FINALLY Got 2nd Opinion / Starting TPO RAs

I finally, after waiting for months, got to go to OHSU for a second opinion on ITP treatments. As I suspected, the hematologist there disagreed with my hemo about the risks of the TPO receptor agonists and about the necessity of a splenectomy. So he put in writing a recommendation to try either NPlate or Promacta and I am now waiting for insurance to approve it. This shouldn't be a problem since I have now failed dex, Rituxan, and WhinRho. The WhinRho landed me in the ER with a fever of 102 and hematuria. No hemolysis though. I think I would rather do the NPlate shot but I'm open to suggestions. My counts have been around 20 for the past few weeks, which isn't terrible, but it's time for one last IVIg infusion before making the switch. Too bad I had to travel out of state all day long to get this recommendation but that's rural medicine for you.

I also had formal neuropsychological/cognitive testing done because I was having issues with memory and concentration that seemed odd to me, as well as sensory sensitivity (to bright lights, noise, movement). The test took all day long. The test found impairments in the areas of working memory, processing speed, and efficiency. I received the diagnoses of "unspecified neurocognitive disorder" and "other specific Trauma-related disorder due to a medical condition" because of the childhood hospitalizations I had to undergo with ITP that caused a sleep disturbance and behavioral problems at school. I screened negative for Major Depressive Disorder and pretty much all other diagnoses. It is unknown what is causing these issues, but two hematologists and the neuropsychologist all suspected it was related to IVIg, possibly some kind of neuroinflammatory response, compounded by traumatic stress. It could also be due to the IV benadryl they've been giving me.

So I'm having a real fun time! I'll post updates about how the NPlate/Promacta goes. I am really excited to get my treatment on a different track and hoping to find something that works, is less of a roller coaster, and lets me have a bit more stability.

That's good news that you found a more modern doctor who isn't just itching to take out your spleen. I'm sorry to hear about some of the other issues. What exactly happened when you were hospitalized for ITP as a child that caused all of those problems? If I'm understanding correctly just receiving IVIg caused neurocognitive issues? The treatments can definitely be worse than the original ITP. Keep us posted on how things go with your new treatments. So many people do well with NPlate and Promacta that I'm hopeful you will join that group and find some stability!

I'm glad you found a doctor that is more realistic! Thanks for the update!

CJ_Goes_Hiking wrote: ... I have now failed dex, Rituxan, and WhinRho. The WhinRho landed me in the ER with a fever of 102 and hematuria. No hemolysis though. I think I would rather do the NPlate shot but I'm open to suggestions...

Oh no! Another 'treatments are worse than the disease' story. Ack!
As you probably already know, I'm predicting a low dose of either. 12.5mg of Promacta or a 1 or 2 dose of Nplate.

... It could also be due to the IV benadryl they've been giving me.

Crossing my fingers that is the case.

momto3boys wrote: What exactly happened when you were hospitalized for ITP as a child that caused all of those problems? If I'm understanding correctly just receiving IVIg caused neurocognitive issues?

I think I need to make it clear for any parents who may be reading that it is NOT thought that just receiving IVIg caused all my issues. I was too young to remember (this started at 18 mos) but according to my parents I was difficult to stick and had multiple instances of struggling/screaming with hospital staff before they could get the IV in. It’s thought that being forcibly restrained and sedated is traumatic for little kids. My experience is atypical. I stopped sleeping entirely around age two (would just stare at ceiling blankly) and became aggressive to other kids. But, neurocognitively I developed normally and had excellent academic achievement and a good childhood overall. Early traumatic stress can sensitize your body to future stress. My recent relapse is similar to people who have PTSD from things like cancer or stroke, even though it is less life threatening. The IVIg I have had recently came with severe headaches and we know it can cause migraines / meningitis so it seems reasonable that given the timing, it has made things worse for me on the cognitive front. Does that make more sense? It feels complicated and confusing even to me.

CJ
My personal experience:- NPlate (Romiplostin) v Promacta (Eltrombopag)
Romiplostim
In the UK we are able to inject ourselves at home. Whereas, if I understand correctly, in the US you will have the hassle of going to a clinic for your injection.
Dose starts at 1mcg/kg weekly and can be increased to a max of 10mcg/kg weekly.
My counts were never stable the whole year I was taking it. Anywhere from single figures to 200ish. I am too symptomatic if counts go below 25. With a count of 6 I gave it up and swapped to Eltrombopag. I may well have lost response to it but wasn't prepared to continue with such volatile counts any longer to find out.
Eltrombopag
Usually starts on 50mg daily tablets. Dose can be adjusted between 12.5mg-75mg a day.
One has to keep an eye on calcium ingestion whilst taking this but is easily got round by avoiding high calcium foods after evening meal and taking tablet last thing at night before going to bed.
Admittedly it took a while to get my dose sorted out to maintain a stable count but even whilst doing this my counts did not swing about alarmingly like they did on Romiplostim.

The target count of either is 50k. I'm currently on 50mg a day Eltrombopag and maintaining a count in the 40s which suits me fine.