TOPIC:

Hal9000 wrote:

I suspect I lived about 8 to 10 years with a count of 12. No 'ledge' here. Still, have you got some ideas about how to treat (because of blood blisters) while dieting? Or, after you've achieved your diet goals?

If I get a significant crash as I go along (by which I guess I mean a bleed or mouth blood blisters, not just a numbers crash), I will probably take a tiny steroid dose... like 10mg one or two times. I am still relatively responsive to steroids and avoid them because of long term effects, but am not against using a tiny pulse (like I have for most of my life!) to just bump me up enough to get out of the danger zone. Upping my Promacta during a crash will not have an instantaneous effect (based on my experiences thus far) and wouldn't work as a rescue treatment. I'm still REALLY liking being on 25mg instead of 50mg from a long-term potential side effects standpoint and am going to fight tooth and nail to stay at that dose. I would reconsider if I were crashing or having symptoms constantly, but so far that isn't the case.

I'm definitely willing to take the low numbers as I gradually lose weight. I'm not willing to give up on finally finding something that is working after all of my attempts and thyroid issues along the way. Lower counts in the short term is a small price to pay. And, hopefully, as I lose weight my Promacta dosage could perhaps become more effective at a lower body weight? I made that up, of course, but it could happen

Hal9000 wrote:

Yes, 'wacky' highly-reactive platelets at work. Do you think there is a possibility that Promacta will keep blood blisters away - for the foreseeable future?

Oh, BTW. I've been meaning to ask you. That was a great insight on the possibility that Danazol might help me long term - ref my thread on Danazol. Have you had ITP friends in the past that were treated with it?

Promacta has DEFINITELY helped with symptoms at lower counts. In the past I would have had blood blisters and runaway monthly bleeds at counts even slightly lower than 30. This is the lowest that I have been able to ride without symptoms ever. Yay Promacta.

Regarding your Danazol, no, definitely no ITP friends (don't know anyone in real life with ITP!) and haven't read about anyone else trying Danazol. Sometimes the body does funny things or achieves new set points after we try something (I've seen this with no symptoms at low counts with my current treatment!), so I was just hoping that you would get some benefit, even if it wasn't a full remission as we all hope for. I'm glad to hear that you are still doing well.

Turned out I have had Grade 1 (early stage) endometrial cancer so have just had a total laparoscopic hysterectomy with a count of 130. Count dropped to 90 after op but rose above 100 again next day. Still on 6x50mg and 1x25 mg eltrombopag per week. I had doses of anti clotting med before and after the op so that may have caused the drop.. Glad I was in a good position to have the op just when I needed it.

Margaret I'm so sorry to hear that! The good news is that it was early stage and that you were able to get the surgery done so quickly. Good thing that you have been our poster child for stability with the Promacta. Keep us updated as you go along.

Love being a poster child at 71!

Margaret wrote:

Turned out I have had Grade 1 (early stage) endometrial cancer ...

LOL, of course it was stage 1 / encapsulated. Between the hyper responsive immune system of someone with ITP and some piddly cancer cells, I'll put my money on ITP any day.

Good news it was caught and dealt with quickly Margaret. Wishing you a speedy recovery

I'm sorry to hear that, Margaret. I hope all is well now!

Margaret wrote:

Love being a poster child at 71!

Never too old to be a poster child!

7/11/18 plt 17,000
25mg Promacta daily

All righty... still having approximately the same low counts. Keeping things the same, but my hematologist has recommended a clinical trial to me that I'm going to start investigating/thinking about.

Georgetown is currently running a trial of Sutimlimab (formerly BIVV009) which approaches the antibody issue from the classical complement pathway. The drug has been also used for Cold Agglutinin disease and is well tolerated so far. Out of a sample of 7 refractory ITP patients, 5 ended up with remissions, but I haven't seen any specifics. I just spoke with the study coordinator today and am going to look into this more.

(Anyone who as heard of this or done anything with this, please chime in. Probably too new for that though )

I'll probably make a new thread to talk about the trial stuff as I look into it.

You don't want to try to raise the Promacta dose first?

Yeah Sandi, I don't think I'm too close to jumping on something new quite yet. It sounds shiny and exciting and so far nothing like the crazy stuff/toxicities with the Rituxan, but everything looks tempting when you get frustrated

I'm perfectly fine where I am with my 25mg right now. I should just sit tight even though my numbers are low because my symptoms are mostly fine. But investigating the idea that I might be able to try something with a completely different mechanism of approaching ITP after a freaking lifetime of ITP is quite tempting! The pie in the sky idea that maybe I could get some kind of a remission and not have to constantly be on medication is, of course, appealing.

Every time I get bone twinges I think of how much I'm pumping up my bone marrow to do more than it ordinarily would and wonder about the long term. It's all good, and I shouldn't complain, but I'm going to learn about the complement system and at least ask some questions since this thing has fallen in my lap.

Okay....you sounded like you were jumping on that train tomorrow. I thought maybe you were abducted by an alien and replaced by a clone! Just didn't sound like you!

8/2/18 plt 20,000
25mg Promacta daily

This count was done at my gynecologist's office. I ended up with a double bleed this month with only 4 days in between (just what I needed of course!). It turns out that I have adenomyosis which is contributing to my issues with managing my monthly bleeds. I ended up taking 10mg of pred on two different occasions to make very sure that the second bleed didn't run out of control. A hospital stay and transfusion are NOT on my to do list! I did not restart the tranexamic acid because I was scared of taking so much of it in a short period of time and potential clotting dysfunction. I also did the mini pred dose because I really needed to get into the dentist and hadn't been able to go while my counts have been in the teens. This count was done about two weeks after the pred spike, so most of the pred effects would have worn off, but I'm guessing that the 20 is still very slightly elevated from what I've been running because of the steroids.

I will be seeing my hematologist later this week and we will be able to discuss all things clinical trial and new issues. I will update after the appointment if we decide to do anything exciting.

Blah, complications. Did the doc mention Danazol? If so, that would be interesting - possibly two birds with one stone.

momto3boys, Sutimlimab does sound interesting, though I have no useful information. Since you mention the trial, I was wondering if you are near Georgetown? We are in the DC area.

Hal9000 wrote:

Blah, complications. Did the doc mention Danazol? If so, that would be interesting - possibly two birds with one stone.

Nope, neither one of the doctors. My gynecologist talked about ablation, the mirena, and progestin based pills.

My hematologist and I just tweaked the promacta dosage for now. When I asked him about Danazol (out of curiosity because my previous hematologist had mentioned it in her laundry list of treatment options several years ago), he said that they really don't see patients respond well to it, meaning there aren't crowds of people getting great counts with Danazol. Of course, this could be because it isn't used much as a treatment option since they tend to go with the newer drugs for ITP. It would take a very high potential for benefit for me to risk taking it. As a woman, permanent voice changes and hair growth would be just too much for me to handle! The psychological effects from those side effects would be bad news

maria3132 wrote:

momto3boys, Sutimlimab does sound interesting, though I have no useful information. Since you mention the trial, I was wondering if you are near Georgetown? We are in the DC area.

Howdy neighbor! Yep, I'm in nova. My hematologist is over at Lombardi in Georgetown.

So I saw my hematologist for an appointment on Friday. At the moment we are going to try alternating days of 25mg and 50mg with the Promacta. I mentioned some of the bone pain that I was experiencing on the higher dosage, and he mentioned that some patients taking other medications that cause similar bone pain find improvement when taking OTC Claritin (Loratidine). I'll give that a try to see if it helps if/when I have that symptom. I talked with him about not having consistent counts in the past when I've tried patterns of alternating days, but he wanted to give it a try before going completely into 50mg daily, and I'm game to give it another shot. You never know when some other factor or variable is playing a role, so it's worth trying again. Let me just say again that I love my hematologist because not only does he ask my opinion about what to do as a next step, but also that he doesn't bat an eye that I have been having counts around 10 and 11. It's so relaxing after having panicky hematologists who always wanted to run full force into treatment decisions because (OMG! Your count is below 30! Emergency!)

An interesting note about something that I learned about the bone pain, it's not that the marrow is somehow exerting force within the bone, or even the act of the increased production in the bone, but rather the inflammatory effects of the cytokines being produced. The joys of more inflammation, yay (ugh!)!

Regarding the adenomyosis, he is fine with me not treating until I go through a few more cycles to see if things might regulate on their own. I'm currently in the midst of bleed 3 since early July, so we'll see what my HGB numbers are and if I can sustain this experimentation before I just have to suck it up and mess with the hormonal side of things (which I really, really don't want to!). I asked my hematologist about splenectomized patients and clotting risks with my various options. He said that if I go the route of an oral progestin pill, that I should opt for a first generation progestin instead of a second or third generation. The clotting risks are higher with the latter two.

I'm not considering the ablation at the moment because I did some reading about the scar tissue created during the procedure having the potential to mask cancer detection as well as presenting the potential for creating a long term pain situation if it causes bleeding to become trapped beyond the newly created myometrial scar tissue. Ugh. Here's hoping that I don't have to choose any of these options that I just hate. I am trying my best to avoid introducing more variables via medications or hormones or procedures into my already complicated biome!

The final item that we discussed was immunizations that I need. I've been keeping up to date with my pneumococcal vaccines after splenectomy for the most recent years (I surely had lapses during the college years!), but I hadn't remembered/been told that I need to have the meningococcal vaccine every five years post splenectomy as well. He wants me to see my GP and get that as well as the flu vaccine for this year. He was all ready to have me get the new shingrix vaccine for shingles, but the recommendation even for immune compromised patients is after age 50. Which is good, because I'm in no hurry to be a guinea pig for that one Who knows what improvements we might have in the next several years?

We spent so long discussing other items that I opted to not discuss the details of the clinical trial with him during this visit. I have the option to go in and meet with the doctor running the trial and ask any questions that I might have, so I'll do that at some point. I'm not in any hurry at the moment (it is a phase 1 trial, which my ophthalmologist brother-in-law equates to "they are just checking the safety and dosages, but those tend to have the least benefit for patients.") and if I really get frustrated and want to do something a little wild down the road, I'll discuss it with my hematologist at that time.

Regarding my labs, I always get those done at labcorp (because Lombardi billing is absolutely insane because it is considered a hospital visit, which means that a CBC costs me 200 freaking dollars because there is a facility/lab fee tacked on to the actual lab test fee.. don't get me started on my rage at their billing practices and how much they want to charge me for breathing the air in their building... a doctor's visit costs my $80 copay plus around 300!)... I got the labs done Friday afternoon, so I won't be able to call in for the results until Monday. I'm mostly interested in the hgb and hct numbers so I will know how much wiggle room I have with all of this bleeding! I've (of course) been so exhausted and short of breath. One step forward and ten steps back, I swear!

That's the end of my current novel! I'll update with my lab numbers when I get them. I sure hope some of this data helps someone down the road. I always found the old threads quite helpful, so I'm doing my part

momto3boys wrote:

maria3132 wrote:

momto3boys, Sutimlimab does sound interesting, though I have no useful information. Since you mention the trial, I was wondering if you are near Georgetown? We are in the DC area.

Howdy neighbor! Yep, I'm in nova. My hematologist is over at Lombardi in Georgetown.

We're in the District, but I used to live in Arlington and loved the more laid back vibe. My son is being treated at Children's, which is affiliated with Johns Hopkins somehow, though I am tempted to take him to Baltimore/JH proper (or Sloan in NYC) for a second opinion. Good to know about Lombardi - I see now that they are doing a lot of exciting research.

It is super helpful! I am learning a ton. Sorry to hear about the bone pain. I hope the Claritin helps (what's the logic there -- is Claritin supposed to be anti-inflammatory?)
I really hope you don't have to go the hormonal route too. And I hear you about the mad lab pricing practices!

I just want to thank you for taking the time to post your journey and for continuing to update. I'm at a point where I need to do something- and so spent the day yesterday researching Promacta and reading the entire thread, and everyone's responses. It helped me so much! I've never been involved in any online discussion group and don't even know what I'm doing so probably need to watch a tutorial or something...but will hopefully post an introduction and log my Promacta path somewhat as well. But thank you and the others who take the time to share information and experience.

I found my old, dead thread of my Promacta Journey! Here I am to give it some closure, though it definitely has died with more of a whimper than a bang.

During the pandemic I bailed on the Promacta entirely because of the bone pain. It was getting worse and I didn't want to go anywhere near a hospital with the COVID-19 numbers that we were having at the time. I managed to just decrease my dose and hop right off of it without any major symptoms. In all honesty, I remained mostly asymptomatic from last May/June 2020 (don't remember exactly when I cut the cord) until now. I just used up my stash of tranexamic acid whenever I had a bad monthly bleed and managed things from home. Now, in February of 2021 I've started having some symptoms and figured out that my count was either in the single digits or the teens. Sure enough, 17k on Friday. So I'm doing 40mg of dexamethasone for 4 days and then starting on Doptelet. I like the idea of no middle of the night alarms to take a pill! That will be nice. I guess I'll start a "My Doptelet Journey" thread for posterity when that starts up. Looks like my doctor is going to want weekly bloodwork until I get out of the danger zone, so more updates from me now that I have some news.

Anyway, just wanted to close this one out and I'm happy to see that some people benefitted from my documentation while I was away! Good luck to all on your ITP journey.

Mom
Good luck with Avatrombopag.
I wish they would hurry up and licence it over here. It’s fascinating how different people have different side effects. I have never experienced bone pain with Eltrombopag whereas whilst on Romiplostim it was almost constant.

mrsb04 wrote:

Mom
Good luck with Avatrombopag.
I wish they would hurry up and licence it over here. It’s fascinating how different people have different side effects. I have never experienced bone pain with Eltrombopag whereas whilst on Romiplostim it was almost constant.

Well, I have to say that I did well with Eltrombopag for years, so I would call that a win for me. Too bad my bones wanted to fuss towards the end there. I'm so glad that there are new treatment options that have come out since I last switched and am excited to see how things go with the Avatrombopag (it's still off label prescribing, so we'll see how my insurance likes that and what the actual costs will be... not 100% sure I will get it until it's in my hand!). I will keep you all posted and fingers crossed that it heads your way as an option soon!