12-year old son newly diagnosed
AnonymousJuly 28, 2012 at 6:31 pm
There doesn’t seem like a lot of activity on this forum, but I am hoping to connect with other parents of children with CIDP. My 12-year old son was just diagnosed in June after approx.8 weeks of what we thought was clumsiness. He had an LP with elevated protein as well as an abnormal EMG. Brain MRI was normal but spine MRI showed enhancements on the C and T spine. So, they are calling his CIDP “atypical.” He also had hearing loss as well.
He was admitted for 2 doses of IVIG and then we went to Florida on vacation. No improvement noted in a week, so the neurologist admitted him again for 4 days of pulse IV methylprednisolone. We saw improvements in balance and muscle strength within 48 hours. He was discharged on a weekly steroid taper and also instructed in some PT to do on his own. (Also, while still not normal, his hearing greatly improved with the steroids as well.) Unfortunately, sometime between the 20mg per day of prednisolone and the 10mg per day, we noticed his abnormal gait coming back. I contacted the neurologist who decided to put him on 10mg per weekday and 60mg per day on Saturday and Sunday of prednisone. They feel this burst dosing causes less side effects.
We have our follow-up with his doctor (Anne Connolly at Washington University School of Medicine’s neuro-muscular dept) on Aug. 9. I am hoping that you all could help me with some questions to ask? Mostly, do we give IVIG a try again or do we stick with the prednisone? And when should we repeat the EMG to see if there is improvement?
And I am also wondering how you parents lived with this roller coaster ride!? Am I just supposed to be constantly watching him for symptoms to look for improvement/relapse? My son has taken to always walking behind me because he knows that I am constantly focusing on his gait and how he is walking! I am just now starting the process of a 504 plan for school and would appreciate any input on that as well.
Thank you! Sorry so long…lots on my mind! (FYI-I am also a pediatric nurse, so I can handle pretty technical info. I’ve been doing a lot of journal searches and have been learning a lot over the past 8 weeks!)
July 29, 2012 at 2:07 am
My daughter was 4 years old when she was dx’s with CIDP. She’s 10 now & lives her life like a completely normal kid. She gets IVIG every 5-6 weeks. As far as constantly watching your son – yep…you will do that. You will worry for the rest of your life for every single ache & pain he has. BUT it gets easier to deal with. You will learn what his CIDP symptoms look like & you will be able to easily decipher between those & other normal issues.
Dawn (Kevin’s Mom) will be able to help you with regards to a 504 plan. I tried to get one for my daughter but was refused because she looks normal. I haven’t pushed the issue again but will if I have to.
As far as his treatment plan…I’m an advocate for IVIG. In my personal opinion, a good neurologist will tailor the IVIG treatment plan to your son. He may need weekly or bi-weekly infusions. Some dr’s are afraid of IVIG. Personally, I would be more worried about the steroids.
My daughter was taken off of IVIG for a few months about 1 year after she was dx’d. We had switched dr’s & the new dr was an advocate for steroids. My daughter got worse while taking them. She went from relapsing every 12 days to every 9. Some variants of CIDP do handle steroids well. We switched back to her original neuro & he prescribed IVIG again & she has been steadily improving since then.
It sounds like your son may have not had the proper dosing for IVIG. He should receive a 5 day loading dose to start with, which is 2 grams per kg of his weight. You figure out how much IVIG he should get by multiplying his weight by 2.2. You take that answer & divide it up by 5 & that is what he should get daily.
For example: My daughter Emily weighs 98 lbs.
Her IVIG would either be rounded down to 40 grams per infusion or rounded up to 45 grams. I would prefer to round up.
He may need to have multiple loading doses before starting on a maintenance dose which is 1 gram per kg. His maintenance dose schedule should depend on how often he relapses. Each CIDP’er should have their own IVIG schedule tailored to them.
Research Dr. Dyck out of Mayo in Minnesota. I recommend his often as I agree with his IVIG dosing.
If you leave your email address I will contact you & perhaps we can talk on the phone. It really helps to speak with people & to know you aren’t alone.
It gets better…I promise.
July 30, 2012 at 2:34 pm
Hi, so sorry this happened, I truly can say I know how you feel. We had about the same time frame of symptoms. We did not have hearing issues but eventually we were not able to walk, hold an item, stand or get dressed. Eventually in PICU for 10 days difficulty breathing and waiting for ivig (there was a shortage when we were dx 6 years ago) To answer your question honestly, yes you will and have to constantly watch him intil you can figure out what his relapse time is between treatment. You have an advantage over us because you are a nurse. About the dosing, it is the standard 2g/kg for a loading dose. You didnot give his weight… Load should have been weight in lbs/2.2 which = weight in kg multiplied by 2 (2g/kg) Unless they gave him an extrmeme amount over the 2 days, it sounds like he may have gotten a maintenance dose. You are a nurse, so I don’t have to tell you about prednisone, especially for a pubescent boy. I would go on the ninds site or print out info on Dr. Dyck from Mayo and give the info to your doc. Not discounting his treatment, because the ivig and steroids together are obviously working, but a proper amount of ivig alone may do the trick. Because of cost and for some patients side affects (we get the aseptic meningits reaction every time, every two weeks for six years) some docs use less ivig. If your doc is a good caring doc, he will listen to your suggestions, ideas, if not time to find a new doc. Eventually you will have to figure out how long ivig will last for your son between doses. I don’t know if you know about ivig, so please forgive me if I am telling you things you know. IVIG has a full life of 42 days, but some people need more than that because their body is producing autoantibodies at a higher/quicker rate therefore necessitating more ivig. In addition it is imperative to keep the treatments in front of the demylienation process. For instance, if your son notices weakness, tingling, pain etc before the end of the 42 days, you know he relapses before the full life. Not to mention if he is feeling it, the demylienation startedlong before. In order to let healing start (distally) you have to make sure no new process starts. We were on loads for a year and then tapered down by 10g amonth until we were at a maint dose (1g/kg) we stayed on the maint for 3 years and then tapered by 10g monthly until we were off (to see if we were in remission) We were not. Symptoms started again after about 6 months. So we loaded again for six months and tapered back to a maint and have been on one since. In total, it will be 6 years this September. We are going to the neuro today in fact to try a wean again to seeif we are in remission yet. Please pray for us. Things do get better, you will be able to understand how he responds as time goes by. Probably the best advice I can give is set the presedence early, both with your son and doc. Let the doc know you are well versed (nurse) delicately of course so as not to insult his ego and that you might have some comments, ideas. As well, let your son know you are there for him unconditionally, but…also keep in mind you will have to side boundaries regarding him taking advantage of situations. There will be a certain amount of depression regarding inabilitie/friends, treatments etc. school will be missed and things build up. It is hard to find the balance between the boundaries and the compassion. There were many days absent from school in the begining w/ hospital stays, pain pt etc. 82 days the first year, 70 or so the next and once we figured thing out, we average about 28 or so. If possible, try to get in contact with the school asap so that you can alert them of the situation and have a 504 plan started. It is an accomadation paln to allow for things he might need because of a chronic condition. TRUST ME!!! It will only help you both. Starting in middle school is helpful so that the HS will already know what works. There is a new law (last year) that no longer requires a student be absent 14 days in a row if there is a chronic condition in order to receive homebound tutoring. You can get up to 10 hours a week of homebound tutoring. We do not use the ten hours presently because we no longer miss as much now that things are stable, but in the begining, we did. Last year freshman year we used probably 20 hours total for the year. Under our plan we are allowed to turn in work as soon as possible with no penalty. Mostly the tutoring was for Math. He was able to do everything else on his own in his time frame. You have to start with the independent accountability right off the bat or habbits will develop. If the school fights you mention due process and that will straighten things out right away. If you would like to talk, let me know and I could leave my # with the foundation. Also, keep a port in mind. Good luck, things will get better.
July 30, 2012 at 3:10 pm
I re read my post and realized there was really no positive info in it. Just so you know, my son is totally functional now, beyond functional actually. He can run five miles, liftts weights (15 yrs now, 9 when this started) has huge biceps, almost a full 6 pack. Everything is normal with the exception of getting tired faster and increased pain at old damage areas when he gets the flu or something. We hope that this time he will be in remission. As I mentioned we go to the doc today to talk about weaning again Things do get better and believe it or not this will make him stronger in life in so many other ways…
About the hearing issue, check in archives for a member named sue or sue from cornwall or something. She is from Canada, she too had ear issues. I don’t remember anyone else on the site besides her and maybe “Jerimy” mentioning hearing loss. Sue did get her hearing back with steroids. What did they find on the c and t spine? Inflamation? Lessions? The most of our pain was centered around the spine as well. Let me know if you would like to talk.
July 30, 2012 at 4:05 pm
Sorry, you asked about the repeat ncv/emg. We had an initial one, then when we were stable the first time and about to start weaning, we did another for a new baseline, it was normal. After the six month period of weaning and symptoms returning, we did another and there were again slowed velocities. this was the tangible proof to show a relapse w/out treatment. Such info is good to have (tangible proof) for the insurance comapny should you have any problems getting back on ivig. As well it can be used to proove that ivig does work. Another unit of measurement, tangible, other than the typical neuro assesmnet is doing a variety of tests through PT. In the begining we had a scrip for PT and did a variety of baseline tests, typical bend and stretch, ability things etc. As well, they used a dimometer to measure grip stregnth. It was something with numbers, real, not subjective measurements. Obviosly not as accurate as a ncv/emg because of variables such as fatigue etc, but it was something. When you do get another ncv/emg, try to insist on the same neuro to do the test. Bring your old reports so that they can replicate the same test, same nerves, same distance measurements etc. Same room temp etc. Apples to apples.
You mention questions to ask, honestly, you have probably learned more from your research than your doc knows. I don’t mean that negatively, it is just that many docs do not have experience with cidp. I would probably ask the doc is she would consider giving loading doses for a year or so and simultaneously wean off the steroids to see if he could do it w/ivig alone. Besides, steroids are not a long term solution, if your son could be off of them before school it would be easier on him with the sleeping issues and mood swings as well as the obvious medical ramifications. True he may need them or some other form of imunosuppressant should ivig alone not work, but finding out through trial and error would be beneficial. Maybe you could also ask about the pt eval for another form of baseline testing. If your doc does do loads, you can get them done at home through home health. That is what we do, same nurse for 6 years, they play xbox and shoot bb guns outside, its like a little man party in the basement, we try to keep it as normal as possible. With you being a nurse, you might be able to have your doc order the ivig through gammaguard or gammunex or whoever and you do the treatment at home. You can also get a fanny pac pump and be mobile if you wanted. We just do the regular pole pump and go directly from the bottles, no pooling like you do for the fanny pac. You can also sign up with the various ivig companies to get on their “frequent flier” program… just kidding, it is a loyalty program, you sign up and they keep track of what you use and should there ever come a time you loose your insurance, they will provide a certain amount for free to bridge the gap. As well, if there was ever a recall, you get a call direct. I would probably ask about considering a port. You might want to have the surgery before school. The first port took about 10 days before it was reasonably healed. If you do go that route make sure they give him an adult port so that it will last. I think ours is called pass port or something, it has three landmarks (little dots) that protrude for easier access. Also have them leave him accessed after the surgery and just flush it so that he is ready for his first treatment. We had so much swelling, they did not have a needle long enough to go through the tissue to the port and we ended up doing peripheral until it healed. For our second port (3 years later, child to adult port) I asked to leave it accessed) I know I am all over the place bombarding you with info, but in case I get tied up with things with my other son going to college and my dad (nursing home ) and Kev at the doc, I wanted to tell you everything I could think of.
AnonymousJuly 30, 2012 at 11:44 pm
Wow, thank you so much to both of you for your responses. Dawn-I hope you received good news from the neurologist today! It is good to hear how well your son is doing. My son really just wants to be able to run and play basketball again!
Mitchell’s spine MRI showed increased intensity, mostly in the posterior column, from c-t spine. That’s fancy for inflammation according to the docs. This is one of the reasons they are categorizing him as atypical CIDP. Our doctor isn’t too disturbed by the spinal cord findings, though, and says there are definitely examples in the literature of CNS involvement with CIDP. She also wonders how many patients are diagnosed with CIDP and never have a spine MRI and how many of them would have had CNS involvement had they been scanned.
I am definitely going to bring up trying IVIG again at our appointment with the neuro-muscular doctor next Thursday. We have only seen that doctor when Mitchell was an inpatient for the steroids a few weeks ago. I am fairly confident in Dr. Connolly’s knowledge of CIDP and treatments for it. When we were in the hospital and she told us that the diagnosis was CIDP, I googled it and found an article from Pediatric Neurology from 2001 and found an article written by Dr. Connolly titled “CIDP in childhood,” so I definitely felt assured by her knowledge! Her colleague at Washington University, Dr. Pestronk, has also contributed to many articles on CIDP. I am praying, though, that she will be open to my questions and that we can have a good working relationship because everything you have all told me and I have read really seems like we need to have a good relationship with our doctor and need to find a doctor who is available and willing to change things up if necessary.
I have printed out our conversations here on the forum and underlined specific topics I want to discuss at our appointment!
I printed out some information from the Foundation’s website and sent it to our school nurse to get started on the 504 plan. She is very willing to let me tailor the plan to his needs. My top concerns are: 1. His hearing loss, while improving, we need to make sure teachers are aware and he has up front seating, 2. Gym class–there is no way he could do running or balance type activities now. At least he couldn’t do them WELL and to have them tied to a grade would be unfair, and 3. The possibility of missing school.
Any other thoughts on school and the 504?
Final question for now–physical therapy. When he was an inpatient, they gave him a stretching routine that he has been doing twice a day. He has his first outpatient physical therapy appointment on Wednesday and I don’t really know what to expect. It won’t be the same therapist as when he was evaluated as an inpatient so I’m not sure if she’ll be able to tell if he has improved or not. Do I push for more outpatient PT? I feel like it can only help, but I’m not sure how much we should be pushing him or how much he should be doing as far as exercise. As much as he can tolerate?
My email address is firstname.lastname@example.org
I would love to continue communicating with you all. Thanks so much for your support so far!
August 4, 2012 at 1:43 pm
Sorry I didn’t email you. The site was down for a few days. I will try to email you later today.
Real quick – my daughter had inflammation from her lower lumbar on down. The neuro said she “lit up like a Christmas tree” on the MRI. She also had cranial nerve inflammation – did your son have a brain MRI with contrast as well?
I can’t really help you with regards to PT. We never put Emily in it as she responded really quickly to the IVIG.
AnonymousNovember 30, 2012 at 3:05 am
My son is also 12 and just had a relapse of CIDP. He was diagnosed at age 4 and got much better by 6. Now he is getting steroids by IV about once a month and doing much better. Feel free to email me at email@example.com. I would be willing to talk to you by phone also. Any questions feel free to ask.
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