Prognosis for Treatment-Refractory CIDP

    • April 20, 2016 at 11:27 pm

      Hi everyone,

      I posted about my mom back in October – she has CIDP and severe tremor. Here’s the post:

      Since getting sick in the Fall of 2014, she has been treated variously with plasma exchange, IVIG, high-dose steroids and Cellcept. Last November she moved into an assisted living facility because she could no longer live alone – the tremor prevented her from being able to do any ADL’s and she was found semi-conscious from dehydration and taken to the hospital. She is wheel-chair bound. Her last treatment regimen was monthly out-patient plasma exchange combined with Prednisone and CellCept. Beginning in late December, she began to get weaker and felt that the plasma exchange was not working as well as it had. She has new doctors at Duke who diagnosed her in March as being treatment refractory, based on her history, the results of yet another EMG and the degree of weakness she presented while still being on monthly plasma exchange, Cellcept and Prednisone. The team recommended trying Rituxan and were able to get her approved and scheduled for her first infusion within a few weeks – which indicates to me how seriously they viewed her condition, especially since they warned us that it could take weeks or months to get approval from her insurance. “Time is of the essence,” they said. Privately, one of the doctors called Rituxan a “Hail Mary” treatment in my mom’s case.

      It’s been four weeks since her first infusion, two weeks since her second. It’s been a total of nine weeks since her last plasma exchange. She’s still taking Cellcept and 30 mg of Prednisone each day. We know that it can take 8-12 weeks or more before we’ll know if Rituxan is going to help. Her doctors have prepared us that she may get worse before she gets better.

      Mom is now experiencing a rapid, precipitous decline in strength and function. She can no longer lift her feet. The PT who has been working with her has determined that any transfers, toileting, etc., must be assisted by 2 caregivers. She’s been moved up to the highest level of care the assisted living facility can provide. I met with the PT and Care Manager today and it is cleared that they are very alarmed at the rate at which she is declining. We’ve had her follow-up appointment moved up to May 16, but both ladies told me that if she continues to experience weakening at the pace they’ve seen over the past two weeks, they believe she’ll need to be hospitalized well in advance of that appointment. They are communicating with her doctors and I hope to hear tomorrow that they can work her in ASAP.

      If you’ve read this far, thank you. What I’m working up to is this: I can’t find any information regarding the likely outcomes for someone who is treatment refractory, older than the average CIDP patient and in poor health. Since her doctors obviously believe that Rituxan is her last best hope, what comes next? I can’t find information regarding any other treatments for CIDP beyond Rituxan when you’ve tried plasma exchange, IVIG, prednisone and steroid-sparing drugs. Does anyone know what happens to someone who does not respond to any treatment? Total paralysis? I’ve read over and over that CIDP is not terminal, but that deaths are usually because of infection due to immune suppression or falls. I can’t fathom what kind of existence she is going to have if nothing will slow down the progression of this disease.

    • jk
      April 21, 2016 at 12:38 pm

      I am sorry to read about your mother’s condition. There are other treatment options available.

      For example, “In intractable cases high-dose cyclophosphamide has been shown to be effective [Gladstone et al. 2007, 2005; Brannagan et al. 2002]. We prefer an initial pulse of three cycles at 350 mg/m2 body surface followed by 600 mg/m2 body surface at an interval of every 6–8 weeks..”


      For more treatment options please refer to this website:

      If you did a search using each of the treatment methods above on this website’s search feature I am confident you will find someone who has talked about the method in the past.

      A small measure of comfort in this data- “The 2007 GBS/CIDP Outcomes Survey indicated that over the last three decades the care of CIDP has improved the overall outcome. The mortality rate has decreased to 1.3 % and patients are less likely to be confined to a wheelchair (7 %). The proportion of patients who recovered or are independent while on treatment has increased remarkably to 31 %. Despite these improvements due to immunotherapy, the majority of cases remain to have some degree of disability with 28 % requiring an assistive device to ambulate (Koski L, 2007 CIDP Outcomes Survey, personal communication).”

      “Conclusions- The long term prognosis of CIDP patients was generally favourable, but 39% of patients still required immune treatments and 13% had severe disabilities. Mode of onset, distribution of symptoms, and electrophysiological characteristics may be prognostic factors for predicting a favourable outcome.”

      Here is another source of information: “When a patient does not respond to or cannot tolerate the first-line agents, other medications may prove beneficial. Azathioprine, cyclophosphamide, cyclosporine, interferon-alpha, interferon-beta, mycophenolate mofetil, and methotrexate have all been reported to be beneficial in CIDP patients not responsive to initial therapies.”

      Pull up a chair to read this one- just as with your post ( a joke, please smile!)

    • April 22, 2016 at 5:40 pm

      Thank you, jk. We’ve been advised by her ASF to begin looking at alternative living arrangements (skilled nursing).