Hoping the new year finds you all well and looking forward to happiness and success in the coming year. Decided it was time to post an update on the ongoing saga of managing PV along with various and sundry other issues.
History - The current saga starts back in January 2018 when I had a god-awful Green Laser PVP/TURP. The docs were not very honest regarding the actual surgery and amongst other negative effects, I ended up with significant reactive thrombocytosis, which put me back on hydroxyurea for a year. This all emerged when I was in the hospital in April 2018 for what turned out to be paroxysmal atrial tachycardia. June 2018 – successful surgery for the tachycardia which was curative.
Started up with phlebotomies in July 2018 when it became clear my ET had progressed to PV. A few months later I started showing signs of toxicity (mouth ulcers, constipation) from the HU. The toxicity grew steadily worse over the next several months (thrush, leukoplakia) despite dropping the dosage steadily back to sub-therapeutic levels. I finally went to a true MPN Specialist who saw that the ET had actually progressed to PV at least 6 years ago. He was very clear that HU was not an appropriate med for me based on my profile. He also recommended that I discontinue the aspirin as I tended to bleed/bruise to much and have never had any thrombosis over the 30+ years with a MPN. He rather prophetically stated “you really do not want to get a brain bleed.” Two weeks later I was diagnosed with a hemorrhagic brain tumor. That was April 2019. I was really glad that I followed his recommendation and got off the aspirin. Aspirin is not a good thing when you have bleeding in the brain. His recommendation for me was phlebotomy-only, which is the treatment regimen I am following now.
For those who have not heard this part of the story before, the tumor turned out to be a pilocytic astrocytoma (Grade 1 tumor). This tumor is related to another genetic condition I have – Neurofibromatosis Type 1. June 2019 – another surgery – this time a craniotomy-resection. The most recent update on this as of November 2019 is that the surgery may have been curative. The tumor was negative for all 27 cancer genes tested for. The edema from the tumor and sequalae from the surgery have abated and I have recovered quite well. Due to the NF1, there is an increased risk of tumor recurrence, but we will be watching and monitoring with MRIs every three months.
Back to the PV – care. A few weeks prior to the brain surgery, the new hematologist who sees me locally called to say “we have been over-phlebotomizing you.” I was so iron deficient that they could not even measure my ferritin levels. Took the iron pills for a few weeks prior to the surgery but discontinued afterwards as the iron pills were worse than the anemia. So since June 2019 I have been monitor-only for the PV. The iron deficiency resulted in erythropenia, where normally I have erythrocytosis. The thrombocytosis continues as usual, cycling between the 500s and 700s. I am totally asymptomatic for all of the primary PV risks – no thrombosis, hemorrhage, splenomegaly, etc. The erythrocytes are gradually returning to “normal” and I will resume phlebotomy when my HGB=14 (HCT approaches 45%). We will be much more careful with the phlebotomies going forward, seeking a better balance between HCT and anemia.
I recently started seeing an Integrative Medicine specialist, who is working with me to see if we can better manage the systemic inflammation that results from the JAK2 mutation. We will be trying some different things to see if there is a way to deal better with the increased load of inflammatory cytokines and decrease their negative impact. If I find something that really helps, I will let you all know.
As if this was not all interesting enough, back in December I was diagnosed with a rather large bone mass on my mandible. At this point we do not know what it is. The maxillofacial surgeon I saw referred me on as he could not deal with it on an outpatient basis. The bone tumor is likely something benign but it is doing some damage due to its location.
So, 2020 will be another interesting year of new medical learning. I am truly fortunate in that I will be going back to Johns Hopkins for this care. The MPN specialist I consult with, the neurosurgeon who did the brain surgery, and the NF1 specialist/neurologist who follows me are all based out of Johns Hopkins. When managing multiple conditions, it really is helpful to have a comprehensive team provide your care. Not looking forward to dealing with this, but it will be what it will be, and I will deal with it as I must.
My newest medical challenge will be just one more thing to overcome. I will be working to better understand the implications of having two kinase-based genetic disorders. NF1 is a RASopathy (RAS-MAPK pathway). The JAK2+PV (JAK-STAT pathway) is a separate kinase system, but the two pathways have crosstalk and impact each other. The interactions are quite complex and none of the docs I consult with really understand how it all works and what the implications are (no one does). I can’t help but to think that this has something to do with the issues I have been dealing with. However, the data base of people with my profile is vanishingly small. Rate of occurrence for all MPNs = 1/300,000 – NF1 = 1/3,000 – adult pilocytic astrocytoma = 1/60,000. Put that all together – there is only a small handful of similar cases. I love being unique, but no so much in this way!
All in all, I consider myself blessed. I have wonderful support from family, friends, faith community, medical professionals, and the members of this MPN forum. I have learned a lot in the last couple of years about the importance of creating a support network for yourself, educating yourself, and taking a strong assertive stance in managing your own medical care. 2020 looks to be an interesting year. I would have preferred something more boring – but oh well, so it goes.
Thanks to all who took the time to read this rather length tale. Thanks to all of my MPN brothers and sisters for your support throughout all of this. We are truly stronger together. All the best to all of you all.
Written by
hunter5582
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And have you had a Myeloid Panel check for additional mutations?
Thoughts on anti inflammatory diet? Early evidence suggests it can reduce symptom burden and possibly impact disease progression. Makes sense since MPNs inflammatory?
Would you, at some future point, consider SCT as an option?
No to monitoring LDH and CRP. Perhaps a thought for the future. Will discuss with Integrative med doc.
I have asked about checking for the additional mutations. the docs agree there is no value in that as the PV is relatively indolent and additional mutations are unlikely. I am keeping it on the back burner though and may pursue it at a later point. The brain tumor tissue was check for mutations (NGS looking for 27 cancer genes). Some of these tumor related genes also have a role in MPN s - really all neoplasms. The tissue was negative for all mutations. I am of course positive for the NF1 mutation. There are some studies indicating increased risk when the NF1 gene is present in addition to the JAK2 mutation. The specific NF1 mutation I have is, fortunately, associated with a typically milder neurofibromatosis symptom burden.
I have considered an anti-inflammatory diet, in fact mostly follow one. What has been the most successful is actually a Turmeric-Curcumin blend that contains the bioactive curcumin formulation CurcuWin. It works better than any NSAID I ever took. I have not taken any NSAIDS since I got started on the supplement. The Integrative Med doc started me on a fish oil derivative called SPM Active. It concentrates the anti-inflammatory agents in fish oil. We will see if it helps any. The doc is also considering Low-dose Naltrexone. Still considering that one. The doc is researching ways to specifically lower the levels of inflammatory cytokines. I like this doc as she believes that JAK2 related systemic inflammation is a real issue that drives a host of the issues I deal with. This doc is very knowledgeable about both traditional Western medicine and complimentary health approaches, so she is a great addition to my care team.
Stem Cell Transplant is not on the table at this point. Perhaps if symptoms progress it is something we will consider.
Thanks for your response and questions. All the best to you.
What is the brand name for your CurcuWin supplement?
Re myeloid panel, I’m PV and had the 31 Panel test. Have TET2 (about 10% of PV are TET2).
My game plan (to avoid too many BMBs) is to monitor my LHD, % JAK2/TET2 and spleen size plus occasional myeloid panel tests. If all stable, then I’ll assume progression on hold. The risk of additional mutations increases the more you have. I agree that borderline worthwhile during PV phase but important during the MF phase if timing SCT.
Do you have significant symptom burden? I’m trying to determine if low symptom burden correlates with low inflammation. And if intolerance to Interferons correlates with high inflammation. eg the success of Ruxo/Pegasys trials
I am using the Spring Valley brand Turmeric Curcumin complex with CurcuWin 550mg. Actually get it at my local WalMart. Seems to work well. The 500mg of CurcuWin is actually a higher dose than the more expensive brand the Integrative Med doc had.
My PV symptom load is low. Thrombocytosis + erythrocytosis. Platelets typically range between 500s and 700s. Erythrocytosis relatively mild. Very mild splenomegaly. No hx of thrombosis despite 30+ years of MPN (used to be diagnosed ET). My von Willebrand factors tend to drop below norm when platelet levels go up. INRs were too long when on aspirin, but in the normal range for now. I did tend to bruise and bleed too much when on aspirin. I was in Stage 1 hypertension (135/95), but phlebotomies decreased viscosity so BP has returned to normal (115/75).
Have had more trouble with secondary inflammation related problems: osteoarthritis, planters fasciitis, eczema, insomnia. Also possibly related are intermittent hypercalcemia, occasional random 24-hour spikes in resting BP, and intermittent elevation in glucose levels (low 100s - prediabetes). I think that the research regarding the impact the JAK2 mutation has on inflammatory cytokine load has validity and may help explain the secondary symptoms many of us experience. The JAK-STAT pathway has significant impact on how the body functions and does more than drive hematopoiesis. I am going to pursue options to better control systemic inflammation / reduce cytokine load. We will see if it works.
I’m really sorry to hear of your ongoing medical issues. You have been through so much and yet, you seem to take it all in your stride. Through all this you continue to be such a supportive and knowledgeable contributor on this forum. Thanks!
Hello hunter - I wanted to say many thanks for all the research you do to find ways in which our lifestyles impact on our MPNs.
I am so sorry that your 2020 is going to be another round of possibly invasive treatments when you have had so much to handle already - it sounds as though you have a super medical team, and I wish you all the very best going forward. Anne-Marie. xx
It is amazing how you take all your experiences and knowledge and pay it forward as you do. Keep fighting the good fight and I thank you personally for being who you are. I am in awe. Greetings from snowy Canada.
I often find I benefit as much by putting my thoughts together and sharing as I hope others do. Will keep fighting the good fight with support from the many wonderful people in my life. Greetings returned from chilly - but not snowy West Virginia.
Thanks Jacquie. I am grateful for the support I get from this forum and the change to share thoughts and resources. All of our MPN brothers and sisters are a real source of strength.
Thanks for a really interesting and informative post. You’ve faced something of a medical onslaught by the sounds of it! I really hope you can find some moments of respite in 2020.
The critical thing is you have an excellent medical team offering a joined up and holistic approach. That together with solid support from family and community networks I’m sure makes a huge difference to outlook and outcomes.
I was particularly interested to read your comments on cytokines and inflammation. I know clinicians and academics are keen to understand more about the relationship of these on MPN symptom burden. It will be interesting to see what the latest research reveals.
Meantime I wish you the very best and thank you for your informed and insightful contributions to this forum.
If nothing else, 2020 will be another year of learning more about how all of this works to create the issues we face. I can't help but to think that the two kinase-based disorders do play a role in al least some of what I deal with. I hope we will all find out more about this and how to manage it better in the coming years.
Thank you so much for sharing your health update! So thankful you have Doctors that you are working with and trust. Please keep us posted on your mandible bone mass and continue to stay strong/positive on your path to healing. You’ve got this!💛
Thanks. I learned the hard way about docs you can't trust. I fired them! The docs I have now are terrific. My new mantra is that "Assertive patients receive higher quality care. Passive patients do not." Any doc that has a problem with that is off the team ASAP. Finding docs who really listen to you, answer questions, and actually spend time with you rather than rushing to their next patient - that is golden. It is what we all deserve.
I completely agree! So glad you have found good Doctors. I will no longer sit back passively while a Doctor drives my health. I’m on a journey now to find quality Doctor(s) that I can trust with my life. I have an excellent MPN Doctor, but still need to find a Doctor like I like for my hashimotos/thyroid disease. I have been reading so much since my MPN diagnosis and I can’t help but wonder if MPNs could actually be another autoimmune disease with its inflammation connection and they just haven’t connected the dots yet.
As you say, you are somewhat unique are you not? Wow! You do have it all going on indeed...
Interesting what you have to say about aspirin too... As I too have have had bleeding episodes over the course of my MPN journey too... Especially around my throat & gums, and an early taste of Portal vein hypertension causing me to wake with blood in my nose and throat that would previously interfere with my respiratory system...
My mutation is CALR+ Type 2, and much of the literature I have consumed suggests that I should not be taking aspirin at all... However, there is a fine line to walk here too, as my Platelets are always so high, (800s-1M). Presently, I am still trialling the MTX drug with some success on platelet reduction. However, I am beginning to again feel like it is no longer having the desired effect. I will know more after next weeks bloods of course... (?) Still taking 25mg b/d of Jakafi as well...
Not sure what my next option might be if my platelets are again on the march... ? Two (2) previous TIAs was enough for me.... and I am now wondering if these headaches I seem to be having are related to the aspirin? Whenever I give the aspirin a short hiatus, I almost instantly feel a loss of circulation in my extremities... It is a fine balancing / juggling act at best, in my view...
In any event, please do keep us all informed as to your ongoing developments Hunter, and stay happy & well...
Agree that it is all a balancing act. The recommendation that everyone with ET/PV take aspirin is likely not what is best for all. Aside from my experience with excessive bleeding/bruising while on aspirin (and the hemorrhagic brain tumor), I seem have a pattern where my von Willebrand factors drop below norm when my platelets get upwards of the high 700s. There is some research out there that indicates that Acquired von Willebrand Disease in much more common than previously thought and that it occurs with platelet levels in the 800s. While I do not appear positive for Acq.vWBD, it is an ongoing caution.
I have also had some issues with blood viscosity (blood pressure, maybe paresthesia). That all went away once the erythrocytes were under control. Aspirin never made any difference in that for me. Sounds like it is different for you. I plan to stay aspirin free for now, but I am taking some anti-inflammatory supplements that are also blood thinning. Maybe this will give me some protection, but not too much blood thinning.
I have followed your posts here and on MPN-Mate, so I know you stay on top of the emerging trends. Thanks for all the information you have been sharing.
Wow Hunter .What a journey you've been on and what an attitude you have . Not a trace of self pity but just a wish to pass on what you've discovered .Well done my man 🙏 I'm interested in the supplements you take for anti inflammatory. I wonder if anyone knows if they're safe to take with hydroxcycarbomide / perindoprel / clopidogrel / atorvastatin ? And if they're available in the UK.. ?Thanks for all the info Hunter and wishing you a healthy , happy and peaceful 2020 . All the best , CA 🙏
Thanks for the kind words. I make a conscious effort to maintain a positive attitude and empower myself to be actively involved in my own health and healing. I learned the hard way what happens when you don't do this.
The supplements I an currently taking include Spring Valley brand Turmeric Curcumin with CurcuWin (500mg of a bioavailable form of curcumin). The CurcuWin formulation is one recommended by the Integrative medicine doc I see. This one supplement has worked better than any NSAID I ever took (high dose Ibuprofen, meloxicam). I have not taken/needed a NSAID since I started with this. It has made a big difference in managing the osteoarthritis and fasciitis. The doc just started me on SPM Active, which is a fish oil derivative that concentrates the anti-inflammatory agents. We will see if this does any good.
Regarding the interactions with traditional Western meds like those you listed, the answer is yes to potential interactions. It seems that just about anything that has anti-inflammatory qualities can also be blood thinning. This means they can potentiate any blood thinners you take. (Note: Aspirin + fish oil is contraindicated). I have found that most Western-trained docs do not have a strong knowledge base regarding many supplements and other complementary health approaches. That is why I sought consultation with an Integrative Medicine (aka Functional Medicine) specialist. The doc I see has a solid foundation in traditional Western medicine, but also expertise in the effects of nutrition, supplements, and other complimentary health approaches. These docs also have an approach that looks at the patient as a whole person with an integrated set of needs.
I am finding that while there are many benefits traditional Western medicine, it does not have all of the answers we need. There are some very real benefits to some complimentary health approaches, some of which are making a big difference in my life. In addition to the judicious use of supplements, I have been practicing Qigong (specifically Nei Gong) which has also been a big help in my health and healing. Add to that practicing good nutrition, exercise, and avoiding contaminants and toxins that pervade our food system and environment.
Thanks again. All the best to you for 2020 and beyond.
Thanks for that Hunter . Agree wholeheartedly. Have taken up tai chi n qi gong 🌟also practice meditation and mindfulness. All helps. Keep on keeping on 🌟😊🙏
Thanks for sharing your story. So sorry to hear about your most recent bone mass going into 2020. You’ve already been through so much. It sounds like you have a great team at John Hopkins.
Sending positive thoughts your way for a happier and healthier New Year.
Thanks for the positive thoughts. I count myself blessed to have such a terrific care team at Johns Hopkins. So many people have trouble finding a provider with MPN expertise. To find a group of skilled and caring professionals across the multiple disciplines I need has been a tremendous asset that I do not take for granted.
All the best to you as well. Hope you have a healthy and happy 2020.
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