This is my first post although I joined the forum about two years ago. Not sure why I haven’t posted sooner and not sure exactly where to start. I would like to say thank you to everyone here for all of the invaluable information and sharing your stories and experiences with MPN’s. I read posts daily and it has been extremely helpful to me. I was diagnosed with ET over 17 years ago after multiple routine blood tests showed elevated platelets. I was then referred to a hematologist where it was confirmed through additional bloodwork that I had ET and was jak2 positive. My platelets have hovered in the mid to high 500’s since diagnosis and I have not really explored treatment until recently. Over the past couple of years my hematologist and I agreed that we could take a wait and see approach since my counts seemed ‘stable’. ( he did try to convince me to try hydroxy which I declined). I spoke with him about Pegasys a few times and at first he declined and told me he wouldn’t give it to his worst enemy due to the side effects. At my last visit we discussed it again and he agreed to put it through insurance to see if it would be approved due to the cost, and to our surprise it was approved. I’m a little nervous about starting it after everything he has told me about it but would appreciate any input. Just a little about me, I am 58 years old and keep myself physically fit, although I do have two cardiac stents. One in 2009 and another in 2017. Heart disease does run in my family and not sure it had anything to do with having ET. I still run and work out four days a week with no real issues. I put some of my latest numbers from my blood results below. My hematocrit is usually between 45 and 47 and my platelets 560’s to 580’s. I have just been thinking a lot about starting treatment to address my ET or to just leave well enough alone. I Also would like to hear any experiences with those of you who have tried Pegasys. I do apologize for the long post and hope I haven’t rambled on too much🙂.
Thank you
WBC
7.83 k/uL
RBC
5.26 M/uL
Hemoglobin
14.8 g/dL
Hematocrit
47.0 %
MCV
89.4 fL
MCH
28.1 pg
MCHC
31.5 g/dL
RDW
13.5 %
MPV
8.6 fL
Platelets
595 k/uL
Written by
pjm03
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Is your Dr longtime in the profession? It's possible he lived through the early INFs. These were hard to use. I actually remember when INF was the latest thing ~30 years ago when I had no reason to care. Next thing it was no longer fashionable because of its troubles.
The current Pegylated INFs (PEG and Besremi) are easier on the body and we see right here that many are doing quite well with these. It is now fashionable for MPN (I am right there myself) But there are those who cannot take it too, just fewer of them than with the old INF. So my opinion is Dr should not automatically reject it.
There is a thought from researchers to use INF preemptively to reduce odds of progression, but there is not deep evidence this is reality. With your stable numbers that might be why Dy does not want to add any risk with INF, but if it might prevent something that is a consideration.
Your blood counts look good to this non-professional, esp after so many years. Your HCT is just over the usual goal for MPN but that is especially applied to PV and if your Dr is ok with it that is good.
You've likely seen posts from Hunter that a large change in PLT may be of more interest that its (reasonable) value.
Welcome to posting! As EPGuy stated, to this untrained, non-professional eye your numbers look pretty good especially two decades in. To treat or not to treat really depends on your and your MPN specialists goals. Until a spike in my platelet counts and some bleeding at the gums, my MPN specialist was content on aspirin and wait (I am 53 Jak2+) - as he usually did not recommend cytoreductive therapy until 60 or 65 (depending on history). We started on hydroxyurea at first but I did not tolerate it well and we switched to Pegasys. If I needed cyto-reduction, my preference was Peg as: 1) I hope to be around a long time and therefore possibly on the medication for decades; 2) the INF (Peg and Besremi) seem to be (today) the only medicines which might be able to induce molecular remission; 3) I was (am) also worried about possible progression given my allele burden and persistent leukocytosis..
I have been on Peg now for 9 months with one month off in the middle. My platelets have dropped from averages around 900 to about 600 (btw 600 is about the numerical goal for me and my MPN specialist) and my WBC has gone from about an average of 13.2 to within range (avg of about 8.5) - all other bloods look really good as well. The only side effect I have had was that my liver reading got a little wonky (liver toxicity is a known potential side effect of the interferons) which is why we had to halve my dose and stop completely for a month. As of my most current readings, the liver has stabilized and we have decided to continue at my current dose (90mcg every 2 weeks). Other than that, really, for me, no side effects of which to speak. I might be somewhat more tired than normal the day after the injection, but it hasn't even changed/stopped my workout routine on that day. My thyroid has fluctuated somewhat as well (might be the medicine might not be - getting it checked out) - overall I have been very happy on Peg.
Like EPGuy stated, your doctor's resistance to Peg might be related to an earlier experience with the interferons prior to the pegylated versions which were much more difficult to tolerate.
If you decide that cytoreduction is the way to go, Peg might be a good choice (I have also discovered that so much of this is trial and error to hit upon the right combo(s) of medicine and dosage).
Hello and welcome to posting on the forum. Glad you decided to reach out with your questions.
When I had ET, my numbers routinely cycled between 500s-700s and I never had any thrombotic events in 22 years. Most of this time all I took was aspirin. After I progressed to PV aout 8 years ago, I still had no issues with thrombosis. However, what I now recognize as MPN symptom burden did increase significantly in the last 8 years. There is more to the JAK2 mutation than the number of blood cells.
On the whole your numbers look pretty good, particularly 17 years into having ET. Normally I would hold off on cytoreduction if I was in your situation/age - except you have stents. This is a risk factor that has to be considered. If I was in your situation I would opt for cytoreduction to minimize my risks. In addition, I would be attending to the secondary symptoms associated with the JAk2 mutation. The overproduction of inflammatory cytokines can be a significant problem, particularly as we age.
You will hear a variety of experiences from members of the forum regarding hydroxyurea and Pegasys. We are all different in how we respond. I have taken HU on three different time periods. The first two were OK, but the last time I have turned HU-intolerant. I experienced toxicity even at a very low dose. HU was also ineffective in managing the PV. I have done much better on the interferons. I started on Pegasys then switched to Besremi. PEG was much easier to tolerate and far more effective than HU. In addition, PEG has a preferable mechanism of action and in the long-term appears to be more effective in preventing MPN progression.
Ultimately, this decision needs to be based on your treatment goals, risk tolerance, and treatment preferences. It sound like you lean towards PEG over HU. Many of us share this opinion/experience. Some of the leading MPN Specialists clearly favor the use of PEG or Besremi for most MPN patients.
Given your relatively low numbers and symptom burden, you would most likely only need a low dose of PEG. Likely 45mcg/week as a starting point. That might be all you ever need, maybe less. I started on 45mcg/week. It took about 8 weeks to drop my PLT by 200 (677 - 477). PLTs have since stabilized and stayed at target.
Please do stay in touch and let us know how things go.
Thank you all for the replies! I appreciate your advice, and sharing your experiences! So the plan from my Hematologist is to start at 90 mcg every seven days with blood work monthly to monitor my counts Etc. I like the sound of starting at 45 mcg and working my way up if needed. I have been so torn on starting cytoreduction since I have been somewhat stable but always have disease progression on my mind. In addition I know I am at a higher risk having cardiovascular disease with two stents. Since my last stent in 2017 my cardiologist started me on Brilinta 90 mg twice daily and so far so good, no blockages. He told me I would most likely be talking it for the long haul. I’ve also noticed just in the past year more muscle injuries, joint and muscle pain, and I developed plantar faciitis on a run which was extremely slow to heal. Im wondering if my ET could be a possible culprit. I have just putting the blame on getting older 🙂. So I hope to start treatment in the very near future once I speak with my hematologist again, and finalize the plan. As a side note, I have been looking for an MPN specialist in / near Virginia for a second opinion but haven’t had much luck. The closest I have found is at John’s Hopkins in Maryland. I thought I saw a list somewhere here in one of the posts but couldn’t find it. Thanks and will keep you posted on the treatment plan!
There are no MPN Specialists in Northern Virginia. I looked as I live in Harpers Ferry and base most of my care in Northern Virginia. I have an excellent hematologist in Leesburg who provides the ongoing care. I see a MPN Specialist at Johns Hopkins. I was seeing Dr. Spivak but he semi-retired. I switched to Dr. Brainstein but he just moved on to Incyte. There are only as few MPN docs left at Johns Hopkins. I will be seeing a new doc in July. We will see how that works out.
On the list Dr. Ambinder, Dr, Smith, and Dr. Jain are possibles Dr. Moliterno is not taking new patients. i will be seeing Dr. Jain.
FYI - the joint pain, muscle pain and plantars fascitis are like all related to the MPN. I experience all three. The proinflammatory state the JAK-STAT deregulation causes has multiple bothersome manifestations.
Hi pjm, you have 3 great replies so far. The only thing I can add would be to consider to see a MPN specialist since you are considering beginning cytoreduction. At your age (young) I would consider interferon over HU due to the long life you have ahead, but that's just me, not medical advice. I would speak with a MPN specialist if possible. I think you do need to consider your cardiovascular status. Best to you.
I think we all feel a bit frightened to start a new drug that has so many side effects listed. I made plans to take it easy for the day of my first jab with the nurse, but found myself carrying on as normal and had no effects at all. I may not have drunk enough water which needs to be a lot and my only feeling was like a slight hangover the next morning. I started on 45mcg weekly to let the body get used to it. They soon upped me to 65mcg which suits me very well. 90 didn’t. It’s worth a try and if it doesn’t suit you, you can change to something else. Let us know how you get on and we are all here to help
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