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ITP relapse after splenectomy

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7 years 5 months ago #58062 by robc
ITP relapse after splenectomy was created by robc
I am curious as to whether any parents here have had their son or daughter relapse from ITP after a splenectomy. We waited 10 months with my daughter to have the splenectomy after trying steroids, IVIG (only works for about a week), rituximab and NPlate. The started on promacta at 25mg, 50mg, and 75mg but that did not work. She had to be in the ICU for 4 nights over Christmas because of a intracranial hemorrhage which the supposed stats say only happens in 1% of patients. Needless to say her ITP is refractory and chronic.

The splenectomy seemed to work at first as her counts went up past one million, but now are crashing down where she is losing 150K platelets a day. She is already at 250K and dropping. This makes us feel she will get below 50K again. The initial doctor decided to let her stay at 1,000 to 4,000 for 3 months straight to see if it would just resolve on its own. That did not work and then came the brain bleed. Now they are taking her condition very seriously and will not let her drop below 50K (they'll just have her do IVIG for 1 night).

So does anyone have experience with ITP relapse after their child had their spleen removed?

Thanks.

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  • Sandi
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  • Sandi Forum Moderator Diagnosed in 1998, currently in remission. Diagnosed with Lupus in 2006. Last Count - 344k - 6-9-18
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7 years 5 months ago #58065 by Sandi
Replied by Sandi on topic ITP relapse after splenectomy
Rob:
How old is your daughter? There have not been many children here who have had splenectomies. I can tell you that they often fail in adults and unfortunately, are not guarantees.

How long was she on N-Plate and Promacta? Some people do not respond right away and it can take time.

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  • Hal9000
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7 years 5 months ago #58067 by Hal9000
Replied by Hal9000 on topic ITP relapse after splenectomy
150K a day, and no spleen on top of that? Would not have thought that was possible. Makes me wonder that NPlate and Promacta might well be working but cant be seen/measured because the platelet destruction rate is so high. I don't see any combination treatments yet. Seems like that is next. I would be tempted to ask the doctor to keep her on Promacta/NPlate when they try the next treatment - a combination treatment.

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  • Sandi
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  • Sandi Forum Moderator Diagnosed in 1998, currently in remission. Diagnosed with Lupus in 2006. Last Count - 344k - 6-9-18
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7 years 5 months ago #58068 by Sandi
Replied by Sandi on topic ITP relapse after splenectomy
A combo of those two might be risky with no spleen. Even one raises the blood clot risk. I've never heard of both being used at the same time.

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  • Hal9000
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7 years 5 months ago - 7 years 5 months ago #58070 by Hal9000
Replied by Hal9000 on topic ITP relapse after splenectomy
To be clear, by 'combination therapy' I mean therapies like those described in this study - which leverages either Promacta(eltrombopag) or NPlate(romiplostim).

onlinelibrary.wiley.com/doi/10.1111/jcpt.12421/full

In this case, combination therapy targets two qualities of ITP at the same time: antibody production and platelet destruction.

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7 years 5 months ago #58073 by momto3boys
Replied by momto3boys on topic ITP relapse after splenectomy
Hi Rob,

I'm sorry to hear that you have been having such difficulties with counts for your daughter. Despite the crashing counts, it's possible that she can stabilize at some point since you haven't yet reached any kind of an equilibrium yet.

I am now in my 40s, but received a splenectomy as a child for my ITP (I was around 8 years old at the time). This was obviously quite some time ago, and they didn't have all of the treatment options that they do now. Regardless, splenectomy was not a magic cure for me, and I have had to manage my disease all of my life. After the surgery, my counts tended to bounce around anywhere between 30,000 to 60,000ish. When I would get sick, they would crash quite a bit lower, but I was able to live a fairly normal life with those relatively safe counts. Not normal, of course, but good enough to not have terrible symptoms other than easier bruising, etc. I was somewhat responsive to prednisone, and would treat with a short course of the medication after crashes in count to get back into my "normal" range of 30-60.

I hope you can find other parents who are in a similar situation now who can perhaps give you more current responses. I think that splenectomy isn't performed much in children any more because of how often they come back from a bout with ITP and achieve remission. It sounds like you had a very extreme case with very severe symptoms that prompted the surgery.

My only advice would be to wait and see where the counts settle out as her body adjusts post-surgery. Just because they moved quickly from a million (woah! scary high!) to 250,000 doesn't mean that they will bottom out in the single digits! Keep us posted and good luck!

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  • Sandi
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7 years 5 months ago #58082 by Sandi
Replied by Sandi on topic ITP relapse after splenectomy
Okay, Hal. I thought you meant to use both N-Plate and Promacta at the same time. Thanks for clearing that up.

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7 years 4 months ago - 7 years 4 months ago #58124 by robc
Replied by robc on topic ITP relapse after splenectomy
Thanks for the replies. Our 8 year old daughter's counts went all the way down to 31K. Then we did another round of IVIG and she was released from the hospital on Sat evening. She is now on promacta again and MMF which is an immunosuppressant. She has not had any positive reactions to the variety of drugs other than a 2 week effect from IVIG. It appears as if the splenectomy did not really work, but somehow did something to spike her counts all the way past a million. We'd love for her to stay even at 50K but that does not seem to be the case.

Has any other parent heard of MMF working? When I read the literature it seems this is another drug that works about 60% of the time. Everything seems to work 50-60% of the time which is very frustrating as or daughter always seems to be in the bottom 40% for each intervention.

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  • Hal9000
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7 years 4 months ago #58137 by Hal9000
Replied by Hal9000 on topic ITP relapse after splenectomy
The combination Promacta and MMF. Sounds good.
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7 years 4 months ago - 7 years 4 months ago #58140 by robc
Replied by robc on topic ITP relapse after splenectomy
Count was 562K today. Of course this is only 4 days after IVIG. We're on the roller coaster again.

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  • Sandi
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7 years 4 months ago #58141 by Sandi
Replied by Sandi on topic ITP relapse after splenectomy
I have not seen MMF being used very often at all on this Forum, so you probably won't get many responses.
One thing I can tell you is that I have seen people have later responses to splenectomy. Counts over a million have often been indicative of a remission and it is odd that didn't happen for your daughter. However, I have seen people crash after initially high counts and eventually go up to a safe number. There is always hope that will happen. Keep us posted please!

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7 years 4 months ago #58229 by robc
Replied by robc on topic ITP relapse after splenectomy
She is at 305K today. So perhaps this combination is working. She went back up past 600K and is now coming down, but not as fast. All we want is for her to level out after having to go through this surgery.

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  • Sandi
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7 years 4 months ago #58240 by Sandi
Replied by Sandi on topic ITP relapse after splenectomy
Good news! I hope it continues!

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7 years 4 months ago #58348 by robc
Replied by robc on topic ITP relapse after splenectomy
Thanks. She has leveled out this week after dropping to 120K. She's at 126K today. If she can stay here by just taking some pills (MMF and 75mg Promacta) each day that would be a miracle in our book, even after putting her through the surgery.

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  • Hal9000
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7 years 4 months ago #58350 by Hal9000
Replied by Hal9000 on topic ITP relapse after splenectomy
Stable is nice. Roller coaster rides just makes one nauseous.
Curious, is she on 1000mg of MMF ?

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7 years 1 week ago #59649 by robc
Replied by robc on topic ITP relapse after splenectomy
Now she is off promacta and MMF. Both seemed not to really work as she has to get IVIG every 4-5 weeks. That is where we are. We usually get her up to around 250K right after IVIG but 4 weeks later she is down near 40,000. We are going to wait a few months before trying another daily drug.

In essence she is a part responder to the splenectomy. Wishing this would just resolve!

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  • Sandi
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7 years 1 week ago - 7 years 1 week ago #59650 by Sandi
Replied by Sandi on topic ITP relapse after splenectomy
Are you trying to get her counts with treatment to normal levels or safe levels (above 30k)? What did her counts do with Promacta and MMF?
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  • Hal9000
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7 years 1 week ago #59651 by Hal9000
Replied by Hal9000 on topic ITP relapse after splenectomy
Oh, she can go 4 weeks now off of IVIG instead of the previous 1 or 2 weeks - pre splenectomy. That is quite a difference/improvement - to say nothing about improved quality of life. I assume the 4 weeks is without Promacta & MMF. One wonders if the 4 weeks will revert back to 2 without the MMF.

With the improvement, has the Doc expressed any thought in retrying NPlate? As I understand NPlate is more potent than Promacta, plus, NPlate would be safer than trying another immune suppressant at this point.
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7 years 4 days ago #59703 by robc
Replied by robc on topic ITP relapse after splenectomy
For the past 4 weeks she has been on nothing other than the IVIG that was about 4 weeks ago. We are going to try a different daily drug perhaps next month. Our doc wanted to see if there was any change in the reaction to IVIG when my daughter was totally off MMF and promacta. Basically we are seeing the same response to IVIG which wears off at about 4 weeks and puts her back under 50k. We are trying to get her to a cure status, but perhaps that never happens.

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  • Hal9000
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6 years 11 months ago #59736 by Hal9000
Replied by Hal9000 on topic ITP relapse after splenectomy
Rob, really difficult situation. I hope the best for her. A delayed splenectomy response would be ideal.
Might I suggest 'Fostamatinib' as a backup plan. It is currently not available but could become FDA approved next year, in 2018. Maybe your doc could look into trying it then.

There was an individual posting on PDSA while on the initial trial for that drug who, like your daughter, did best with regular IVIG treatments. Apparently Fostamatinib, without any other drug, worked well for him.

The drug seems to work well for only a small set of folks - 18% of ITP'ers. So the drug hasn't received overwhelming praise:
www.pmlive.com/pharma_news/rigel_shaken_after_fostamatinib_misses_phase_iii_trial_target_1173284

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6 years 11 months ago #59855 by robc
Replied by robc on topic ITP relapse after splenectomy
Thanks Hal. We will look into that. I will ask her doctor Dr. Michele Lambert at CHOP about that.
She's at 274K today one week after IVIG and still on no daily drug for now.
We take things week by week.

Thanks,
Rob

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  • Hal9000
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6 years 11 months ago #59874 by Hal9000
Replied by Hal9000 on topic ITP relapse after splenectomy
Oh my that sounds like a nice number off of IVIG. A couple of questions.
- Can you tell me what happened when NPlate was tried. Did it work for a few weeks and then stop, or ? How did the counts go?
- Now that she's off Promacta and MMF, is it clear that it was helping or not helping at all? Did the Doctor think MMF was helping maybe a little bit?

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6 years 11 months ago #59887 by robc
Replied by robc on topic ITP relapse after splenectomy
NPlate did not work at all for her. That was tried probably a year ago. We have not tried it since the splenectomy but the doctor is looking at other agents first. It appears as if MMF and promacta were not doing anything substantial which is why we weaned her off of that. They can't seem to figure out what type of pathway is still causing the platelet destruction.

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  • Hal9000
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6 years 11 months ago - 6 years 11 months ago #59889 by Hal9000
Replied by Hal9000 on topic ITP relapse after splenectomy
Ok. Through a process of elimination, kinda wonder if two 'pathways' could be at work. IMHO, that Promacta & MMF combo test was astute. I wonder if combining either steroids(Dex or Pred) or Imuran to MMF may have yielded a useful response. Perhaps adding Promacta to the two if the response is week.
Good luck...

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