Splenectomy Failure?

I am an 18 year old girl who was diagnosed at 8. I have tried every treatment to pretty much no success, so when I turned 18 at the end of June it was decided to have my spleen removed. The date was set for August 15, 2018. They gave me steroids to prep and going into surgery my count was 55k. Two days later my count was 53k. I was told not to worry but to restart my Promacta which I took a daily dose of 75mg. Two weeks later my count had climbed to 139k! (Which was higher than it had been since I was diagnosed. Even on the 75mg daily my count bounced around between 20k and 30k.) However at my last blood draw on September 30, 2018, my count was 59k. Does this mean my surgery has failed or is it normal for counts to bounce around following surgery? I have heard about people's counts soaring after surgery but mine never did. Is it to earlier to declare failure?

Hi Rors. If it ends up not working, most people in your situation would go to Nplate - a higher dose of Nplate. The higher doses can stimulate more platelet production than 75mg of Promacta can. Nplate doses range from '1' to '10' micro grams per kg of weight. It will probably take around a 5 to 7 dose.

I assume you failed Rituxan? Also, that IVIG gets your counts up for about a week?

Yes, I have failed Rituxan. IVIG only manages to raise it a little. I was on Nplate for 7 months however it gave me horrible joint pain that resulted in trouble moving my smaller joints.

Rors:
Generally, if counts don't respond right after the surgery, it probably didn't work. However, I have seen a few people who had counts that bounced around for a while and settled into a safe range. I wouldn't give up hope just yet.

Everyone has a different idea of 'success'. Some would only be happy to have counts in the normal range without treatment. Some would be happy to have safe counts with treatment.

Mine bounced around for 6 months before settling down, so I would give it a bit longer.

What does your physician say?

Hi guys! I was diagnosticed with ITP 2.5yrs ago. Since then I`m taking corticosteroids and from time to time IVIg. Respond to both threatments was none last two -three months (platelet counts were 2,000-8,000).
After all, I decided to remove my spleen in search for a more permanent solution. The procedure was two weeks ago. One week after the splenectomy my counts were 406,000. Today, two weeks after the operation, my counts have dropped at 58,000. Please help!!!
What are you experiences? What does that mean? Could it be possible that the whole procedure was unsuccessful? I˙m a 34yrs old mom of a baby-girl and soon I will have to be back at work and take care of her. What are my options? What to expect?
When will I know for sure the outcomes of the procedure?
Many thanks, Valentina

Valentina:
It's difficult to tell whether the surgery worked or not. Many times, it does not. At this point, your counts could continue to drop or they could stabilize. Only time will tell.

If counts do drop too low, you have other options. Ask your doctor about Promacta or N-Plate; they both have fairly good success rates for getting counts up but both are maintenance treatments.

I hope for the best! But if I have to take other drugs which one is a better option, having in mind side effects?

Valentina, if counts continue around that number it sounds like you got a 'partial' remission from the splenectomy. Everyone that I've seen here with a partial remission has NOT sought further treatment to raise counts higher.

From your overview, about losing responses (row 1 to row 1 & 2), counts going up to 406 (row 2 resolution), a partial remission seems possible. Likely? I dunno. It would seem both full remission, and collapse with Promacta required, outcomes are still possible too. The 406 was nice.

Let us know how things go.

Both of those meds have tolerable side effects, some people don't have any. The decision basically comes down to taking a daily pill (Promacta) or getting a weekly injection (N-Plate). Promacta is usually easier and counts stabilize better.

Dear all,

here I`m again - 3 weeks after the splenectomy my counts are even lower - 16.
My hematologist made a conclusion: the procedure is without reply and we have to start with new therapy: Promacta or N-plate. The decision is mine.
I have a few questions:

1. Can we be completely sure that splenectomy was unsuccessful? Are there any examples when organism needs more time to stabilize and get custom to a "new situation" (to be without spleen). Maybe some of you have personal experience to share?

2. Are there any time restrictions for taking this two new drugs (Promacta, N-Plate). For how long I can take them - a lifetime or there is some suggested length period of usage? What are your experiences: how long you took them and what happened after the threatment? Did you menage at some pont to live drug free?

3. Which one you would recommend (having on mind side-effects). I have to meke my choice by tomorrow.

4. If both medicaments failed - what are my options?

I`m relly confused now - this situation is new for me and I would like to be good informed and make the best possible choice. Please help!
Many thanks and best regards from Croatia,

Valentina

Having done Romiplostim (N Plate) & Eltrombopag (Promacta aka Revolade) I would opt for Eltrombopag

My son just started Promacta little over a week ago and so far we did not have any side effects. The only difference between Promacta and NPlate is Promacta is a once a day pill and NPlate is a weekly injection. Also with Promacta you need to follow some dietary restriction. But it is very manageable. My son just does not eat anything after 7 pm and take Promacta right before bedtime around 9:30 pm. I read a lot of posts, it seems that Promacta is better to keep our counts stable since you take it once a day.

I agree, I'd start with Promacta (preferably a low dose). Some doctors start with a high dose which can shoot counts up too fast and places the patient at risk for a blood clot. A slow climb is much better.

It is possible to go into remission with Promacta. I would never say that you'd be on it for life....anything can happen.

Hi guys!
Thank you all for your helpful and informative answers.
I recieved IVIG last Friday and soon going to start my treatment with Promacta.
Like Sandi said - we`ll see what future brings. Let`s hope for the best.
Kind regards,
Valentina

Hello all, here I`m again with the updates.
After I received IVIg at Friday , today - 4 days after - my counts are 92.
It`s good to that before splenectomy IVIg could raise counts max to 20 - even when I received it two or three days in a row (40mg always).
How to explain that? Is it possible that I have "partial remission" after the splenectomy after all?
My doctor said we could postphone the beginning of Promacta intake for a while.
Do you have any thoughts, experiences, recommendations?
Thanks a lot!
Valentina

Hi Valentina,
Good that your counts went to 92! How exciting! Just a comment from my experience with ITP -9 years and 5 different drugs, still have my spleen. Do you have any kind of serious bleeding issues when your counts are under 20K, under 10K? If you don't have a problem with bleeding, I would just wait and not start Promacta. I would want to wait to see whats going on. Its really best to do only one treatment at a time. The target count for Promacta is 50K so you are already higher than whats recommended for that drug. There is a risk of stroke and blood clots with Promacta and Nplate if counts get too high, best to keep counts under 100K. It sounds like you doctor is sensibly cautious to even suggest waiting. Good luck- hope you are in remission!

Valentina - I wouldn't quite commit to saying that you have a partial remission just yet. When you can go six months without needing treatment, that would be a partial remission. Sometimes treatments work a bit better after splenectomy; only time will tell. Please keep us updated!

Hi guys,

10 days after IVIG my counts are 18.
Tomorrow I`m starting eltrombopag / 50mg.

Kind regards,
Valentina

I'm sorry. I hope Promacta works for you.

Give the Promacta time to work. It can take a while

Ha Clia. I'd be interested to hear what dose of Promacta you end up on. I'm secretly hoping a partial remission is possible.

Dear Hal,
thank you so much for your kindness and superb wishes!
I wish to you and everybody here all the best in a new year!
Here are the updates. I started with 50mg of eltrombopag (Promacta/Revolade). My counts were below 10 back than. After two weeks of usage counts were 35 and one week later they dropped at 25.
In a few days I will finish my first package (28 tablests), so my hemo has suggested that for a next cyclus I start using 75mg daily.

I don`t know what has happened? Why my counts have dropped? What can be the reason behind? Can drug become ineffective somewhere in the middle of the therapy? Also I have cought a small cold, so maybe that`s the answer.

Best,
Valentina

My count always drops when I have a cold.

Ok, 75mg for awhile. Then see how it goes.

Dear all, can you pls help.
My counts after 28days of Promacta/50mg when they were 25K, after 7 days on even higher dose of Promacta775 mg are even lower - 24K.
I feel many side-effects on this higher dose (I`m 58kg).

What to do if that droug failed? I have zero effect on steroids (I`ve been taking them for 2+yrs), ivig is not a solution for me (when I received that therapy two or three days in a row every week / 40mg my counts are around 20), I don`t have my spleen any more...

Any solution, recommendation or your own experiences to share?

Thanks guys,
best, Valentina

Difficult situation Valentina. Most who respond to steroids respond to Promacta as well. Are you taking a small amount of steroids now? Some folks though, can take a while to respond. Perhaps that is your situation? Don't know if this is the case, or, you're just not responding to Promacta. Too early to know. In the mean time, a 24 and 25 count isn't too bad. No bleeding symptoms?

Is the doctor wanting to stay on 75 mg for a little while longer? Maybe switch over to Nplate after a few weeks if counts don't rise? Are you going to be able to stick it out for that long with side effects from the 75mg?

Hello Valentina,

A couple of questions:
1.) first...did you have an Indium Scan prior to spleen removal as a way to know if your spleen or the liver was doing the destruction.
2.) Did you ever have a serum thrombopoietin blood test done. See link: ash.confex.com/ash/2018/webprogram/Paper117267.html
This paper from December of 2018 (fairly recent) indicates that if your thrombopoietin levels in the blood are above a certain amount...you will have a less likelihood of responding to NPlate (weekly shot) or Promacta (daily pill). The higher the level..the less chance of a response to either drug. The levels for a response are indicated within the paper itself.

3.) Did your platelet counts skyrocket to over 150k after an IVIG since you were diagnosed?
4.) Fostamatinib is an option but did not work for me at 100mg and 150mg daily.

Here is my situation...I went for an Indium Scan that shows that my spleen is primarily the culprit and I have an appointment with my surgeon on Feb. 6 to schedule my surgery. I am going to have my serum thrombopoietin levels checked within 2 weeks because I was not responsive to the highest dose of NPlate (level 10) and I am now 2 weeks in to 75 mg of promacta without a response. Believe it or not ...Dr. Provan (ITP specialist from London) has indicated that I can go up to 100 mg of Promacta to try for a response prior to my spleen removal. My hemo just put the order in for 2nd week of February to increase to 100 mg of Promacta daily. Please keep in mind that I am on 20mg of prednisone daily + 75mg of Promacta daily + mycophenolate mofetil (MMF) 2000mg daily = platelet counts under 20k. So I can share in your frustration.

Dear Hal,
thanks a lot for your kind reply. It means a lot to have you here. You are such an awesome supporter, as a someone who knows the problebs of this area and what to share the knowledge as well as excellent empathetic human being.
After 2.5yrs we quit on steroids, no results with them and many negative side-effects. At the moment I`m only taking Promacta. I spoke with my hemo and she decided to come back to 50mg dose. We`ll see the counts after one week. I`m not happy with 75mg dose. Can`t stand it.
I˙m not happy with my history of taking quite strong drugs for a quite long now. i found out that I have some kind of cyst on my liver. I didn`t have that before taking steroids, ivig, eltrombopag. Feel like an experimental bunny.
I would be happy that my organism can hold the platelets around 20 or 30 on its own. Its like we are healing something relying on the method of trial and error. The drugs I`ve been taking aren`t helping to solve the problem. Actually, they are causing the new ones in some different places/organs. Whats the use of taking Revolade anyway when we know that it is not solving the problem with low platelets permanently. I know that we have to raise the number of platelets at the present moment. It is not desirable to live below 10K, it can be quite dangerous, but on the other hand I`m quite happy because I don`t have massive bleedings. It can be rough during my periods for a couple of days but that`s all. I used to have massive bleedings from my nose and gums, but not any more. With the present counts I don`t even have the bruises, only petehiae.

Thanks for the super nice compliment Valentina. Sandi is my roll model.

Ha ha, yes. It is easy to feel like an experimental bunny/rat with ITP. Promacta needs to start working, soon, to shake that feeling. As I recall, it took 'momto3boys' a month before she started responding. Sometimes a bit of steroids can speed things up.
Hopefully you're getting a bit higher counts now with the splenectomy.

Question. You mentioned IVIG. How do you do with that? Does it elevate counts for a couple of weeks or ? Also, has IVIG always worked that well?