Wasnt sure where to post, Aaron is now 22 dx at age 5. We did Rituxan at age 8 in a drug trial, minimal response over a year made it to 30k from 2k but he leveled up slowly. Remission for 5 years and age 16 we tried Rituxan again, no real response but still in midst of puberty. He pretty much was 2K for a year or so and then we did Nplate for the last 5 years but has stopped working and his count today 1k, no treatments for 2 weeks. Considering trying Rituxan again now that he is an adult. Anyone know of any stories where it didn't work and then maybe it did.
Not big on treatments in general. Thanks for any advice. It's been years since I've been here. When I found this site in 2001, it was so helpful.
I've read/skimmed through many old PDSA postings, such as yours, and made notes along the way. The goal being to try and collect what does and what does not work given one's steroid and IVIG responses. The results can be seen in my
ITP treatments table.
From my notes, what I would ask you is if you've asked your doctor about 'Fostamatinib' - which is a newly FDA approved drug for ITP?
Thanks. The chart is confusing but will look at closer. Aaron often doesn't follow anything...always different lol. He used to have response to Prednisone then when came out of remission, it didn't work. With the NPlate, prednisone worked again. He has only been off 2 weeks but will definitely have to test whether he still gets a response from Prednisone.
He never responded to IVIG as a child so never tried again.
I will look at the new ones.
I prefer not to treat but it's scary being at 1-2k all the time. Got spoiled last 5 years.
Thanks for your advice.
He was at the highest dose (10) of nplate for over 4 years. Earlier this year he hit 200k so they started lower the dosage, we got down to 7 (not sure the exact verbage) and he went down to 20k and stayed there so started upping the dosage and his counts went up slightly but once maxed again and he started dropping down again every week to only 10-15k with the max dosage. We all feel it stopped working. Though in ITP world better than the current 1-2k. But its a lot of work to get the injections for such a minimal bump and it was a great run while it lasted.
I opted against Promacta 5 years ago because of the liver issues. Scared me. And considering he is non active and overweight, to me, not an option at this time.
Yes, Rituxan is one that I have over the many years that I saw people do well on consistently enough that I am reconsidering it. As you mentioned, I guess he did a slow bump and we are pulling his records from 2012 to see how he did. I have all his counts somewhere but after my divorce and moving from a 4 bed house to tiny apt, ugh I think in storage.
After your comment of minimal response, after I find out how he did in 2012, going up slowly even to 20k is better than living at 2k, which he did 2011-2012. Aaron does well at these low numbers, he had 2 bone marrow biopsies with no bruising.
We had great results on special diet but I was stay home mom than and any slip caused crashes (and a father who liked sabotage), I gave up on that. If I could Aaron to take charge of his diet, I know that would be good for him and his ITP. But he won't. Will keep working on that, his crazy work schedule doesn't help.
Thanks for your input. It was helpful. I had not thought that 30k is better than nothing. Other issue I know with Rituxan is the vaccine immunity. At age 8, they said it didn't affect his but that was fresh in his system.
Tidbit of info. When he was in remission, we sent him to Sweden to Boy Scout World Jamboree in 2011. Before the trip I opted to give him the Meningococcal vaccine since he was gonna be with kids from all over the world. It was within that 6 months that he started his decline. I believe this vaccine affected his ITP.
But the alternative if I didn't give was worse.
Thanks again. Feel free to put any other thoughts out. I'm so out of loop and frankly overwhelmed from my life...
Peg, sounds like you need a vacation or at least staycation. (I will gladly join virtually.) I am sorry to hear about the recurrence with your son, but yes, 30K is much, much better than 20, 10, or 1.
A thought: this is the fourth time i see mention of Sweden in the last few days on this board (one girl's mom, Johnny, peg, and myself). I know Sardinia in Italy has a high incidence of a thrombocytopenic disorder (I forget which), but could there be something in the Swedish environment too? :/
Peg ok. Thanks for that tidbit for sure. The vaccination, or maybe an exposure to an unusual local strain of flu, sure upset the apple cart.
It appears that there are two antibodies at work in this case. Let me explain. The previous partial remission is good evidence of row 1 in my table. That's one antibody. The flaky/unusual steroid response supports this notion as well. The high dose of Nplate is good evidence of row 4, the second antibody. The one week IVIG response supports the existence of row 4 as well.
In this particular dynamic the contribution from each antibody is 'additive'. It can be viewed as: the contribution from row 1 plus the contribution of row 4 adds up to the Nplate '10' dose. For example, a 2 Nplate dose can compensate for row 1 and a 8 Nplate dose can compensate for row 4. That gives: 2 + 8 = 10. As you can see, row 4 is very difficult to overcome. The numbers here are just examples but are fairly typical.
On the vaccination/flu making things worse. The question that comes to my mind is which of the following happened.
- Row 1 antibody became worse
- Row 4 antibody became worse
- A new antibody is now in play, either row 2 or 3
I could easily be wrong but I wonder if the first one of these may be the case.
The nice thing about row 1 is the number of options available - as you can see in my table. Certainly Rituxan is one option. With that, the only thing I would suggest would be see if the doc could jack up the steroid dose during the four treatments. I'm not that familiar with steroids but I wonder if 1 to 2 mg per kg would be good. Those that get higher steroid doses during Rituxan seem to have a higher likelihood of good results. A more profound option with greater success chances would be to start Azathioprine treatments and then do Rituxan. Finding a doctor with experience with that treatment regimen might be a problem.
Another row 1 option is just a Dex pulse. But in this case taking large doses of Nplate concurrently bears a risk of higher than desired counts with the combination. Maybe a lower 20 mg pulse first, then a higher/normal 40 mg pulse.
There are lots of other ways to attack the problem. Lots! Trying Fostamatinib (safer) or Cyclosporine make sense as well. Those would have a large impact on row 4 antibodies but little/no effect on row 1. So, even if Cyclosporine treatments caused a row 4 remission, an Nplate dose of 2 or so would still be needed if row 1 antibodies were not in remission.
Diagnosis of ITP in 2014. Ex renal specialist nurse. I retired in Nov 2019 after 46 years on the front line. I firmly believe in empowering patients to be involved as much as possible in their care; always question medics about the evidence base they use.
Thank you received: 424
Or maybe try Promacta (Eltrombopag). Takes a while to find correct dosage but has kept me more stable than anything else I have tried. Have never had IVIg as over here in the UK best practice is only use it as an emergency rescue or pre surgery treatment
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