Spouse with new onset CIDP

Hi Skytim,
I am newly diagnosed with CIDP, and have a wee bit of med knowledge as well, (actually became dependant on my ICU colleagues to care for me for 2 admissions, initially GBS then subsequent admission 12 days post initial discharge with /CIDP which has been confirmed/)
From the bit I have read,(Mostly of Gareth Parry, a med advisor on this support board) and from the discussions with my neuro (and his discussions with 6 other neuro) and intensivits the first line treatment seems to be a five day course of ivig. Plasma exchange may also be an option. After this they seem to try prednisone either highish dose oral or high dose pusle therapy. Apparently there is some new reasearch results coming out soon from Dr Parry supporting this pulse dosing. After the ivig and pred which seem to be classed as the proven treatments they then look at the so called unproven treatments including immunosuppressants and cyclophosphamide, cyclosporin etc, possibly in combination with some of the already mentioned above proven therapies.
Basically from my personal short experience with CIDP, if your wife is deteriorating, she may well need her treatment reassessed. in my case if i deteriorate for 3 days in a row it means im relapsing and need treatment reviewed. If not within approx 7 days i am paralysed except for head control, and resp function.
As you will have read everyone is so different, but this is treatable so hang in there and keep going back for more assistance to your neuro.
Good luck, and all the best
Kia Kaha (stay strong)
kathy

Sorry Skytim,
forgot to mention re some of your questions, as far as the conduction blocks i am sure someone here will set you straight, my understanding though is that a block indicates there is inflammation also hence the need for some form of prednisone.
In a nutshell my emgs and conductions tests showed blocks and prolonged f waves etc indicating, demylenation adn inflammation.
I have also had sensory symptoms, more severly of late during my last relapse, i wasnt that aware of extent of loss of sensation as is gradual and once hands lose sensation you cant regulary or accurately check yourself (unless use the pinprick test etc ) Had burning and tingling in hands and feet at times and a few days prior to my worst i lost full sensation for full length of arms and legs. couldnt feel pin ***** or hot or cold etc up to thighs and alsmost shoulders.
As you ask about response time to ivig, usually on day 2 am a bit worse, day 3 no worse and day 4 showing slight increase in strengh and all upwards from then, saying that the last relaspe wsnt typical, the whole five day course just held me with no improvement really then after that, and while changing off o pred onto pulse methly pred i went on huge dive into full paralysis so started back up on anther 5 day course ivig and then twice weekly ivig of whcih i have reamined on along with starting the pulse methylpred and immunosuppressant. all good so far!!!!
hope this helps. look forward to hearing of similar success for your wife.
Kathy
I know you will read this as my own experience.

Hi Skytim, Welcome to The Family. Everyone responds to ivig differently. Be Patient. Treat her pain now! Is she on any kind of pain meds? Neurontin, Lyrica, or antidepressants work well on nerve pain. The pain issue can have alot to do with how she is reacting to ivig, pain drains the body of energy-which is in short supply as it is in a cidp person. Exercise at a min time while in such a state. But keep doing it as it will also help with recovery. Rest big time. Please feel free to ask as many questions as you have or just vent when you need to. The dx sounds about right. How are your wife’s b levels, potassium levels etc?

Hi,

I’m sorry you and your wife are going through all this, but congradulations on getting such a quick diagnosis. It might not seem like it, but given how many months/years it takes others, please find comfort in the fact that your wife has the best chances at reversing some of the damage since she was diagnosised so quickly.

As for IVIG, when I first started getting it nearly a year and a half ago, I remember the main difference, at first, was I could climb a flight of stairs without feeling like my legs were going to fall off. That’s it, just stairs. And it’s gotten better from there – but very slowly. I’ve heard it can take up to two years of IVIG to take effect (though I don’t know if it’s true), but certainly in my experience, my docot wanted to wait 3 months to see what was what as far as it working.

All medicines need time to build up in your bloodstream and take effect – it’s why tylenol doesn’t eleviate a headache the moment you pop it in your mouth. Don’t give up hope, get her medications if she’s in pain, and good luck to you both.

though I truly wish it was for different reasons?
Also, it is unfortunate that you have had to learn about this disease in such a personal way. What IS fortunate tho, is that your wife was diagnosed so rapidly and received IVIG so soon after ‘onset’. It could be that the doses she is getting are not enough or a whole slew of other issues could be interplaying with treatment. Many who have CIDP also have other immune issues and it can be a case of which came first? or, Who’s on first?
I first when I’d been hit with this, was mis-diagnosed, I was not internet-wise at the time and followed my own instincts in the ‘This is NOT right!’ vein and got second opinions until I found pro-active physicians who did the testing [as your wife has had] and received IVIG treatments. It took me over a year to get that diagnosis. Damage had been done, but is improving now over 4 years later.
Web up the following: ‘LizaJane Neuro lab sheets’ and you find a menu [of sorts] put together by many Peripheral Neuropathy sufferers and many of the possible tests and labs that go into a diagnosis. If nothing else, the worksheets help one keep track of all the tests over time? I only discovered this info over a year after I was ‘smitten’ with this all. It did truly tell me that my current and diagnosing doctors did ‘all the right’ things!
You and your wife are near to my heart and prayers.
Kathy, Dana and Cheryl are both right about checking the whole metabolic panel including thyroid [autoimmune] aspects.. I like to think of it this way…I have a wide range of my immune system going haywire! The IVIG keeps it from getting worse and also a bit better! It is hard to ‘think positive’ when such a devastating thing occurs, but other options aren’t acceptable!

Hi Tim,
my husband’s CIDP started similar as your wife’s, none of the treatments worked in his case, docs didn’t have enough evidence to diagnose him either then CIDP. After 2 years he’s ordered ( THE NEURO ) a PET scan which showed a lytic lesion in his sacrum consistent with a plasmacytoma- a type of blood cancer- which can cause severe neuropathy in other words it is a different syndrome which very often is being wrongly diagnosed with CIDP, called POEMS syndrome.If you are interested to read about this syndrome on the net, I wanna let you know that my husband doesn’t have any other symptoms that this syndrome generates. We are still waiting for the results of the bone marrow biopsy to confirm plasmacytoma. Now I don’t wanna say that your wife suffers of Poems Syndrome, all I wanna say the immune diseases are getting very easy misdiagnosed and in atypical cases is better to investigate further or to ask a second or third or n opinion . My heart goes to you both and I wish you the very best of luck.

I found IVIG effective when first diagnosed, but somehow after 3 months it had no effect at all.

Plasmatheresis works well for me, and a good dose of methylpresnisone inbetween.

Also im not getting more anti inflamatory natural foods into me. green tea and honey. plenty of fresh air and natural health sups like immune boost berocca and these garlic and zinc tablets…

ST,

Please send your wife my regards.

We have a 3 year old with CIDP. Onset about 5 months, dx. at 19 months, therefore much damage was done before dx. He has never walked on his own (only about 10 feet now) but uses a walker.

We have a great ped. neuro. that could not figure out why ivig was not working. We did it for 1 year, it worked off and on. Now, he’s on solumedrol each week and ivig every 4 weeks.

He has made great progress. Remember, each patient is different. What might help one, might not help the other.

Good luck and never, never give up. We’ve learned that from going to dr to dr to dr.

If you have any questions, I’ll be happy to try and help.

Love, Lori

ST,

I read the post before mine so I wanted to put in my 2 cents about solumedrol vs. pills. Dell has not experienced the pie face with solumedrol nor the weight gain. That is HIS experience. Also, one of the leading dr. in the country on neuro. muscular at Johns Hopkins, Thomas Crawford, believes the solumedrol is better than predisolone.

Good luck,
Lori

My 6 year old daughter has CIDP, dx’d at 4. She’s had too many IVIG treatments to count & was treated briefly with oral Prednisone. She has all the side effects from the Prednisone without any of the benefits.

I’ve researched treatment options and I’ve come to the conclusion the IV steroids (Solumedrol) are safer than oral Prednisone. Oral Prednisone should be used as a short term treatment option due to it’s side effects. It is sometimes not absorbed into the bloodstream & therefore isn’t really worth all the side effects that come with it.

Solumedol has fewer side effects, can be used long term, and is more easily absorbed (obviously as it’s an infused steroid).

If I ever had to make the decision about giving my child steroids, I would go with infused as opposed to oral.

Since your wife is newly dx’d, I would hold off on any steroid treatment. Steroids should not be given to someone with GBS. You want to make sure that she absolutely has CIDP before going that route.

My daughter responded to IVIG quickly but required a lot of it to stay stable (sometimes over 150 grams per month!). Some people take a little longer. Most people start off with 2k per gram over 5 consecutive days. There has been some research done that women, in particular, should have the 5 dose treatment every other day as opposed to consecutive days. However, I find that the consecutive treatment always worse better for my daughter.

Personally, I think treating your wife every 2 weeks is a good idea. Most people do end up going onto a treatment plan of 1kg per gram every 4 weeks. I think it’s worth it to be more aggressive in the beginning of treatment.

I used Google to learn about CIDP. You can find out anything you need to know by doing that.

Also, I urge you to encourage your wife to take something for the pain. It can be unbearable & there’s no sense in her suffering anymore than she has to.

Good luck,
Kelly

I was diagnosed with CIDP in sept. and was immediatly treated with 2 rounds of IVIG over a 2 day period, am not sure what the dosage was but it took just over 12 hours for each IV to run, then my neuro. switched me over to Solumedrol infusions, I think they did 1000 mg a day for 4 days then started in on 1000 mg a week, they started out doing it all at once then when I didn’t respond the way they wanted they switched me over to 500 mg twice a week and that seems to be doing the trick. So far the only side effects I really see are that I can’t sleep on infusion day and for about 12 hours after my infusions I’m am so hot that I feel like I am sitting in a suana. I think he must be onto something tho….. When they started the solumedrol was totally paralyzed from the neck down, came home from hospital 2 1/2 weeks ago barely able to walk with a walker……..now am walking inside the house totally unasisted and the only time I need to use my whelchair is to go to the gocery store or places like that….other than that I just use my cane…..:D

Skytim,

I am sorry that Frani has this disease. With some luck, it will be manageable. It may take a while to learn the treatment(s) that will manage it, though. It is tough to go through; I can offer information and an understanding ear.

On the question of IV solumedrol vs. oral prednisone, it seems that most consider IV solumedrol superior, as it seems to have fewer side effects. I don’t know about oral methyl prednisolone vs. oral prednisone. You asked about initial dose and length of trial for steroids. I think I was given a typical regimen. I received 1 g IV solumedrol each day for 5 days, followed by 1 mg/kg of oral prednisone every other day for four weeks, then starting a 10 mg per week taper. So the steroid trial runs for about 5 to 8 weeks, but perhaps longer. In the long run, prednisone alone was not enough and I now have a combination therapy of azathioprine, prednisone, and plasma exchange. I will PM you about this therapy, if you wish.

I think that administering prednisone as a double dose every other day is a big improvement over a single dose every day. The adrenal glands don’t atrophy as badly in the every other day regimen. Keep the dose as low as possible, of course, but don’t go over 2 mg/kg every other day. If the side effects are severe, they taper as quickly as tolerated until they are manageable.

I think most people would agree that you should keep to IVIg alone for 8 weeks. There are several protocols. This site ([URL/http://www.neuro.wustl.edu/neuromuscular/antibody/pnimdem.html#cidp[/URL]) has two. The Peripheral Nerve Society has CIDP guidelines available on their website ([URL/http://www.pns.org[/URL]). Another fairly common protocol is 400 mg/kg/day x 5 days; followed by 400 mg/kg once per week for several weeks. Not to rain on the parade, but it is possible that Frani won’t respond to IVIg; about 30% of patients don’t. Depending on your wife’s vascular access, the next treatment would be to try would be either plasma exchange or steroids.

As for pain, I found nortriptylline effective, but I did not like the side effects, so I switched to gabapentin. It has different side effects, but I find them more tolerable. I trade some pain for a lower dose, as I found gabapentin slowed my thinking. If Frani’s pain is worse in the evening, I would suggest 600 mg at about 2:00 pm, 600 mg more at about 6:00 pm, and another 600 mg at bedtime. Depending on how severe her pain is, she may need to start earlier and take 2400 mg total per day. Read the PI on the drug, as you, Frani, and the neurologist will need to figure out what works for her and the information in the PI is invaluable.

Godspeed with an effective treatment,
MarkEns

Hi ST,
I missed how long she has been symptomatic. My 10 y/o had the same jelloie feeling. After his second day of ivig he was able to run around. True atrophy is a concearn, but how long she has been down for would be relative.

Maybe you could start moving her legs for her if it is a concearn. It might even help her from a confidence issue when she sees the concearn and caring when YOU help her.

If you do PT, I personaly would be sure the person has experience in working with gbs/cidp patients. Overworking only causes more fatigue and stresses the nerves that are still working. Other nerves form other areas are now trying to pick up the job of the affected nerves. So if you over work these helping nerves, now you have even more fatigued areas. (sorry if I confused you) Additionally, I noticed that when we did pt for month, that in the beginning my son would get disappointed with how badly he had lost stregnth. In the clinical setting with other patients far worse, it was very depressing for him. The pt we had was very kind to explain all of the exersizes to me and we did them at home and only returned to the rehab but once a week for eval and new exersizes.

I do not know the facts of your wifes condition, so what I have explained may not even be an option. I wish you luck in this journey. Exude love and patience to your wife, I am sure this will go miles in helping her to recover.

Sincerely,
Dawn Kevies mom

ST,
I am so sorry, I read your initial post. I did not realize you are a doc. I hope I was not too condescending. Something of interest that I did notice is that it does not seem like you did the full loading dose. Perhaps I read wrong. Upon my son’s first symptoms, tripping, to the worst, bareley able to walk, could not hold a pencil, wash rag, or even button or zipper his pants, to treatment, a total of aprox 6 weeks passed. At that time we did a full loading dose which consists of 2g/kg over 3-5 days consecutively. Unless I have misread, it appears as though you are spacing them weekly. If so, weekly would be a maintanence dose AFTER a loading dose. If you have time, read my post of recent regarding ivig for Kevin is over, but confused. Maybe it could give you some info regarding tx and symptoms.

Since you are a physician, maybe you could have some “pull” so to speak and make contact with a Dr. Saperstein. He is on the board and runs a clinic out of Arizona. He also writes for the gbs/cidp foundation news letter. Contact the foundation and have them send you a packet. There is a thicker white book that has so much info! It is like a minny manual for gbs/cidp. The info is astounding, it explains all of the variants, treatments and lists doctors. I highly reccommend it! One year later I still refer to it. Good luck to you! Your wife will come through the other end! Kevin has. It has been a year and a month since our dx and Kevin is doing much better. There have been hurdles along the way as there was speculation as to Kevin having gbs or cidp. Now we know.
You mentioned your wife has issues with accessability to her veins, Kevin just had a port placed a week ago and received the start of our New loading dose 3 days later. We completed the tx Thursday, even with recooperating from the surgery, he was better w/in 48 hours of the start of treatment. THERE IS HOPE AND THERE IS PROGRESS. There may be bumps along the way, but you will get through them. At least we have a medicine for this illness unlike other illnesses. For that I am thankful.
Dawn Kevies mom

I’m not sure how often she will receive ivig but if her veins aren’t the greatest, I would consider a port. I had to plead and beg our neuro. to get Dell’s port but he’s had it since April and it’s heaven.

I only have 1 nurse access it (we do home health) and I can access it. I know you are a dr. but I’m not sure your specialty and we are all in the learning process with this horrible disease.

I’m glad she’s doing a little better.

Lori

PT is very important for keeping the muscles from atrophy. at least doing a small amount of range of motion daily helps that. I have no feeling in my left leg and the right is almost that bad now. doing some range of motion daily has helped me not atrophy but can be exhausting so that is why I do most of it in our hot tub which my physiatrist ordred for my, it is much less exhausting doing hydro. it also helpd with my fibromyalgia. the days i am able to get into the tub are nights hat i sleep a little better. hope this helps a little about the therapy issue. also even though my therapists had never dealt with CIDP they looked up as much info as they could efore beginning so mention that to the ones you work with.
thoughts and prayers are with you
Jody