How do you know which drug?

There are lots of options for treatment with drugs for ITP. I read many posts and it seems like everybody is on something different than the next person. Why? Is it just preference or is it what the doctor thinks is best for any given body? I was offered Rituximab. Four treatments for four weeks. The sheet I was given on it is so scary I can't yet bring myself to get through reading the whole thing. It is not paid for in my case, in Canada anyway. I had a call from Roche tonight to talk about my medical situation. It was surreal. My doctor is trying to get it for me on compassionate grounds. That sounds scary too. It must be very expensive. Does anybody know $$$?
Still not sure it is for me. Sandy Too

Sandy - most people go down the treatment line until they find something that works. There is no particular order, although Prednisone is usually tried first. Treatment options are usually suggested by a doctor based on their experiences with a particular drug. Patients can also make suggestions too once they become familiar with the options available.

Yes, Rituxan is expensive. In 2004, my four infusions were about $60,000. I don't know what they cost now. Most treatments do cost a lot. Steroids are the cheapest thing.

Sandy, I did Rituxan back in 2011 and have been in remission since. You say you're in Canada? May I ask where? My doctor got it for me but I didn't pay for it. Couldn't have afforded it if I had to pay for it. Two of us got it but it only worked for me.

Hi there

I too am from Canada where Rituxan is not covered. The first time I got rituxan was in a double blinded drug trial it gave me a 6 year remission. The second time I received it was through the compassion program.

It was fairly easy to get into the compassion program you just have to show your insurance won't cover it. For me all it took was a 3 way call between the compassion program lady, myself and my insurance.

Last time I had it was approx 2 years ago got a remission of 1 year. If I remember correctly the cost ( Ontario Canada) was 3,300.00 a dose needing 4 doses for a total of 13,200,00 approx. Since my drug coverage per year is 2,000.00 a year I was accepted into the compassion program and found out I was covered in less then 2 weeks.

OMG, I had no idea. $60,000 Sandi, how do you folks pay for something like this? Is it through private insurance coverage? Thanks CindyL and Winnifred, I am from Ontario. The compassion program is what my hemo said she was applying to. I had a telephone conference with a Roche rep yesterday. It went well. I am to hear from another Roche rep today or tomorrow about the funding part. A little scary as I too could not afford this kind of money.
Question. What makes an ITP patient choose the timing to take the medication? If only so much will ever be paid or for so long by the compassion program, why would one not wait until they absolutely had to do it, knowing that down the road the option might not be there or run out?
It is very encouraging to hear about the remissions. I know Sandi does, but have you two ladies got assorted auto-immune problems as well? How did the treatment go? This is something that is worrisome for me. I don't do well on medication period and this medication is in a whole new world. thanks Sandy Too

Sandy - My private insurance wouldn't cover it since back then it was considered an off-label treatment for ITP. At the time, the drug company would cover costs for those who had insurance denials. I only had to pay for the infusion costs from my doctors office and the pre-meds. I shouldn't say 'only'. It was thousands.

When it comes to timing for ITP, when do you consider it to be absolutely necessary to treat? When counts hit 20k or 10k or 5k....? The thing with Rituxan is that it can take 4 to 12 weeks to work, so most people choose to do it as soon as counts get below their comfort zone. They worry about later, later. There are other options that people can try after Rituxan so if it doesn't work or you can't use it any longer, you move on to the next thing. There are some that don't think a thing of using Rituxan and others that are hesitant like you are. It can be a very difficult decision.

Sandy,
Here is a discussion of the use of a "low dose Rituxan" protocol which has gotten excellent results.
pdsa.org/forum-sp-534/7-treatment-general/27993-low-dose-rituximab-rituxan-research.html?limitstart=0#40349

For me, my counts started going up right away and kept going with treatment. Had no problems during infusion. I was actually kind of surprised I got the last dose. I have low thyroid issues but I had that long before my ITP. No other issues.

I'm in NB by the way.

Sandy Too, you are thinking Rituxin is a best next step for you, or ?

I don't know Hal, I have allot of trouble with drugs of any kind. I have had a major reaction to IVIG. I have multiple auto-immune things going on. I had my blood done today again and I am back up to 197 after 3 weeks of pred, 50, 40 and 30, down to 25 tomorrow.
I have a terrible time with GI issues even on the pred. I got a sinus infection second week the first round of pred 80 mg. This time although only 50 as I could not do the 80, still got a sore throat and sinus thing by the second week and today a UTI so bad I was bleeding.
It's not that I would want to dismiss the ITP and I am grateful for the chance at Rituximab, but I know my body with all this stuff and I just don't have a good track record. As Rob's link read at the end post, "anything can happen" and it sure rings true here. I am really having a time with it. The IVIG was the same, so I called and talked to my doctor..."you'll be fine"""...then I end up on the floor fighting for my life...doctors running asking the nurses "has this ever happened before"...they are shell shocked and so "no, not ever"...I warned them too.
If I were to have a bad reaction which I read is pretty common, I have no working adrenals to help me. I know this will sound funny but I can't be so sick that I can't fight. Fighting at the worst times is what has kept me here so far I believe. The serum sickness sounds just hideous. I think if I were going to try it, I would talk to my doctors about low dose for sure, and about having IV hydrocortisone at the same time and ask to stay in hospital for a day or two. My little town hospital would not have a clue if I walked in there in that kind of state as they don't know what to do for me now most of the time even with the flu, I have to tell them! :huh:
I will continue to read, and post questions and talk to my rheumy on Oct 19, she knows me better than any of them, then I will think some more. I do know that my platelets drop low so doing nothing really is not an option. Thanks, Sandy Too

Sandy - if you are at 197 then you do not need to treat. Your counts could stay up for a while. Do not let anyone talk you into treating with high counts. I understand that you'll need a plan in case they drop again, but that might not happen for a while.

It's not low counts that need treated, symptoms do. If counts are low and you have few symptoms, it's okay to wait a while and see what happens. Low counts are not an emergency unless symptoms are.

If you are not comfortable with Rituxan, then ask your doctor to discuss other options. You don't have to do it.

I'm on Pred & N plate keeping my counts at about 50 as been waiting for minor surgery which is planned for 8th October. . Once recovered from that I fully intend to reduce meds much as I can to maintain a count of around 30. My haemo is happy for an even lower count than that but I am always frozen stiff and absolutely shattered they drop below 25.

Sandy, I don't remember which SLE symptoms you have, but Rituxan is effective for more than one, especially dermatological ones. you might try googling rituximab AND __________ (fill in the blank dor whatever condition) That might be a factor in your decision.

Rituxan is supposed to be helpful for Lupus, but over the years I truly have seen more people with Lupus have reactions to Rituxan than those who do not have Lupus. Mainly serum sickness and hives.

So the first time my platelets, went from 13 to 258 in about 3 weeks. Then when I got back to my reg dose of pred 7.5 they dropped again within the month to 13. That was the end of August. Now again I am back to 197. Started at 50 this time ,and am still on 25 mg pred, then down 5 mg weekly to 20 then 15 and then staying on 10. So here's hoping they will stay up this time. Hemo say she knows by the way I responded the first time, that they won't. As far as symptoms, well I have so many on any given day for other things that it doesn't really matter. They are all the same fatigue, nausea, GI upset usually are my worst. I just keep going when I can and rest when I can't. The pred has me pretty messed up right now but I remember the first time hoping that I would get right again, and I did..so I am sure things will be good again in a while.

I too think Rob and Sandi, that both of my doctors are thinking the same thing, that if they can give me the medication perhaps it would help with multiple issues, the ITP and the Lupus and the rest which has been difficult for a year now. I must admit I am thinking about that too as I have never had such a rough go for so long before. I don't mean to sound whinny but I am so over it! Plus there is the damage that is being done (to what) all this time too. Also as you say mrsb04 my rheumy said she would be very happy to leave me right alone if my platelets would stay at 20 even.Again here's hoping.

My lupus symptoms are not skin really, although my face tells me when I have had too much sun which in turn tells me when I am in trouble tomorrow inflammation-wise, it's more systemic, more organ issues for me. My lungs are scarred, bronchiectasis so easy infection and terrible time fighting off colds and just breathing. Sjogren's is about dryness and smooth muscle movement, causing easy infection of the sinus, and lungs, and gut, and bladder and slow motility in the GI tract....which is my problem re tummy upset, my meds just sit there and hurt my tummy as my it doesn't empty so well, about 1/3 as fast as it should. The high dose pred plays heck with that I can hardly eat right now. IgA and IgG issues, again easy infection. Liver has antibodies but all is well so far only with the occasional elevated enzymes, but there is cirrhosis from the long term pred. Osteoperposis from the pred and from staying out of he sun, and my bones, spine and neck hurt (Lupus?) which in turn cause me headaches when I lay down, Addison's which can cause fatigue as there is no way to measure levels on any given day or situation or increase them, and metabolic stuff, due to no aldosterone, so dehydration is always difficult. Hypothyroid, maybe fatigue if it get out of wack, which it does on occasion. Then of coarse there is lots of pain and inflammation at the drop of a hat. Some vision troubles sometimes too. Oh, ya, BRAIN-FOG :lol: ...I hope the things I am trying to write even make sense...and I am sorry about the spelling but I gave up on that long ago at these times. Life's too short. Apologies.

It sounds an awful lot I know, almost unbelievable as I look at it typed out..but I just keep going! AND I am a pretty content, interested and happy person for the most part.
I am in awe everyday of just what a gift we have all been given that of this amazing machine called the human body...and even if it's a little bit broken. Thanks every one, Sandy Too

Making the decision to treat is hard. You need to look at everything your life style, side effects, how you feel, your numbers. Rob is right search the drug and _______ you will find lots of answers. Some you'll like and some you won't. It will help you to make your decision!

My new doctor is all about being prepared. We discussed at my last appointment what to do next if and when my count was to drop Last visit my counts were pretty much normal last week my count was 80. Who knows what it is today or what it will be next visit. All treatments come with side effects for me that means switching to different medications after a while.


Rituxan worked great 1st time. I had a reaction first infusion but nothing the 2nd, 3rd or 4th one. It gave me a 6 year remission. 2nd round of Rituxan I had some side effects after 2nd dose. Then during the 3rd dose they started up. I got a year with good count. The side effect the 2nd time was parathesia. Because of that I won't ever get Rituxan again.

Well, I sure have learned allot in a short time. Trial by fire. I think the thing that is making me feel un-easy at all is the way my hemo gave me my options. Matter of fact and carved in stone. Remove the spleen or rituximab. Plus really no explaination of the drug, just a couple of pages of the drug description and side effects. It seems a little "take it or leave it" and "cover our butts" hospital wise, and not so much working with me as a person with this problem. ITP is all about individualized diagnosis and treatment. Having said that maybe it is just because my hemo already knows this stuff, maybe she has the answers about my situation and just feels it un-necessary to debate. Lord knows I have been going to the Kingston General and Hotel Dieu for long enough she has access and could read volumes about me.
I am at the point where I can now begin to study each of the second line drugs. Also the possibility of low dose vs what she wants to do which is standard dose.
There is always he hope still that after tapering the pred to 10mg and staying there, that my platelets will stabilize at a count she and I are content with. Fingers crossed.
I will have to read about the side effects too, there's lots of them. I will investigate your "parathesia" Winnifred. As I don't know what that is yet. Sounds as though you have had some good luck with remission. Glad to hear it for you. Thanks, Sandy Too

Sandy Too wrote: I am at the point where I can now begin to study each of the second line drugs. Also the possibility of low dose vs what she wants to do which is standard dose.

If you decide to try for the low-dose Rituxan protocol, the following email that my wife sent to her hematologist might help:

Dear Dr. ______________,

There are at least seven studies that show that a much lower dose of Rituxan can be as effective as the standard dose. The low dose Rituxan protocol is typically 100 mg x 4 weeks, compared to the standard dose Rituxan at 375 mg/square meter, averaging about 700 mg x 4 weeks. It may or may not be combined with high dose dexamethasone 40 mg x 4 days the first week of treatment.

In each of these seven studies, which included a total of 199 patients treated for ITP with low dose Rituxan, responses were comparable to typical standard dose treatment. In at least one study, infusion-related side effects were significantly fewer, so at least some side effects seem to be dose-dependent, though that may not be the case for all side effects.

Three of the studies, totaling 96 treatment subjects, used low dose Rituxan along with high dose dexamethasone with the first treatment. The other four studies, totaling 103 treatment subjects, used low dose Rituxan alone. It appears on the surface that adding HDD to the treatment may increase the effectiveness, though the studies do not report their results consistently, making comparison difficult.

I am leaning toward the low dose approach, if you would be willing.

I have included the relevant articles below with links.

Respectfully,



Here are the low dose Rituxan studies:

1. High response rate to low‐dose rituximab plus high‐dose dexamethasone as frontline therapy in adult patients with primary immune thrombocytopenia
David Gómez-Almaguer, et al First published: 2 April 2013
N=21 Complete sustained response at 6 month 76.2%
onlinelibrary.wiley.com/enhanced/doi/10.1111/ejh.12102/#author1
www5.medicine.wisc.edu/~williams/ld_ritux_dex_itp.pdf

2. Low-dose rituximab combined with short-term glucocorticoids up-regulates Treg cell levels in patients with immune thrombocytopenia.
Li Z, Mou W, Lu G, Cao J, He X, Pan X, Xu K
International journal of hematology 93:1 2011 Jan pg 91-8
N=31 plus controls. Sustained response 77.4%.
www.unboundmedicine.com/medline/citation...ne_thrombocytopenia_

3. [Effect of different therapeutic regimens on regulatory T cells in patients of primary immune thrombocytopenia].
Zhonghua Xue Ye Xue Za Zhi. 2013 Jun;34(6):478-81.
Li ZY1, Li DP, Yan ZL, Xing WW, Liu KG, Cao J, He XP, Pan XY, Xu KL.
N=44 plus other treatment groups. Overall response (OR) rates of low dose Rituximab + HDD at day 28: 79.5%. 12 months sustained response (SR) : 66.7%.
www.ncbi.nlm.nih.gov/pubmed/23827100

4. Activity and safety profile of low-dose rituximab for the treatment of autoimmune cytopenias in adults.
Provan D1, Butler T, Evangelista ML, Amadori S, Newland AC, Stasi R.
Haematologica. 2007 Dec;92(12):1695-8.
N=7. Sustained complete responses 57.1% (no glucocorticoids)
www.haematologica.org/content/92/12/1695.full.pdf

5. Low-dose rituximab in adult patients with primary immune thrombocytopenia.
Zaja F, et al
Eur J Haematol. 2010 Oct
N=48 Overall response Overall and complete responses (CR) (platelet level ≥ 50 and 100 × 10(9) /L) were 60.5% and 39.5%, respectively. The 12- and 24-month cumulative relapse-free survival was 61% and 45%, respectively. (no glucocorticoids)
www.ncbi.nlm.nih.gov/pubmed/20546023

6. Lower dose rituximab is active in adults patients with idiopathic thrombocytopenic purpura.
Zaja F, Battista ML, Pirrotta MT, et. al.
haematol June 1, 2008 vol. 93 no. 6 930-933
N=28. Overall (platelet count > 50×109/L) and complete responses (platelet count > 100×109/L) were achieved in 21/28 (75%) and 12/28 (43%) patients respectively.
After a median follow-up of 11 months (range 3–18), 7/21 (33%) patients relapsed and 3 needed further treatments. In patients with idiopathic thrombocytopenic purpura, lower dose rituximab seems to show similar activity to standard dose.
www.haematologica.org/content/93/6/930.full

7. Clinical efficacy of lower dose rituximab for chronic refractory immune thrombocytopenic purpura
Li Y; Wang XM; Mao M; Zhang XY; Fu L; Ai HM; Zhang LX
Zhonghua Xue Ye Xue Za Zhi. 2012 Mar;33(3):204-6.
Department of Hematologe, People's Hospital of Xinjiang Uygur Autonomous Region, Urumqi, China.
N=20. The responses were of 11(55%) CR, 4 (20%) R and 5 (25%) NR, respectively, with median response duration of 8 months (5 - 23 months).
www.ncbi.nlm.nih.gov/pubmed/22781608#

Thanks Rob, I think if I do decide to go ahead with the Rituximab it would be low dose.
But the issue I have are the reactions. I read the Rituximab Monograph from Roche today and it explained something called cytokine reaction syndrome. It's pretty serious. I can't help but think about the IVIG reaction I had, sure seems similar, and add that I am affected by every tiny teeny sniffle, infection, the sun, stress, drugs, skin cream, you name it as well and maybe there is a reason? I was never given a reason why I had the IVIG reaction I did.
If there is something to this, about me, then I would think it wouldn't matter if it was a teaspoon or a cup the reaction might be the same with the Rituximab?

I also read some about Cellcept. It seems to be a gentler option, if there is one.
I posted about serum sickness, cytokine reaction syndrome, cytokine storm and infusion reaction as I read about them all and wonder if they are all exactly the same thing caused by the same thing? I will do more reading.

Saw my GP tonight about the sore throat and such and talked about the whole thing. She said she would not even want to hazard a guess for me, with my problems with medications in the past. She advised me to talk to my rheumy tell her everything I have learned and then ask her directly what to do and why. Your information post and letter will be very helpful. thanks a bunch, Sandy Too

Sandy - the problem with all of these immunosuppressants is that they raise the cancer risk. I refuse CellCept and Imuran for that reason. Steroids raise that risk as well and since I've been on that for a long time, I try to stay away from other immunosuppressants. I've tried both CellCept and Imuran. They caused GI problems and I was sick all the time with bronchitis and URI's. Of course you can consider CellCept, but I wouldn't call it gentle.

So the rituximab is what you are treating with then Sandi? That and the prednisone.
I thought of you today as I found a study about the Efficacy and Safety of Rituximab in SLE and Sjogren's Syndrome Patients with Refractory Thromobocytopenia. I am unsure how to post it, I am not great with computers. It is from the JCR:Journal of Clinical Rhuematology. Volume 21, Number 5, August 2015. Maybe you have read it. It is what I have kind of been looking for. Something that gives me both the information why I should, and the courage to try something.

However it was a study of 21 cases, with just over 80% overall response rate, and states that it was safe and well tolerated by just over 71% but there were two fatalities. One of infection, pneumonia, this person had pulmonary hypertension. The other was subsequently diagnosed with lymphoma and later died from refractory AITP. That hits home hard. My lungs are a mess, and I too like you say struggle with bronchial issues. Like you also I struggle with GI problems that can become out of my control and very painful but also make it very difficult to eat properly or at all or to take my meds. I am too new to ITP to know how it will play out. I hold out hope that staying on the 10mg of pred will keep my platelets at the 20 mark my hemo would be happy with.
I also learned that sjogren's patients have up to a eight-teen times greater risk of lymphoma due to continuous B-Cell activation. So it tells me about the potential for damage in my body now and into the future, and that perhaps now is the time to try and arrest it. There were 5 persons who had to end the treatment due to toxicity.
The study helps me understand why my long time rheumatologist might have agreed that this was my next step. There is good evidence that it promising for the treatment of many auto-immune diseases and then the ITP too.
But it is not going to be easy for me, at this point in my learning I feel like it's something I have to do, but I feel as uneasy as I did before the IVIG.
Then there is all the immunoglobulin stuff that perhaps is the reason for my difficulties with treatment. I have deficiencies and antibodies to everything, I re-read the first bunch of blood work back in 2003 when I was first told by the rheumy at the time "I would like to tell you you have lupus, but it's bigger than that"....I had absolutely no clue what he was saying. I do now.
Well, at least this time I know better, and everybody in the place will know too or they won't be infusing me with anything.
Education is empowering, and now I know why I have to do this, I guess it has given me the determination, courage and strength to do what I have to do. Thanks again for everything.
Sandy Too.

Hi Sandi too

Education is the key the more you read the more you learn. When you make an informed choice then it is the right choice for you!


PS: what ever happens you make the decision not your doctor!

Sandy too,

Here is the link you wanted to post:

journals.lww.com/jclinrheum/fulltext/2015/08000/Efficacy_and_Safety_of_Rituximab_in_Systemic_Lupus.3.aspx
Efficacy and Safety of Rituximab in Systemic Lupus Erythematosus and Sjögren Syndrome Patients With Refractory Thrombocytopenia: A Retrospective Study of 21 Cases

I don't know what to make of the two cases of pneumonia, one case of lymphoma, and two deaths out of 21 patients. I could not determine whether other immunosuppressants were given concurrently or only previously to the Rituxan therapy (the report is ambiguous in its wording).

To insert a link the easy way, just go to the address bar of the web page you want to link, highlight and copy the URL, then return to where you are writing your post, and paste the URL into your post. (using the link icon is more complicated)