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Living with Polyneuropathy CIDP, GBS, & CMT

Screaming screaming screaming

Do you ever want to just scream and scream and scream because of what disease is doing to your body and your life? The hopelessness of CIDP is dragging me down. No hope of ever getting substantially better. The constant danger of getting worse. Relapses that wipe out every bit of progress. My neuro ups my IVIG dose, I feel a little better for a while, then I crash again. I stumble around the house with a cane and feel as if I’m losing even that limited mobility. My doctor’s response? “Oh, stop being pessimistic.” But he admits he can’t offer me hope of anything beyond maintenance of the level I’m at. I know a lot of people are worse off than I am, so I don’t need that lecture. My own life is the only one I’m living, and I can’t set aside my feelings just because somebody else has it worse.

Sandra-

I’m sorry. I’m also in the exact same boat you are and often times feel like I am paddling upstream against the current.

I wish I could wave a magic wand for all of us and fix it- I can suggest what I did with my doctors but as a pt with multiple medically complex issues it may or may not work for you.

Know what is and isn’t working for you… do your research into what’s available and the dosages and success rates for those dosages. Be prepared to advocate for you and your own health care. For me it has done ok in some ways and so so in others depending on the doctor.

If you need to talk or just vent please feel free to text me

K

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Sandra and Kris:
Me too! Just spiraling down. Cane no good anymore, must use a walker, actually a rollator. Next year, who knows. Now affecting my hands so that they are weak and I can’t close one finger on each hand.
A long term relationship ended because of my increasing limitations. I was alone and not able to live on my own. I was rocked by the breakup, and also had no place to.live. A real low point for.me. I lived with a friend for a while.
I knew I needed to try to cobble together a new life somehow. I went to online dating 3 years ago at the age of 73 and I ultimately met Judy, an amazing woman. She is very good to me and really enables me to have a life. We bought a condo together and I just ran for the board and got elected. We have made many friends and are involved in many social and Civic aspects of our community.
Yes, I get discouraged about my rapidly diminishing abilities. I decided 2 years ago to discontinue further treatments because they seemed oointless. However there are two things that allow me to have the happy life I enjoy. First is Judy who makes everything possible. Second, and this is huge, I don’t have any pain. My heart goes out to those CIDP patients who suffer unrelenting pain. That changes everything.
So I get discouraged, but I am fortunate that I am still able to have a happy life as of today… Who knows what’s around the corner, Driving is becoming difficult for me and if I lose that, it will be a tremendous, devastating blow, but the present is ok.
I write this partly because I am so grateful and just need to say it out loud, so to speak. And partly I write to urge people not to give up on having a life. You may not be able to do what you used to, but you can do some things, and new things. I’m not naive - i
hope you have a partner/spouse/significant other or companion who is supportive, understanding, and helpful because that makes all the difference. I havs no advice - I’m just telling my story. Like Kris, I wish I could tell everyone of a miraculous breakthrough, but all I can offer is my wishes for permanent plateau and remission.
Mike

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I saw my neuro this morning, and he is not happy with how sick I am. This is my second major relapse in a year. He is increasing my IVIG by another 5 grams per infusion for now (so I’ll be getting 40 grams each time). I can hope, but I’m afraid this will continue until I’m entirely wheelchair-bound. I am beyond exhausted every second of every day. I feel worse now than I did when I was diagnosed. I have plenty of friends, but I no longer have the energy to go anywhere and do anything with them and having visitors is beyond me. Until I developed CIDP, I had a life, I had a career as a novelist, I traveled to promote my books, I spoke to groups and conferences. That seems like someone else’s life now. I’ve tried many times to push myself to work, but my concentration is gone along with my energy. All I can hope for is a little boost from the increased IVIG.

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Mile

Thanks for your story. Mine is a lot different. My family and my ex decided I was faking- seriously- I can sort of laugh about it now but it’s irksome that they told my children mommy wasn’t really ill- lol like I faked my LP, nerve biopsy, mri, etc. I understand it’s easier for people to believe that it’s not really happening so they don’t have to deal with the issues as they arise. I am only 52 and truthfully I don’t know what causes my pain- the CIDP stuff; the MS, or EDS which is a rare genetic disorder that affects collagen. I’m grateful you found someone who’s supporting and allows you to live your best life.

K

Saandra

I just had an extra day added to my maintenance dose- from 35gm/4 days to 35 gems/5 days. I have been wheelchair bound for several years but have the use of my hands for the most part. I am a teacher but my students have to help me with passing computer, papers, we trade and grade anything but exams; I have been really lucky for the most part in my teaching as I teach older students. One of them actually taught me I could use google doc to write on instead of using the board. I might not have the perfect life and often I feel like I want to quit- when I go home I hurt so bad I usually sit and cry even though I did not walk a step.
I own a small business so when I’m not totally wiped- ok I’m usually wipes I make my products- anyhow I think I simply have to fill every day and every minute so I don’t simply quit.

K

Kris,

If you spend time reading posts on this forum you will see that there are many people whose families accuse them of faking! That is unbelievable to me but apparently is very common. And, many relationships have been destroyed by the onset of this affliction. Sad.

You are only 52 and very attractive. Meet somebody. You really need to now, but also for the long run. I think we all need someone but especially people dealing with an illness like ours. It’s certainly not easy for us with our baggage, but you never know. Go online -There are all different kinds of people out there, many who are lonely. You don’t have to meet the love of your life, but a companion would make a huge difference in your world.

I didn’t intend to be preachy. Sorry if it comes off that way. I know you are struggling with the harsh realities of this debilitating disease and I don’t mean to minimize what you are dealing with. Neither do i think that there are simple dolutions. I just hope you can find ways to cope and to make some adjustments that may imrove your quality of life.

Best,

Mike

SsandraP and Kriss66 - Your neurologist has nothing else but for there is something that will STOP your CIDP and no more IVIG ever.

I have CIDP and CMT. I am part of the 5 to 7% of CIDP patients that it crosses over into your core and is life-threatening. It will kill me if can’t find a regiment to stop progression. For the last 18 years, I spent 7 days out of every 28 days in the hospital getting high dose IVIG to slow my disease-it doesn’t stop it. My body has always responded horrible to IVIG but without it my CIDP takes over very quickly. When I tried to spread out IVIG past 28 days, only 4 days late it took over what was left of my arms & effected my breathing more. I am in a motorized wheelchair, have no sensation or movement from mid thighs down, no sensation and extremely weak hands to forearms and have partial paralysis of my diaphram that required 18 to 20 hours of oxygen daily.
In addition to my planned hospitalizations, I have numerous complications and infections that require hospitalizations as well. I spent 208 days in the hospital last year. I have had 16 port-a-caths- the last 4 have been in my thigh run up my femerol line to my heart. The 239 rounds of high dose IVIG itself caused chronic red blood cell anemia, iron deficent anemia requiring IV Iron monthly, hypomagnesium so severe required IV magnesium 4 or 5 days a month, nausea & GI issues to point needed TPN to maintain weight and more.

Then after 18 yrs, in May 2019, I had a HSCT (Hematopoietic Stem Cell Transplant) that saved my life. -This is a TRUE Stem Cell Transplant (SCT) including Chemo same as used to treat cancers.(NOT Stem Cell Therapy or Stem Cell Treatment that have no chemo & do nothing)
True SCT’s have been used for Autoimmune diseases including CIDP, MS, & Lupus for 17 plus yrs.

It has stopped my CIDP progression cold. Since the day I finished SCT, I have had no IVIG or any medication for CIDP. For 18 yrs, nothing could stop it. SCT stopped it immediately. It can stop your CIDP. Imagine a world with no meds and not getting worse.
–I have actually had some improvement as well. I can now tighten the muscles in both thighs and lift them off my wheelchair. I use my oxygen about half as much- can go 4 hours in a row without it. After being off IVIG for 5 months, my chronic red blood cell anemia and hypomagnesium are completely gone. My Iron Deficent anemia is improving and been 2 months without IV Iron.
I hope you will consider HSCT. The change is unbeleivable.
Let me know if you have any questions. I spent 6 years trying to get SCT. I have all the most recent data and research.
Keep fighting!

Susan from NC (Note-Kriss66 I responded to something you wrote with some of this info as well)

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Kris — I have five infusions in every four-week cycle — one a week for three weeks, then two in the fourth week. I can’t have infusions any closer together, not even two on consecutive days, without horrible headaches. As long as they’re separated, I have no side effects. I get the impression, from all the posts I’ve seen in support groups, that a LOT of doctors don’t prescribe a high enough dose and patients get worse as a result.

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Susan — I can consider stem cell transplant all I want, but Medicare won’t pay for it. I’ve already been told that. It costs a fortune, and I was told the patient has to spend several weeks in the hospital and only a couple of hospitals in the US do it. Was that your experience?

It is a last resort treatment for people who are very sick, getting worse, and haven’t responded to any conventional treatment such as IVIG, steroids, plasma exchange. Medicare classifies it as experimental and unproven. I don’t meet any of the requirements.

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Stem cell transplant is not necessarily permanent. There are reports in medical journals of relapses. This is just one. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2117563/

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There are additional things to be hopeful for even in the painful grips of cidp. I was at a weekly infusion of IG for 3 years then 1 year of steroids which destroyed my attitude and excited my appetite! I gave up treatment, to try and reclaim who I was before roids and try to save marriage which I did.
I went into remission and was able to stop the expansion of pain, and regain muscle and strength and some stamina.
I am still there but declining after this 11 year trial of pain, progression and permanence.
My mind went to faith and I felt healing in church. I told my wife that I felt healing in my legs. After that was reclaiming strength and maintaining all the pain of permanent nerve damage due to demyelination. I hate my myelin sheath!
Neurostimulator is best pain management ever! It greatly reduces leg pain!!! It doesn’t help my painful feet!! Just doesn’t reach despite numerous modulation reprogramming.
Daily pain is hard! It’s the hardest thing I’ve ever done and I’m still doing it.
You can improve from where you are now!!
I repeat: your current condition does not reflect your future! There is healing sometimes. Why not you?
Today I wrote a friend that I’d give up my legs or feet if I had reason to think it might stop the pain. I’m sorry for those who struggle like me. I wish we could get together and visit about our struggles. While I’m in many ways restored, then thorn in my flesh is chronic foot pain and the meds I take barely fill in the pain hole I’m in. I never feel comfortable and have to fake enjoyment to try and break out. I feel for the first time in many years that I’m losing this battle with CIDP. This discouraged me and so I write encouragement to expect some healing and tomorrow does not equal today. Pray for yourselves and believe in GOD.

Thank you for sharing of your struggle! You are very encouraging to think of potential improvement.
What physical remnant, or thorn in you flesh, did cidp leave to you? Pain, deadness, weakness? May you continue to be restored to better health and walk without pain into your glorious future!

SsandraP----I Note your Stem Cell Transplant article is from 2007. That patient is actually the relapse of the first CIDP patient EVER to get a SCT in the world in 2002. His was done with a BEAM protocol, which is no longer used. He had a second stem cell transplant using the non-myeloablative protocol in 2007 and has not relapsed in 12 years.

World wide 92% of CIDP patients using the non-myeloablative SCT protocol have no progression at 5 years plus. If you are in the 8% that relapses, you have the same options to treat as before and if past 3 years can chose SCT again.

That success rate is far beyond anything else offered. The non-myeloablative protocol, which is used as the accepted protocol, is not as intense on your body as the previous BEAM and myeloblative ones. You have less chance of serious complications(including death) doing SCT than using monthly IVIG for 5 years.

IVIG was never intended to use for decades- no matter if subq or IV. It was intended to do 3 months stop and CIDP be stopped. If didn’t work, then was set to use 3 months or further between treatments. Everytime you use it- can cause acute kidney failure, acute red blood cell anemia, even if no problem for years of doses. It causes many other medical problems, not just side effects. So using it long term to maintain comes with a high medical cost.

Uninformed neurologists will tell you is only as last resort and should only be used if very disabled. To the contrary, the first US patient, Jennifer Osman, had CIDP only 2 years was still walking, when done at Northwestern in 2005. She got everything back that CIDP had effected. Still symptom free 14 years later.

The requirement to qualify for the clinical trial done for the last 13 years at Northwestern is- “Failure to tolerate or respond to, or an incomplete response to, or relapse after at least 3 months of conventional treatment consisting of corticosteroids or plasmapheresis”. As shown here https://clinicaltrials.gov/ct2/show/NCT00278629 . SsandaP you would qualify.

The earlier SCT done, less non-reversable damage, faster and better long term response. Once its determined IVIG isn’t going to work you can consider SCT. IVIG was intended to stop the progression and recover from the disease. So Northwestern’s definition of “incomplete response to IVIG” is if disease progresses when you stop IVIG. Ironically, you can be to effected to get SCT.

Obviously, if doing IVIG every 3 months and it kepts CIDP completely controlled SCT not for you. If plasma exchange or other milder things work- SCT not for you.

If in 4 years I have a relapse from my May SCT, that will be 4 years with no IVIG, no IVIG related medical issues and side effects and no progression. It will be a relapse from whatever I got back physically. That is success.

FYI Medicare also classifies high dose IVIG for CIDP to be unproven and experimental for more than 3 doses if it is inpatient. You can only do the highest dose inpatient because of the risks.

Few people have total relief of symptoms from IVIG, cortisone, etc. But you have to be desperately ill for insurance to cover a stem cell transplant. I’ve been over this with doctors, I’ve been over it with insurance reps. Basically, you have to be at death’s door before they will shell out the money to put you in the hospital for weeks under completely sterile conditions, obliterate your immune system, and give you new stem cells. This is not a routine procedure by any means, and we shouldn’t give CIDP patients the idea that it is easy to have done. It is still regarded as “experimental and unproven.” It’s also seen primarily as a treatment for severe MS. It would be great if we could all have our CIDP cured, but the requirements are stringent because the cost is enormous — and it’s potentially deadly if something goes wrong. I’m quoting knowledgeable doctors.

I suggest that those interested in stem cell replacement talk to their neurologists about it. And their insurance companies. Come back and tell us what you learn.

SsandraP ----Your information on Autoimmune Diseases Stem Cell Transplants is old and wholly incorrect. Stem Cell Transplants (SCT) are done for CIDP, Lupos, MS, Schleraderma, Chron’s and more. You haven’t quoted any knowledgeable doctors and your only article link was over 12 years old.
I am quoting and provided links to Doctors DOING SCT for CIDP in US for 13 years. Also attaching links to the other facts.
The reason to put current accurate SCT information here is precisely because you really think you are getting up to date info and you aren’t. By including all the links, patients can both examine all the data on their own and take printable info to discuss with their doctors if they choose.

(1) Many insurance companies have/will pay, especially for IVIG dependent patients. You went over it with insurance reps. I went through it 2 times,They can say no on phone or in policy but isn’t a binding no until they process the written request for approval for a SCT for the individual patient. If they say no, federal law requires within 3 days a Peer to Peer review and 1 more review if needed. They would rather pay $120,000 (cost at Northwestern) once, than continue to pay for long term IVIG. Humana, Aetna, BC/BS, UHC have all paid. Burt at Northwestern got Primary Medicare to pay. I was accepted in 2 different places & times. UHC agreed to pay 1 time but had to appeal the other.

(2) If your on death’s door, you won’t be accepted-it is too late. Every patient must pass pulmonology, cardiology, and hematology testing and requirements to make sure your body is strong enough to do SCT. List of specific exclusionary criteria here https://clinicaltrials.gov/ct2/show/NCT00278629

(3) For the last 13 years, this is the ACTUAL ongoing Acceptance Criteria to get a SCT for CIDP at Northwestern “Failure to tolerate or respond to, or an incomplete response to, or relapse after at least 3 months of conventional treatment consisting of corticosteroids or plasmapheresis or IVIG.” This clearly shows SCT is and has being done early into CIDP disease. This link includes the quote and is the actual clinical trial. https://clinicaltrials.gov/ct2/show/NCT00278629 ).

Numerous data shows, if IVIG or other high level treatments aren’t stopping progression, you should start considering SCT early on because "To improve outcome, treatment should preferably be initiated before irreversible axonal damage has occurred".From 2017 Autologous Haematopoietic Stem Cell Transplantation for Neurological Diseases (https://jnnp.bmj.com/content/jnnp/early/2017/09/02/jnnp-2017-316271.full.pdf)

(4) Current Autoimmune SCT protocols DON’T require weeks in the hospital under complete sterile conditions. About 30% are done completely outpatient in medical apartments near the hospitals. (Similar to Sloan Ketterings outpatient SCT program https://www.mskcc.org/cancer-care/diagnosis-treatment/cancer-treatments/blood-stem-cell-transplantation/transplantation-process/outpatient-care.) The protocol has been tailored for autoimmune diseases and results in fewer days neutropenic below ANC 0.5, when the immune system is completely suppressed and largest risk of infection. Previously was 3 weeks or more. Now about 7 days. If you do inpatient, average stay is 12 to 14 days.

Printed in 2017, Evolution,Trends, Outcomes and Economics of Hematopoietic Stem Cell Transplantation in Severe Autoimmune Diseases (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5745133/ explains in great detail everything I said above about protocols. It shows the evolution of Autoimmune SCT over the last 20 years. It includes data from 1951 SCTs. Discusses changes, results per protocol, charts of successes and relapses, long term outcomes, and more.

We should be providing these up to date SCT facts, with supporting links, to CIDP patients. The documentation empowers patients and allows them to provide knowledge to their neurologists-many, like the ones you talked to, aren’t up-to-date.

We shouldn’t be scarring them with false statements of how it is done, who can do it, what happens and false claims saying is more dangerous than other treatments. It has a Lower death rate than IVIG use for 5 years. Stem Cell Transplant for CIDP has a 0.26% death rate (including the year after transplant) vs IVIG’s 4% death rate when used more than 5 years. You have more of a chance of dying from IVIG than Stem Cell Transplant. That’s a fact. I’ve provided the links to support everything I said.