MarkEns

Your Replies

  • March 5, 2012 at 2:51 am

    Hello Lori,

    I can only comment on my case, but it was clear that PE alone was not going to do the job and that I needed something else. PE worked very well for me, returning me to nearly full function; it just did not last for any reasonable length of time. I wanted to stay away from steroids if I could, so CellCept, with the shortest induction time, looked like a reasonable choice. Unfortunately, it did not work out, so I went with the regime I posted above. If I have been using IVIg and working at nearly full function, no, I would not have added an immunosuppressant. If the IVIg was only keeping me from getting worse, I first would try a very aggressive IVIg protocol, perhaps getting loading doses every other week for a few months (my insurance would have allowed it). If that did not work well, I would then add an immunosuppressant.

    However, like GH said, you need to discuss these options with your neurologist. My opinion, as a well-informed patient, is that your IVIg protocol could be more aggressive. Yes, it would be more aggressive than typical, but there is reason to that it might be beneficial. There may be extenuating circumstances that argue against an aggressive protocol, but again, you need to discuss those with your neurologist.

    ~MarkEns

    March 5, 2012 at 2:03 am

    Hello Lori,

    There is a host of immunosuppressants that could be taken. Probably the four most common for CIDP are CellCept (mycophenolate mofetil), Imuran (azathioprine), Cytoxan (cyclophosphamide), and Rituxan (rituximab). The first two work mostly but not exclusively by interrupting T-cells. Cytoxan seems to work by suppressing bone marrow and also by interrupting T-cells. Rituxan works by interrupting B-cells. CellCept and Imuran are most often taken by mouth. Depending on dose, Cytoxan can be administered orally or by IV. Rituxan is administered by IV. Cyclosporin has also been used.

    What works for one person may not work for another, so you just have to try them. Many doctors hoped CellCept would be the most effective, but that has not proven to be the case. If it does work though, it carries the fewest severe side effects. Imuran is often considered more effective than CellCept, but it carries more serious side effects. Cytoxan is riskier still, but might be more effective. Rituxan seems to have less risk overall, but it is relatively new, so the long-term effects are not known.

    I have to take some exception to what Dawn said. All immunosuppressants carry a cancer risk, it is just a matter of degree. As an indication of risk, CellCept and Rituximab are not listed as carcinogens by IARC (International Agency for Research on Cancer), while Imuran and Cytoxan are listed as known human carcinogens. Dr. Lewis’s comment about CellCept’s CNS involvement concerns the very small risk of developing progressive multifocal leukoencephalopathy (PML). Nearly everybody has the virus that cause PML, but CellCept suppress the immune response to it in some people, leading to PML. PML is a demyelinatiing disease of the CNS, similar to MS, but much more rapidly progressive. CellCept is a commonly prescribed anti-rejection drug, but fewer than 30 cases of PML have been reported. The risk of PML is small.

    Discuss these with your doctor. I think all of these carry what is called a “Black Box Warning”, which means that your doctor must have experience in using them before prescribing them. This warning is also an indication that these are riskier than average drugs.

    ~MarkEns

    March 5, 2012 at 1:36 am

    Hello Lori,

    Doing badly on prednisone does not rule out other immunosuppressants. They act in different ways and so have different effects in different people. Immunosuppressants, except for prednisone (it is both an anti-inflammatory and an immunosuppressant), generally take a long time to have noticeable effects. CellCept is reputed to 3 to 6 months, while azathioprine (Imuran) takes even longer, up to a year. There are others to try as well, each with varying times to effectiveness and each with different side effects. You may need to try several, in sequence, until you find one that works for you.

    I had to reduce the amount of time away from work, so I also used prednisone in disgustingly large doses for a few months while waiting for azathioprine to kick in, after not responding to CellCept. The combination of PE, prednisone, and azathioprine worked very well for me. I was able to finally taper off the prednisone. Prednisone caused other problems, but it was, on balance, a reasonable trade-off for me.

    ~MarkEns

    February 21, 2012 at 5:46 am

    PattyO,

    The Foundation newsletter, which comes out about once a quarter, has an insert of all of the liaison’s contact information. You then call or e-mail the liaison to initiate the contact. If you don’t get the Foundation newsletter, you can call the Foundation. They will then give you the name and contact information of your nearest liaison. Some liaisons have secondary contacts that they call upon. If you want to talk with one of them, the liaison will give you his/her contact information.

    Another option is to ask your neurologist or infusion nurse if any of his/her patients are willing to talk with others. It is a little difficult for this coordination to happen because of HIPAA restrictions, but it can happen.

    February 11, 2012 at 11:22 pm

    WTF,

    I have been diagnosed with CIDP for 6 1/2 years, so I have been at it a while. My main treatment is plasma exchange, but I did some research into IVIg. The European Federation of Neurological Societies and the the Peripheral Nerve Society have published these guidelines: http://www.pnsociety.com/Guidelines_CIDP.pdf. There are divergent views about treatment, of course, even among the doctors considered experts on CIDP, but this is a good place to start.

    As far as your treatment goes, I think that it is close to the guidelines. You get 0.615 g/kg every two weeks. The guideline for maintenance is 1 g/kg every three weeks. That works out to nearly the same amount of IVIg. However, it is not working for you. I would not be unreasonable to try 1 g/kg every 2 weeks, or even to repeat the loading dose. Your initial loading dose seemed like it was a bit on the low side, 1.85 g/kg, and was administered over slightly too long a period. I mention it just for thoroughness; I am not sure I would make an issue of it with my doctor.

    The sensory symptoms seem to be harder to treat and slower to respond to treatment. However, if you gain your strength back, there is a good chance that your sensory symptoms will abate some.

    Treatment for some can be very effective very quickly. For others, no treatment works well at all. Nobody knows why. The best you can do is press you doctor for aggressive treatments. Ironically, though, you also have to be patient and allow time for the treatments to work. You have been at IVIg in a more or less normal protocol for three months. It is time to step it up to something more aggressive and give that another three or four months. If that does not work, it is time to try plasma exchange, corticosteroids, immunosuppressants, or some combination.

    What worked best for me was a combination of plasma exchange, immunosuppression with azathioprine (Imuran), and a blast of prednisone, followed by a taper.

    Godspeed in finding an effective treatment.
    MarkEns

    February 11, 2012 at 10:59 pm

    WTF,

    Actually, you are not too far off. Last I knew, there were all of four people on paid staff with the Foundation.

    If we had the private message thing we had before, I would send you a PM with the names and numbers. If the liaisons’ phone numbers were listed, I would give them here. Unfortunately they are not. Try again on Monday and let me know. If you don’t get anywhere, let me know and I will figure out something else.

    Best regards,
    MarkEns

    February 8, 2012 at 4:29 am

    Lori,

    Typically, you should notice an improvement in a couple of weeks, if not sooner. However, you might need two or three rounds in order to see effect. Further, most textbooks say that PE has to be repeated every couple of weeks to maintain effectiveness. Taking those two ideas together suggest that two or three rounds of treatments spaced every 10 days to 2 weeks should be done before deciding that plasma exchange is of limited value. Waiting a couple of months to see if it worked seems to me to be out of line with standard experience. I would press your doctor for another round much sooner.

    MarkEns

    February 8, 2012 at 4:28 am

    I miss the ability to delete a post. Posted a reply here that I meant for elsewhere and now find that I can only edit it, not delete it.

    February 8, 2012 at 4:23 am

    Exosurf,

    The 2 g/kg dose is what is often called the “loading” dose. It is just 0.4 g/kg given for five days straight as the first line treatment. If there is improvement, more IVIg, with a dose and frequency dependent on your clinical response, is usually administered. If you have kidney problems, there are good reasons for limiting the dose. However, a slow step-up in dose, along with blood and urine tests to monitor your kidneys might allow you to determine if 1 g/kg might not bring improvement without kidney damage.

    Godspeed in finding a treatment to improvement your symptoms,
    MarkEns

    February 6, 2012 at 12:25 am

    Hello WTF,

    It is not uncommon for the IVIg dose and frequency to need adjusting. You don’t say what the doses are. Perhaps you need more a larger dose than typical to show a response. If you and your insurance can afford it, it would be reasonable to try 2 g/kg every two or three weeks for three or four months before deciding IVIg does not work. Emily, a very young girl when she came down with an unusual presentation of CIDP, needed very large doses very often to stop and then reverse her symptoms. It was an unusual regimen, but it worked for her.

    Godspeed in finding a treatment that is effective for you,
    MarkEns

    February 6, 2012 at 12:14 am

    Try this link and filling in the appropriate information: http://www.gbs-cidp.org/home/get-support/chapters/. I don’t know exactly what happens: if you get contact information right away or if the Foundation will e-mail you with contact information.

    February 3, 2012 at 5:03 am

    Hello Lori,

    I am glad the process went reasonably well. It is too bad that the initial round did not yield dramatic results, but if you noticed any improvement, then at least is was a worthwhile experiment.

    The low BP is not uncommon at first. If you do plasma exchange on a routine basis, you will learn to recognize the symptoms early and the staff can make the appropriate adjustments.

    Typically, you should notice an improvement in a couple of weeks. Most textbooks say that PE has to be repeated every couple of weeks to maintain effectiveness. That is not necessarily true, but waiting a couple of months to see if it worked seems to me to be out of line with standard experience. I would press your doctor for another round much sooner.

    The reason people opt for a more permanent catheter is not because the insertion process is so difficult. It is because a) they are getting PE often enough that they may as well leave one in, b) the risk of infection with the jugular catheter is higher than with the permanent ones, and c) constantly inserting a jugular catheter creates significant scarring of the vein.

    I hope you find a good treatment.
    MarkEns

    February 2, 2012 at 4:56 am

    Hello Lori,

    So, how did it go?

    MarkEns