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?