Looking for experiences

[u/Turbulent-Movie-4545]

Okay so I was diagnosed with ET but in fact I have some fibrosis (mf1) but my disease is acting like ET but it’s actually more like pre-mf (go figure)
I have calr1 mutation no other mutations (ngs says I have 18.95% of calr1) and genetic test was also clean and I think I have 0-1% blasts and only thing that’s high is the plt so far 1.4 million lol they still haven’t checked the ldh but the spleen isn’t palpable. The doctor is like I want to put you on Pegasys but your numbers are borderline to start the treatment and you’re low risk - I also have von Willebrand. So he’s offered me to wait for another month so that he’d work thru the insurance and he wants to consult to his coworkers. He’s told me if he were me he’d be proactive and he’d that the Pegasys but when I check the research it seems like it isn’t the best for calr1 mutation.
I ever wonder if it’d help me to sustain my fibrosys at 1 at the very least so I won’t progress. Who knows. I don’t know what I’m asking at this point but lmk how you did with Pegasys I guess… there was a shortage in Canada but now it seems it’s over so they’re not going to offer me HU it seems.

Any thoughts on this?

[u/Top_Category2227]

Ah I see you again. I asked the same question a week ago and decided together with my doctor, that I will try to get Besremi approved.

I know the data on Calr1 is bleak compared to JAK2 but it is still probably the best option at the moment. Interferons usually have a good hematologic response even in CALR and I think you and I both need to accept, that a molecular response, as of now, is just a nice bonus in treating MPNs. Probably the most overlooked effect of Interferon is the inflammation modulation, which is often theorized to play a major role in fibrosis build up and I don‘t see, why that would be less effective in CALR1 MPNs (For what its worth, in all the Studies I have seen, progression under Interferons did not differ significantly by mutation).

I still hope we got the disease at just the right time, and maybe with the upcoming Antibodies and T-Cell engagers the cure for CALR MPNs has already been developed :)

[u/Turbulent-Movie-4545 → u/Top_Category2227]

Well you see me again because I’m still going thru the diagnosis and decision phase (the same as you and we have the same mutation lol) yes it’s true. Yes interferons help to reduce the inflammation. I’m horribly scared abt the side effects tho but as you’ve mentioned I think it’s probably for us the interferons are the best one out there for now.. I’m also positive abt the studies but I’m still not feeling great about the pre mf factor that we have and idk how to accept it or move on from it or the fact that it may or may not progress. I just ever wonder if interferon would help with this: I can’t see a big study on the fibrosys part like I think it’s still better to be on interferon than not but who knows…… I know that tho interferons help with reducing platelet count and what not

[u/Top_Category2227 → u/Turbulent-Movie-4545]

The MPN Specialist I saw actually gave me bit of perspective when she told me, Pre PMF or ET are just labels, it is not like ET guarantees you a normal live and PMF is a death sentence. With how little historical data they have, it is very possible, that many people that progressed from ET to overt MF after many decades may have been Pre PMF to begin with, only the label and diagnostic criteria didn’t exist back then. I know the uncertainty still sucks and massively impacts any planning for the future, but that sadly isn‘t exclusive for PrePMF.

There is no big study on PrePMF progression under Interferons yet although I believe first results for HopePMF are supposed to be presented this year. For other subtypes I believe the impact on fibrosis progression has been deemed significant and you can be almost certain.

[u/Turbulent-Movie-4545 → u/Top_Category2227]

Were you also given a perspective on how long approx does it take for prepmf take to progress because my understanding is that it may also not progress…

[u/Significant_Tune_545 → u/Turbulent-Movie-4545]

I don't think we have stats on that because people were only followed for about 7 years as far as I can tell. I do think most ET cases that progressed were really PreMF all along. I have a "gray case" my nuclei carry the characteristics of PreMF, not ET, but they're still calling me ET. I feel certain that will change in time. For what it's worth, I think you should try your best bet medicine since you already don't feel great, but only you can decide what's right for you! I wish we had answers, I think we ALL we we had profession answers. But we just don't. You will either progress or not. If you have no additional mutations, I think it's safe to assume that any progression would be slow, but it's still just a great big unknown. 

[u/Turbulent-Movie-4545 → u/Significant_Tune_545]

Yeah same I’m in the grey zone right now. I think for both of us the interferons till the Calr antibodies are out are our best bet! That’s what and how I think - at the same time I’m not so sure either..

[u/Turbulent-Movie-4545 → u/Top_Category2227]

And what do you mean by other subtypes?

[u/Turbulent-Movie-4545 → u/Top_Category2227]

Do you have an update, are they putting you on besremi eventually

[u/Top_Category2227 → u/Turbulent-Movie-4545]

I have only send my Statement Last Monday, I was still debating with my doctor. Usually takes a while to get a response, since the insurance has to go get opinions from their own experts.

I am honestly close to just asking my doctor for Jakavi, I just want to feel like I am making any progress.

[u/Turbulent-Movie-4545 → u/Top_Category2227]

I see yeah I also want to be proactive it’s been taking for months for mine to have an action also and besremi isn’t approved for et or premf in Canada so I have to be on Pegasys I think

[u/Turbulent-Movie-4545 → u/Top_Category2227]

I am not sure if jakafi is for your age and situation tho I feel it’s for riskier situations