new member hello
AnonymousApril 13, 2007 at 1:22 pm
My son, Cole is 10 years old. He was diagnosed with CIDP about 6 years ago. I stumbled across this site a few weeks ago and have been monitoring posts for a while, (we are a bit shy). We are in Ontario Canada, just west of Toronto and I will be attending the conference in TO next week. I’m hoping to make some connection here and at the conference and get some guidance.
Cole was born normal and healthy. When he was about 2 he started missing physical milestones — stopped jumping, running, began to struggle with stairs, etc, and he stopped growing. Doctors, (at least 4) said we were crazy…. you know the routine. When he was 4 our GP retired. The new GP took one look at Cole and knew somethign was wrong. We were referred to Sick Kids in Toronto. After about 24 months of wait lists and many, many tests including NCV, LP, gallons of blood, cell/muscle/nerve biopsy, the diagnosis of CIDP was handed in despite the fact the LP did not show the typical elevated protein level. I will try to summarize the treatments we have tried since then.
Treated by Dr. Banwell at Sickkids, he was on a 5 week cycle of iIgG to start. We saw moderate improvement but NCV showed no change so they discontinued after 4 treatments. His condition deteriorited, so they started again after about 6 months at 3 week cycle. Although he did not regain lost strength, the igG slowed the detrioration. They added oral steroid, (15 mg/day when he wieghed 40 lb) and we saw rapid improvement. He could climb stairs again, but when they started to reduce the dosage he began to detiorate again. He remains on 5 mg/day. (its been 4 years now).
In 2005, Dr. Banwell referred us to Dr. Dycke at Mayo in Rochester to see if he had any ideas. They repeated all the tests including another biopsy and agreed with the diagnosis, and suggested we be more agressive with the treatment.
In 2006, Cole’s treatment was transferred to Macmaster Children’s Hospital in Hamilton under the care of Dr. Tarnopolsky. Cole had a port put in and IVIG was changed to weekly, (2 /kg). We observed improvements in energy level but no dramatic strength increase, though the doctor’s strength tests suggest the large muscles were getting stronger. After about 6 months of weekly treatments, over concerns of blood viscosity they decreased ivig dosage to 1g/kg. He began to deterioriate again. After 1 month they restored dosage to 2 g/kg and have added solumedrol to the weekly infusion. He has not regained the lost strength. We really don’t see any positive impact from the solumedrol.
Cole continues to slowly get worse. The treatment seems to control the rate of deterioration but not stop it. His strength loss is assymetrical, (left is much stronger than right), ideopathic but predominantly peripheral, and he does have sensory loss. The nerve damage is predominantly axonal. He has some demylenation, but it is secondary to the axonal damage. He has bilateral foot drop, had tendon transfer 2 years ago, and can’t walk without his AFO’s. He has severe growth delay. Although he has gained weight with the steriods, he has not gained much in height. He is the same size as his 5 year old brother. This is just as painful for him as the strength issue. He does not have muscle pain, but sometimes reports tingling. He used to get fevers regularly, but now is just always warm. He gets headaches at least a few times a week, but they are managed with analgesics.
What we are doing now isn’t working well enough. Cole continues to lose function very slowly. Despite this, he is still a happy, well adjusted kid. He goes to school, gets average marks in Grade 5, (in spite of all this), but his friends are less likely to sit and play with him or wait for him every year.
We are looking for options, alternatives, anything!!!
Sorry about the length of this post, and thanks for your patience.
Betty, (Cole’s mom)
AnonymousApril 13, 2007 at 2:05 pm
Welcome to our GBS/CIDP family 🙂 What an amazing struggle your family has been thru. I’m so glad that you found “us”. We’ll be around to help you all that we can. And as you’ve probably read, this is a great place to vent!!
We have more and more families arriving on this site with sick kids. I hurt for each little one that is experiencing these diseases. And you parents are amazing caregivers. I salute you! Keep coming back so that we can get to know you all. Big hugs 🙂
AnonymousApril 13, 2007 at 4:50 pm
I too am very sorry for your struggles. We have an almost 3 year old with cidp, contracted at about 5-8 months of age.
I feel your pain. Although out son is very lucky and does not hurt, he is also not growing very much, about 1% on scale, and is also disabled. He can cruise the furniture and walks with a walker with wheels.
We are doing ivig every week, about to go every 10 days. His problem is: he gets stable, then takes a nosedive. We can’t get the disease into remission.
My only advise. Talk to everyone you can. I love my doctor and he’s been great but the ppl. that have helped me on this forum have been priceless. My son probably would not be doing as good as he’s doing if it were not from learning from different ppl. on this forum.
Aside from our regular neurologist, we also went to John Hopkins. There, the dr. has his doubts that Dell has cidp because of the age of onset but we believe he does. His protein is 93, suppose to be 30 something and the IL2 blood test was 1600, suppose to be 100-500. The muscle biopsy confirmed it too.
Dell also has the fevers like you mention. You can read the posting that I posted yesterday on the main forum about fevers.
Good luck, please write whenever you need to. This forum is also therapy.
AnonymousApril 14, 2007 at 2:52 am
Hi Betty, (Cole’s Mom)
My name is Robin, (Hunter’s Mom) I feel your frustrations and despair. My son is seven. My son has lost the use of his left hand and wrist. One finger at a time, and now his ankle reflex has diminished and last two toes can’t curl. When Hunter was about 2 (2001) he started complaining of leg pain and down hill from there. We too have been shuffled and we have also seen Dr. Dyke at Mayo in Rochester and Dr. Kuntz and Dr. Spinner for nerve biopsy. (I really liked him) Hunter also did not have elevated protein. They have spoke to us about tendon transfer. We finally got a diagnosis of CIDP in 2005. I’m very interested in hearing more about the transfer.
Hunter gets IVIG every week and has been for 2 years now. It has slowed the progession, but has not stopped it! His stamina has improved and pain improved. but now things are starting to happen a little faster again and it scares me. It is soooooooo difficult to watch your child loose function, in pain and not be able to do anything to stop it. My motto is that I won’t give up. We live in Florida, and have an appt on April 24 with another specialist in Kansas. If I’m not satisfied or he cant help, I’ll just keep finding another Dr. There has to be one out there that can help my son.
This site is great to have people to relate to. I’m pretty new, still learning. Sometimes it confuses me a little. It seems there is no one set way of treating CIDP. It is all over the board. The dosages, rates, frequency etc.
I do have a post or if you would like email me at [email]firstname.lastname@example.org[/email]
Your family and Cole will be added in our prayers,
I will post anything I find out at the Doctors. Wish us Luck!
Thanks for your post, I can relate to your story!
Robin (Hunters Mom)
AnonymousApril 14, 2007 at 3:31 pm
Hi there –
My daughter was 4 when she was dx’d. I think we are very lucky in that we got a quick dx & quick treatment. She gets IVIG 1 time a week right now because she has right eye paralysis.
This is what I’m thinking about your son’s case…
It took about 2 years to get a dx & treatment, correct? In that time the disease was wreaking havoc on his nerves & probably his muscles too. The long wait (through NO fault of your own – you were trying to find answers) probably caused the axonal damage. From what I’ve read, axonal damage cannot be repaired. Therefore I would focus more on repairing the demyelination, since you CAN do something about that.
Have you discussed with his dr how well he was doing on the oral steroids? Why did they take him off of those? I ask because some people respond better to oral steroids & others to intravenous. Just as this disease affects every person differently, each person reacts to the treatments differently.
(My daughter was put on 20 mg’s of oral steroids & it actually made her worse. She lost 3 days in the time it takes for her eye to relapse. Like I said before, it works for some & not for others.)
Your son’s growth may have been slowed down from the steroids. I read that is a possible side effect. I would revisit the solumedrol infusions with his dr. If they aren’t working & you are seeing no difference then why keep doing them? At that same time I would remind the dr about how well he did on the 15mg’s of oral steroids.
What really ticks me off is how dr’s want to go by what EMG/NCV tests show in children. We switched neuros for a few months & she wanted to perform them every 2 months on my daughter. They are PAINFUL so I don’t see the reasoning. I personally feel that in children the BEST way to determine if they are getting better is by how mobile they are. I take my daughter to the park regularly & base her progress on how well she does there playing on the toys. If she has no trouble running, climbing, jumping then I know things are good. If she starts to show any problems with doing those activities then I know it’s time to get her in to see the dr.
Also, it takes longer than 2 months for nerves to be repaired. So doing an EMG/NCV & not seeing improvement doesn’t mean that there isn’t any. It doesn’t always show up on the test. There’s little tiny repairs being made every single day as long as you can keep the disease from attacking the myelin. As long as the myelin isn’t being attacked the Schwann cell can begin to work on repairing it.
Welcome to our site & I am glad you found us. While I’m sad that your son has CIDP I’m glad that you’re here. For a long time there were only a few of us here whose children had the disease. It’s good to see that other parents are finding us. The BEST way to learn about this disease is by talking to anyone that you can who has it. I’ve learned SO much from the people here.
AnonymousApril 15, 2007 at 6:13 pm
Thank you all for your replies. A few answers and a few more questions:
Thoughout the whole process and even now Cole’s diagnosis with CIDP is mostly by default. Because Cole did not have a precipitating event, had early onset, (we are told about 18 months based on growth patterns), absence of elevated protein in SF, insignificant response to igG or steriods, etc, the doctors have always felt that they would uncover some congenital cause. Indeed, even now we are awaiting the results for some new gene is ID’d, (and that’s ok). Becasue of that they have been reluctant to proceed with steriods because of the side effects. They have been content to administer IgG because it does no harm. (Except for that period when they discontinued.) Ironically, that deterioration when they stopped the IgG supported the CIDP diagnosis. Were there members who were eventually diagnosed with some congenital condition?
In his first year, Cole was at about the 40th percentile. At 4 he was at the 0th. He started with steriods when he was 6, he was off the scale. With steriods he started to eat and grow and appeared to gain strength. He climbed to the 20th percentile in weight and 10th in height. The neurologist was reluctant to leave Cole on the high dosage for obvious reasons. She said typically with CIDP, the benefits are retained after weaning and his failure to sustain did not support the CIDP diagnosis. She though the strength increase could be attributed to the fact that he was finally eating. Growth is definitely affected by steriods. He continues to gain weight, but no height.
We started infused steriods when Cole was 9 after a 2nd biopsy showed inflammatory cells in the nerve, supporting CIDP. We started with steriods in every 2nd IgG, (ie biweekly). With the first few infusions we thought we were seeing an improvement but it was not sustained, so we started including solumedrol with every igG infusion, (weekly). No one wants to increase the dosage further. I plan to suggest stopping infused steriods at next neuro appointment. Is there any one out there who responded to oral but NOT infused?
Cole’s biopsies showed significant axonal damage, even empty strands and very minor demyelination. I don’t really understand the disease process, but believe that in Cole, the immune system is directly attacking the axon and the myelin damage occurs after. I am told that the axon can repair itself but much much slower than the myelin. Does anyone else out there have nerve damage that is predominantly axonal? I wonder if those with axonal damage
respond similarly to treatments.
Cole is currently on 2 /kg IgG, weekly. I think this is manufacturer’s rec. I have read that some are receiving up to 4/kg. At what frequency? What triggers lead to increased dosage? What is max dosage and frequency? Our neuro has been concerned about blood viscosity. Is this a real concern?
Cole seems to initially respond to any increase in treatment agression, but that reponse always tails off, like his immune system figures it out and rises to the challenge. Are ther others who respond similarly? In the absence of other alternatives, we are starting to talk about supressors. Are there any other children who are on suppressors? What drug, dosage….
Cole has worn AFO’s on both feet for his foot drop since he was 5. When he was 8 he developed pressure points and eventually open sores from the AFO’s and had to go in a wheelchair because he couldn’t where AFO’s and couldn’t walk without them. This despite persistant and agressive effort from his orthotist and physiotherapist, including several sessions of serial casting.
For Cole, they cast the feet for a few weeks before surgery to maximize the tendon stretch. In ~2 hour surgery, they thinned the achilles tendon to stretch it and spit the tendon that runs down the inside of the lower leg to the foot and attached a piece of that tendon to the outside of the foot. (ie a peice of tendon is transferred from inside of lower leg to outside.) This requires long incisions on both sides of the foot around the bend of the ankle). The feet are cast ASAP after the surgery to maximize the stretch. (Cole was actually cast in surgery, but there are lots of arguments against that.) Cole was released, with casts on both feet next day. He had moderate pain. He was in wheel chair for another week and walking on casts for 6 more weeks. When casts come off he had incredible range of foot motion. Caution – Cole thought that the surgery would “fix” his feet. He was disappointed even though everyone else thought the surgery was successful as it made him mobile again.
Further, it is quite likely that further surgey will be required unless the “cause” is addressed. I know others who have had ankle fused so that the foot can’t drop. I think that this surgey is more painful, (boney – not just soft tissue) and reduces ankle flexibility significantly, (can’t point or flex foot).
Are there any members in Canada that are attending the conference next week?
AnonymousApril 18, 2007 at 3:01 pm
That is an awsome explanation of everything.
I have to go through my emails with the dr but I think Dell has every sensory problem known to man. It will take me awhile to find it but I’ve been thinking about looking for it anyway.
Dell took oral steroids for 1-1/2 years. We saw no improvement or decline because he can’t get stable, therefore, he goes from strong to weak constantly. Since Dec, we have been on 200 mg of solumedrol with the ivig treatments. I still don’t know if it is working or not, doesn’t seem like. Our dr. wants to put him on suppresants too but I am so worried. Don’t know what to do.
I am very frustrated as I write this, I just wrote to my priest. Dell has been stable for a few weeks but got weak last night. It makes me so depressed.
I haven’t answered too many of your questions because I have my own. I look forward to the day when all our children can become well and cured.
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