Starting Rux Next Month

[mfh7]

After five years of phlebotomy only with PV I started besremi two years ago at the minimum dose since I wanted to take a more early intervention approach. Also my platelets were extremely high from all of the phlebs. Been on max dose of besremi now for over a year. Besremi lowered my platelets and normalized everything but the red count/hct. Unfortunately lowering the red count is the primary goal and I still have phlebotomy every two months. In that regard besremi has failed as it does for a certain number of users. They don’t know why. Could be secondary mutations is the working theory I think. My MPN has recommended I switch to rux since for whatever reason the interferon is not controlling my hct even after the max dose for over a year and the hct control is the primary goal for my situation.

My question is whether anyone here had no hct control with interferon but had better luck with rux/jakafi in that regard. Also has anyone here had allele burden reduction with rux?

Thanks in advance.

[Ovidess]

I have only taken Besremi, and am now on it at its smallest dose. My platelets came way down from over a million into the normal zone for a while (currently slightly above), and my HCT has pretty much been in the decent zone the whole time, but I notice the red blood cells never get into the normal zone, and the doctor does not even seem to think think that a goal. It makes me wonder if he is focusing on the right thing. When I was first introduced to a diagnosis of ET in 2023, it seemed to be mainly about controlling the platelets, but as I learn more, platelets become less important and stickiness of the blood looms large. I got upped to two 81 mg aspirins a day. Is it true that medicine really has no answer for the sticky blood issue?

[mfh7 in reply to Ovidess]

We are all different but I also had good results in terms of lower platelets from besremi but for what it is worth my mpn doc seems insistent that for me it is the control of the hct i.e that is the most important factor and since besremi has been unable to do that after two years we need to try something else. Fingers crossed the jakafi will work for me and eliminate the need for phlebotomy. Best of luck to you as well!

[jon1972]

I am in the same boat with the ineffective HCT control on IFN. Homozygous for PV is another reason for HCT resistance. Peg sort of helped for a while before becoming ineffective and bad side effects, and I finally had to stop taking it. I recently tried to get put on Jakafai and got denied by insurance since the label says to try hydroxyurea first. So I started taking HU a week ago, but almost hope that i'm intolerant since I have always had a bad feeling about HU. Very toxic with Leukemia and skin cancer risk (according to the label). Good for you for being able to get on Rux/Jakafai. I've been having the same questions you asked.

[mfh7 in reply to jon1972]

Thanks for your reply. Are you in the US? If so I would recommend appealing the decision of your insurance company in denying Jakafi. The insurance companies make their decisions solely based on cost concerns as I’m sure you are aware. My MPN specialist helped me with the appeal and got it done for both besremi and now jakafi. What does Homozygous for PV mean and how do you know it exists for you?

[jon1972 in reply to mfh7]

Actually I'm not certain that I'm homozygous, but on my bone marrow biopsy they said my allele burden was very high, I want to say 60%+. They had a side note saying that this is associated with more aggressive disease characteristics, which is often associated with a homozygous mutation. Homozygous means both alleles on the gene are positive for the mutation instead of just one (heterozygous). Someone can feel free to correct me if my terminology is off with any of this. Under the same insurance I have been previous approved for Pegasys and Besremi, but they didn't like the request for Jakafai. I think it's the most expensive of them all. My doc didn't want to try and fight it saying that given their reasoning that I didn't have much chance of winning an appeal. So I elected to take the route of going on HU first. I'm most likely going to have some side effects that I'll ultimately use in my case for an appeal. But I guess this is the easiest route to get through the appeal process. Yes, I'm in the US and go to Cleveland Clinic.

[EPguy]

I've had good blood counts on HU, IFN and Rux, so not a match there.

But I've also had continuing allele burden (VAF) reduction on Rux as of last. There are other members who have also posted good reductions on Rux, consistent with the trials.

There are regular posts of members here with weak HCT control on IFN, as you refer.

What secondary mutations do you have?

[mfh7 in reply to EPguy]

Asxl but only 10%. 40% jak burden for what that stat is worth. I’m familiar with the references to the possibility of a potentially poorer prognosis with that additional asxl mutation but hoping that doesn’t apply to me. I’m 57 and was diagnosed at age 50. Im having slight spleen enlargement for the first time this year even after two years of high dose besremi so hoping the jakafi will help there. My two primary concerns are hct control and progression. Hoping jakafi will help with both. I tolerated besremi well at max dose for over a year and it was great at lowering platelets but I think the lack of hct control is relevant to the spleen so need to try something new .