Anyone with Sensory Predominant CIDP?

Hi,

Yes I have CIDP (chronic inflammatory sensory demyelinating polyradiculoneuropathy) It is very rare (one case in 1m). I have had IVIg (borderline response) twice, been on steroids (no luck) and now I have plasma exchange every three weeks, originally over 5 days several times, then over 4 (just the one time) and then over three days it three wasn’t enough so I am going back up to 4 days when i’m next in hospital. We’re trying to get funding for Rituximab (not easy) and if we don’t have luck there I will have a Portacath inserted and I think the plan is every three weeks of two days of PE. I get a lot of pain, sharp, cutting in pain, stinging sore feet, pain and prickles in the hands (feet and hands also go purple (autonomic neuropathy), burning, prickles, crawling, tightness and pain in my nose, forehead, head, shooting pains in my head, cutting in pain in my neck etc, tightness, pulling etc around my gums too. I hope this helps. People who respond to PE generally respond to Rituximab which in some cases pushes the condition into remission. It’s a long road! I work.

O.

I made a mistake in my reply, i meant to say that I have PE every six weeks. The plan is to have PE every three weekends over two days if I don’t get Rituximab and if contractually my consultant can’t administer it to me if I have paid for the drug (we need to check NHS rules). PE is a big help and has reduced my many symptoms but six weeks is not often enough. Week 1 say is my treatment week and then weeks three and four I will notice a big improvement. Symptoms then start creeping back week 5. I had numbness in my feet and hands when the condition started 18 months ago and I don’t suffer with numbness any more but where my hands and feet were going numb at the start my sensation in my finger tips and toes isn’t quite as sharp as it is further up the fingers and feet. I’m aware of that when the pin prick test is done. After the first IVIg I had improvement for two weeks but after the second treatment I had improvement for only one week and nerve conduction studies showed only boarderline improvement. That’s when I was taken off the IVIg and put on Prednisoline. That caused a flare up in symptoms plus a bone scan showed I have osteopenia so I was taken off the steroids. I’m in the UK by the way but am having difficulty getting funding for Rituximab in circumstances where PE costs £1,000 per day and Rituximab £2,500 and if it works I may only need it every 10-12 months or never again. If it works it would clearly save £1,000s of pounds. My CIDP started in July 2016 with a whole host of bizarre symptoms and I started IVIg in the December. I started PE in June last year and nerve conduction studies after only theee weeks showed between 50 and 100% improvement. I hope this helps. You have had it a long time. How have you been coping? I would be interested to hear how you get on. Cyclophosphamide is very immunosuppressant but my consultant won’t give it to me on safety grounds. I really hope we both push this horrible thing into remission.

Regards O

Hi
Yes I imagine you can’t wait for the IVIg and I imagine you will get some improvement. Presumably you are in the States? Does your insurance pay? After that you need to find out how often you need it. PE apart from having a line inserted in your groin each time is pretty harmless. It drains away your plasma and you see it filling up in a bag and it’s dark yellow. Clotting Levels become an issue but it’s all monitored. I have my next PE the week of 26th February but I go into hospital on the Sunday. I wish you all the very best and please stay in touch because I have never come across anyone with CISP and we may be able to pass on useful information to one another. Do you have any idea why you might have got this? I always blame it on chronic stress that I went through. The problem with CISP my consultant has told me is that because it’s so rare no drug company will pay for trials and so it’s trial and error for the patient and clinician. Once you have tried the three frontline treatments (steroids (I know you and I can’t have these), IVIg and PE) you can then look at immunosuppressants but they come with an obvious risk. Do let me know how you get on with IVIg and similarly I will let you know about the Rituximab.

Regards O

PE apart from having a line inserted in your groin each time is pretty harmless. It drains away your plasma and you see it filling up in a bag and it’s dark yellow. Clotting Levels become an issue but it’s all monitored.

In my case the line has been inserted in my neck but still no issues other than one treatment my BP dropped. I have had the clotting factor issue a few times requiring a tranfusion the day after PE.

Has anyone been “cured” with immmune suppression?

Pik,

I don’t have the same CIDP as you but I have sensory type and no weakness so far. I do get burning in feet and reduced feelings in hands/feet. I had trouble with balance and ploped my feet when I walked. I started IVIG in November and after the first 2-day treatment my balance got better and I stopped ploping my feet. I get infusion every three weeks of 80G Gamunex. I am 65 so I have Medicare (original) and a supplimental plan which pays for my treatments. I had to have doctor switch from original prescription for home infusion becasue that would fall under Medicare D Prescription plan which would have been very expensive for me. I still have burning in feet and when someting cold is placed on foot it still feels warm. My left hand usage has improved but is still reduced. I see my nurologist in April to review my progress and treatment plan. I have moderate fatique which is worse for a week after each infusion. I drink lots of /coffee/caffeine each morning but it doesn’t help the fatigue. I am able to still work but it is challenging at times. I hope and pray IVIG will help you as it has for me. Let us know how it goes.

I get 2 bottles of infusion each of the two day treatment every 3 weeks. I’m there usually for 4 hours each day. I guess becasue i had never gotten IVIG before and my age, my doctor’s orders state not to excede 150ml drip. At the infusion center the nurses told me their normal full rate is 200ml. They infusion always starts slowly [50ml] then increases to max. This is to reduce the chance of side effects. The doctor’s orders also state to give benedryl and acetaminophen. Some patients get headache or rash/itching. THe only side effects I’ve expierenced is the increased fatigue and if i’m given the infusion at wrist or on hand i will bruise. Bruise doesn’t show until arround day 5 and last 2-3 days…no big deal. As I said, the only other side effect is the increased fatigue. I haven’t talked to my doctor about it. I’ll wait until my next visit since i read about CIDP fatigue and gather one has to learn to live with it and adjust accordingly. Fortunately my work understands and my job is such that I can schudule my workload to not do anything physically strenuous for several days after each treatment. The positive effects of the treatment do outweigh the negative. Research indicates that left untreated CIDP will continue to degrade a person’s quality of life. About 1/3 of untreated end up wheelchair bound.

I wish you all the best. I think when I was having IVIg improvement started pretty quickly. With plasma exchange I feel improvement weeks three and four. My consultant says 40% of cases go into spontaneous remission but I have given up on that! What is a big help if you aren’t one of the 40% is knowing that you aren’t alone. I’d never heard of CIDP/CISP and no-one I spoke to had either so it took a while to get my head round it. What is also a comfort is that there is treatment out there although finding the right one and frequency can be a lengthy and frustrating process because everyone is different. There is even potential remission through immunosuppressants. That’s what I’m hoping for one day but if i’m not that lucky I just have to resign myself. Let us know how you get on.

Regards O

Hi PikNIk
why did it take so long to diagnose? did you ever have a nerve biopsy?
(I myself am undiagnosed but similar loss of sensation hands feet…)

Hi, that’s the first time I have heard of a connection with the herpes virus and CISP. I’ve not read it in any of the literature either. CISP is extremely rare so I don’t think one can say most people. I’ve not been tested for it. I think you should be confident with your diagnosis because having a diagnosis is a big help and it’s not helpful if someone tells you you should have herpes too to fit the diagnosis. CIDP is on a wide spectrum. I had a frustrating time also getting a diagnosis. My GP had no idea my symptoms were neurological, I had to tell her that (I came to my own conclusion after talking to various people) and the neurologist she sent me to was a Parkinson’s specialist (and subsequently told me he had 12 CIDP patients) and yet insisted nothing was wrong. All the numerous blood tests kept coming back normal and astonishingly for a neurologist he told me I didn’t need any further tests. Luckily my uncle is a neurologist. He lives in Austria and when I caught up with him he couldn’t believe my then neurologist hadn’t requested NCS. It was quite a battle and I got a second opninion from another neurologist and then went and saw my neurologist again and when the NCS showed pronounced demyelination I changed neurologist.

Regards O

Hello,
I am late to this thread but thought I would reply. I was diagnosed with GBS in 1982, and CIDP over a span from 2000-2003. I have both sensory and motor neuropathy, but the sensory neuropathy has always been most troublesome. IVIG and steroids provided a little relief, but I stopped these in 2006. An underlying condition was not discovered until 2014, and since addressing that, I have seen some unexpected improvement. I have a rare type of carnitine deficiency that is causing several problems. When I started daily supplementation with carnitine, the sensory neuropathy improved greatly, along with balance, and time to fatigue when walking, etc. Not one neurologist offered this as a potential cause, or as a possible therapy. And I saw some well respected neurologists. My point is, rare cases will many times escape good doctors, and sometimes it pays to look outside of the specialty of neurology for an answer.

I’m curious. How is your blood sugar? I asked My Doc, if this may be a player in my CIDP. He checked my blood work and I am High normal, just under 95 for the last 5-6 tests. He kinda inferred My A1C would be low, not border line High.

I been receiving IVIG along with prednisone, for almost a year now. I have kinda hit a plateau. I am better than I was. But still looking for improvement.

I got through the loading-dose okay. on my second infusion 3 weeks later. I started noticing small bumps on my forearms, shins. This was a sign that I ignored. They got a little worse and were itching pretty bad. I was afraid to say anything about a little bumps and decided I will see if it gets bad. Well the next infusion it went into high gear.

I had to do a 3 day course of prednisone. They next tried stretching the interval to 4 weeks with a lower dose prior to my infusion, in my IV.

I have been on that for 6 months now and am slowly getting better every month.
I takes time and the treatment are kinda tailored to you condition and results.

I been to 3 neurologists before finding out about CIPD. So keep informed and don’t be afraid to ask questions.

There is a wealth of information here as well as support. Hope all goes well with you treatments! This is a long haul, but you CAN get through it!

Thank you for sharing!

both my glucose My A1c, have gone up slightly, The A1C is 5.2 was 5.0 My exercise routine is really gone down hill. I need to get back on my program. The winter makes it difficult, I joined the Y, but right after had car problems and then Snow hit. So I gotta get off my duff.

I’m pretty sure I am going back to every 3 weeks. I’m hoping that will help too. I’m also going to ask about my carnitine levels.

Well it is nice to have a place to go and compare notes!

Thank you
Steve

Yes, my CIDP symptoms stayed primarily in my sensory nerves for the last 5 years, and contributed to the very slow CIDP diagnosis, which didn’t come until a month ago.

I had progressive nerve pain in my feet, starting in big toes and steadily moving upward (all toes, balls of feet, etc.). I tried to chase down the cause with several doctors, but the diagnosis remained Peripheral Neuropathy Idiopathic (unknown).

But over the past year, the pain and progression steepened, and numbness went all the way through the top halves of my feet. Everyday felt like there was a war in the tops of my feet! I pushed and had another EMG done about a month ago, and several of my amplitudes fell to near zero. I also lost reflexes in arms. All within 6 months. This spurred my neurologist to diagnose me with CIDP and now I’m taking Mycophenalate and Prednisone, and I’ll soon start IVIg.

I feel lucky – my muscles even in my totally numb painful toes work at 90%. The downside has been that my sensory-primary impacts made my CIDP even more difficult to diagnose.

I’m three weeks into the Prednisone 60 mg and it seems clear that the war inside my feet is no longer being waged. The pain is less. I’m optimistic for IVIg to work as well.

PikNik77 – my story is similar to yours. After years of idiopathic recently diagnosed with CIDP. I sm on Day 20 of Prednisone and Dsy 1 of IVIg with brand Flebagamma.

I am happy to learn and share through the chat string that PikNik77 started. I will be back soon. I am open to questions.

One small thing – i wrote up my history on another chat string. Yours has more momentum so i will sunset mine and send anyone who replied over here. But if anyone wants to read my sensory-predominant hishistory it is at shttps://forum.gbs-cidp.org/forums/topic/any-knowledge-my-cidp-stayed-primarily-in-the-sensory-nerves-for-5-years/

Thanks
Greg with CIDP

I have apparently had sensory-predominant CIDP for six years. I studied everything i saw twelve or more docs including four neurologists. Was diagnosed as idiopathic until last month now its very very rare CIDP. As i read this chat string i realize that its even more specific snd more rare its SP-CIDP (Sensory Predominant CIDP). I also picked up on the link between SP-CIDP and Herpes. In five minutes i found the document at this link in which medical researchers believe theres a very strong causal connection between Herpes and SP-CIDP. http://www.ewg-board.eu/archiv-euromedica/archiv-euromedica-2-2014/Pages%20from%20archiv_euromedica_02_2014_maket_29_12_2014-16.pdf

I had Herpes outbreaks in my past.

Now after six years of increasing pain and numbness and as many years of exhaustive attempts – now I have a very likely answer. And I have treatments underway (Prednisone and Flebagamma).

I write all this to say: (a) thank you for your contributions to this chat string they are extremely vsluable to me; and (b) i will answer any question posed to me on this chat string to try to give back.

I know a lot about Peripheral Neuropathy. I chased through dozens of hypotheses to try to figure this out and stop my debilitating progression.

SP-CIDP i finally caught you.

Hi PikNik

Re your question about complete and constant lack of sensation:

My experience has been very similar. Over the six years and especially in the last year the numbness has become complete in my toes and front of feet. Top to bottom. Pinch as hard as you can and i wont know. Also feet are cold even in summer in winter they feel like ice cubes even under blankets.

My biggest problem though has been weight bearing pain. Even though they are completely numb if i step down on something sharp on one of my foot balls it feels like a stab. I sm not sure how the numbness and the stab co-exist. I am highly stressed walking even on carpet in bare feet. I have recently taken to wearing protectors like these nearly 24/7 -https://www.amazon.com/gp/aw/d/B00O80Y39W#immersive-view_1534793580717

Regarding my hopes for my current Prednisone + IVIg treatments:

I have to limit myself to hoping to stop the SP-CIDP progression. And the burning. I am having to accept that the numbness and the cold and the weight bearing pain will remain. My family and i are downscaling and moving to a warmer climate and a new career for me. I may come out better but after this six year chase i have to be conservative in my optimism.

Still i feel lucky i have learned how hard CIDP can hit and I am on the lucky side especially if i can stop the progression.

Does anyone have any clues regarding the differences between SP-CIDP and CISP? I see them both used apparently inter-changeably on this forum and in other medical literature.

I like to know these things and I will share what else I learn.

The difference between SP-CIDP and CISP may be trivial. But then again my chances for a positive outcome leapt forward when after five darn years I learned the differences between Peripheral Neuropathy and CIDP. And my chances leapt forward again in just the last three days when I learned the differences between most types of CIDP – and the rarer sensory predominant type which is the topic of this forum.

So i am curious.

SP-CIDP vs. CISP.

Anyone know any clues to their differences?

Hi Slate. Perhaps you will find some clues in the linked studies. CISP is a sensory form of AIDP and/or CIDP according to a study documented by The American Academy of Neurology in 2012. You can read the detailed study here:
https://pdfs.semanticscholar.org/fc11/952797e2122cf2a0fb13f18d7378d69f03c3.pdf

The study concluded (excerpt) ‘… CIDP patients differ from CISP patients in that their distal sensory nerves usually show evidence of demyelination, whereas in CISP patients, the pathologic process appears to be localized to the sensory roots (thickened lumbosacral nerve roots). Similar to other varieties (CIDP, MMN, DADS, Lewis-Sumner, etc.), the syndrome of CISP presented here may well be an additional phenotype in the spectrum of CIDP…’

Another study, from Brazil in 2013, is summarized in the link below:
https://www.ncbi.nlm.nih.gov/pubmed/23732204

Jim-LA

Thanks for sharing your knowledge your last post gave me exactly what I needed. I now better understand the differences between SP-CIDP and CISP (they are not synonymous). I now feel even more certain that my variant is SP-CIDP. I could have spent weeks figuring that out but with your help I got there in a couple days. Very generous of you. Thanks again.

I did not know there was a sensory edition of this stuff. My GBS (?) started in 1984. We did steroids at first. Then we went to Mayo Rochester in the winter.Dr Bartelson told me to go home and try PLASMA Phresis. I had over 15 PP up to 1984. I had a 15 year break and I felt good. I like to exercise, run ride or just jumping.Last year CIDP attacked again. This time IVIG was the big boy on the block. It did nothing for me. After about 8 months we decided that IVIG was not helping. My Neuro decided to go back to PP for a short period of time. After 2 weeks and 6 PP we stopped. My Neuro told me thats it I have nothing else to offer. He (A Mayo trained ,by Bartleson) that may be we should go to Mayo again (Florida the time) So within 4 weeks I’m all set to go to a more pleasant weather. My neuro did send them a letter and my hospital is affiliated with Mayo. So they already have all my hospital records. Ive got a plan to stay for three days and a life time driver to take me., I’m having autonomic systems to malfunction. The biggest on is BP. I’ve about had to argue with a good DR. He just would not listen. He was called about every other day until he’s started to allow us to medicate as we needed. He has a real hang up about PRN but he finally conceded. We saw him just last week and ask him if MAYO should examine any special areas. He said no.
So I check in on Monday Sept 15th. The only thing I don’t like about this is the nerve conduction tests. After about a half hour Im so jump I can’t even think.The nerve biopsy is painful but its over.I think they are going to try to separate autonomic system and CIDP.

Hi there- I just joined this forum and saw your questions. You and I have the exact same CIDP…sensory. It started in 2003 with numb big toes. Finally in 2007 was told that I had MS. Went with that till 2012, when I couldn’t lift my legs to drive a car. Mayo, Jacksonville finally correctly diagnosed after many tests including spinal tap and EMG.. I use hand controls to drive, eat and exercise (pool) the best I can and 3 ½ years ago get IVIG every 4 weeks. (2 consecutive days). Pain is more like random shooting pain. extreme exhaustion in legs, and little sensation in hands. So far, it is all that is being recommended by my Neurologist who is head of Clinical Trial/Research center. I pray someday they will come up with a cure/remission for me. Staying positive and living with my “new normal” life has daily challenges. I have good support and care from my partner. He helps me continually. I would like to hear how you are doing since your post!

Hi again- See my previous post explaining my journey w CIDP.

So my question is after having complete leg numbness up to my pelvis, and hands, and balance issues, my walk is getting slower and slower. What can I do to increase my walking ability? I do exercise.

My problems started in 2003, so now 16 years later, I need new options and answers. I don’t want this anymore!!

CISP is a very rare variant of CIDP and research into it is incomplete at best. However, renown researchers, such as Dr Richard Lewis, have been covering CISP. Here is an article he updated a few weeks ago that includes recognition of the variant:
https://www.uptodate.com/contents/chronic-inflammatory-demyelinating-polyneuropathy-etiology-clinical-features-and-diagnosis

The following 2017 case study of CISP treatment has demonstrated that varying IVIg dosage and frequencies can have a favorable effect on treating CISP:
https://bmcneurol.biomedcentral.com/articles/10.1186/s12883-017-0906-2

The half-life of SCIg does not differ appreciably from that of IVIg, and it can be administered at lower dosages and more frequent intervals than IVIg resulting in higher and more stable serum IgG levels, potentially improving efficacy and reducing adverse effects and ‘end-of-dose’ effect (treatment wearing-off before the next dose is due). Patient self-administered dosing is well-tolerated up to 50 grams per week administered in 2-3 injections. Studies of cost-effectiveness and health-related quality of life favor SCIg use in treating CISP.

SCIg is a subcutaneous injection/shot given into the fat layer between the skin and muscle. Many people can administer SCIg themselves; I’m not one of those. I’m overly sensitive to anything puncturing my skin and these shots really stinged me, so I discontinued them. I’ve since had a few other types of subcutaneous shots and tolerated them as long as they were given v e r y slowly. SCIg is discussed here: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2817783

Some other options you may wish to review include – Consider taking Vitamin B12 (Methylcobalamin 5000MCG) and Alpha Lipoic Acid (1200MG) daily to help with peripheral neuropathy while waiting for something other than IVIg/SCIg. These over-the-counter supplements have helped some who have suffered from GBS/CIDP and variants. HSCT is a newer treatment option (still in clinical trials) that has worked for some. You can read more about HSCT here:

Problems with IVIG

SCT Futuristic Science or down home bone marrow transplant?