Steroid taper-better, then symptoms return

My 12-year old son was dx with CIDP in early June. He had one round (2 doses) of IVIG and we didn’t see any improvement. 2 weeks later he was admitted to the hospital for pulse IV steroids for 4 days. We saw immediate improvement in balance and gait. He has been doing his PT morning and night and has been doing an oral steroid wean for the past 4 weeks-40mg once a day, then 20mg once a day, last week was 10mg once a day, today is supposed to start 5mg once a day. After the taper the plan was to see our neuromuscular doc again and most likely start pulses oral steroids on the weekends.
Unfortunately, over the last week we have noticed some of his symptoms returning…awkward gait, stumbling, etc. they are really subtle but myself and other family members who have been around him for the past month jar notices as well.
I have a call in to the nurses at the neurologists office. Any ideas what is going on? Was the steroid taper done too quickly?
All this constant watching and observing him is really stressing us all out. He has started always walking behind me so that I’m not watching him walk!
I was so hopeful when he started getting “better” with the steroids. Now I’m just sad again.
Background info-he just started having symptoms in April. Awkward gait, saying his legs felt “weird” and Al’s saying he couldn’t run.

I say if it works, don't fix it. It and the least medication the better. I don't know your son's height and weight so I'm unable to help you with the steroids, but it sounds like your neurologist needs this information and his meds. need to be re-ajusted. Gary

Normally pulse steroids are not advisable for this age group. This will have serious side effects later. Pls consult a senior neuro physician in your area and get an expert opinion.

I'm pretty confident in our doctors. He is being treated by the neuro muscular team at Washington University School of Medicine. I have read many articles that actually say the opposite, that pulse/burst steroids have less long term effects than low dose daily steroids. I appreciate all the feedback, though, as we are REALLY new to this! His doctors stopped the steroid taper and switched him to 10mg of Prednisone during the week and 60mg each day on the weekend. I think the goal is to wean the 10mg during the week and head to just weekend dosing which they've said he will be on for a while.

VKPN-I would be interested in the literature you have or where you've heard that pulse steroids aren't advisable.

Our follow-up appt. with the neuro-muscular team is in two weeks. I am trying to keep a list of questions to ask. One of them is in regards to IVIG. If he didn't respond after just 2 infusions, do we just give up? Or do we try it again? I know it is way more expensive, but has fewer side effects that long-term steroids.

Thanks all. It was very disheartening to see him improve, then regress when the steroid dose was reduced. What a roller coaster this is!

As far as height and weight-my son is 5'3 and weighs approx. 108lb.

Don't rule out the possibility that the IVIG actually helped. I get regular IVIG, and it usually only takes me 2 or 3 days to see improvement. But, everyone is different, and for some people it takes much longer for the effects of the IVIG to kick in. But, IVIG does "wear off" and you generally need repeat treatments. So, it is possible that that is what you're observing.

Of course, it is also possible that the tapering off of the steroids was too quick, or too drastic, and he might need some kind of continuous maintenance dose.

As you read this blog, you will see a common thread that every case is different, and everyone responds differently. In my case, prednisone did very little, but IVIG is like a miracle. It's great that you have found something that works, now you just need to work with the doctors to determine the right dose.

Good luck!

Bill is probably the most up-to-date site for information you can count on. Gary

I'm so sorry that he is having a hard time. My thoughts are with you and him.

Steroid taper is common, and seems to have been done appropriately. However, especially while starting IVIG therapy, he may have to be on a constant dose, but the dose is not a constant for everyone of the same age and size. Do not do this until talking with his doctor, but for now, it seems reasonable for him to be on the lowest dose that was effective.

The reason for tapering is potential side effects of prolonged steroid use, which are concerning. Also, steroids cannot be abruptly stopped for any use, since symptoms can receive, or the body also can slow down the production of it’s own cortisol, causing adrenal insufficiency.

Definitely talk with his doctor. It may be that intermittent steroid injections with an intermediate lasting agent (such as Kenalog) would work better. Hopefully, he won’t need steroids long term. Prayers are with you both.

Had our follow-up with the neuro-muscular doctor yesterday. The plan is to taper his Mon-Fri prednisone down to zero and taper up his Sat & Sun. prednisone to (gulp) 225mg on Sat and Sun. She said she has treated approx. 150 pediatric patients this way over the past 10 years and they have found that this weekend dosing has less side effects and works well...something about CIDP being B-cell mediated. She also said they have very good results, even cure, in a majority of the children. I asked about IVIG and she said her personal opinion is that she likes to try the prednisone protocol first and if it doesn't work, then move on to IVIG as IVIG is a pooled blood product which is as concerning as the possible side effects from the prednisone.

At this point I am going to trust her and her experience and the fact that the neurology department at our hospital is in the top 5 of Children's Hospitals in the U.S. It does seem like the majority of people I am seeing on CIDP forums like this one are being treated with IVIG, but most of them aren't children. We are going for a follow-up in 2 months. For now, my son will continue with outpatient PT and we will be praying for improvement.

Like Uncle Bill, I usually see an improvement in my neuropathy shortly after an infusion. Therefore, I assumed that the IVIG from a particular infusion kicked in a few days after that infusion. However, that assumption appears to be incorrect, at least in my case. I got permission for an experiment when I was getting infusions every other week. I moved up a single infusion by one week, which would result in unusually high immunoglobulin levels in my blood. I expected to see some improvement from this experiment a few days after the infusion that I moved up by one week. I did see a big improvement, but it took more than three weeks for the improvement to appear. Apparently, it takes a while for the myelin to grow back. When I got an increase of about 4% in dosage (remaining on a two week schedule), I didn't see the improvement in my neuropathy for more than four weeks. I think that the improvement can be observed earlier if the increase in blood immunoglobulin is larger. I suspect that, if I missed an infusion, I would still see an improvement in my neuropathy a few days after the normal infusion date. I don't think it's at all surprising that it would take more than two infusions and a few days to see an improvement from IVIG.