Hi, I have ET Jak2 +, probably had it at least 16 years. To be fair, it has never given me any trouble at all, but annoyingly my numbers keep rising. Now >1500, so doc very keen for me to start on a cytoreductive drug. I am considering it.
I am going for a second opinion with an mpn specialist, so want to be well prepared with all the right questions to make the most of this appt.
So, to tap in to your wealth of knowledge and years of experience, what questions do you wish you had asked your heamatologist before commencing treatment?
Did you feel your doc gave you sufficient info regarding side effects?
Do you have any regrets about your decisions and why?
I am 53. Should I push for one drug in particular?
Plus anything else you can think of that is should know ahead of time.
Thanks in advance
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Amethist
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If you can afford to see a functional practitioner or Ayurvedic, to support you that would be helpful. Someone who has good knowledge of western meds and interested in keeping an eye on blood tests etc.
I think if platelets are so high I would combine conventional and natural approach .
Hi, I'm ET Jak2 for about 5 years now and just on 75mg aspirin. I'd want a full run down of the different meds and the investigative ones like Ruxolitinib. I don't like the sound of some of the side effects to be honest but they've told me I have to consider them by the time I'm 60.Just make sure you ask everything you need to ask. Write it down, and don't leave with any unanswered questions.
Docs have a tendency to answer one and then try to move you on. It leaves you feeling like no one is listening.
I hear some of the treatments are well tolerated on the whole, so your mission is to arm yourself with the knowledge.
I think by answering you, I also just had a little talk with myself 😊
Have you looked at the MPN Voice website? Reading that may guide your questions. Be sure to write down everything you can think of no matter how insignificant it may seem. Best wishes.
I would be looking at several things in your situation. One would be a thorough check for Acquired von Willebrand Disease. Start with a standard von Willebrand Panel, but consider a ore comprehensive check if needed. Here is some info on that.hematologyandoncology.net/f...
This does matter as aspirin is contraindicated in the presences of Acquire von Willebrand Disease. If your regular hematologist has not already dome this, the MPN Specialist certainly will.
Regarding cytoreduction, assuming you do need to initiate it - hydroxyurea would be considered contraindicated ay your age by many docs. Many docs do not initiate HU in patients age <60 years due to concerns about long-term leukemic risk (10+years). Per the American Society of Health System Pharmacists "Hydroxyurea is a highly toxic medication with a low therapeutic index." Do note that some people are able to tolerate it and benefit. It does often reduce hematopoiesis rapidly, but is does so at a cost for many. I am one of those who is HU-intolerant. I had toxicity even at low doses. Here is some info on HU.
There are no drugs FDA approved for ET in the USA, including HU. They are all off-label. HU is much cheaper than the other options ($70/month vs $4,000/month or more). Insurance companies prefer cheaper drugs, even when there are potentially better drugs available. Jakafi and Pegasys are also used for ET (off label). So is anagrelide (another cheaper med). Something in favor of Pegasys (and the newer version of interferon - Besremi) is the the IFNs appear to be more effective at preventing disease progression. There is the possibility of hematologic and molecular remission with the IFNs, which is very promising.
Jakafi is known for its effectiveness in treating secondary MPN symptoms like pruritis. People have used it successfully to treat ET symptoms, which kames sense since it is a JAK-inhibitor.
You can find more information about the other medication options at the same two websites listed above. Do note that ALL of the meds used to treat MPNs have a risk/benefit profile. We each respond differently to each of them. It is important to decide what your treatment priorities are, along with your preferences and risk tolerance. It is ultimately our decision about what you should take. Hopefully you will find a MPN specialist who will you be able to work well with to make the decisions you need to make.
Thank you very much for all the information. Yesterday I also came across a paper from 2019, that also talked about low risk ET pts with extremely high platelets, and that just because they were high, it doesn’t necessarily mean they have to take cytoreductive drugs, so that gave me some hope too.
Jevans, I have a stash of papers, cant find the exact one (note to self: must sort out my online filing system!), but here are some of the ones i took to the specialist to discuss, all basically say the same thing., ie, its not so much the high platelets by themselves but there are other important factors too.
1. Clinical Advances in Hematology & Oncology. October 2017 - Volume 15, Issue 10 “Current Challenges in the Management of Essential Thrombocythemia” by Ariel Kleman, MD, Arun K. Singavi, MD, and Laura C. Michaelis, MD
2. “Approach to patients with essential thrombocythaemia and very high platelet counts: what is the evidence for treatment?” By Lorenzo Falchi Prithviraj Bose Kate J. Newberry Srdan Verstovsek First published: 16 December 2016. doi.org/10.1111/bjh.14443
3. “Essential thrombocythemia treatment algorithm 2018” by Ayalew Tefferi , Alessandro M. Vannucchi and Tiziano Barbui , Tefferi et al. Blood Cancer Journal (2018)8:2 DOI 10.1038/s41408-017-0041-8
4. “How I treat essential thrombocythemia” by Elisa Rumi1,2 and Mario Cazzola BLOOD, 17 NOVEMBER 2016 x VOLUME 128, NUMBER 20
5. PerspectivePerspectives on thrombosis in essential thrombocythemia and polycythemia vera:is leukocytosis a causative factor?Tiziano Barbui,1Alessandra Carobbio,1Alessandro Rambaldi,1and Guido Finazzi. BLOOD 23 July 2009
6. “Correlation of Thrombosis With Increased Platelet Turnover in Thrombocytosis” | Blood | American Society of Hematology 18/01/21
7. “Hydroxycarbamide Plus Aspirin Versus Aspirin Alone in Patients With Essential Thrombocythemia Age 40 to 59 Years Without High-Risk Features”
8. Guideline for investigation and management of adults and children presenting with a thrombocytosis, Br J Haematol . 2010 May;149(3):352-75. doi: 10.1111/j.1365-141.2010.08122.x.
9. Correlation of blood counts with vascular complications in essential thrombocythemia: analysis of the prospective PT1 cohort , doi.org/10.1182/blood-2012-...
10. The quantitative JAK2 V617F neutrophil allele burden does not correlate with thrombotic risk in essential thrombocytosis, November 2007 Leukemia 21(10):2210-2
Hi Amethist, I've just been reading this very old thread. It caught my attention because you seem to have a very similar position to me on treatment. I've had ET for 15 years since I was 40 ( now 55). Never been on any treatment except aspirin and have consistently had a platelet count between 1000 and 1300 that whole time. I do occasionally get a bit of pressure to consider going on to treatment by my consultant but I have resisted it so far. They say if I go over 1500 or I get to 60 years old then treatment should start, but I will still be thinking very carefully about it, even then. I have no other risk factors. I was just wondering if you did go on to start treatment or not and how you got on ?
I’m now 57, and moved back to uk 2 ago . Since returning I haven’t even bothered to register with a gp, let alone any other medical professional.
So no, other than ½ regular aspirin every 3-5 days ( depends when my toes tingle,) that all I do. Or ever plan to do. The age criteria is rediculous and completely unjustified.
A few years back my platelets went up to approx 1600 so haem wanted me to start HU. I stalled him and managed to get a 2nd opinion from a MPN specialist who agreed with me and the regime I had put myself on. I never went back to the haem. Then moved country. So for the last 3-4 yrs, i haven’t had a regular blood test. I currently have 0 faith in the medical establishment.
Moving forward- I have researched which ‘ cancer’ pathways are involved in ET, worked out which supplements block them, and have pursued this route. I did do a private blood test 6 mths ago as a starting point, still around 1450. Probably will check again in a bit. However I know these tests are just a snap shot and certainly very unreliable. On 3 occasions I’ve had 2 blood tests in the hospital taken within 1 hr, where the difference has been around 300, sometimes higher, sometimes lower. Haematologist could not explain it. Yet they base treatments on these results?!
I think it more important how you feel. The initial symptoms that I had when I first saw gp ALL went away when I sorted out my vitamin deficiencies especially B12 and D. Maybe if ET made me feel unwell, I might consider trying a medication, but it really does not impact my life in any way, so what’s the point. I certainly don’t want to risk any side effects.
So for now, a little aspirin ( as too much is a potential problem with bleeds) and supplements/ vitamins and that’s fine for me .
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My Heamatologist said I most likely have ET and mutation tests showed I have JAK2
bone pain??
ET , newly diagnosed and have a couple of questions.
What questions should I ask the consultant?
I am new to PV and this website
