Diagnosed with ET in 2016 and PV a few years later. Recently started on Hydrea after platelet counts reached 1,000,000. Have been having phelbotomies for several years for the PV.
I'm a recent transplant from the Midwest to the Central Coast of California...arrived just before the pandemic. My symptoms started to worsen shortly after the move...I was fortunate to find an awesome oncologist here. Until recently, I've been able to manage the symptoms with the help of an Integrated Health specialist but a recent heart attack brought about by a blood clot, changed my thinking on how I was going to continue to manage these cancers and still be active and healthy.
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K-itty
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Welcome to the forum. Glad you found your way here.
Like you, I have PV with thrombocytosis. I was diagnosed with ET about 30 years ago. It progressed to PV about 8 years ago. Many of us actually had a masked PV while others do truly progress from one disease to the other.
If you had a heart attack due to thrombosis then more aggressive treatment is certainly in order. Controlling the erythropoiesis is the key to reducing thrombotic risk. it is more important than the level of thrombocytosis. Anti-platelet therapy (e.g. aspirin) is also a critical part of treatment. It helps with both thrombotic risk and controlling microvascular symptoms. Do be aware that phlebotomies and the resulting iron deficiency can drive up thrombocytosis.
If you found a good oncologist, then that doc should be able to help you sort it all out. I was on a phlebotomy-only protocol too, but was having trouble with the iron deficiency symptoms. I started on Pegasys back in May. So far things are going well and the 45mcg dose seems both to be working and not causing adverse effects.
Note - I also see an Integrative medicine doc. This is not a conflict and supports my overall treatment plan. This doc diagnosed several nutritional deficiencies and is consulting on the complimentary health approaches I use to control the systemic inflammation that is caused by dysregulation of the JAK-STAT pathway. I always assume that if something is biologically active enough to help you, it can also hurt you - and interact with things. I hope you have found a good Integrative Medicine doc where you are now.
Thanks Hunter! I have frequent CBC tests ...every 3 weeks, to see where platelet levels and hematocrit levels are, as well as iron deficiencies... tso far, so good...the iron deficiencies, that is. Just recently, as I shared, just started on Hydrea to lower platelet counts. I've read up some on Pegasys. It's it pill form or injections? Why did you switch from the Hydrea?
Next week I'm having an angiogram to see what's what in the heart. An MRI this year revealed a blood clot in the apex of the left Ventrical caused by an old heart attack which caused an aneurysm which in turn caused the blood clot...Whew! 🤔 Somewhat apprehensive about this, but felt it was necessary.
The Integrated Health Dr. has also helped with many nutritional deficiencies, which I feel has helped me stay well while dealing with everything else surrounding these cancers.
My first Hemotologist/Oncologist said I was just one of the "lucky ones" to have the Jake gene! Uh, thanks?
Pegasys is an injectable medication. Usually taken 1x/week. I was back on hydroxyurea for 1 year 2018 - 2019 but I was not able to tolerate it it this time. I experienced adverse effects even at very low doses. Even at higher doses, the HU was not adequate to control the erythropoiesis and I had to also use phlebotomies every three weeks. Then I was over-phlebotomized and became so iron-deficient that my HCT dropped to 32%. After consultation with a MPN Specialist, I shifted to a phlebotomy-only protocol for nearly two years. This worked well for a while, but then the long-term consequences of iron deficiency became problematic. These consequences included driving up the thrombocytosis by nearly 200K.
The decision to opt for Pegasys was driven by several things. I needed to attain better symptom and risk control than phlebotomy only could offer. PEGylated Interferons are the only treatment option that we currently have that are potentially disease modifying. Hematologic and molecular remission is attained for some people. The long-term research also indicates that progression=free survival is better with PEG than with HU or other options. Based on my treatment goals, priorities and risk tolerances the risk/benefit profile of Pegasys is better than hydroxyurea. Hydroxyurea is a highly toxic medication and does have significant risks. I did not in fact respond well to it. That is not to say that there are not risks in using PEG, there certainly are; however, these are the risks I would rather take particularly in light of the better benefits offered by PEG.
I have responded very well to Pegasys. I have experienced no adverse effects at all at the 45mcg/week dose. The medication is controlling erythrocytosis and thrombocytosis. HCT and PLT are both within normal limits. My iron levels are coming back up without an increase in HCT. Do note that not everyone responds as well nor as quickly as I have. Some people cannot tolerate PEG at an effective dose. Others do better on Jakafi. Some do better on HU. We are all different in how our MPN presents and how we respond to our treatment options.
I hope you find your current treatment plan effective and that you tolerate it well. The good news is that if it does not work, there are other options.
Hello K-itty and welcome to our forum. Really pleased that you have found a good oncologist and that between you, you find a treatment plan that suits you. Take care and best wishes, Maz
Hey Kitty, this may sound stupid but is there a climate difference for you in moving from the mid west to California? The reason I say that is warmer weather worsens all my symptoms.
Your question isn't strange at all! Yes, hugh climate change from the Midwest to the Central Coast of California! Most people think it's warm everywhere in California...I know I did! The Central Coast is right off of the Monterey Bay, so we typically have fog in the morning and warm, arid afternoons and cool evenings-unlike the Midwest with heat and humidity in the summer and cold winters. I've noticed that it's more damp, cold air that bothers me...specifically my joints, and not the warmth. In fact, my body lives the dry heat!
Hi, k-Itty, I am also in the US. But I have post ET MF, having been diagnosed with Et in 2008 which transitioned to Mf a few years ago. I agree with Hunter, that you probably need a more aggressive treatment since now you’ve had a heart attack. I also have a functional medicine doctor but I started experiencing nausea a few months ago, and have stopped all supplements, which seems to have made my bouts of nausea less frequent.
Take care and welcome to the forum. It has been extremely helpful to me in so many ways.
Thanks for the concern and reply...I am having an angiogram this coming Tuesday to address an MRI finding that I have an old blood clot in the apex of the left Ventrical of my heart due to a heart attack which caused an aneurysm that in turn caused the blood clot-Whew! I'm more than certain is due to a high platelet count, which I've tried in vain, with no success, in lowering without the use of Hydrea.Will see next week what is what and whether or not stents are going to be required?
Well, I’ve been on and off Hydroxyurea since 2008. In 2010 my numbers stabilized and I went off my medication without consulting my doctor and I ended up in the hospital with a blood clot in my spleen. I was in the hospital for three days. Over the years, I’ve been on anagrelide, Jakafi, and more recently, in the last 2 years, I’ve been on Fedratinib with Hydroxyurea for the MF. I’m having another bone marrow biopsy on Monday to see if my MF has progressed.
I’ve been on hydrea for five years for ET. It has suited me well and I have no side effects. I am comforted to know it offers me protection against thrombotic events.
When I first started this drug I developed the odd mouth ulcer. As my body became accustomed to the hydrea this side effect completely disappeared.
Return of canker sores that had been absent for 20 years
Thrush
Leukoplakia (severe dyschromia of the tongue)
Adverse effects continued even when dose reduced to 500mg every other day. Did not resolve until several months after HU was d/c. There was a permanent change to the surface of the tongue (now have geographic tongue/fissured tongue).
Just because something happens to someone else, does not mean it will happen to you. We each react differently. Here are some links to information about HU. Bear in mind the part about "could happen" vs "will happen."
I would note that with a history of a heart attack and a clot that cytoreduction is certainly indicated. You appear in your picture to be shy of 60, but despite younger age, the cardio-vascular risk must be considered. If you are choosing to use HU or any other medication, it is important to understand how it works, what the risks/benefits are and how to handle it.
Hunter, your vast knowledge indicates that either you've done your research or you are in the health care profession or both? I am actually in my late 60s and I too do vasts amounts of research...if you're not your own advocate, who will be? I was diagnosed with ET during routine blood work to have hip replacement surgery. At the time, 2016, my platelet count was in the 600s. I chose to do aspirin therapy at that time and later, wanting a second opinion, saw a Specialist at Mayo Hospital, who also told me I had PV. The course of action was still baby aspirin. Skipping forward to present day, my platelet count is 1,100+ and because of heart attack I chose to start Hydroxyurea...against my better judgment as putting a toxic nontheraputic drug into my body, goes against everything I believe in. However, neither do I have death wish and want to live a healthy productive live. Since starting on HU, I have experienced mouth sores, GI upset and fatigue. I have been able to control both by drinking aloe versa juice-something I've actually done for years, and supplements that have helped with the constipation. Having an angiogram in a few days, will also determine a course of action, i.e. blood thinners? These seem to be tricky cancers that as always, there's no set path as each of us experience them differently. I'm appreciative for all of the sharing on this site...it helps me on this journey.
My background is actually in Clinical Psychology, which is where I learned how to do research. I realized when it became clear that my ET had progressed to PV and my old hematologist missed it that I would need to become better educated about MPNs. At the same time I had a very negative experience with a medical procedure that was very badly handled by those docs. This all taught me a very important lesson about the price of ignorance we can pay when dealing with a medical condition and how important it is for us to advocate for ourselves. Assertive patients receive higher quality care. Passive patients do not. I am glad to hear you take the same approach.
It sounds like you are having some trouble tolerating the HU. Unfortunately it is a toxic medication and we cannot all tolerate it. The good news is that there are other options. After HU + phlebotomy did not work for me, I consulted with a MPN Specialist and shifted to a phlebotomy-only strategy. This worked OK for a while and did control the HCT; however over time the chronic iron deficiency was causing problems. This included increasing thrombocytosis, pushing PLT up into the 900s at times. I had researched my options thoroughly and knew a different approach was needed. I did check into the rusfertide clinical trial but did not qualify ( I was too iron deficient- not needing enough phlebotomies anymore). Based on the current research, I felt strongly that a PEGylate Interferon was the best choice. I had hoped for Besremi to be approved in time, but it was delayed. I decided to opt for Pegasys until Besremi becomes available. I started in May of this year on 45mcg/week. My response has been amazingly positive. HCT and PLT are well controlled after a very short time. I have had no adverse effects at all. I am very fortunate. Not everyone responds as well as I have.
The most important factor to control in PV is the erythrocytosis. This is the greatest risk factor for thrombosis. For females, HCT<43% is the important target as I expect you know. On its own, thombocytosis level does not correlate with thrombosis risk. At higher levels, it does correlate with hemorrhage risk however. There is also the issue of microvascular symptoms we need to deal with. While I have never had a thrombotic event, I have had some microvascular issues. They are quite bothersome.
The MPN Specialist told me 65 is the new 35. (I like that doc). We do not apply the age-based protocol to my treatment plan (now 66 years old/young). We decided on cytoreduction with PEG based on my actual presentation of PV, my treatment goals and priorities, and my preferences. One of the factors is that in addition to the JAK2v617f mutation, I have another mutation that increases my risk of progression (NF1:c5425C>T). I decided to opt for the treatment that offers the greatest chance of preventing progression.
Hope the angiogram goes well and that you get a clearer picture of your treatment plan going forward. If you do need to opt for a more powerful blood thinner, i would be sure to get input from the MPN Specialist. We are also at higher risk for hemorrhage and I would think the blood thinners with a lower risk of bleeding (e.g. Eliquis) would be a safer choice.
Hi K-itty. It’s nice to “meet” you, and to hear you’re in California, too! I’m in Northern California. I was just diagnosed with ET in March and you’re the first MPNer I’ve crossed paths with that lives “nearby”. It feels good to know of someone familiar with this type of disease who is just a few hours away! Best to you!
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