questions from recently diagnosed CIDP

There is a great deal of variation from one case of CIDP to another. The rate of progression can be very slow or relatively rapid (a few weeks). Response to treatments also varies. It often takes some experimentation to find the treatment protocol that works best for you.

IvIg is a standard treatment for CIDP, so should be considered a medical necessity if your neurologist wants to do it. Unfortunately, some insurance companies try to weasel out of their obligations at every opportunity. I was fortunate to have very little difficulty with insurance coverage. I had two five-day rounds of IvIg which were covered, even though they were not of much help. If you need IvIg, but have trouble with side effects, you can try reducing the infusion rate, or changing brands if possible. Also, there are medications you can take when having IvIg which make it more tolerable.

Prednisone is generally used as a short term treatment, starting at a full dose, then tapering down to zero over the course of a year. I followed this regimen with no adverse side effects. While taking prednisone, I started taking mycophenolate mofetil, an immune system suppressant. This takes awhile to have an effect, but by the time the prednisone is dropped, it is sufficient by itself.

You definitely should try to remain optimistic. My case looked pretty bleak. I continued to deteriorate after IvIg. I spent about two weeks in the ICU and was a quadraplegic for a few weeks. I was a paraplegic for a few months. I knew recovery was beginning when I could lift one finger independently of the others.

It was a long road back, but today I walk without any assistive devices, I do not require pain meds or IvIg infusions, and the only medications I take are mycophenolate mofetil and two optional nonprescription supplements. I am not fully recovered — I can’t run, I’m still a little unsteady on my feet, and my feet hurt most of the time. I’ll take it. I hope your recovery is as good as mine or better.

It is important to watch for signs of relapse. It is important to distinguish from general exhaustion and relapse. Exhaustion is typical of CIDP, and will come from overexerting yourself. You must have physical exercize, but must pace yourself to avoid exhaustion and to rest as needed. Relapse is not merely tiredness, but progressive weakness specific to affected nerves and muscles. You need objective ways of monitoring this. Your neurologist does this when he tests your strength in various ways. You can do this at home very easily with small weights and elastic therapy bands. If you have a persistent loss of strength, evidenced by inability to lift a given weight in a particular way, then it could be a sign of relapse, which calls for an examination by your neurologist.

I know that I am in remission because my ability to lift weights has not declined, my balance has improved, and my pain has lessened, even though the improvement has been very slow. But I take nothing for granted. At the start of every day, I try out my legs and think about whether they seem as good as the day before. I do a few exercizes now and then, and test my balance. Then I just go on with my usual activities. I am very fortunate in that there has been no backsliding so far.

It seems that you are still trying to arrive at the best treatment plan for you, which will lead to the best recovery. I wish you the best and urge you not to give up hope.

I recall that there was a period when I felt chronic fatigue before I began having noticeable symptoms of weakness in the legs. I am sure now that this was from the early stages of the disease. I don’t remember how long this went on, but it doesn’t matter as the timing would vary from one person to another.

By the way, the nerve biopsy is generally used to support the diagnosis when there is uncertainty about it. As you have had all the usual tests except this one, and your symptoms are consistent with CIDP, I expect your neurologist is confident of the diagnosis. Your insurance company has no business asking for another test, especially an invasive one, if your neurologist doesn’t need it, in my layman’s opinion. Every medical procedure has an associated risk.

jane—you shouldn’t  have to have a nerve biopsy just for your insurance company to cover your treatment, but if your Dr suggests it’s a good idea or for some reason you do need one, just wanted to let you know I did have it done at the Mayo in MN.  They used my sural nerve as my feet are the weakest.  It really was not that bad, I had it done under local anesthesia, was able to walk on it immediately after.  There was some nerve pain in the area after for a while, but not bad.

I know everyone’s experience with it is different. I opted to go through with it because I was already in MN, figured  Mayo would be one of the best places to do it.  I actually had less problems with the nerve biopsy than i had with the lumbar puncture.

I do have an atypical case where it was suggested to help with diagnosis.

sorry i can’t help you much with your questions on relapse,recovery etc. Im a little over three years in with this diagnosis. Have tried prednisone, ivig, plasma x/c, rituxin and now am currently back on ivig.   The ivig seems to slow down the progression, but nothing has helped me gain back strength or sensory loss  so far.   it is discouraging and can take time to find treatment that works.

I just recently started with a new neurologist I found online listed as a cidp center of excellence (st elizabeths in Boston).  I also got accepted to Chicago for the 3 day consult to see if i am a candidate for a stem cell transplant.   Wish i had applied for this sooner, i kept hoping the other treatments were going to work……oh well hindsight.

It is a hard disease to deal with and normal to wish for your old life back—-I go through this a lot too.  Seems like most of us that have this were avid exercisers and led very active busy lives prior.    Best of luck in getting the ivig approved by your ins co.  im still in the appeal process for the one set of  rituxin infusions i received back in June for $17,000 that they did not deny until after i received the infusion.   insurance companies are just so joyous to deal with!!!

As I wrote, I transitioned from prednisone to mycophenolate mofetil (CellCept). MM is a long-term replacement for prednisone, whicj I took for only a year. My neurologist does not like to use prednisone for a long term, because of the side effects. MM is an immunosuppresant drug.

A “typical” pattern is for muscle weakness to be bilaterally symmetric, and to begin in the legs and progress upwards. Any diversion from this could be called “atypical.” Rate of progression also figures in. GBS reaches its maximum extent in eight weeks or less. CIDP tends to be much slower, typically developing over several months, and following either a relapsing course, alternating between weakening and recovery, or a long, weakening progression. My case is atypical in that I reached maximum weakness in eight to ten weeks (I was originally diagnosed with GBS), then have recovered steadily for about two years without relapse. There is a great deal of variation, and treatment must be individualized.

Jane12,

I believe it is possible to have a very mild CIDP for years before a major episode of symptoms. For at least 8 years before my major episode, I had trouble getting up from a crouched position. I thought at the time that it was just age, fat, and deconditioning. It may have been partly that, but I now think it was a low-level CIDP. My major episode was not as bad as many that you will read about here, but without treatment, it would have been much worse.

As to treatment, as I said in another post, IVIg is the standard treatment, so your insurance company should cover it. However, any treatment that works is better than none at all. If you are in fact stable, you might be able to wait a bit. Make sure you are not getting worse, though. Keep a diary of symptoms and review it every few days. If you find things are worse this week than last and have not resolved the problem with insurance coverage for IVIg, I would recommend starting prednisone. Yes, the side effects can be severe. However, by not arresting the inflammation and demyelination, you leave your nerves more susceptible to damage to the axons.

As for stress, people react to it very differently. Many people here have seen a correlation, sometime a very strong one, with stress and severity of symptoms. My major episode was unrelated to stress. Once I was treated, I had very rapid improvement, returning to acceptable, if not full, function within days.

I was not physically active before my major episode, so I don’t have the benchmark you have. I was left with some weakness, such that it is awkward for me to get down to the floor or up from it. I have some minor pains and other deficits. But that is OK, because I can do nearly everything I want to do. I am content to be where I am now.

Godspeed in finding your treatment soon,
MarkEns

By the way, here is a link to a recent newsletter of the Foundation which contains an article on recommended exercises for rehabilitation from CIDP:

http://www.gbs-cidp.org/newsletters/pdf/Newsletter_Summer_2010_Med.pdf

Just diagnoised this Feb, landed in Seattle Swedish Cherry Hill.  Medically stabilized (5 days) and then transferred to the Swedish Intensive Rehab.  Made amazing progressive thru Feb early March and was scheduled to go home 3/18, but had a major relapse starting 3/14 and continuing to today.  It really has gotten to me. The Docs here seem to think it was caused by reducing my predasone dosage and maybe the start of a preventive antibiotic Dapsone.  The upped dosage of predasone back to 40 mg orally per day and stopped the dapsone and are considering adding Imuran.  I have went from independent to dependent and after a month plus I believe I am out of benefits and just don’t know what to do or anything –  a very scarey time for me.  Please advise (1st posting here)

JohnQ, this should probably be a new thread.

sorry to hear what you have been going through… Im not sure if the fact that your ankles are strong that would affect your nerve biopsy – I would think it would though.. Did your EMG/NCV show demylenation/typical CIDP??    I hear the results of the nerve biopsy can be patchy- I would think if your ankles aren’t weak it would increase the chances of the biopsy being normal dispite you having a disease process going on.. but who knows!?!   They did an epidermal nerve biopsy on me (it is a less invasive skin biopsy).. I guess it’s a new way of doing it. .. that’s what helped them with my diagnosis.. it was very minimally invasive – they jsut take a punch biopsy of three areas (ankle, calf and thigh).. mine came back significantly abnormal.. I think it was then that my neurologist said – huh, something weird is in fact going on- im not just totally crazy! 🙂

I get IVIG.. I tried only three days of prednisone, they started me out at 60mg, which was just too much for me – other people on here might be good to ask if they started out at lower doses and worked up, or higher doses and tapererd down.. The 60mg was too much during an exacerbation and I felt it worsened me.. I wish I would have tried lower and worked up. Otherwise, I hear great things about it working great, especially along with an immunosuppressant like cellcept or immuran . Good luck!

ahhhh.. same thing happened to me!! wrote a long post and it got deleted …. I am SOOOO sorry you are going through all of this.. I remember just waiting for IVIG.. I was waiting for ANY treatment, and it seemed like the dr’s didnt’ care, ore didn’t realize just how fast i was declining.. I felt like I was going to wither away – i coudln’t take care of my kids, work, hardly eat, you name it … it was devastating..

I also had that heavy concrete in my shoes and hands.. it was so awful.. I”m glad they aren’t making you get the nerve biopsy, but am shocked especially if your test clearly show CIDP why insurance isnt’ approving it yet?  Did the prednisone seem to make things way worse? Have they talked about other immunosuppression/cellcelpt etc?    So sorry you are going through this.. I really feel for you.. I’ve been there

Immunosuppresants generally take weeks to months to reach maximum effect. If you are declining rapidly, you need the five-day treatment of IvIg promptly. If you continue to decline, you may need more IvIg or even plasma exchange. I had two five-day regimens of IvIg, continued to decline, then had plasma exchange. By the time I received PE, I was already mostly paralyzed below the shoulders. If you are getting weaker at a noticeable rate daily, you need more aggressive treatment as soon as it can be arranged.

You may have to do the biopsy then, but the insurance company is just hoping for a loophole, in my opinion. The AAN does seem to have a “mandatory” criterion of a nerve bopsy, but this appears to be for research purposes. It is not usual for neurologists to require this when all other indicators are present. I am unable to find a link to the AAN document, because it is available by subscription only. However, here is a link to an interesting report on the usefulness of the nerve biopsy:

http://www.bioline.org.br/request?ni10145

Good luck to you. I hope you get the treatment you need soon.

From the Foundation newsletter:
“Established Therapy of CIDP”
http://www.gbs-cidp.org/newsletters/winter98established.htm

“Corticosteroids have been the mainstay of treatment for CIDP. Retrospective analyses document improvement in 65 to 95% of CIDP patients. To achieve this benefit, high-dose Prednisone has to be prescribed for a minimum of six months (initial dose 1 mg/kg. body weight/day for 6 to 8 weeks, subsequent tapering by 10 mg/month and change to alternate-day dosing). First signs of improvement were seen at mean 1.9 months and maximum benefit was reached only after a mean 6.6 months.”

This is treatment for a slowly progressing course, not a rapid one. If you are getting weaker daily and cannot curl a one-lb weight, I am surprised you are not already in a hospital preparing to receive IvIg. When I was hospitalized, I had no use of my legs, but could still push my wheelchair around. Eventually, I lost even that.

But here’s an encouraging note: two years out of the hospital, I walk without a cane or any other assistive devices. You need to get proper treatment soon to maximize your potential for recovery.