IVIG not working, next step Cellcept….

I don’t have time at this moment for a response to cell cept or other immuno. but check out this website and it gives you a diagram of rec. treatment protocol. It is a md. sight and one of the docs is actually on the gbs foundation advisory board. Kevin has somwthing strange happening to his mouth and we are going to the doc. Here is the site cidpus or [url]www.cidpinfo.com[/url]
Dawn

Welcome to the forum! I knose several people here had to have several rounds of IVIG before they had improvement. I have had two rounds and the second they added SoluMedrol which helped. Others have tried Plasmapheresis if the IVIG didn’t work. Cellcept, from what I understand would be tried if those don’t work. Why haven’t they done an LP? I don’t know at this point if it would be helpful or not but it may be worthwhile to either find out if there is protein, or if something else is going on.

Has your mother had 1 treatment (meaning 1 day) of IVIG 1 month apart? That isn’t going to cut it, if so. She needs to have a loading dose of IVIG, which is usually 2g per kg over a 3-5 day period. Then she can go on a maintenance dose of 1-2g per kg. Most people can go 4 weeks between treaments but some need them closer together.

Usually if IVIG is not working then the dr’s will add oral Prednisone or Solumedrol. Then if that doesn’t work you go onto the “heavier” immuno-suppressants like Methotrexate then Cellcept.

Good luck.
Kelly

Hi,
I am a little confused, you write that her immune system is compromised, why do you say that? With cidp, a trigger happens, say a cold or what ever and then the immune syst. turns on and fights. With cidp, it does not turn off and attacks the myelin sheath around the nerves. So it is not compromised in a weakened sense, it is kind of overactive if you will. How do they know it is cidp w/out a ncv or l/p? What makes them sure, did the emg indicate something? How long has this progressed? Ivig is the first defense for cidp, sometimes it takes a few rounds meaning a couple of loading doses. My son is 114 lbs and gets 100g over 4 days every month. 2g/kg is typical protocol. Not everyone reacts on the first loading dose, it may take another 3-5 day course. If this does not help, as Gab. mentioned, some dr.s add solumedrol, this seems to be common with members on the site as of late. Next, is pp sometimes even followed w/ ivig. If this does not work then immunosup. are introduced. This wipes out the immune system and can be risky regarding getting sick or what not. Google Cellcept to see the risks, they are high. Some NEED cell cept or other immunosup. so you have to do what you have to do. Some on the site have used these drugs and gone into full remission. I feel the risks are too high for my 11y/o. Give the ivig a try again. Have her symptoms worsened, how long has she been this way? The longer you wait and do nothing, the more damage that happens. You have much to think about and it all happens so quickly, just try to take a deep breath, be strong for your mom and come here often, there are so many nice people here who can offer so much, sometimes even more than the docs because they have already experienced it. I have learned much from this site and it has helped us through many a dark, confused day. The people listen to everything here, medical problems regarding your mother, and problems you have as a care giver. Be strong, you will get through this with your mom, and there is medicine, it sometimes just takes a while to figure out the plan!
Dawn Kevies mom

2 days of consecutive IVIG is not enough of a loading dose. She needs the full 3-5 days then maintenance doses. Some people even have 10 consecutive days of IVIG…in extreme cases.

Kelly

Hi,
I would concur with everyone is saying that she needs the loading dose of IVIG i.e. the five days of IVIG and then a monthly regime calculated on her weight.

My husband was diagnosed with CIDP Lewis Sumner variant last summer, and started with the above regime and has done remarkably well.

However, the neurologist added Imuran at the beginning of December and we hadn’t done much reading at that point. He became very ill on Dec.22nd and was hospitalized. Looking at his bloodwork from the hospitalization it looks like he was sensitive to the Imuran and developed symptoms of the sensitivity within 12 days of starting the Imuran. The neurologist is waffling over what his illness was i.e. maybe Imuran sensitivity and maybe underlying infection that surfaced when his immune system was knocked out with the Imuran.

Now she wants to start him on Cellcept, but we have a 3 month reprieve where she is keeping him on the IVIG and will reassess his response in April by re-doing his EMG and nerve conduction tests.

Our game plan is to refuse the Cellcept due to lack of long term studies about how effective it actually is, and the possible horrendous side effects. We live in Canada too and it seems cost of IVIG is a factor in adding other drugs so quickly. If we get pushed against the wall over the cost of IVIG, we will discuss with my husband’s neurologist IV Prednisolone.

My husband is 60 years old, and due to having CIDP undiagnosed for so many years he is not a hardy fellow. Cellcept has many side effects and I think the very young and the older population in particular needs to think carefully before accepting treatment with cytotoxic drugs. We discussed my husband’s case with our GP and she said we are well read and informed and if we are not comfortable with a treatment offered we have the right to refuse it.

I have searched endlessly for articles on efficacy of Cellcept on treatment of CIDP and the best I have come up with is one abstract in which the authors say “that MMF(Cellcept) induced a modest benefit in 20% of their patients and stabilized patient condition, allowing reduction of steroid or IVIG therapy”. http:[url]www.neurology/org/cgi/content/abstract/63/4/715[/url]

Dawn Kevies Mom has been very helpful with sending me lots of info. I think it is key that you do lots of reading and be an advocate for your mother. Just from our experience thus far, I would advocate for a proper protocol being followed for the IVIG before it gets ruled out. And then I would ask questions about why IV Prednisolone couldn’t be tried next before the cytotoxic drugs are used.
Laurel

I understand giving the 3-5 day dose over a 2 day period but sometimes that STILL is not enough. The same thing was given to my 4 year old daughter. It didn’t have the desired effect, so she received 3 more IVIG treatments 1 time a week for 3 weeks. That still did not completely repair her eye damage & she ended up needing IVIG for 5 consecutive days of 2g per kg, then IVIG every other day for 2 weeks at the same dosage & then IVIG 2 times per week for 1 week before her eye was better. Sometimes it takes more IVIG than what people think in order to see results. It depends on the individual & the extent of damage. Each person with CIDP should be treated per their specific needs, not always typical protocol.

What I’m trying to suggest is that you speak with the neurologists and ask for a longer course of consecutive IVIG to see if that helps. If it doesn’t then you move on to the next drugs, which normally are steroids. It is EXTREMELY rare, in the US, for dr’s to go from IVIG straight to cytotoxic drugs. The possible side effects must be worth the risks to take those drugs. You’ll never truly know if they are worth it unless you try everything else before that. Cytotoxic drugs are the last line of defense in treating CIDP.

I know you received quite a few responses from Dawn & myself. Both of our children have CIDP & we’ve both researched this disease constantly. It could be argued that mother’s of chronically ill children with rare diseases know more about the disease & treatments than the dr’s do. I’m telling you this just so you know that both Dawn & myself really do know what we’re talking about, LOL.

Good luck to you & to your mom. I hope she starts to mend soon.

Kelly

PS, an l/p is a lumbar puncture also known as a spinal tap.

I have Lewis-Sumner variant of CIDP,I have been on 1000mg of Cellcept twice daily for over 3 years and 60gram of IVIg treatment every 5 weeks for over a year now. I have rountine blood work done, and my doctors have been keeping an eye on me, so far no side effects from the cellcept.After trying different treatments for the last 5 plus years this has gotten the best results for me. The side effects look scarey on paperwork for most drugs, luckily I’ve had none of them.

Ashley, it took 4 months of IVIg before I noticed a difference but because of my stupid kidneys I get a very low dose. I agree with the others about Cellcept but given the option between immobility and a compromised immune system I know which I’d pick. Have you considered plasmapheresis? Why not try MORE IVIG and/or more often before trying the other nasties. My nurse has one CIDP patient who gets IVIg weekly, had a big loading dose and now gets it once a week – that is what finally worked for him.

At any rate, don’t give up and tell your Mom we’re thinking of her. :p

Julie

Ive posted this a couple of times since November. The neurologist who spoke at the meeting is the new neuro that has joined the Foundations advisory board.

Copied from a previous post.
[quote]
Last weekend, the Philidelphia group had a chapter meeting. The speaker was Dr. (sorry, I am going to get his name again from the foundation and post it for you)…… There were a number of CIDP patients in the group and he answered a number of questions regarding treatment, what was effective, what to do if certain treatements werent effective etc. Here are some of the notes I took from his answer and question time. ….

# As we know, certain varients of CIDP react better to certain treatments. He said that in his experience, if the patient did not respond after 2 or 3 IVIg therapies then he added sterroids to see if there was a response.

# next step would be (I think the IVIg would have been discontinued when following this step), High dose sterroids everyday, then change it to every other day and add Cellcept or similar (up to 3g), and then lower the sterroids more.

# Then if that didnt work, he would try Plasmapharesis

# consider Retuxan (sp?)

These are from my notes, and I was trying to write them down as quickly as he was speaking. He was responding to a patient who asked him what would his treatments be if IVIg failed and what steps would he follow to find one that did work. He also mentioned a couple of treatments that may work for CIDP, but they have been removed from the market because they are so dangerous, so there is a possibility way down the line that some trials may be considered with some of these drugs, but that is still way down the line if it happens at all.

[/quote]

Ashley,

No disrespect intended to many doctors and neuro’s, but I do believe that many people on this forum are far more expert than the average medical professional. There are some wonderful doctors out there who dedicate a great deal of the time to peripheral neuropathies, GBS and CIDP, or those who are prepared to admit to not knowing much, but spend time trying to find the correct way to treat a particular patient – and that includes listening to research the patient or family has done. If you have been able to get one of those wonderful doctors, you are truly blessed. Many patients on the forum have spent months or years researching our particular syndrome, whether it be GBS or CIDP. You are taking the right approach I believe in questioning and being proactive in this case. It is however so unfortunate that each person reacts so differently to each treatment given, and you are going to make the most informed decision that you could possibly make drawing the knowledge on the forum, the doctors knowledge and of course, the personal knowledge you have of your mother.

[FONT=”Comic Sans MS”][B][SIZE=”2″]Ashley,
I couldn’t agree more with Ali about the people at this site.
I am 59 and have enjoyed life-long good health, then suddenly on my 59th birthday, Wham!
I find myself unable to walk an in not inconsiderable pain.
My first two hospitalizations, I was seen by a Gastro-Interologist who removed my gall bladder of all things, then treated me for an infection resulting from that procedure, and for pain I was on a morphine pump. On both occasions, my request to be seen by a neurologist was ignored.
When I finally got to the right Hospital, I was immediately given a lumbar puncture and an EMG and it was determined that I had GBS.
By the third IvIg treatment (in ICU) I was improving and after the fifth, dramatically improved. Alas, after I’d been home for 10 days I suffered a relapse and had another course with less dramatic results, still I was able to go home and get around with the help of a cane and a walker.
After that though, I went into a depression and allowed myself to get weaker and worse for nearly 2 months, till I revisited this site and was inspired to get back up in the saddle, so to speak.
Plasmapherisis worked wonderfully for me, no longer need a cane but carry it for balance issures, and I’m taking Prednisone, which we are trying to reduce by using the scary,( but hopefully worth it) Mycophenolate (CellCept)
For me, this is kind of like an AA meeting, reading everyone’s story just inspires you to keep searching, keep going and keep smiling.
Best to you Mum[/SIZE][/B][/FONT]

[U][I]Please listen to your Mum. If she asks to be turned she [I][U]needs [U][I]to be turned![/I][/U][/U][/I][/I][/U] No one can adequately explain the pain from being in one spot to long 😮 Yes, there is harm that can be done ~ her skin could break down “in the blink of an eye” and there’s all kinds of complications that could come from it. Trust me, you don’t want to go there!

There are no words adequate to be able to help you know our pain. Please, you must trust that your Mum knows what she “really” needs. Her body is telling her. I remember saying that the sheet was to heavy on my legs and how would a healthy person understand that 🙁

Of course her caregiver is taxed (as all of ours’ have been) but she is not trying to be difficult or demanding. She is in a new world of helplessness, pain, and its totally foreign to her, first and foremost. This is not an “easy” disease especially with all the uniqueness that we each present and experience.

Please let your Mum know that there is a whole other family out here that knows what she’s going thru and cares deeply. Reassure her of the hope for better days. And we look forward to when she can make her first post to us.

Please keep asking the questions so that we can be of help to all of you.

Hi DR Ashley I got CIDP in the fall of 2004 from a flu shot. I had a spinal Tap in the spring of 2005 and in June of 2005 I had a nerve biopsy. Both were normal. I still have pain to this day from the nerve biopsy.

I am seeing DR Zwicker at the Ottawa Civic Hospital. I started taking Gabapentin for the pain from my nerve biopsy. I was at 1800 mg then I switched to Lyrica. The nerve test and the muscle test is what told her I had CIDP.

I saw a local Neurologist in Dec 2004 and again in March 2005 it was him that referred me to the Ottawa Civic Hospital. I was there within 3 weeks. I got the first DR who could see me. I wasn’t getting worse by then my still it affected me in my daily and work life and it got worse last year so last summer I had 25 grams a day for 5 days and just came back today from 5 more days of IVIG Gammunex 25 grams a day for 5 days.

I go back on March 4th for a follow up to see if it improved me even more. When I saw her last November the tests showed I did improve but I was still having problems with my body muscle and weakness. Hopefully I will improve so much more I won’t need anymore IVIG.

I weight 144 lbs and got 125grams of IVIG the first around and the second time around. Also people say if the IVIG doesn’t work on somebody even after getting many doses it may be time to consider P.P (Plasmapherisis) (sp?).

Hang in there and keep us posted.

Sue

From Cornwall Ontario

Ashley,

I have just finished a post regarding the bowels. Unfotunately I think I probably discuss it more than anyone else on the forum 😮 , only because it effected me so intensley when I was paralyzed. I think that the most important thing to remember here is that the patient is not originally constipated – but becomes so because they are unable to evacuate and water is drawn from the stool which makes it harder and even ‘more unmoveable’ (not great language). The lack of muscle strength prevents the patient from ‘pushing’, the body is paralyzed and unble to pass the stool naturally. Unfortunately the only thing that worked for me were the enema’s – and not to be horribly curde here, but they were not the little fleet enema’s, but manual and more industrial (to put it nicely). Feeling the way your mom feels before she has been relieved by the fleet, is [I]the most awful[/I] feeling! and I really dont say that lightly. Once I was able to stand up on my own, and start moving, I no longer required enema’s, but seemed to be on a constant diet of prunes…..which I HATE, but they did help tremendously (the soft ones). Later of course I was able to stop eating them. Hope that helps.

Hi Ashley,
Now concerning bowels, I am a bit of an expert. Hubby has had lots of trouble with constipation over the past few years probably related to the CIDP and he tends to get a little overly focused on that issue . I found that the stool softeners were critical on a regular basis in the beginning until he added Metamucil capsules (4 per day) to add fiber and bulk. But the biggest benefit was adding probiotics three or four times a week. The best were Udo’s Choce Super 8 Hi-Potency. No complaints of constipation since routinely taking Metamucil capsules and Probiotics.
We have horses and they have a delicate digestive system with colic being life threatening to them. Our thoroughbred had frequent gas and impaction colics until I added probiotics to her routine as well. It was a family physician that gave us the advice to add probiotics to my husband’s routine.
Laurel

Ashley,

If the fleet’s are working, maybe stick to them, or maybe try two at a time, alternatively, give them more often. The larger ones often bring on an anxiety level that is somthing else to deal with entirely, but if needed, obviously worth it. I just wanted to give you an idea of what can happen if things aren’t taken care of initially, and how really awful and painful it is for someone to have impacted bowels. 😮 (Isnt it amazing what we will talk about as we age – my 16 year old will be horrified if she read this 😀 )

Hello,

I’ve read all your posts, not the other’s ,just yours. I’m sorry for what’s going on with your mum. I wish you the best of luck. I’m sure it is so frustrating.

We have a 3 year old with CIDP. He went up and down for awhile and finally stabilized with ivig and solumedrol. I agree with Kelly, Emily’s mom. You have to make sure she had ENOUGH of ivig.

We are going to start Rituxan in March to kill his B-cells. He’s not quite in remission and would really like to see if we can get him into remission before he would start school. I know you worry about everything with your mum, just think hard it is when you have a 3 year old with the disease.

Love to you both,
Lori

Tylenol isn’t really going to do too much for the pain that she’s in. It’s not “regular, normal” pain. It’s searing, burning, nerve pain. I also don’t think that any kind of numbing cream will work either since that is mostly for muscle pain…unless she is having muscle pain as well then it will help a little with that. She needs something like Neurontin for the nerve pain. We gave Emily Motrin when she was in extreme pain…but she was 4 years old when all of this started & it just took the edge off.

Nerve pain, from what I’ve been told & what I can guess from Emi’s description, feels similar to when your feet go to sleep & you try to walk on them anyways. Emily describes it as “mushy strawberries”. She says that the muscle pain feels like “nuts”, which I’m assuming feels like a “charlie horse” or a knot in the muscle.

Pain management is VERY important, especially if she’s bedridden & can’t move around. She needs the best meds to make her feel most comfortable.

Hope that helps,
Kelly

Ashley Tylenol and pain reliever cream just won’t cut it. She will need pain reliever that will help for the nerve pain. There are many kinds people use. The main one is Gabapentin. Also some take Lyrica and there are others.

Sue

Ditto Sue ~ this is not the “usual” pain issue. Mum needs neurontin, lyrica or one of the others because this is the pain of neuropathy. Narcotics don’t make the difference either. And don’t be concerned about her becoming “addicted”. She [U]needs[/U] the pain relief. As her pain decreases she will be able to taper down. Only she will know what she really needs for pain relief. No doctor, text book or any other source will know the amount she needs better than she does. We have all had to discover the “right amount” by trial and error. Hang in there!

[FONT=”Comic Sans MS”][SIZE=”2″]Ashley,
The pain that accompanies GBS/CIDP is difficult to convey, and different for everyone. For me it felt as if I had been stabbed in the sternum and right through to the spine. Fortunately for me, my neuro felt pain should be treated as aggressively as the other symptoms, In hospital, at one point, I needed a Morphine pump, and went home with a fairly high dose of sustained release Oxycontin, (40mg) plus Baclofen (which is for spinal cord injury) and Neurontin. I am now weaned down to 10mg of the Oxy and off the Bac but still taking Neuronitin. The Neurontin or Gabapentin is very important so please ask about it if she’s not already taking it.
The other issue is ‘perceived pain’ or pressure sensitivity, like having a mere blanket over your feet feel like a ten pound weight or feeling like there is gravel in your shoes, even though your wearing soft slippers.
In other words, keep bugging your doctors up there about getting adequet pain relief for your Mum.
You’re a very good son
Best wishes [/SIZE][/FONT]

Dear Ashley,
I am so sorry to read this dreadful news. It felt like it took the wind out of me reading it so I can only imagine how upset you all are. May God bless your mother and you and your family. I will say prayers for her.
Laurel

[FONT=”Comic Sans MS”]Ashley,
I’m so sorry. My prayers going up for all of you.[/FONT]

[FONT=”Comic Sans MS”][SIZE=”2″]Ashley,
I know you are probably still reeling from the news and perhaps haven’t had time to do much research yet so I poked around a bit and found this web site for the ALS Society of Canada
als.ca/_units/ontario.aspx or in case I messed that up, simply
als.ca
Since all the sites visited stressed the importance of a 2nd opinion, specifically from those who have had training in ALS diagnosis and treatment, I took the liberty of copying down this information for you:

Ontario Chapter
265 Yorkland Blvd
Suite 300
Toronto M2J1S5
Phone 416-497-8548 Toll free 866-611-8545

Please don’t lose heart, you have been so strong, and if you were my son I would be very proud of you. Come to think of it, I am very proud of you since we are [I]all[/I] family here.
Whatever happens, please keep in touch as we have become quite invested in your Mum and you.

[/SIZE][/FONT]

It’s nice to meet you Dr.

Lyrica and Tramadol have helped me considerably over the past 4 years. I did take Neurontin for a long time but the dosage made me tired and comatose. Since your mum is older, perhaps Lyrica would be a better option for her. Good luck with everything, you family and mum are in my thoughts and prayers.

Jerimy

Ashley,

I am sorry for the ALS diagnosis. My aunt had ALS and I helped my mom take care of her every evening for nearly a year. I was in my mid 20’s at the time and now, looking back, I am so glad I had the chance to be able to be with her and get to know her. It is a difficult disease to say the least but she is lucky to have you . Take one day at a time and make the best of every moment. Try to also take care of yourself so that you can be there for her. Even tho it’s not CIDP or GBS, you have met a great group of very caring people on this forum that will reach out for you with support and prayers.

My best to you and your mum.

Stacey