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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.

Kelly

Thank you all for your replies. A few answers and a few more questions:

Diagnosis

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?

Growth

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.

Steriods

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?

Nerve Damage

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.

Treatment

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….

Tendon Transfer

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?