Promacta

I am in my third day of 75mg of promacta and I really do not know anything about it. I still have two more rounds of ritux tomorrow being my third. How is promacta working for others and what should I expect?

Hello. I am 52 yrs old and new to this site and pretty new to ITP. Was diagnosed "by accident" during a routine blood test in August 2014, and my Platelet count then was only 30. Went to the hematologist, did a "wait and see" period of 12 weeks with rechecks and counts did not increase, so started on Prednisone which did not work for more than a week. Then did Retuxin IV treatments, 7 of them, again only worked for a week or two. Was sent to hospital 3 times in the middle of all this for platelet counts below 10 (7, 5, 3) for a transfusion and then sent back home same morning. Finally started on Promacta the last week of my IV treatment (went to hospital with count of 3 at end of that week) and it finally started kicking in but good!! My first blood test after being on Promacta for about a week was in 600's, next count was in 800's so they reduced me from 50 mg/day to 25 mg/day and now my counts are in normal range (240-250 range). I have had 2 recommendations from 2 hematologists to get my spleen removed while my counts are up, since I don't know how long my system/body will keep reacting well to the Promacta, plus they claim they don't know the long term usage side effects since it's a newer drug. Am meeting with a surgeon next week to consult and am seriously considering removing my spleen if it works and gets me off the medicine. Would appreciate if anyone else has similar experience and/or can advise if they recommend getting spleen removed or staying on the Promacta?

Spin:

Splenectomies do not always work and studies show that they work less often in those over 40. When they don't work, you end up back on the same meds you are using now and risks can go up (clots, etc). Also, sometimes ITP resolves within a year and if it does, you will have had the splenectomy for nothing. Spleens can be important. Also, the vaccines that you need prior to splenectomy are not very effective within 6 months of having Rituxan.

Promacta is a great way to manage ITP if you respond to it, and it seems that you respond well to a lower dose. You only need counts around 50k, so the dose can be adjusted to maintain that level. It is a newer drug, but studies have shown that people who have been on Promacta for 10+ years are doing well with few problems.

Good luck with your decision. I know it's a tough one.

Spin, just my story: I had my spleen out when I was 47 but it didn't work. In 2011 I had Rituxan and have been in remission since. If I had to decide to have a splenectomy at my age now, (55) or not, I don't know if I would. Of course everyone is different.

Thank you for the info. I already had my vaccinations to prepare for the splenectomy, as recommended by my hematologist but assume they may not be effective since I was only off the Retuxin for about 4 or 5 weeks before I got the shots. It doesn't seem like my hematologist is very up on things concerning ITP...she is more of an oncology specialist so I think I need to change to a more ITP experienced hematologist! I also just went to a rheumatologist last week and she thinks I have Lupus since the ANA and DNA tests the hematologist did last August were positive - but my hematologist never mentioned I should go see a rheumatologist and that I could have Lupus or other auto-immune based on the test results. Very frustrating...have now started on Lupus meds and staying on Promacta for now.

Hi Spin; I've been on Promacta now for two years and have had absolutely no side effects. As Sandi noted, you seem to be particularly affected by this drug, as I have never had counts above 115K even on the 75 mg dose! The literature on Promacta states that certain groups are especially sensitive to this drug (especially people of Asian descent) and so they typically recommend starting with the lower 25 mg dose.

As for having the splenectomy or not, I went to England for the Indium scan--a test which radioactively labels your platelets and then scans your body to locate where the platelets are being destroyed. The "good" news was that my platelets were "mostly" being destroyed in my spleen, indicating that I would be a good candidate for splenectomy. The "bad" news is that the spleen is only one site of platelet destruction, which means a splenectomy does not guarantee that our clever little bodies won't come up with another way to destroy the platelets (hence this is why no medical facilities in the US will do the Indium scan). Unfortunately, this is exactly what has happened to Rachaely--see her recent posts about post-splenectomy platelet count drops--which makes us all rethink the option of having a splenectomy.

My advice to you is to not rush into a splenectomy. Like you, my hematologists--who all seem to be oncologic hematologists who treat ITP as a sort of "sideline"--had strongly advised me to have the surgery. I also had the requisite vaccinations and a surgeon lined up,but then I started to read the stories of other people like us on this PDSA website, and decided to study the issues further. There is no magic bullet for ITP but as is oft repeated on this site, you need to find what works best for you. Promacta has worked for me, but it is no panacea. My counts have been as low as 14K on this drug, but typically range around 20-30K (50 mg dose), which is good enough that I don't have bruising or bleeding and can live a "normalish" life (e.g no more downhill skiing or building Habitat houses!) Still, keeping my spleen and using the newer medications is what I have found is the best personal solution.

Finally, depending on where you live, there are some great ITP specialists around and talking with someone who treats hundreds of people like us--versus a smattering in most oncology hematalogy practices--would be a great comfort to you; I know it was for me. Just ask and we'll see if we can point you to one. Good Luck!!

Spin:

Those two test results can indicate Lupus, but do you have any symptoms? It can take more than just those two results for a diagnosis, although the dsDNA is a big one. Which meds are you on? I can't believe that the Hemo didn't tell you about those results.

Keeping your spleen is even more important if you have Lupus for several reasons. If you can get by on Promacta, that would be my choice.

I am on Plaquenil (spelling?) for the Lupus and I do have some other symptoms besides the ANA and DNA tests. Mostly arthritis in all my major joints, makes it hard to sleep at night as I constantly toss and turn to relieve the aches, as well as the low platelets (aka ITP) and some other symptoms that were previously attributed to either my hypothyroidism or a result of my bariatric surgery for verticle sleeve (I lost about 70 lbs since surgery) about 2 years ago - freezing cold hands and feet, hair loss, sensitivity to sun, etc. I met with my bariatric surgeon this week to talk about the splenectomy and she agreed with me it should only be done as a last resort if meds don't work. Good thing is that she would be able to do it laproscopically if I need it since my stomach is no longer touching the spleen due to my surgery (which she says is part of the risk - to move the stomach away from the spleen which she already did 2 years ago). So...will stay on Promacta and the Lupus meds for now and I asked to switch to a more experienced hematologist in the same practice for my next visit - hopefully he has more experience treating ITP. Plus I have an appointment with my rheumatologist in a few weeks to get the results of the Lupus blood tests. Just a never ending cycle of tests, doctors, putting more pieces of the puzzle together..... I don't know how everyone who has had this for years does it! It's just exhausting, plus working full time and trying to carry on life as "normal".

I forgot to mention, since I've been on the Lupus meds for a week now, plus continuing the Promacta, my last platelet count went up to around 355! I'm hoping that the Lupus meds are helping the PLT counts and that they may be able to reduce the amount of Promacta I take in the near future, but I know it's too soon to tell - but I have hope!

Just checking in - it's been a while since I shared or asked anything. I am still on Promacta and Plaquenil (for Lupus) and my counts have been so good I have been reduced down to the lowest Promacta dosage available, which is 12.5 mg pill once a day. My last platelet count (last week) was 179. I am so thankful that the Promacta has worked so well with my system - since I started taking it in February all my counts have been in the normal range (for normal, non-ITP, people!) I'm also very happy that I read up on things with everyone's help on this website and did NOT proceed to have my spleen removed as I was being urged to by my previous hemo. I have been feeling like a "normal" person again, one who doesn't have to worry about bleeding, bumps and bruises, but I always have that nagging worry in the back of my mind that my body will start rejecting the Promacta and my counts will drop to unsafe levels again unexpectedly (like it did when I tried Prednisone and Retuxin treatments). I guess that fear just comes with the territory, once you've been in the danger zone you don't forget what that feels like or how it affects your whole life. My prayers go out to everyone who suffers with this horrible disease and I hope they find a cure for it in our lifetime. It makes me appreciate just how precious our time here is, and I will make the most of it while my counts are up and my health holds out - vacation in Florida, here I come this weekend!

Spin:

I have been in remission since I began taking Plaquenil 9 years ago. I don't know if that's what did it or if it is due to the Prednisone I've also been on since then. Anyway, I have read that Plaquenil can help ITP so it's not so far-fetched. I'm glad things are going so well for you!

www.ncbi.nlm.nih.gov/pmc/articles/PMC3623781/

www.ncbi.nlm.nih.gov/pubmed/24254965