Promacta - ?'s for those who have had it.

Good day everyone,

I have failed at rituxan. The best I was at was 82K (after four weeks after the last rituxan) for a week or two then back to the 50's. Which should be good enough, right? The word should is the issue. I still bleed like there is no tomorrow and I have to trek back to the specialist in Tucson, AZ to see if Promacta is the next step. So far, all I was told is that Promacta is very expensive and since I am at 50K, I should not be taking it, but why I am I still bleeding? Yes, I bleed from everywhere you can think of, nose, mouth, urine, stool, etc. Not fun laundry time.
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I am now at the point with my local hemo that I am frustrated at the he said she said. If I get clots and blood in my urine, it is a urological issue. But no, the urologist says my counts are too low, so it is ITP. But the hematologist says it’s urological. And so on and so on and so on. The local hemo wants me to start Promacta, but I cannot get a hold of my insurance to see how much it will cost, and I am not getting the good feelings from reading about Promacta.

I say the not good feeling, as my hemo told me that my “spike” to 82K and then back down, is why I am now bleeding more than usual. Really? A spike of 30K is that traumatic that I now bleed more? I am confused and my laundry is looking more like an episode from CSI every day. It has gotten to the point that if I can use the bathroom and not stop because of a clot, I should be OK. Of course my hemocrit is still OK, so they are not that worried. Can someone please tell that to my laundry?
So has anyone here had any experience with Promacta being given to you at counts above 50K and when you were tapered or discontinued from it, did you experience the crash or excessive bleeding? I am thinking that my platelets are useless at any number and they just sometimes work or just don’t. Color me bleach stained and faded blood blotches.

AzThumper

Just a weird question, AZ, but have you ever had any platelet function tests? Your doctors are frustrating me too.

First off, thank you for asking that question, it is a very long answer however. From my recollection and paperwork we have the following (first please have lots of coffee and try to get through my stuff, it’s long and messy, but hey it matches my pillow cases and clothing in the AM):

When first diagnosed as a child (we are talking about 36 years ago!) I went through the normal scare the parent time, so I got a bone marrow biopsy and of course the coagulation tests and more blood work than I think children should get. The result – I had no platelets for quite some time, my marrow is producing what appear to be good cells or at least the marrow is not “bad”, I have a really long bleeding time, and functional tests were deemed inconclusive as the chart of criteria for me was all over the place. So they stuck with ITP and that was why I bleed so much, but why my platelets functionality was all over the place, well that is why there is an I or idiopathic in ITP.

So I continue being a lab rat for about 5 years and get the same results. Coagulation tests are prolonged and functional tests for platelets are just as all over the place as they can be, but this is still just ITP so can we rip out my spleen already? Mom and I say no, I keep the spleen and my functional tests are something for the doctor to scratch his head over.

Now we flash forward to my twenties, at this point I have been off of prednisone for over 12 years and do the normal bleed like no tomorrow when sick, but otherwise manageable bleeding. Given IVIG and steroids to no effect and if all looks bad, pump me with packed platelets. This of course “dopes” my count just long enough to get discharged, but nothing is really done. My doctor says that the ITP is just that and going to a hematologist is really just wasting my time and money as there was nothing new discovered and my blood work is still as confusing as before, so just live with it. Right.

Now as I reach my thirties, all heck and hand basket goes wrong. I have systemic inflammation, my gallbladder gives up the ghost, I get nerve damage in all my limbs, and my eyesight goes out the door. Of course at the same time my platelets discover that the new high is going to be 50K. More blood work, more marrow withdrawn, but no bleeding time, as I have ITP why do that? Again no results from prednisone, IVIG, given WinRho (go into anaphylaxis and get the ZERO platelets!) and kept in hospital in and out for about 3 to 4 years.

Again, not a damn reason given for my platelet functionality being all over the place other than that is what it means to have ITP. Yes, that is the term my latest hematologist has given me; I am all over the place. I guess there is like a chart of some type that shows if items A to C are checked then you have this. For me I am all over from A to Z, so I really don’t match any real disease criteria just I have low platelets for some reason and I bleed with whatever count you want to say is safe or even good or bad. I do not bleed with ZERO platelets, but get me to 50K and the damn has burst or maybe not. Raise them; we bleed like I need to rid myself of blood. So at this point with insurance all in its knots and wanting me to go through this step by step mess, I am left with Promacta.

My latest hematologist needs me to see the “father” or big cheese of ITP, but that guy just does research and I cannot see him. Of course that would mean me flying to him, so even if he said yes, that would be a trek and half. So I have to find someone in my insurance plan who can figure out my condition.

So this is why I am going back to Tucson again. I was told the first time that my all over the place functionality means well nothing really. I am just that simple ITP person who just is really hard to treat. But, he did say if I fail on rituximab, then I am refractory. Now of course the local hemo now wants the Promacta as my bleeding everywhere is getting really messy, so I need to go to the Tucson doctor and ask again, what in the heck is wrong with me.

So the general consensus is that either my platelets are bad or really not that functional – about two hematologists ago and my past internal medicine doctor and my current internal medicine doctor, platelets are just messy and not going into the ITP box because ITP has no real box – the specialist in Tucson, the platelet functionality means nothing at all – Mayo Clinic, older testing and functionality tests are the sign of ITP – my first hematologist when I was a child.

So to every other doctor, 50K is too low, ITP people will bleed and that is why I have eye issues, pain in various parts of my body from blood masses, why I have blood in my stool and urine, and so on. ITP should not make me bleed at 50K, current hematologist and I really have something like ITP but not really as I seem to make all definitive testing and treatment rather pointless, but nice and messy for me to deal with.

SO with all of that bible of stuff, I have had enough functionality tests done that either mean something or nothing, I just cannot find a doctor with the skills high enough to say I have something else other than just ITP or I just really have one messed up not budging and not very treatable case of ITP.

PS – Still have my spleen, which is one thing everyone agrees on. At my age of 41 with all my other conditions, ripping out the spleen is not going to do anything good. Well at least so far, maybe that will change, like my counts or my bleeding symptoms or just about anything else at this current time.

AzThumper

I was just wondering how in-depth the doctors have gotten with you, because I know there are some newer function tests that are performed in research, but are not usually done on patients as a rule. Maybe that is what your doctor wanted the research doctor to do.

This is really off the wall, but do you know if you've been tested for APS?

www.apsfa.org/

I am going to see the specialist in Tucson on Monday, so I will ask him about APS testing. I know I have been tested for Lupus and I was told that I am negative for that. I will also ask the specialist about the coagulation tests and see what his take is on them now that I have failed rituxan and they want me on Promacta. At this point, I have more questions, but I am going to see if someone can give me some sort of answer. This laundry really sucks.

AzThumper

AZ - there really isn't such a thing as being 'negative' for Lupus. A Lupus diagnosis is not given because of a result of one or more tests, it is given by meeting a list of criteria. The common tests could be negative one day and be elevated three months later which could lead to a diagnosis. Somtimes, symptoms appear before labs show anything, so the diagnosis can be hard to get even with corresponding physical problems.

Good luck with the specialist and please let me know!