My Promacta Journey

Today's lab results

3/1/17 plt 127,000
dosage 75mg promacta daily (except for the last 2 days at 50mg)

Yikes! I knew my count was high because of some obvious pathology with my monthly cycle and just decreased my dosage because of ever growing clotting fears. This number is terrifying, and I am continuing my newly decreased dosage of 50mg daily. I have been eating mostly paleo except for a few lapses here and there. Maybe this means the diet does so well for me that I will need way less medication. Or maybe the act of losing a few pounds (nothing too significant yet) triggered the uptick. Whatever it is, this is a scary scary number for me. I am picturing clots flowing around my body and I don't like it. I really want to be on the low low end of safe counts with this drug that is producing crazy huge platelets. Hopefully I'll go down with the dosage decrease.

My consultant says that the protocol for Promacta states that the preferred count should be between 50 and 150. After a crash to 1 in 2015 my count has gradually increased to 110 after 14 months on 75 mg Promacta daily. I asked today about the clotting risk but he stuck to his guns and said he would not be reducing my dose unless I got to 150. He is worried that I may crash again and so am I . I think it is not safe for you to be tinkering with your dosage yourself. There is no clear link between dosage and count , you run the risk of a crash. The first step should probably be to reduce to 50 mg twice a week and wait and see what happens but you really need to talk it through with your consultant. I eat anything and everything and drink alcohol in moderation and my count has not been adversely affected.

The prescribing information calls for increasing the dose with counts <50 and decreasing the dose with counts > 200, so it's hard to argue with the 150 benchmark, especially short term.

Margaret k wrote: My consultant says that the protocol for Promacta states that the preferred count should be between 50 and 150. After a crash to 1 in 2015 my count has gradually increased to 110 after 14 months on 75 mg Promacta daily. I asked today about the clotting risk but he stuck to his guns and said he would not be reducing my dose unless I got to 150. He is worried that I may crash again and so am I . I think it is not safe for you to be tinkering with your dosage yourself. There is no clear link between dosage and count , you run the risk of a crash. The first step should probably be to reduce to 50 mg twice a week and wait and see what happens but you really need to talk it through with your consultant. I eat anything and everything and drink alcohol in moderation and my count has not been adversely affected.

Thanks for the thoughts Margaret. My doctor has decreased my dose when I've gone above 100,000 in the past. When I look at others on this medication, I don't find a lot of people who have already undergone a splenectomy, as I have. This makes me more "high count" averse than I would be if I still had my spleen to manage clotting behavior.

In particular, when I am observing extremely abnormal clotting behavior in my body at the moment, I would much rather have my counts crash back down than to have complications from numbers that are too high. Even at my relatively recent lowest of 18,000, I was having no symptoms. I would much rather be there than dealing with a stroke or DVT. You can't undo a stroke, but you can work the dosage back up from a low count. Risk all around though, definitely.

I don't know. There are no easy answers for sure, but lacking a spleen makes me VERY high count averse Honestly, regarding the doctor and dosage, I don't feel like she has tons of experience and she mostly agrees when I tweak the dosage here and there.

Thanks for the input!

Rob16 wrote: The prescribing information calls for increasing the dose with counts <50 and decreasing the dose with counts > 200, so it's hard to argue with the 150 benchmark, especially short term.

I fully concur that I am probably overreacting (but my doctor is on board as I'm driving this train, haha). It's scary when things go strange with our bodies, and I need to research more about whether there is good data for asplenic patients on promacta and risk of thrombosis. I mostly read about people who have managed to keep their spleens on this medication.

Maybe lower counts are like a security blanket for me :lol:

I completely agree with you Mom, risks of a CVA / PE/ DVT terrify me too. I'm very early on in this Promacta journey and fully intend to sort my own doses out to maintain a count as near to 50 as possible.

For what it's worth, I'd rather have counts on the lower end too. It's mostly about comfort zones which depend on past experiences and treatments. Being asplenic would make me more cautious too. Once you do this for a while, crashes are not as scary if you know that you can deal with them and get counts back up.

My comment above was directed to Margaret k, who wrote, "After a crash to 1 in 2015 my count has gradually increased to 110 after 14 months on 75 mg Promacta daily."
momto3boys has a different situation, with a history of fairly decent counts at a lower dose, except in times of illness. As long as she and her doctor are making very gradual changes, I am in favor of tweaking the dosage to keep it in the 50 - 100 range, or even slightly lower. I am a little concerned that minor illnesses seem to cause significant dips in platelet counts.

The problem is that at least for me it is not easy to tweak the dosage to get a desired count as the condition seems so variable in itself and in the response to medication.The last time I was on Promacta I went from 3 to a count of 200 on 50 mg( in order to get neurosurgery) in 3 months around Nov 2012 . After that the dosage was 'tweaked ' to some days at 25 mg but my responsiveness really tailed off and by Aug 2013 I had difficulty in getting to a count of 50 on 50 mg( in order to have minor surgery) .In 2014 my count hovered around the 50 to 30 level and by May 2015 it had gradually declined to 10 on 50 mg so I was changed to Romiplostin (no sustained improvement.) I then hit 1 when awaiting Rituximab.So you can see why I am reluctant to play about with the dosage now.I think my consultant regrets not sticking with the Promacta through the low patch and is wary of rocking the boat now. Although I too can function normally from 10 to 20 it is restrictive being at that level in that I can't fly and I do love to travel! At my current rate of improvement it might only be about 3 months before experimental tweaking could begin - I' ll keep you posted .

Margaret - it's like I said above. It all depends on comfort level and past experiences with counts and treatments. Everyone has a different situation and you have to do what suits you best.

Margaret k wrote: ... At my current rate of improvement it might only be about 3 months before experimental tweaking could begin...

Margaret, forgive the Promacta novice question.
So you are saying that after being on 75 mg for awhile your counts consistently rise slowly? If so, would you know if that is generally true for others as well?

Reading more about your history Margaret makes me understand why you would be more reluctant than I am to have a count crash. It sounds like it's been REALLY hard to get you up into good numbers and safe counts. I, on the other hand, haven't been lower than 18 in recent memory (and don't recall ever being below 10) and haven't had the experience of extremely low scary counts. I have been responding fine with upping my dosage on the promacta (and most of my life had a really great response to prednisone, which meant that I could fix low counts with really short bursts of it), and this makes low numbers less of a problem for me thus far. (In my particular case, it's easier to fix a low count than to risk an adverse event at a high count)

We are all so different, and respond so differently to various treatments. This is one of the reasons that I love reading a wide variety of personal histories and responses. Thanks for contributing your experiences, and I'm so happy to hear that your numbers are good for you now, especially with the scary numbers you've had to deal with in the past!

Just a little note regarding symptoms as of late:

I have been having chilled extremities which has been particularly bad the last three days. My ears are driving me insane, and feel cold all the time. I have never had symptoms like this before, and of course reading about circulation and little clots blocking flow isn't helping my high count paranoia. I'm planning on going in for an extra count tomorrow to make sure my numbers haven't gone any higher since last time.

Keeping track of all the little things on my promacta road. I will update after my next labs. I may have to try to find some ear muffs, ugh! It is really annoying!

mom,

Read up on Raynaud's disease (--phenomenon, --syndrome) and see if it might fit. It can even affect the ears, though less commonly.

Thanks for the tip Rob! Maybe this is something that I have in a really mild form. The tip of my nose also gets colder than that of the average person, and in the last few days a few toes on one foot were chilled on one occasion (but no weird paleness as in some of the photos). I don't know what's been up for the last 3 or so days, maybe sudden onset, lol! I'll have to watch these symptoms and look into this further (especially if my count has gone back down tomorrow, as I hope it has).

Thanks as always for your useful input!

That's how I get having Lupus, cold all the time. It can also be a thyroid symptom, or maybe you're getting sick.

Hi All,

Speaking of Promacta Journey's I 've been on a solid 50 mg dose for more than 2 years and have always had counts in the 100's+. I thought about tapering back the dosage to 25 mg recently and my doctor was for seeing what would happen. So far its been going well!! My counts are in the 60's range and my doctor wants me to try taking 25 mg every other week. He thinks, knock on wood! I might be able to ween off !! I don't want to get overly excited because this whole ITP thing has been a roller-coaster, but man would that be something !

I wanted to share some good news!

Thanks,
Dan

aero8991 wrote: Hi All,

Speaking of Promacta Journey's I 've been on a solid 50 mg dose for more than 2 years and have always had counts in the 100's+. I thought about tapering back the dosage to 25 mg recently and my doctor was for seeing what would happen. So far its been going well!! My counts are in the 60's range and my doctor wants me to try taking 25 mg every other week. He thinks, knock on wood! I might be able to ween off !! I don't want to get overly excited because this whole ITP thing has been a roller-coaster, but man would that be something !

I wanted to share some good news!

Thanks,
Dan

That sounds great! Thanks for bringing some good news to my thread (now that I'm about to post some crappy counts, lol!) If you have had really stable numbers it sounds like you are a great candidate for slow dosage decreases. Keep us posted

Here are the labs from yesterday:

3/8/17 plt 16,000
dosage 50mg promacta daily

So, that's quite a drop from the 127,000 of last week. In some upsetting news I found out that my hematologist had a brain aneurysm and is on medical leave until May at the earliest. That certainly puts everything in perspective.

It's definitely my fault that I had such a big crash (dropping dose from 75mg to 50mg, though I'm still surprised that it happened in just one week) , but I don't regret it a bit. All of my spidey senses are tingling that there is something weird going on with me and that my clotting behavior is still completely out of whack. I just don't feel right. I have NO low count symptoms, and there was hardly any blood on the cotton ball after the blood draw, which is completely unheard of for me. I find myself wondering about margins for errors on CBC counts in cases where platelets are absolutely huge and clumping together. (I need to research more)

Because of the low count, I triggered the "must see the doctor" valve and spoke with another hematologist in the practice. I told him my current dosage and that I'm unwilling to go back up to 75mg for now. I explained the lack of symptoms and my extreme skittishness because of my lack of a spleen for filtering. All of my instincts are telling me that being on that long stretch of 75mg has changed something significant in my body, just observing how everything is behaving. As long as I'm asymptomatic he's fine with me coming back next week for labs to see where I stand, and just to follow up sooner if any symptoms crop up. I sort of feel like saying "stop the ride, I wanna get off!" and to go back to no treatment, but I have to see this out for the moment. Whenever I can next have a good long "sit down" with a doctor, I'm going to talk about ways of backing off on the promacta. All of these fresh, delicious platelets have surely amped up my immune system so I can't just quit cold turkey. Maybe they can manage the effects with prednisone for a period to get me off. Or maybe after I'm on lower doses, my body will return to bleeding like it's supposed to and I'll stop freaking out, lol.

Tune in next week for the next episode in the saga

FYI, about the amount of blood on the cotton from a draw. From what I've seen the amount depends on which vein is punctured. It appears there are two main veins in the arm (fold) that are the biggest. When looking at my right arm, the vein on the right produces a lot of blood on the cotton and the vein on the left does not. I think the amount has to do with how far below the skin surface the vein is. Your millage may vary...

It's called the antecubital fossa; & personally I don't think it matters which vein is used, it depends on the technique of the phlebotomist. I bruise every time with 1 and rarely with another irrespective of my platelet count.

Agree Mrs. B. I've had a lot of bleeding and even hematomas with high counts and no bleeding at all with low counts. Some techs are good and some are not. Vein scarring might also have something to do with it when you've had it done for years on end. I have one arm that they can never get blood from any more. I once had four unsuccessful sticks for an MRI with contrast and they ended up using my foot...couldn't get a needle in my arm veins.

Sometimes that needle going in hurts like heck and other times, you barely feel a thing. It's the tech.

Hi mom-- I don't want to offend and I could be wrong, but in my humble experience with ITP, I would just like to encourage you to not worry about NOT having bleeding symptoms. ITP and our bodies are always changing, there are so many factors. Is there anything you are doing differently that could be helping your bleeding symptoms?
I don't normally have bleeding symptoms at low counts. But this week my counts are at 52 and I have two bruises! I was surprised, I would have guessed my counts were in the teens. I took Ibruprophen last week so perhaps that caused the bruising? hard to say
Once I went to two different labs for two different doctors. At one lab my counts were 15K and at my hemo's lab my counts were 30K. That is a huge difference to me! but my hemo was not surprised. He said it made sense as there is a big variation between labs. And told me that I should stick to one lab. Also as you said there can be platelet clumping esp in the violet topped tubes.

I have been on Promacta several times since 2011 and also Nplate currently. I totally agree with your decision to stay on the 50mg. My counts always drop when I change doses. Give it a couple weeks and they will likely come back up. Thats how it goes with me anyway. I believe its best to find the dose you like and stay with it. I have seen how my immune system will settle down if I keep the TPO dose steady.
All said though, I do think Promacta is a bit of a weird drug- just my opinion. So I understand if you just want off of it. Once I thought I was having some kind of DVT/ vascular event in my legs from Promacta. My counts went over 100K and the skin on my legs turned white like marble! So freaky- I quit Promacta immediately. The color came back in about 24hrs. I saw the nurse, she sent me to a cardio-vascular surgeon who did ultrasound. My veins and arteries were fine. Year or so later I read that "pale skin" can be a side effect of Promacta (?!) anyway, good luck!

poseymint wrote: Hi mom-- I don't want to offend and I could be wrong, but in my humble experience with ITP, I would just like to encourage you to not worry about NOT having bleeding symptoms. ITP and our bodies are always changing, there are so many factors. Is there anything you are doing differently that could be helping your bleeding symptoms?
I don't normally have bleeding symptoms at low counts. But this week my counts are at 52 and I have two bruises! I was surprised, I would have guessed my counts were in the teens. I took Ibruprophen last week so perhaps that caused the bruising? hard to say
Once I went to two different labs for two different doctors. At one lab my counts were 15K and at my hemo's lab my counts were 30K. That is a huge difference to me! but my hemo was not surprised. He said it made sense as there is a big variation between labs. And told me that I should stick to one lab. Also as you said there can be platelet clumping esp in the violet topped tubes.

Hi poseymint! Thanks for taking the time to give your input. I will never be offended by more advice and input . It sounds like I've come to a lot of the same conclusions as you regarding my path forward. I've been mucking around with my diet, so that can surely change things a bit here and there.

poseymint wrote: I have been on Promacta several times since 2011 and also Nplate currently. I totally agree with your decision to stay on the 50mg. My counts always drop when I change doses. Give it a couple weeks and they will likely come back up. Thats how it goes with me anyway. I believe its best to find the dose you like and stay with it. I have seen how my immune system will settle down if I keep the TPO dose steady.
All said though, I do think Promacta is a bit of a weird drug- just my opinion. So I understand if you just want off of it. Once I thought I was having some kind of DVT/ vascular event in my legs from Promacta. My counts went over 100K and the skin on my legs turned white like marble! So freaky- I quit Promacta immediately. The color came back in about 24hrs. I saw the nurse, she sent me to a cardio-vascular surgeon who did ultrasound. My veins and arteries were fine. Year or so later I read that "pale skin" can be a side effect of Promacta (?!) anyway, good luck!

I've pretty much decided that I'm fine staying at 50mg for the immediate future provided I don't have any additional really funky symptoms, unusual unexplained headaches, etc. I plan on sticking here and seeing what happens over the next few weeks. I'm probably going to do weekly counts for the short term, just to get more data points. I've calmed down some from my "stop the drug!" fit and am just going to play things by ear. Psychologically I'm somehow more comfortable with 50 than 75, so that's what we'll do for now. If I got a white leg like you did, I'd surely freak out again and want to get off. So I'm just going to observe my body and proceed with caution. Thanks as always for your advice. I love "been there done that" anecdotes, even though we are all so different

I know everyone is waiting with bated breath for today's lab results, hahaha:

3/15/17 plt 37,000
dosage 50mg promacta daily

Who knows what the dip last week was about, but I'm great with today's number. I'm just going to stick at 50mg and mess with nothing for the immediate future (unless I get a crazy symptom like a white leg like you, poseymint!).

I'm going back in 2 weeks, so we'll see what happens. Last week's crash was probably unrelated to my dosage change because it was so soon after I had adjusted it. Maybe it wasn't really a crash and just clumping. Mysteries mysteries! Maybe the dosage decrease will be reflected in future results. Always something exciting to look forward to!

Still no symptoms of any kind (bleeding gums, bruising, etc.).

Great count! Good that you stayed with the 50mg. Its important to feel comfortable with a drug.
I believe Drew Provan, ITP expert in the UK once advised that counts be between 35K- 50K on the TPOs.

That's a good count momtoboys! Glad you stayed with 50 mg. I think the less of this med we can be on the better !

Just got the results online from my last labwork done last Thursday:

3/23/17 plt 61,000
dosage 50mg promacta daily

Lots of changes to report. I have now switched to a new hematologist. My previous hematologist is in physical therapy after a brain aneurysm and the office was giving me a hard time about coming in for lab work without seeing the doctor. They wanted me to follow up with a nurse practitioner and see her every time I came in for labs (i.e. collect my $70 copay more often). No thanks. It takes 20 minutes to get someone on the phone to schedule or change appointments, so I decided it was time to leave the practice since I had really stayed because I liked my doctor.

I found a new hematologist and had my first appointment with her last Thursday. I definitely get the impression that she doesn't see many refractory ITP patients. She seems to like the idea of splenectomies (70% remission she says) and doesn't want to take IVIg or Pred "off the table" for me... Ummm.. none of these are particularly good signs. I already don't have a spleen, so I guess her misguided thoughts on that topic don't necessarily affect me for now. I have no idea why we would visit the idea of IVIg as a treatment. Her idea was that some people can get a few months of remission out of an infusion, but that doesn't seem like a good plan to me. I haven't had it since prior to splenectomy as a child, and I know people use it as a rescue treatment. Regardless, I told her that I'm on 50mg Promacta for the moment and want to stick with that for the immediate future. She was fine with this and just wants to get weekly labs on me for now and see me in a month. That 61 was a lot higher than I was expecting, but maybe I still haven't seen the effects from dropping my dose quickly to 50. She didn't seem to share or relate to my thrombosis fears, but she seemed okay when I emphasized that I don't want to chase numbers and my preference is absolute minimal treatment. She did throw out Cyclosporine and Danazol (hell no) as other things she may suggest as needed.

I'll see how she works out, but the initial signs aren't great. Good thing I can do my own research! Her office is fairly convenient and the staff is great, so I'm going to try it for a while and see. It certainly would have been nice to find someone who could be more of a "partner" in planning treatment, but honestly there are a lot of mediocre and bad hematologists out there.

That's my update!

Maybe you just need to break her in a bit.
An appointment every time you get labs? Are they serious? Yikes!

Sounds like your new hema will need to be broken in. You have a life time of experience to share on the topic.

FWIW, stumbled across this recently. After trying a combination of Promacta and Danazol, they tried a combination of Promacta and Cyclophosphamide. This success suggests that Cyclophosphamide has the ability to provide a immune system boost needed for a TPO agonist like spurred remission. From figure 1, looks like it took them a while to get the dosage right for maximum beneficial response.
www.hindawi.com/journals/crihem/2015/583451/#B5