TOPIC: This Syndrome and Promacta usage

My previous hema said I shouldn't take Promacta because of the clotting warning. My new one says it is fine as long as we don't allow my platelets to get too high.
So, I'm to cap out at 100 or perhaps ever so slightly higher. Then, immediately reduce my Promacta dosage. I took Promacta and in one week my platelets tripled and we are already planning the reduction of dosage to 25 mgs.
Anyone have any experience with this?

I'd be a bit skeptical that counts can be kept under 100 while taking both Promacta and 10mg of Pred. Even 25mg of Promacta may prove to be too much. On the other hand, 25 to 50mg of Promacta would be a typical dose if one wasn't taking any Pred.

I don't have experience myself with Promacta and the Lupus Coagulant. I've never used Promacta. I have the Anticardiolipin Antibody though so I am familiar with APS. I know a woman with the LA who was not treating ITP at all because nothing worked. Her counts were below 20k and she still had several serious clots. Clots can occur even with low counts and the risk goes up as the counts go up. Usually the target range for counts while on Promacta is 50k. If it were me, I'd rather stick to that as a target.

Have you ever had a clot? If so, I'd be more apt to worry about it. Some doctors take that combination (TPO's and APS) very seriously, and some are pretty casual about it. I'd rather err on the side of caution. What is your count?

I’m basically off Pred now. I’m taking 25 mg of Promacta. Last count was 125 but I was taking a little prednisone. He feels the drop in pred (was on 2.5 and did a rapid taper) will push my platelets to below 100. Not sure he is taking the APS seriously. I’ve never had a clot before and this might be why. He doesn’t even want anoutger platelet count until 3/1. I’m going to push for another count this week.

I would want another count this week too. You really have to be your own advocate. This is a good video for you to watch.

www.onclive.com/insights-archive/immune-thrombocytopenia/risk-of-thrombosis-in-patients-with-itp

Recently he took me from 5 to 2.5 mg of prednisone from one day to the next. Then from 2.5 to 1 mg in one week.
Now, I have some hair loss, weird fatigue and body aches.
I have sjogrens syndrome (active)
and lupus basically in remission with the exception of APS.
I didn’t notice any side effects from the Promacta. Do you think this crummy feeling I have is from going down very fast on Prednisone or is it more likely from Promacta? When I was on 5 mg of pred and Promacta I felt much better. Each fast jump downward in pred seem to bring discomfort, but this last one down to 1 mg is the worst. Yes, I need to speak up and I will likely request a blood test this week. Thank you.

The hair loss, fatigue and body aches are steroid withdrawal symptoms. I have Lupus and Sjogren's too and take 10 mg's a day. I feel much better on Prednisone than I do off. I'd take 50 mg's a day if I could. It's the only drug that allows me to function at a somewhat normal level. The higher the dose, the better I feel. 10 mg's doesn't do much, but I'd be the Tin Man if I didn't take it. You need to take it slow, especially at the end of the taper.

Far too fast a taper of Pred in my opinion.

I’m on 25 mg if Promacta and 1 mg of Prednisone. I refused to stop the prednisone because that fast taper almost did me in.
My recent count was 69. It was a fairly big drop. Actually, I’m fine with this count. He seems Concerned. I guess he wants to see if it’s spiralling down. I’m nit sure I’m willing to take more than 25 mgs of Promacta.
Another count in about ten days.
A number of 50-70 seems great to me. So if it stays steady at 69 I think I would be okay with that. Don’t want any high numbers.
I am having off and on leg pains. Hair loss has greatly slowed down and knock on wood might have stopped. A tiny improvement recently in leg pain.
A bit frustrated.

Yes, classic steroid withdrawal symptoms- aches, joint and muscle pain, hair loss, deep fatigue. I couldn't go through the day without a nap, felt fluish when I tapered too fast- plus was irritable and depressed. As the others have said and you now know, you tapered too fast. I usually go down by .5 when I have tapered. But I was on it over a year- length of time on the drug makes a big difference to how fast you can taper. You must give the adrenals time to recover and start working again. Not all doctors understand tapering prednisone- that we've seen again and again on this forum!

Unless you have bleeding issues, there is no reason for counts to be over 50 for most ITPers. I am on TPOs and try to keep my counts between 30- 70. My average count for the past year has been 43. Dr Drew Provan, an international ITP expert from the UK recommends that patients on TPOs keep counts between 35-50, He is very aware of clotting and stroke risks. A lot depends on if you bleed at low numbers. I don't have bleeding problems. but I'm over 60yrs with increased risk of heart/stroke due to Lupus, Sjogrens, my age, blood pressure, overweight etc., so looking at the whole picture I am more comfortable with lower numbers of 30-60. Absolutely I try to keep counts under 100. Good luck, take care and hope you feel better soon!

Thank you so much Poseymint. I am turning 60 next month. I am currently about twenty pounds overweight , mostly from the prednisone. We moved six months ago ..so add big stress to that. I have dropped five pounds in the last month since being in only 1 mg of prednisone.
What you say makes sense. I am very worried about the stroke risk. I don’t know if the hematologist is just trying to keep me calm or if he is not taking it as seriously as I think he should.
We seem to have the same health concerns. I too have high blood pressure.
I joined Weight Watchers and I’m determined to get the extra weight off as my blood pressure tends to go down with weight loss.
I agree that a count around 50 or so is absolutely fine. I’m going to tell the doc this. Since I’m not all that open to any higher dosage of this drug, I think that would be the case anyway .
Thank you for your response. I’m finding this medication a little nerve wracking....calming down a little. A difficult month or so.
PS what does TPO stand for?

TPO = Thrombopoietin

I'm with you as far as keeping counts between 30k and maybe 70k. I'd much rather do whatever I could to avoid a clot. Once it happens, you end up on blood thinners then you have to juggle both.

I wouldn't increase the Promacta dose. A count of 69 is absolutely perfect. Protocol is take the lowest dose possible to keep count above 50. If it were me I would insist on staying on 25mg for a while and see what happens. Like Poseymint I am more than happy with a count above 30. If I could maintain a count of 30 without any medications I would be delighted.
Counts do sometimes dip. Mine plummets when I have a cold for example but rights itself once the cold clears. I do not alter my Promacta dose to compensate for the dip.
Good news about your weight loss, hopefully this will continue.
Having experienced steroid withdrawal I would stick on 1mg of Pred for a while to give your adrenals time to recover. I doubt a dose that small is having any effect on your counts.

Thank you everyone.
Ive learned so much here.
Yes, I definitely am fine with these lower counts we have been talking about.

I see the doctor Monday and I plan on discussing this in detail.

Two questions.
1. I have deep leg pains. It is slightly better than perhaps a week ago. This started with the pred taper. First, I thought it was just the taper as so much happened with that. Now I’m wondering if the Promacta is causing the pain andvif the pred was previously covering it up. It can get very painful and I’ve never had anything like this before. Is this likely a Promacta side effect?
2. Periodically, the dr and his office seem confused re my exact count. Last visit at first they told me my count was 39. They did it again and said it was 69. Does this happen to others? They have their own on premises lab. Could they (platelets) be clumping? Any thoughts at all?

Thank you.

1. The only way to know about the leg pain is to give it time. Both of those reasons are plausible (Prednisone taper or Promacta).

2. Platelets won't normally clump if the sample is run quickly. It clumps when it sits in the vial for any period of time. That's an odd jump for a same sample test. I'd chalk that one up to a fluke and hope it doesn't happen again. You have to rely on the accuracy of their tests so hopefully they fix whatever problem they have. The margin of error shouldn't be that much. I'd ask them and see how they stammer through an explanation.

Had hema appt. yesterday.
Ok. But don’t know if these oddities should be concerning.

1. Count was 40. Seems to have gone down almost 30 k in about twelve days. I personally think it was the fast taper of pred that caused this. (Currently on 1 mg of pred and 25 of Promacta)
2. I told the doctor about the leg pains I’ve been having. He thinks it might be fibromyalgia. Other docs here and there have mentioned this possibility over the years. I said it started with the last drop in pred and I felt the taper was too fast. He said when the pred dosage is small you can go a little faster. That would be a big NO in my book. At least not for autoimmune patients.
3. There was a delay getting back to me with my count after the blood draw in the office. This usually means something. He said that they think my platelets are clumping even though they did them stat. Last time when I had the 69 they sent the sample to Quest and it came back 90. Soooo... I’m to go to Quest every 11 -12 days for a test and see him every 14 days for a test and they will get a handle on it. No special chemical in the tube to prevent clotting. Just two counts , two places, very near one another.
Thoughts?

I noticed when my platelets were on the low side I had leg/hip pains. In fact when I was diagnosed I went to the doctor for hip pain that would not go away and mentioned I had some unusual bruising. They did a blood test and the next day I was in the hospital. Mine was noticeably worse the lower the count.

No way am I interested in increasing Promacta dosage. This stuff creeps me out. I appreciate getting off prednisone, but this stuff is a bit scary.

Update: My hema no longer trusts his machines to do the platelet count. He says they seem to be clumping. That is all he says on the subject. Last count at Quest was 100.
He does not want me to go off the 1 mg of prednisone. Does not offer an explanation. At this point I think it would be ok to do .5. But, the 1 mg isn’t causing problems, so I suppose it’s fine to leave it alone.
I’m still on 25 mgs of Promacta.
I asked him if 100 platelets was too high in my case. He said “This is a below normal count and his fine.”
He seemed much more rushed than typical.
He gave me a script for a CBC at Quest and told me to see him in a month.
My leg pains had improved.
Oddly, just today they seem to be worse than yesterday. Very close to how they were about a month ago when they hurt a lot.
I have not noticed any pattern.

Also, my GERD is back (from years ago) and I’m back on Zantac 75 mg twice a day. This is a low-ish dose.
I suspect the Promacta is the cause of the gerd. I time the Med carefully.
On my own I have decided to take my next count in two weeks and see the doctor in three weeks instead of four.
I personally am unsure of what my highest platelet count should be with APS and taking Promacta. I think at this time I can’t wait too long between tests in case it creeps up. The dr is not addressing all these concerns. I suspect 100 is maxed out. I’m concerned and frustrated. And until this is resolved and a bit more steady, I think I should get counts and see him no more than theee weeks apart.
This is not meant to be disparaging to the dr. I think he doesn’t have experience with APS.
Any thoughts?

I was shaking my head as I read this. His comment that "this is a below normal count and is fine" blew me away. He's right, it IS a below normal count but is on the high side for a TPO, and he's not taking the LA seriously enough. You're situation is special. It is much better to err on the side of caution and try to avoid a clot rather than have to treat one if it occurs. I've read that once you clot with LA, you're on blood thinners for life.

You're right to stay on top of it.

I continue to have concerns. I took a count and it inched up to 114. The doc was on vacation. On my own I lowered the Promacta dosage to 12.5 one day and 25 the next. When I saw the doc next, he said to lower it to 12.5 daily (oddly implying its because I’m overly worried)
I take a blood test tomorrow.
He admonished me again for being concerned about having a count over 100. He said he’s had patients with counts as high as 5 million who did not have a blood clot or similar event. I explained again if it weren’t for the APS I would not be this concerned at all. It is very clear he either has no knowledge of APS or no concern about it.
Then I said a count of 50 would be ok. And I certainly never want to go over 100.
Then he said something soooo incongruent with the other things he was saying....something like “well if you want a count of 30, that’s ok.”
STRANGE.....Hard to say for sure but perhaps someone else like the other doc who seems VERY concerned for me suggested a count of 30.
Would a count as low as 30 be ok in terms of keeping me safe for every day things like dental procedures?
I’m thinking more like 40-50 from personal experience.
He is having me take all my blood work at Quest. The other doc had Quest put a chemical in my tube which prevents clumping. When this is done, my counts come back MUCH higher. This doc (my usual one) says the chemical in the tube to prevent clumping is not needed. He is not specific.
>I’ve been having what feels like Raynauds in my ARMS. I rarely have raynauds, but it’s only in my hands when I do. Now, it’s all the way up to my elbows. It’s worse if I drink coffee.
I’m frightened.
I am so confused , I’ve skipped some dosages of my meds.
I now have some bruises.
No meds seem all that good for me for a variety of reasons. For example, with Rituxan...you seem to get on a regimen for four treatments and your count could inadvertently go very high and you wouldn’t be able to stop it.
I’m getting names of doctors for a second opinion. As a side note, I’m relatively new living in this city....so I don’t know the doctor’s here well...something I knew very well in my hometown.
Thanks for letting me get this off my chest.

Nomad - I think the best thing you could possibly do is get a new Hemo. You need one who knows how to manage both disorders. I would not be comfortable with that doctor either. I had a Hemo who fully admitted that he wasn't familiar with APS so he sent me to another Hemo that did know.

It seems as though you might have something going on with blood vessels if you have new symptoms. Do your arms turn colors or get white? APS can cause a lattice like appearance on arms and legs. I've had that many times.

There is pain in my hands and arms. The arms are more noticeable to me as I’ve never had this before. I’m reacting to cold and coffee. Significantly more than ever before.

I have had a very faint blue color in my hands. No Lattace...although I’vehad that before.

I just called another doc’s Office. Omg. They want my records and a letter from my doc stating I haveITP. Ive had this for forty years...I felt like screaming. I don’t necessarily want my current hema to know and there will be delays with my gp. My insurance does not require any of this.

I fear the arms and hand discomfort that is either raynauds or something much like it is related in some way to Promacta. I’ve never had this much discomfort before. It usually has to be extremely cold. I live in a warm state. I’ve been wearing mittens , long sleeves, cut way back on coffe and have raised the ac. Sometimes I use a heating pad.

I did not take the Promacta today. I’m nervous about it.

I would want to scream if I were you too. I feel frustrated for you. Like you'd just make up the fact that you have ITP?

The doctors in this new city I’m in often seem to have a different way of doing things. Not necessarily an efficient way.

I’m putting the word out looking for a doctor familiar with both issues.

Meanwhile, even though my current doc seems to think I’m silly or something (?) for having concerns about my counts getting too high due to APS, he did agree that he would work with me on this. It would be better if he understood the need. But, at least I have his cooperation.

Thank you for your support. This has been odd and at times stressful.

You are not being silly or overreacting. Don't let him make you feel that way. I know a woman that has APS who had serious blood clots with counts under 10k. She wasn't even using a TPO at the time. You cannot be too cautious.

"Use the lowest dose of Promacta to achieve and maintain a platelet count greater than or equal to 50 x 109/L as necessary to reduce the risk for bleeding. Dose adjustments are based upon the platelet count response. Do not use Promacta to normalize platelet counts [see Warnings and Precautions (5.4)]. In clinical trials, platelet counts generally increased within 1 to 2 weeks after starting Promacta and decreased within 1 to 2 weeks after discontinuing Promacta [see Clinical Studies (14.1)].
Thrombotic/thromboembolic complications may result from increases in platelet counts with Promacta. Reported thrombotic/thromboembolic complications included both venous and arterial events and were observed at low and at normal platelet counts.

Consider the potential for an increased risk of thromboembolism when administering Promacta to patients with known risk factors for thromboembolism (e.g., Factor V Leiden, ATIII deficiency, antiphospholipid syndrome, chronic liver disease). To minimize the risk for thrombotic/thromboembolic complications, do not use Promacta in an attempt to normalize platelet counts. Follow the dose adjustment guidelines to achieve and maintain target platelet counts [see Dosage and Administration (2.1, 2.2, 2.3)]."

www.drugs.com/pro/promacta.html

Thank you VERY much Sandi.

It does say “greater than or equal to” I was surprised to see “greater than.” I suppose some people might bleed at 50. I don’t believe I would.

In the next section it CLEARLY warns of the greater risks associated with APS and other similar conditions. Serious risks.

I do not bleed at 50. There is no reason for me to be above 50 when on this Med after reading this warning.

I am glad this doc finally gave into me , but very concerned that it took so much of my energy to convince him of something that makes extremely good medical sense. Some gaslighting here too. I think that would be the right term.

Sandi...I can’t thank you enough.

You're welcome! When I first found out that I had APS antibodies, it scared me more than ITP ever did. I totally understand your concerns. I had two doctors recommend that I take an aspirin a day as long as my counts were over 50k. There is no data to suggest that it helps any, but it's a protective measure. These days, my counts are way to high for my liking!

The article did say, twice, not to 'normalize platelet counts', so you are right to want them in the 50k to 100k range (or lower).

All these months later and for the most part I’m still having weird issues with the doctor.


I was on 25 mg of Promacta (no prednisone) and out of the blue my count dropped to 18. I went to 37.5 of Promacta. It slowly went up to 92. I asked him if I could take 25 one day alternating with 37.5 the next for awhile. I’m hoping for a count around 60-75. He said that my desire to have a count around this number is not reasonable. Also he doesn’t like alternating dosages.
Then he said take 25 daily. Then he switched to stay with 37.5 daily but when my count reaches 100 switch down to 25.
What does this mean? He hinted that he doesn’t like my “excessive”worry about blood clots. He feels it s unreasonable.
I just don’t know what to think anymore.

Also...he is having me take all my blood work at Quest and not in his office anymore with no explanation. Ive had ITP for forty years or so and I’ve always had my blood work at the doctors office.

You know your body better than him. I agree with you wholeheartedly and understand your worry re clots. He is the unreasonable one here. I would dose yourself how you want and see what happens to your count.

I somehow missed this. I second MrsB. There is nothing unreasonable about your request. You are still shooting for a safe target range over 50k, so why should he care. You don't need high counts for daily life. You need to do what makes you comfortable...he's rude!