Daughter with GBS later catogorised to CIDP and new lne of treatment
AnonymousJune 6, 2013 at 5:38 pm
Hello I am new to the forum and alike most of you we are going to through a juicer seeing our daughter so sick and no idea how or who to see to make thing better and this story behind us.
In late November 2012 our little girl complain that she was having double vision. I took her to an ophthalmologist who saw a light strabismus that we attributed to her being tired in school. 3rd grade , 8 years old and getting to school every day at 7:20 we thought that was our answer, this was on December 10th, took a long while to get the appointment. On December 12th she was falling, tripping and would find reason as to why she was falling, ones she said she trip on a horse cover and another that she missed a step. On the 13th she was riding and the horse trainer said that she had a hard time getting on her pony and that her ankle was showing a odd positioning. on the 14th the teacher told me that during an exercise fire drill she could not get up by herself and someone had to carry her outside, ad she added that she could not run during PE class.
I made an appointment with the Pediatrician ( Ex Pediatrician) and she said not to worry that all was fine and we could try to see a neuro after the new year…. Dec 16th we were in the emergency room as our little girl could not go upstairs and had zero strength. after the spinal tap, cat scan and later nerve conduction study we were told that she had GBS.
We were in the hospital for 6 days with a loading dose of IVIG and we went from not walking to running down the hall except from the terrible side effect of headaches and vomiting the strength was back.
Dec 21st we were released from the hospital ready for christmas with an excuse from PE class and some PT.
She went back to school and on the second day of school January 8th the double vision came back. Went to the neuro and he initiated another course of ivig 1g/kg on the 23rd of January ( tool very long thanks to the insurance to keep this in the pipes for so long) . Result perfect running after a few days apart from the side effects that ran for a few days.
day 18th after the IVIG re relapse and we were at that time seeing a specialist in Baltmore who told us right before the second relaspse ” she might just have a hick up of the GBS but not CIPD but I can be wrong”
Well sure enough he was wrong as on the way back she relapsed, double vision first and then progressive weaknesses, when I say progressive I mean rapid progressive as she goes from a broad/long jump of 28 inches to a zero in 10 days. Goes from going up the stairs fine to not at all in one week or less. IS this typical to CIPD or GBS?
Now we have made graph of her broad jumps and she improves very much with the IVIG and seems to go down hill at day 12th of the ivig. The specialist in Baltimore refused to give her IVIG more often and decided to add to the treatment this past Monday 600ml of pulse solumedrol… I have been telling her that this will most certainly make her feel better even if I was just against the whole thing and wanted her to have ivig more often.
Today we are at day 3 after the new treatment IVIG 1g/kg + 600ml ivMP and she feels worse than with the IVIG alone. She used to be able to go up stairs alone after three days and today it is very difficult. With IVig only she can jump 16 inches on day 3 and today only 6 inches and she almost falls when she lands.
I have so many questions and I am French so the medical system for me here is so confusing. In France you typically can talk to the doctor easily than here where you left zillion messages to zillions messengers.
After relapses for six months can we still talk about GBS or we definitively speak of CIP.
Why he refused to give ivig more often when we know she respond, yes she relapses but she responds very well to it…. can the ivig only prevent from going into remission ? and does it needs to be pushed by something else. I would not even go into detail on the methodology he used to forced us into the pulse therapy.
Can her not being as strong on day 3 as she was with the ivig only be just delaying the effect but on the long run it can make her be better longer…
Have heard or are you a case of GBS that last for months before going away…
Who should I see to have yet another opinion as I do not want my little girl to be on so much medications.
She never had any pain only weakness she had never complained of pain other than legs growing pain behind her knees but I guess many kids have this. And even now she does not even have this growing pain.
I also heard that children go into remission for most of them when dealing with CIDP but all I read is kids having meds for years is there literature on this or no everyone is different.
Thanks so so much for any answer that you may have as this tornado has been lasting for six months and we still have no answer.
June 6, 2013 at 11:22 pm
It is not unusual for a diagnosis of GBS to be changed to CIDP, because the initial presentation can be similar. As for CIDP, it is hard to say what is typical, because there is a lot of individual variation.
IvIg is a treatment but not a cure. If a patient responds to it, then it is best if the treatment schedule is adjusted to that which gives the best result. Unfortunately, IvIg is a limited and expensive resource, so there may be economic reasons why it is not administered as often as is best for a particular patient.
There is no one best treatment for all who have CIDP. The treatment must be individualized for best results. I have no experience with Solumedrol, so will leave it to others to comment. I did take Prednisone for a year, and continue to take an immune-suppressant drug.
June 11, 2013 at 1:03 am
Your daughter’s symptoms sound an awful like my daughter’s symptoms. Emily was 4 was she was dx’d with GBS & then later on, CIDP.
She too had double vision – it’s called diplopia. And her relapses ALWAYS started with eye issues. Her right eye, at one point, was turned in looking toward her nose.
Has your daughter had an MRI with contrast of her brain to check for inflammation? Emily had inflammation on her cranial nerves (also along her spine from the lower lumbar down).
It took MASSIVE amounts of IVIG to get Emily back on track. She was getting around 200 grams a month for awhile. It’s been 7 years since her diagnosis & she lives life like a “normal” kid. We just stopped her IVIG to see if she can go without it (fingers crossed!).
When Emily was 5, she was getting IVIG every 10 days. We saw a specialist who decided to lengthen the time between treatments & give her Prednisone. After 2 months of the ‘roids, she went from relapsing every 12 days to every 9. I took her to another dr & we tapered her off the ‘roids. We found that she would need IVIG every 7-10 days, when she was relapsing every 12. We slowly lengthened the time between treatments but she always received the same amount (20 grams).
You said something about taking your daughter to Baltimore? Have you contacted John Hopkins Hospital?
AnonymousJune 25, 2013 at 11:47 am
Thanks for your long response and explanation how things went with Emily. I hope that she is hanging in there with the discontinuation of the ig.
It took me that long to get back on as our Madeleine was not well at all. Yes we see a specialist in John Hopkins and he works with our local Neuro in between visits. We are in Atlanta.
The past two weeks were hell as the JH dr had decided to add Solumedrol pulse in IV to the ivig at the last treatment of ivIg. Terrible results we had a Madeleine who barely could go up the stairs for 3 weeks and had the life of a 90 years old so she was reduced in her mobility ( 3 weeks was the interval between treatments). The JH dr was not aboard at the time for having ivig more regularly and wanted to go his way with the steroid…He had to force me in as 1 I had a guts feeling that it was not right 2 I am terrified by steroids. But he told us that this was the best way to make the ig effect last longer as she starts to relapse at 11 days.
It was like if the Solumedrol had stopped the ivig from doing its job and kept the process or remyelination.
Botton line we landed in the ER last Wednesday and were in the hospital until Friday night.She had MRI and they reloaded Madeleine with 2g/kilo and the ivig will be given to her at smaller interval. They have also talked to us about subcutaneous IG and it is very new. NO more solumedrol which I had the sentiment made her so much worse. we are at day four after the last ig and she can run and go up unaided things that she has not been able to do in a month now.
I must be in total denial but what I read is that 90% of cidp respond to steroid and that steroid makes GBS patients worse off. So now I am wondering if we are not dealing with the later as this precipitated her.
June 26, 2013 at 3:17 am
Stephanie – I’m so sorry to hear Madeleine had to go through all of that. I know it is so very hard to watch your child go through all of that. I had to fight tooth & nail to get Emily’s IVIG treatments. I kept a calendar with all of her IVIG’s & all of her relapses. It took a few months, but when I took it to her neuro & he saw on paper what I had been telling him, there was no way he could deny that she needed IVIG more often. Are you keeping a log of her treatments & relapses? I highly recommend keeping a medical binder with all of her test results, IVIG info, etc in it.
I personally believe there is a variant of CIDP that does not respond well to steroids. I think I remember reading about it – it was so long ago though. Maybe you can use Google & find some articles. That is what I did when Emily got 1st got sick.
Emily is doing well so far. It has only been a few weeks since her last treatment. The longest she has gone without IVIG is 8 weeks – so at 8 weeks since her last treatment I know I will be a nervous wreck looking for any sign or symptom.
Have you researched other CIDP specialists? There is a dr out of Mayo in Minnesota that I really like. His name is Dr. Dyck. Emily has never seen him but I have done some research on him & I know of 2 other kids with CIDP who have seen him. As far as I know, both parents were pleased.
AnonymousJuly 26, 2013 at 3:07 pm
How is Emily doing? I was thinking about her and used a lot of your advise in Madeleine’s case. We have not yet been able to convince the Dr to give her the IVIG before she relapses and they seems to every time wait that she relapses before the treatment. This time again they waited 18 days, net Monday, and she relapsed at 12 and this drives me nuts.
I hope you are enjoying the summer.
July 29, 2013 at 6:39 pm
Hi Stephanie, we were 9 when the nightmare started. If I were you I would find another doctor. We went through 4 until we found our present one whom we have been with for 6.5 years. You cannot wait for a relapse to happen. The demylienation process starts prior to the symptoms being physically present and the damage starts at that point. You have to time the ivig ahead of any symptoms of a relapse. Each time there is a relapse more damage is done to the nerves and they never heal 100% The healing process is like a chain, the nodes of ranier (spelling sorry) unfortunately do not heal in sync if you will, during the healing process some are skipped over and that is where the chain is broke and healing is not complete. That is why it is imperative to find the dosing necessary to beat the demylienation process to the punch. In the beginning we would get a load every 4 weeks for 4 days in a row, eventually we went to every 2 weeks over 2 days just to break up the dosage time AND keep the ivig constant so that it never got to its end life, the titers would be more balanced instead of running to empty. Your daughter obviously needs more treatment than we did and that is the most important thing to remember about cidp, every one responds differently both to the ivig and rate of repair/damage. I have read some studies that stated children can get up to 3g/kg perhaps you can convince your doc to do a loading dose over 4 days and then in a week after completion start a weekly one day infusion. So essentially you would be getting a monthly load but instead of once a month over 4 days, once a week over one day. If you do this immediately after the initial load (7 days later) you will be ahead of any relapse. You may have to do this for a while. For instance we did loads for a year and then took 2 years to wean down to a maint dose. We are hopefully going to try a full wean this August as we are hoping to be in remission. We shall see. Something else to consider, your daughter may require more than just the ivig. Another possibility might be to try plasma pheresis, cleansing the blood of all of the autoantibodies and then pumping in the ivig. This would give the ivig a jumpstart because the autoantibodies would be removed prior to the administration of the ivig allowing the ivig the opportunity to have to focus only on newly produced autoantibodies. As well, another option could be ivig and some sort ofimmunosuppressant. Unfortunately these drugs come with their own problems. If there is no other alternative than that has to be considered. Obviously steroids have not worked. If it were me, I would try the 4 day load and follow in a week with a once a week load amount If that showed no results I would move to the pp ivig combo if that failed the only other option would be the ivig imunosupp. combination. Good luck prayers sent your way
AnonymousJuly 30, 2013 at 12:41 am
Hi we have convince the neuro in Atlanta to give 1g/kilo every 11 days as she relapse well noticeably at 15 days.
We have decided not to take the immunosuppressants as she responds to ivig and due to the risk.
We have become pro ivig since the Solumedrol pulse therapy that precipated our madeleine. Yet we have talked about loading doses but on 2gr she lasts 17 days which is why we decided to do the 12 days treatment plans.
My fear is how long will take to be on remission and this is the unknown factor. I have read that kids tends to grow out of this as the system eventually resets. Is this due to puberty change and what is the percentage of this spontaneous break free from the nightmare is again something that I have not found. I hang out on small drops of hope that this will go away. She has no pain just weaknesses in the legs going up stairs and the drop foot. No pain at all and again which is one positive. We just want her to have a normal life all month long and not only 10 days out of the month.
Thanks for the information I take note and hope that your daughter will wean off well. For me the day it happens will be the best day after her birth.
July 30, 2013 at 12:24 pm
Without getting too technical, the reason puberty is mentioned as a possible time line for remission is that at birth and puberty the thymus gland is the largest. At this time the immune system re-configurates and the t-cell receptors have a chance to do their job. People use the term you out grow the illness, such as allergies, it is not that you are out growing your allergies, your system just rebooted. By the time we are 50, the thymus gland has shrunk so much it is pretty much non existant thus making it harder to go into remission. That would explain why children are said to have the best chance of recovery. Since we have had this some 6.5 years ago I personally have only read one article that actually gave statistics. Personally, I did not really take anything conclusive from the article regarding a time line. The results were all over for all different ages which pretty much blows a hole in the thymus gland theory. For instance, there was one patient in early twenties who had it 3 years and went into remission, a few 50 to 60 y/o patients were 5-7 years on ivig and a 70 y/o was on ivig for 10 years. I will say that if I remember correctly the study was in early 2000. Research on cidp and the way statistics are gathered regarding treatment type and duration have changed. As well diagnositic criteria has changed significantly, even since we had it. In my opinion, once we stopped thinking about when we were going to get better and just continued on with life and incorporated cidp into our life as a part of it, we were able to relax and deal with things better. Honestly that took about 3 years. this August will be 7 years.
AnonymousJuly 30, 2013 at 8:50 pm
Thanks yes this is indeed our new normal and we try to adapt as do her brothers. Madeleine is 9 as well and I hope every time that it wont happen again and every time is does and she start having trouble going up the stairs and starts to see double again. We will see how she does with the 12 days schedule for her ivig and make her life as normal as possible. At this point she is the strong one who told me this morning ” today is not great but by Thursday I can go ride and canter the pony again” so she is strong and take all of this with courage. I am worried now with the school absences as she will be out of school every 12 days for who knows how long. We have already changed her to a private Montessori school but still she has fallen behind so any tip you could give me on how to make sure we succeed with this road block would be helpful.
AnonymousAugust 1, 2013 at 8:23 pm
Raiyaan is 9 and we got diagnosed about the same time as your daughter did. Maybe we can help each other out. Dawn has been so helpful. I have the exact same concerns/questions/fears as you. When I open my eyes in the morning, after a prayer, this is the first thing on mind and the last thing before I go to sleep. It is emotionally draining. But more so for our kids. We have been to Baltimore too for a second opinion. We are in central nj and getting treated at a hospital here. But we have been to all the big doctors in area for opinions. My son has also responsed well to IVIG, but very slowly. We started seeing changes a month after his first 5 day infusion. Any improvement comes right after about 2 to 3 weeks of dosage and he has had 3 5 day so far. Scheduled for one next week. I just pray that this goes away for all our kids.
August 4, 2013 at 4:33 pm
Hi Stephanie regarding school and missed days. If it is any consolation to you in the beginning while trying to figure this out, we would miss many days of school. Actually the first year was 82 days second year about the same, 3rd year about 45 once we switched to a a schedule of loading doses 2 days every 2 weeks instead of 4 days in a row. Currently the last 3 years we have missed less than 7 days a year. It is important to think about setting up a 504 now. There will be missed days!! The 504 entitles your child to have a plan devised for their needs. Such as additional time to complete assignments with no penalty, extra time to write things(some of things we needed) because hands had to re-learn to write and tremors made it difficult. Gym was a huge issue because he could not do what he used to not even close. So for instance while volleyball, basketball etc were being done, he would be scorekeeper or captain, swimming he would just do things at own pace. The idea was to try to do as much as possible but not be over fatigued or in danger. Without a doubt, the most beneficial aspect of setting up a 504 plan was the at home tutoring. It is a law across the board, nation wide and besides accomadations there is the benefit of at home tutoring. That was truly a life saver to us, a Godsend. The at home tutor is allowed to come for 5 hours for every day missed, to your HOUSE!! Just my opinion, we did not abuse the right for 2 reasons, one it is at a cost to the school district but more importantly, I did not want us to become reliant on the tutor. Should we have cidp lifelong, I felt from an early age it was important to learn to adapt by ourselves. We were able to do all missed work even missing 82 days a year by ourselves except Math. So the tutor would come 1-5 hours a week just for math and some weeks not at all. I helped establish a plan with each teacher how they would like to approach the absenteeism and we went from there. From 4th grade to now junior year high school we have done that every year. I contact each teacher in beginning of year sometime before school starts, explain illness, deficits, ivig schedule and ASK HOW THEY would like to handle things. Then I go over it w/ my son and he handles things by himself now. In 4th grade and throughout grammar school obviously I had to make sure he was completing work and keeping up and we would do things together. Now it is so easy to keep on top of things with the schools having assignments updated daily on the computer!! In the beginning it was still old school. As soon as possible, speak with your childs school counselor and principle, set up the 504. It takes some work, letters from doc explaining treatment, treatment schedule, deficits etc. Also several meetings w/counselor and principle, usually nurse too to write on paper what plan will consist of. The sooner you tackle this the better. If you have a plan established early on, there is less ressitance in middle school and especially high school. Making school as stress free as possible is so important on so many fronts. Stress makes the whole cidp issue more intense, stress from failure in school leads to self confidence/esteem issues which are already compromised w/cidp and failure in school obviously has a lifetime impact on future goals. Don’t use the 504 as a crutch to get out of assignments/work, some people do. It is a detriment to your child. Also, some teachers will allow the child to skip assignments because they genuinely want to help the child, however this sets a presedence early on that hey I can get out of things because I have cidp. It will not be that way in the adult world, so better to face it head onnow. Don’t misunderstand me, the teachers are well intended because they want to help in the moment, but it is the moments thereafter that have to be considered as well. If you have an open dialogue with the teacher and explain these things they are understanding and helpful. It is all in the delivery!! If you go into this with a positive attitude and include others in what you are hoping to achiev, things will be great. I can honestly tell you in the almost 7 years we have been doing this we have had NO complaints/concerns, and all the help and compassion in the world from all 3 schools. I have a friend who went in gangbusters, lawyers etc and it did not go so well for her son. Just be honest. If you want to leave an email we can exchange phone numbers
August 5, 2013 at 1:03 am
Stephanie, I just re-read your post and noticed Madeline is in pre-school. Luckily at this point socialization is what is important. Maybe you can set up extra play dates and then you can focus on curriculum based teaching at home on the days she misses. If she misses many days as we did maybe you could consider putting her baack in public Montessori to save yourself some money. When it comes time for kindergarten maybe you could ask if she could be in morning and afternoon kindergarten (in our school district kindergarten is half day) if it is not all day. That way she can make up time for the missed days. Maybe summer school could be an option as well. Not sure how a 504 would be implemented in kindergarten. I do know that this new common core curriculum decides by seven years old which trac a child is going to be on for their school career, so it is important to not fall behind. Maybe you could see if your school district offers early intervention preschool to supplement days she misses.
BTW have you guys had a port put in yet? If not, you may want to consider it, venous access is going to get difficult real fast and then you will be in a conundrum when you are due for treatment and they cannot access a vein. If you do get a port time the surgery a couple of days before surgery so that the surgeon can leave her accessed after the surgery so you will be ready to go when it is treatment time. After the surgery the area remains swollen for about 3 weeks and it would make the first access painful. Plus the incision where the port was put in was where it needed to be accessed. Oh well, just thinking out loud
AnonymousAugust 5, 2013 at 12:19 pm
Madeleine is in fact 9 years old and a fourth grader and the school that she has been missing was last year mostly as she would go for her ivig and then was having terrible side effects for days. Fortunately she is now drinking a lot and for the past three treatments has not had any side effects of headaches and vomiting which is a big relief. She is starting school next week and we shall see how things go. As for the 504 we will ask for it probably if we go back public next year but again all will depends on her health improvement. What is unbelievable is the rapid effect of the ivig. Madeleine never had any pain but just weakness in her legs her upper body is always fine apart from the double vision. She declines when the ivig wears off (day14) in the matter of three or four days she goes from running to having a drop foot and having horrible time raising from a sitting position or going up stairs. Once she had the ivig she is fine and after two days she can run upstairs , ride the pony and canter ( she is proud of this part) and really has a normal life and resumes business as usual/ Nobody could tell that she has this. At this point I am still convinced that we are still dealing with GBS and not CIDP, even if Dr have to say CIDP because this is what the books say after 3 relapses. I am not buying it yet.
Regarding the port I was talking with her nurse at the hospital where she has now the ivig every 12 days and she assures me that we will be fine without this invasive solution we will either be changing hands or going up in her arms. It is true that Madeleine’s veins have not been a problem at all and changing where we do it will be the way we do it. We want to say that the less medicalize the better and that after the treatment her life is back to normal again. This is why we now refuse anything else than IVIG after the detrimental response to the initial Pulse Solumedrol that we discontinued straight away. We stay with the team that wins ( Madeleine+ IVIG and nothing else)
Here is my email and I can give you my phone number via this route. firstname.lastname@example.org.
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