CIDP maybe?

    • Anonymous
      February 13, 2008 at 8:48 pm

      I am new to the forum. Thanks for the opportunity to share my story. I have a “demyelinating neuropathy” that my neurologist called CIDP this week. I’ve seen this doc 3 times now and was started on CellCept, and have previously been on IVIG for Common Variable Immune Deficiency. I have significant transfusion reactions with IVIG. I also have had severe side effects to steroids (with avascular necrosis, adrenal insufficiency etc).
      My question is this: My doc is from the local community and has excellent credentials. I, however, am progressing fairly rapidly and wonder if I need to seek a second opinion in a research center. I live in the Atlanta area.
      There is some hesitation to increase my dose of IVIG, and the communication is not great between the doc and myself (even though I am in the medical profession.)
      I feel a sense of urgency about my deterioration in the last month. I have progressive leg and arm weakness (ascending) with bad paresthesias. Actually, I had a muscle biopsy last week because of the dramatic muscle weakness. I have “severe neuropathy” per the Nerve Conduction studies.
      Any thoughts? Has anyone been treated by Emory University Neurology in Atlanta?

    • Anonymous
      February 13, 2008 at 10:05 pm

      I just wanted to wish you well. I take Imuran well the generic verision. The bad thing about the immune suppressents is they can take up to 6 months to start working. Has your Neuro considered Plasmapheresis (Plasma Exchange)? Good luck

    • Anonymous
      February 13, 2008 at 10:21 pm

      Thanks for the note:
      No one has suggested Plasma Exchange. I am going to ask the docs to increase the IVIG. I’ve only received 10 grams once (this week) and I’m not scheduled again for 2 weeks.

      Are there are specific lab tests that can identify what antibody is attacking the myelin? If so, are they primarily done in a referral center or do community-based neurologists have the ability/knowledge to order them? It seems to me that CIDP is a diagnosis of exclusion….

      Thanks, and God bless

    • Anonymous
      February 13, 2008 at 11:07 pm

      Hey Melissa,

      Nice to meet you and wish it wasn’t under these circumstances. Your IVIG dose is usually based on weight. There are several tests your Neuro. can run to see how you are doing on treatment. If I can ever help or you have a question, my email is [email][/email]. Feel free to contact me.


    • Anonymous
      February 13, 2008 at 11:08 pm

      I was dx with CIDP at Emory back in 2003. Dr Linton Hopkins was my Neurologist.The whole process was pretty straightforward and I really did not have any complaints. At the time he dx me with a mild case of cidp, and did not put me on any meds. I have since moved to South Carolina and am now seeing a local Neuro. Overall, I was pleased with my experience at Emory, but if I was starting over again I would look at UAB (medical university at Birmingham) There is Neuro that has a lot of background with CIDP. I don’t remember his name right off, but I could find it if you are interested.
      Hope you find a Dr. that you feel comfortable with.

    • February 14, 2008 at 11:11 am

      Hi Melissa,
      My son is now 11 and weighs 114lbs. He gets 25grams a day over four days once a month. (2g/kg) Ten grams is NOTHING, that would be like using a garden hose to put out a wildfire. What reactions did you have to the ivig? Was the flo rate low enough? Kevin’s max flo rate is 39. Some adults on this site have 50 as a max, some go as high as 200. Pre meds also assist in keeping the reactions down. (benadyly,tylenol, some even get a low dose iv solumedrol prior to infusion).

      You asked about a blood test for a specific antibody, I do not recall Us personally getting that test. Our confirmation was a ncv/emg, clinical presentation and finally a spinal checking for elevated protein. There are blood test that check IGg,IGa levels so that you can see what ivig is best for you (s/d,liquid) There are also other blood tests to determine some varients, but I know nothing about them or their names, as this was not something we did, but others will chime in.

      I guess since you are rapidly declining, I would ask the doc to do something asap. If you are hesitant about more ivig, pp is an option. Usually, ivig, ivigw/ steroids, pp, then some sort of immunosuppressant. You mentioned being in the med. field. Immunosuppressants may be difficult, since you are around others who are ill. Besides, they are a last resort, and I personally would give ivig another try.
      Regarding a doc. Try Emory, I know there is a peds doc there that is on the gbs board, we were going to see him while we were in a state of confusion, but we did not need too. I am sure there would be others there that could help. Maybe you could call his office and ask for a referal. (Dr. Sladky) Good luck!
      Dawn Kevies mom

    • Anonymous
      February 14, 2008 at 12:04 pm

      My daughter is 6 years old, 50 lbs & gets 20 grams of IVIG every 2 weeks. 10 grams isn’t going to touch anything when you are in a flare. Usually a person will get 2g per kg for a loading dose then you can find a maintenance schedule which is suitable for you.

      What problems do you have with tolerating IVIG?


    • Anonymous
      February 14, 2008 at 6:02 pm

      Hi Melissa, It doesn’t sound like you received enough IVIG, My son is 9 and he gets 50grams in a 5 day period, the infussion rate needs to start out slowly, he starts at 18 and ends at 187. We start the week with Benadryl, this helps with any alergic reaction,he also takes tylonal every 4 hours for the 5 days. I sure hope you can find a neuro that you feel comfortable with, you can email me if you would like. [email][/email]

    • Anonymous
      February 16, 2008 at 5:55 pm

      [COLOR=black]Welcome Melissa, although we wish you did not have CIDP. [/COLOR]

      [COLOR=black]If your neurologist were to do a typical IVIg treatment, it would start with 2 g/kg, spread equally over 5 days. If you weigh 60 kg, then you would be getting 120 g of Ig, or 24 g/day. I am not a doctor, but if you already know that you have significant transfusion reactions with IVIG, I would be very careful about getting lots of IVIg. Perhaps the problem is related to the brand or the infusion rate. You might discuss the problem with your CVID doctor. Assuming you could overcome the infusion problems, you should immediately talk to your neurologist about your dose.[/COLOR]

      [COLOR=black]After this “loading” dose, it is a wait-and-see game. If you stop losing function and instead gain, he would probably put you on a “maintenance” schedule of IVIg. If not, he might try another couple of loading doses. The maintenance schedule would be tailored to your response. A typical schedule is a dose of 0.4 g/kg every other week for a couple of months, followed by 0.4 g/kg every four weeks for many months, then trying to lengthen the time. [/COLOR]

      [COLOR=black]If you have problems with steroids, then they are not really an option for treating CIDP. The typical course is to start with pretty large doses, between 0.5 and 1.0 mg/kg per day.[/COLOR]

      [COLOR=black]Plasmapheresis is a typical option for CIDP. Because not many of the white blood cells are removed, I would think it would be OK for someone with CVID, but I would check with your CVID doctor first. Plasmapheresis requires good vascular access, either by having really good veins or a central venous access device.[/COLOR]

      [COLOR=black]As far as a lab test, no, I don’t think there is. Still, you should be checked for anti-MAG antibodies (myelin-associated glycoprotein). Depending on how knowledgeable your doctor is he will have already done this test. He might have requested various electrophoresis chromatograms. He will not be able to have them done in your community, but there are labs throughout the country that can do the tests. [/COLOR]

      [COLOR=black]Your real question, though, seems to be should a specialist at a large institution see you. It really depends on how comfortable you are with your local neurologist and how knowledge he is with CIDP. Ask if he knows about the PNS guidelines and if he could find them in less than 10 minutes. If he can, he is probably pretty up on his knowledge. Still, if your insurance will allow it, a second opinion will not hurt and could be helpful.[/COLOR]

      [COLOR=black]Godspeed in finding a treatment that will work for you.[/COLOR]

    • Anonymous
      February 16, 2008 at 8:56 pm

      [QUOTE=Jerimy]Hey Melissa,

      Nice to meet you and wish it wasn’t under these circumstances. Your IVIG dose is usually based on weight. There are several tests your Neuro. can run to see how you are doing on treatment. If I can ever help or you have a question, my email is [email][/email]. Feel free to contact me.

      I am currently on IVIg every other week. What test are you referring to to see if my treatment is helping? My neuro went soley on my response on whether I felt I had more strength or not. Feel free to contact me via (email)at [email][/email](email). Thanks, David.