I was diagnosed with PV in January and a BMB showed I have stage 1 Fibrosis on the bone marrow so I have been diagnosed with PV/early MF.
My spleen is enlarged but up until very recently my bloods were normal. Now my hematocrit has risen slightly. I had portal and splenic vein thrombosis 3 1/2 years ago and take blood thinners.
I am due to start on Ruxolitinib in the next few weeks but I am quite worried about the side effects, the specialist has been advised to put me on the highest does I can manage.
I am relieved that these drugs are on the market but I worry about how they will affect my life and of course I worry about weight gain, has anyone taken this medication and not gained weight? How much weight am I likely to gain?
I know there are a few other drugs on the market and I have heard that the side effects aren’t quite so bad with Interferons, is this true?
Should I be considering something else as a first line treatment?
Also, I’m 41 and I can’t help worrying about my life expectancy, is it possible to have PV/MF and life a long life or is it the case that a potential transplant is looming and it’s just a matter of time?
I am grateful for any advice offered as I am finding this situation very daunting.
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DarcyShepp
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Based on what you indicate above, it sounds like you have post-PV MF, with splenomegaly and a history of thrombosis. Ruxolitinib would be recommended by many MPN experts in this situation.
I do not think that I would say that the interferons have fewer side effects than the JAK-inhibitors. These two classes of medications have different side effects along with their different mechanisms of action. We are all different in our treatment needs and how we respond to these medications.
It sounds like you may be seeing a regular hematologist who has consulted with a MPN Specialist. If you have not consulted directly with a MPN Specialist, it would be advisable to do so. This would be the best way to address your questions and concerns.
It would be very reasonable to want to minimize risk of adverse effects while achieving your goal of preserving both quality and length of life. Suggest that you speak directly to a MPN Specialist about how best to achieve these treatment goals.
Since your blood counts have been normal, except for a slight rise in hematocrit, and your fibrosis level is only 1, you may be have a case of early PV, in which case a low weekly dose of Pegasys interferon, that has minimal side effects, could potentially slow or prevent progression to the transplant stage for many decades.
But alot depends on whether your spleen is slightly, mildly or greatly enlarged. Were you given an idea of how enlarged it is, either descriptively or in centimeters? Did your BMB report mention a disease driver mutation and it's allele burden percentage? FYI: Normal Life Expectancy for Polycythemia Vera Patients Is Possible: sciencedirect.com/science/a...
Thank you so much for your reply, in 2020 when I had the blood clots my spleen was 16 cm’s in April 2024 it was 19 cm’s which I think is quite large. I’m Jak 2 positive and my BMB revealed an allele burden of 71%. I haven’t been told I have any other mutations but the hematologist said we can test for them.
I had thought they would have been picked up in the BMB but he hasn’t confirmed that.
I’ve had itching as as symptom for decades and it’s only since being diagnosed with PV that I made the connection. Even when I had the blood clots my blood tests presented as normal.
Thank you again for your reply, this group is so helpful!
A 71% allele burden, history of blood clots and growing spleen at the age of only 41 strongly suggests a rather aggressive and/or complicated case of disease. On the other hand, your near normal hematocrit and hemoglobin suggests mild disease.
IMHO your case is too complicated for a regular hematologist to handle and you should consider seeing a hematologist that specializes in MPN's and could offer treatments like interferon or the combination of interferon and Jakafi and offer a more informed prognosis and long term care plan via a Next Generation Sequencing gene panel blood test.
Sounds expensive, and it could be, depending on where you live, but here again the true specialists are aware of some of the financial work arounds - like easy to qualify grants that cover the cost of the Next Generation Sequencing gene panel.
Re side affects: very often on drug leaflets things are said like 1 in 10 people will experience X. Remember that means 9 in 10 won't. The ruxolitinib weight gain is partially in your hands. You may need to watch the amount you eat and exercise. When I was on ruxolitinib the "side affect" I had was it stopping itching! I did put on several pounds but I love food. I had to change to fedratinib because my platelets fell too low
I don’t have same diagnosis but if you choose Interferon I haven’t experienced any side effects, apart from a few muscle aches post injection. I haven’t gained weight, infact I’ve actually lost a little which is great for me👌. Good luck on your journey.
the simple answer is most have less sides on Rux than Peg , weight can be an issue but only if you let it, it’s the same mechanism either less and better quality calories or more exercise or both.
I do wonder whether it would be wise to get a second opinion from an MPN expert to establish if you have PV or MF. Stage 1 fibrosis is not so bad, I discussed fibrosis with my expert at Mount Sinai and he said they now think fibrosis is not the big deal they thought before.
If you are PV or mild MF I wonder why switch to Rux , Peg might do the job as well for you.
As usual we are just giving comments , we don’t have the records and full medical picture.
If it were me I would get a second option from a MPN expert.
I am 42 and have PV. I have been on interferon for over a year and done well. So I know this feeling all to well. Luckily my spleen has been ok. But I was diagnosed about 4 years ago. You really need a MPN specialist if you don’t have one. They know best. If you have any specific questions just reach out!
Hi. Just for your knowledge, a recent article discussed the link between this drug and skin cancers so be sure to use good sun protection if you take it. Btw I loved that you said Hi “Guys”. I’m in the U.S. and use “guys” all the time when talking to more than one person. I’,m happy to see someone else use it. Best of luck in your journey. Katie
I was diagnosed with PV back in 2015 and have been on Rux for almost two years. My blood count numbers have been stable ever since the correct dosage was determined after my first few months on Rux. I've had no trouble with weight gain, rather the opposite.
You're right about all our options for treatments. I have another condition that is untreatable so I appreciate the MPN options too.
Your Dr likely has pointed you to Rux because of your enlarged spleen. Rux is especially known to rapidly help many pts here. But interferon (IFN) can also provide this result. Both can reduce the Jak2 allelic burden (VAF).
You say "the specialist has been advised to put me on the highest dose I can manage". This specialist should be the one doing the advising. What is this Dr's specialty? As others say here, you should find an MPN specialist. What outcome are they looking for with this high dose?
I've been on all three common MPN meds, HU, IFN, Rux. For me Rux has been the mildest. On side effects, on the forum re Rux we hear most often about weight gain, and less often Shingles and non-melanoma skin cancer. You should be up to date on the current shingles vaccine before or while starting Rux. Ask your Dr for more details here. If you're at risk for skin (history of skin cancers, light complexion/sun sensitive) this risk is higher. It may also increase if there is past HU use. Standard sun protection habits are good for all including Rux pts. I've had increased hair growth on Rux, one pleasant side effect that is known to relate to Rux.
As Hunter says, IFN has a different side effect profile. It is more varied and thus harder to describe in one sentence. High liver blood counts and low WBC counts are common dose limitations. IFN is the only one with an FDA black box warning for worst case dangerous outcomes. Rare but can arise with short warning. I have suffered this. But IFN can be especially effective for lowering high VAF and no worries about causing weight gain, shingles, or treatment induced skin cancer.
I experienced malaise and fatigue on IFN, but it worked great on VAF and blood counts.
With all the meds, more dose makes sides more likely while many have no troubles at all.
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However you say you're not aware of being tested for any other mutations. These can be tested by BMB or blood draw. This is increasingly standard procedure to check for "non-driver" mutations. You should inquire further and request if it has not been done.
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As monarch5000 notes, you could be a candidate for the combo therapy of IFN/Rux. It can allow lower doses of each and get the best of each. But getting insurance to pay may be a problem. If I could start over, I would try to get this option.
Thank you so much for such a detailed answer, it really does help!
My hematologist requested the advice of a very well known mpn specialist as my case was tricky. She reviewed my files and gave him advice on the course of action. He wanted her specifically to provide her comments as she has worked in the field for such a long time and has a lot of experience. I will do some more research on the options you suggested. Thanks again
Totally understand your concern. I was diagnosed 15 years ago with PV and it’s perfectly ok to find it daunting. It’s on your mind constantly.
I promise things will get better as you adjust to the new normal.
I do confirm that you need an Mpn specialist.
Itching wise - more commonly known as aquagenic pruritis. Like having thrush under your skin, a blow torch on the top and about a million insects nibbling at the same time?
I had this in a very severe form. Absolutely horrendous. I decided to make a note of when it happened, and what might have sparked it off.
The main cause for me of course was bodily contact with water so I gave that up. I haven’t bathed showered or swum for 14 years.
Fortunately I can wash my face, my hair ( over the bath) and we have a bidet.
Hugely recommended you try it. Plastic bidets available on Amazon in you don’t have one. They fit over the loo.
Things will get better, ask Maz at mpn voice.org.uk for a buddy. She will pair you up with a friendly voice who knows exactly how you feel because they ‘ve been there.
Think of your brain as having two Mpn boxes.
In one, a very tiny box which will shrink as time goes on, is the part that’s frightened concerned daunted - in the other box a great BIG positivity box which will get bigger all the time, is the part of you that thinks “ right. This is NOT going to get the better of me. I know that although it’s a bit tough at the moment things will be under control, there’s loads of help out there, and in the end ALL WILL BE WELL”
I have also had "aquagenic" pruritus [although I don't need water exposure to be intensely bother by a stinging "itch" sensation] for well over a decade, while my blood counts remained normal- although my Hct and Hgb were elevated for most of that time.
Then in 4/22 my CBC finally showed elevated RBC's, and prompted referral to a local Heme/Onc, and when they repeated my CBC in 6/22, all my counts- RBC, WBC, and platelets were in the very high range.
They started me on HU immediately after my BMB and Abd U/S- where a 48% allele burden and borderline splenomegaly were found.- but the side-effects were nearly immediately intolerable, and even if they had not been, my reading aimed me at getting on interferon ASAP because of its potential for achieving "MRD" - minimal residual disease burden.
Thus, i asked the local Heme/Onc to be prescribed it, but they were uncomfortable with it, having never used it, so I looked here:
and found Dr. Wang at Roswell Park CCI in Buffalo, NY, who I contacted by email, and who agreed to manage my transition to Besremi.
Thus, I am currently on the Besremi 'flavor' of INF, and while it has been intermittently helpful in lowering my blood, counts [especially my WBC and Platelets] I haven't really felt any relief from itching yet.
I also haven't really had any side-effects apart from 1 episode of body aches and malaise the next day after one of my middle level doses- I'm at the max dosage of 500 mcg every 2 weeks at present.
What has helped my itching is taking a beta alanine supplement- which I also learned about here on healthunlocked.
I take a 3-gram dose in a soft drink twice daily and have had a significant improvement in my quality of life- and if I'm ever not completely sure how much it helps, skipping or even delaying a dose makes me very sure it helps.
The only side-effect i have ever had from it was some facial flushing [which I usually have often anyway] and transient hot sensation in my face that is way less troublesome than my painful itching.
For what it's worth, I think that if you can, you should go to see the MPN specialist nearest you so that your information [and care] come to you directly and not filtered by transmission through someone else's understanding.
I hope you find it works for you as well. I have to admit I can't say that it has increased my strength or stamina [which is why athletes and especially body builders take it], but I'll settle for the itch relief.
Keep us apprised of your results and ongoing concerns/issues, that's why we're all here.
I also have PV and stage 1 fibrosis but have not been diagnosed with early MF. I experienced aquagenic pruritus for 6 years and it dominated my life. I was diagnosed in 2019 and took hydroxy for nearly 2 years which controlled my bloods but not the itch. In 2021 I switched to Rux as part of the Mithridate trial and the itch stopped almost immediately but I have never lost the burning sensation in my legs. All my bloods except platelets are well below normal so I have been anaemic for several years and recently it has been discovered I have heart failure for which I have an appointment with a cardiologist in June. I have no idea if the heart condition has anything to do with my MPN.I have also gained weight but I think that is because fatigue and breathlessness have made me less active.
I should say at 72 I am a lot older than you so probably more medical issues are to be expected. I am still happy that I went on Rux because the itch was all consuming and impacting on my life. I realise this post is a bit gloomy but I live a very full life and am very happy keeping busy with family events, lots of walking, gardening and as many weekends and holidays as the budget will allow .
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