Trying to avoid splenectomy

Would love to hear your opinions and experiences. Me:

Diagnosed with ITP 2 plus years ago. Did prednisone for almost 18 months. Initially boosted count but eventually stopped working. Did 4 Rituxin treatments. Same result: initially boosted numbers, then they dropped again. I have very few to no symptoms (sometimes I bruise easily, but not always) so I never know when my numbers are low. I was testing weekly but switched to monthly. As long as I stay above 30 my Drs let me go.

Just dropped to 16 and now they are pushing the splenectomy again. I've read too may accounts from people having the surgery and then their counts go back down... and now they have no spleen as well. I've read both positive and negatives about both NPlate and Promacta. I'm being approved for Promacta right now. I am never sick and very rarely have side effects from any medications and, as I said, have very few ITP symptoms. I was diagnosed with celiac disease and eat gluten-free, (although I also have no symptoms of that either.) I have low ferritin and have had issues with anemia. I'm a distance runner- gained weight on the prednisone but just lost 30 lbs and am back to where I want to be. They are hesitant about me running with counts this low, but as I have no symptoms it's hard to reconcile that with any issues.

I'm a bit perplexed as to why I keep getting the surgery pushed on me when we haven't tried NPlate or Promacta.

What are your experiences/results, positive and/or negative with Promacta and/or the surgery?

Thanks!!!

I'm a bit perplexed as to why they keep pushing the surgery without even trying the latest ITP treatments as well. I'd be thinking strongly about shopping around for another hematologist. Some of them are just in love with splenectomies. I've found that ones who are more focused on other conditions (oncology, anemia, other blood disorders) tend to be more surgery pushing. It's like they have this new, cool thing they can try out on you. Uhhh, no thanks.

Regarding surgery, yep, I was given a splenectomy around age 9. They didn't have many treatment options back then (prednisone, IVIg, or splenectomy), so I don't blame them too much (but I reserve the right to be a little bitter since I didn't get to choose, lol!). My presence here so many years later is a tribute to the fact that the surgery didn't work for me. In addition to treating my ITP and low platelets, I have the additional worry about infections and an increased risk of clots because of my lack of a spleen.

Promacta, on the other hand, has been working really well for me. I have been on the medication for more than a year and have kind of settled out with a dosage of 50mg. My last few counts were in the 40s, which is very close to the target count of 50. Safe, but obviously not normal numbers. I haven't had much of anything that I'm aware of in the way of side-effects. It sounds like you have had a fairly good run of avoiding treatment with counts above 30, which is exactly what you'd want to do. Before starting any treatment, I'd wait and see if your count of 16 was just an outlier. We all get random things that drop our counts here and there, and if you are generally running above 30 and the 16 is a bit of an anomaly, I'd continue to avoid treatment. If you get a bunch of counts below 30, then NPlate or Promacta are great options!

penlyn, I notice from previous posts that your counts fluctuate. Do you suppose that 16 is just a low outlier, as mom23boys mentions?

Also, you've taken IVIG once before? How did that turn out. No response and had to use a Dex pulse, or, rise to normal for a couple of weeks, or ?

It seems that folks that have counts (and steroid dosages) that wander/fluctuate tend to have trouble with stability similarly when on Promacta/Nplate.

I'm not sure why they keep pushing splenectomy either. So many doctors believe that it's a quick fix and no big deal. There are so many other options out now and there is always a chance of remission (without it). It took me 8 years, but I did go into remission and have been okay since 2004. The TPO's were not available then but if they had been, I would have tried them.

I agree with everything that Mom ^ said!

My spleen was removed on August 1 this year. There are a lot of complications and concerns that you should consider before going that route. I did because nothing was working for me, the only drug I haven't tried is NPlate. I have had quite a few months since surgery. You can see my Post Spleen Journey in the recent posts.

Hal... I've been all over the place since diagnosis. Even in the middle of my month of Rituxan treatments I went way up one week and then way down the next. We've been hovering between 30 and 70 the past 6 - 7 months. I did IVIG once and Dex once, both right after super low counts (of less than 20.) Both worked. I am very lucky that EVERYTHING has worked initially. And, of course, having zero symptoms... I never know until the CBC where I am. I'm going to try the Promacta, if it becomes financially feasible. I guess I was looking for verification (and maybe support) here that it's NOT time for the surgery!

Thank you everyone. I am definitely going to continue with the Promacta plan. I appreciate your feedback. I'd like to keep my spleen as long as possible!

P

penlyn63, thanks for the info - like to add ITP experiences onto my list.
Hopefully that 16 is actually a outlier. But I have to tell you. That Rituxan treatment fail was an 'outlier' as well. That isn't common for folks that respond to steroids and IVIG from what I've seen. If your fluctuating platelet counts has a cause other than your immune system, that would explain why Rituxan didn't work. I'm no doctor. Other culprits have been found and reported here on the forum but I have no comprehensive list of those. The only one that immediately comes to mind is thyroid issues.

So here's an interesting new wrinkle! I just got a call from the drug company who has the Dr's request for my Promacta. My insurance company denied me the Promacta "BECAUSE I HAVE NOT HAD A SPLENECTOMY." What the.....??????????????????? That's why I want the damn drug, people!!!

penlyn63,
Have your doctor challenge the decline - I was able to get it approved with a call from my Hemo and documentation showing falling counts on Prednisone. My Hemo was also able to get me on the assistance program with Promacta and I only have to pay a $25 copay instead of the $4,000+ cost.

Thanks Cindy. That is the plan! I'm concerned also because my Dr WANTS me to have the splenectomy. I'm the one fighting it. But at this point I feel like we still haven't determined whether the spleen is even really responsible for the destruction of my platelets. I've never had my bone marrow checked. Maybe I'm not creating enough platelets? I haven't been able to have a colonoscopy or endoscopy either because of my low counts. I just think at this point it is premature and the Promacta may help.

Penlyn:

If this doctor will not fight your insurance denial, get another doctor. Many people that use Promacta have not had a splenectomy, so that isn't always a requirement. I've had drug denials approved several times by appealing. Don't get discouraged yet.

A bone marrow biopsy will not determine if the spleen is the place of destruction. Only an Indium Scan can do that, and as far as I know, it is only performed in the UK. Most people with ITP have both destruction and production problems as per recent research. At this point, unfortunately, it takes trial and error with a treatment to successfully treat ITP. (Or you can ask Hal to figure out what you might respond to).

Thanks Sandi! I guess my point was really that I still don't know whether I'm creating platelets and my body is destroying them, or whether I'm just not creating enough to begin with. (And probably both!) So why assume it's the spleen and remove it at this point? I intend to fight the denial and the PA-C that works for my Hem, who I see most of the time, is on my side on this one!

Thanks!!

Most people do not know the answer to that question. Those tests are not normally done in the average doctor's office, only for research purposes at this point. Many doctors are still under the assumption that a bone marrow biopsy will determine if a person is making enough platelets. That's not true; it won't. This information came straight from some of the top ITP Specialists associated with the PDSA. That became a big question a few years ago when the TPO's came out. Patients and doctors were assuming that the TPO's wouldn't work or shouldn't be used on someone who had a 'normal' bone marrow biopsy.

This thread might help:
pdsa.org/discussion-group/6-general-itp-discussion/11698-bone-marrow-biopsy-and-production-answers.html

Great! Thanks for the info!

Sorry kid, but the splenectomy is nothing but a shot in the dark as far as success. The only way to know where the main site of destruction is is to have the Indium Scan. Even then, it will not guarantee success, but can show possible failure. We've had very few people here have it done, but one woman had a splenectomy based on the Indium results which showed primary destruction in the spleen. The splenectomy did not work. Another woman had the Indium and it showed primary destruction in the liver. She saved her spleen. Many times, the results are not conclusive. For example, you can end up with 40% liver destruction and 60% splenic destruction. What then? There is also the chance that after the splenectomy which is initially successful, it will fail down the line because the liver takes over destruction.

The type of antibodies that a person has also plays a big role. At this time, those tests are also not routinely performed and there is little correlation between antibodies and what treatment a patient will respond to. That is actually in the initial stages of research, but has not hit doctor's offices as a protocol for treatment. It's all getting there, but is taking time.

Sandi is correct on all counts. An Indium scan will show where the platelet destruction happens. If is shows only splenic destruction then there may be a hope splenectomy will work. However the rest of the reticuloendothelial system will take over the spleen's job ergo the liver will destroy the antibodies attached to your platelets.

In the UK Eltrombopag (aka Promacta or Revolade depending on the country you live in) is authorised if a splenectomy is contra indicated. If a patient does not want a splenectomy then that is a contra indication. Either your haemo takes this on board and supports you or you get a new haemo who does.

My insurance denied payment for Nplate(more expensive than Promacta). The criteria was that I needed to fail 3 treatments OR fail a splenectomy. I actually had failed 3 treatments which is pretty easy to do- prednisone, dexy, Rituxin. The staff at the hemo's office who handle insurance issues appealed the decision. It took a long time, the bills were piling up! but it finally was approved. My hemotologist was also in favor of me getting a splenectomy before going on an expensive drug like Nplate. He was pretty angry at me but I held my ground. Luckily it was the wonderful women in the business office that fought the insurance company and not my doctor. But coincidentally, he had another patient later that year who did go ahead with splenectomy. Her counts went way up and then after just a few days crashed to lower than they were before the surgery. And the counts would not return to pre-surgery levels. She went onto Nplate then. He told me that story I think as kind of his way of saying it was okay that I refused splenectomy.
As Sandi has said in the past, you never know when you'll really need your spleen. I believe its much more important than its given credit. And as DMann's story goes, I've read several accounts of people having blood clots after splenectomy. So do what you think is best for YOU!