team, and a surgical team since he is on their floor for the Plasmapheresis. I do feel that this causes confusion and possible lack of understanding about his functional weakness since each person that does the basic MMT muscle testing is using their own interpretations. Plus things get said on rounds that I am not sure where it is has come from. Our last attending (who happens to be the one who told us at our first admission and again at followup that this wasn’t neurological) said to me after I confronted him about not “seeing” the true functional weakness. “Well he was able to stand this morning in front of his commode. That is good” I looked at him baffled and said “No I told you he can’t stand” he bumbled and said “Well I know that was said at rounds this morning..I know he can’t rise but once he was up he stood for a moment” UH NO! he can barely roll to his side to use a hand held urinal I have to bring his legs over for him…it is this that frustrates me because I don’t know what is in his chart or how they will know that this syndrome has stopped and is on the recovery side if they don’t even know what his baseline is to judge decreases.

      @ Dawn – it is so good to hear from a parent who has experienced something similar. Chad’s IVIG was started the day after his diagnosis. I believe the neuromuscular Dr. said that while they could go for a 5 day treatment plan they are able to treat with a 2 day course it is just basically done at a faster flow rate once they determine by starting slow that there will be no adverse reaction but apparently is the same amount of immunoglobulin. I will have to research this more. After the two weeks with progressive worsening of his symptoms (weakness) and continued pain they readmitted him and decided to do the plasmapheresis for 5 every other day treatments immediately followed by 2 IVIG treatments again. Now it has been 3 fulls day past that and we are still waiting to see visible improvement. It is hard because he has such bad muscle contractures in his knee area and somewhat in his arms that the pain is pretty intense especially when he is moved or his legs are unsupported and try to fall straight. He still can not move his legs out or in but can rotate side to side a bit as long as they are pulled up near his hips. Your son had also gotten to the point of being unable to walk? How long were his symptoms evident before he received a diagnosis? The complete lack of any resolving of his symptoms is what has me worried. They call him on the severe end and yet he has no difficulty breathing or respiratory involvement (Thankfully) Now I am hearing from PT that all of his recent areas of increasing weakness is strictly from deconditioning not syndrome progression even though he was walking and even attending school (with difficulty) up until his admission on 4/29. Also the ability to pull his legs towards his body while laying in bed was just lost several days ago. So I would find it amazing that he has lost all of his muscle tone and had so much atrophy from really just 2 weeks..I guess it can happen but I would have thought he would at least be able to push the keys on a keyboard as a sign of recovery. It is all just so confusing. I am looking to possibly get a second opinion but there are no other pediatric neurologists in the state of Washington. Do you know of someone who sees children that is well versed in GBS/CIDP and has successfully treated at least several children? I have seen the list of Centers of Excellence but would like to know which ones have treated maybe difficult cases. I would also love to communicate more if you would like to contact me my email address is nunamaker6@gmail.com.
      Thanks again!

    • GH
      May 30, 2013 at 10:55 pm

      Mom.., it is true that a loading dose is sometimes administered in two days, although a five-day regimen seems to be the usual.

      Seattle Children’s should be a good place, but the doctor you described above sounds like a dim bulb to me. A doctor should keep an open mind about the diagnosis until it’s perfectly clear, because there is variability in the various tests they use to diagnose neurological disorders. The remark about standing was particularly stupid. I could “stand” for a short time a week before I was hospitalized, provided I could get upright using my arms, but if my knees buckled I went down like a rock.

      I know that there are lots of doctors and therapists coming around when one is hospitalizec, especially in a teaching hospital, but the notion of several “attending” physicians doesn’t make sense. One neurologist who understands peripheral neuropathies should be the attending physician coordinating treatment of your son’s neuropathy. The rest are hospitalists, or consulting specialists, or something.

    • Anonymous
      June 27, 2013 at 8:49 pm

      I would try KU Medical Center on the Centers for Excellence. Dr. Barohn, or Dr. Dimachkie I think would be very good Doctors to contact.