Just saw this one. While back from 2016 so a little dated, this is a nice explanation of actual risks in ET. (e.g. Microvascular events can correlate with platelet levels but thrombotic events do not.) Very interesting and an easy to understand explanation of some of the basic science regarding ET and risk.
ET Myths and Actual Risks: Just saw this one... - MPN Voice
ET Myths and Actual Risks
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hunter5582
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Thanks for this.
I have actually seen it before, but it’s good to revisit. I picked up something there too which probably wouldn’t have made much sense the first time I viewed it. Some women who have ET with the Jak2 V617f mutation only have the mutation in their platelets!!
Gobsmacked by that.
Scrap that. It’s V617f negative🤷🏽♀️
About 50% of folks with ET have the JAK2 mutation. ET also results from CALR and MPL. Likely some yet to be discovered mutations too. The interesting thing is how the "same" mutation can result in different disease presentations.
Dr. Spivak was my MPN consultant before he semi-retired. His perspective is not shared by all, but is well thought out. I agree with the notion that we have to carefully evaluate the need for treatment interventions and base decisions on an objective risk-benefit analysis. Lab numbers that do not correlate with actual risks are not a good way to make decisions. We really need to look at our actual symptoms and risks to make good decisions.
Hey Hunter... 
Interesting presentation diffused by the presenter's sense of levity...
Might have to listen through this a couple more times though because there were many assertions I am not totally sure I agree with, specifically concerning higher Platelet counts & thrombosis events for example.
Doesn't seem to hold much faith in HU either, and that is no surprise I guess... 
Thanks & best wishes buddy...
Steve
Dr. Spivak has views that are not universally accepted, but are based in a sound understanding of the science of MPNs. The notion that levels of thrombocytosis does not correlate with risk of thrombosis is well supported in the underlying science. Note the difference between true thrombotic events and microvascular events, which he addresses in the presentation. Most of the risk is not in the numbers of platelets, but in how the blood cells behave. Dr. Spivak's core approach to treating ET and PV is to treat the actually symptoms and risks associated with the patient's presentation of the MPN - not just to "sanitize" blood cell numbers when there is not any evidence to support that this is an effective treatment strategy, This is not to say he does not use medications to treat ET or PV, rather that he tailors the treatment to match the needs of the patient based on their presentation of the MPN.
You are correct to think Dr. Spivak would not likely include HU in his treatment strategies as he believes PEG-IFN, ruxolitinib and other options have a better risk/benefit profile for the patient when medication is needed. I expect that Silver, Kiladjian, Hasselbalch and others are in agreement that there are now options that are preferable to HU. My own negative experience with HU certainly influences my opinion on it, but I think the emerging options really are far better based on the evidence as well. I am especially excited by both PTG-300 and Besremi, which certainly appear to be much better choices for PV treatment.
I hope that the different views on MPNs will continue to be available to us so we can better understand out options and the underlying science. While it would be nice if there were some absolute certainties, the science is not there yet.
All the best my friend.
I agree buddy...
The jury is still out in many ways...
Steve 
We may end up with a hung jury on some issues!
I hope for more clear answers for all of us with MPNs. Until then, we need to watch the debates and disagreements and draw our own conclusions.
The other Steve - not the tanned Aussie guy!
Very true my friend...
It has simply become vividly apparent to myself that there is not a "one size fits all..." explanation...
For example Steve, I have just had another type of stroke over the Xmas break. Naturally, both my doctors were unavailable, and I am also caring for my 85yo immobile mum who had just returned from a couple of weeks in hospital, and required close monitoring etc...
Hence, my testing regime was as a negative consequence of Post ipso facto...
This time around, & unlike my two (2) previous TIAs, there was a residual physical effect of numbness & spasticity more specifically to my left leg & upper arm, and like my TIAs, my platelets were around the 1M days afterwards upon first test. I couldn't walk w/out a stick for the first week, and am only really now beginning to mend again w/ some physio helping a little...
Hence, on every occasion in my MPN journey thus far, high Platelet regimes have always been present. Others might wish to call such an occurrence a coincidence, however, personally I am a great believer in 'cause & effect'...
There is much to learn my friend...
Steve
Sorry to hear about this Steve. I hope the effects are reducing now -if only you could pinpoint the cause -not heat and dehydration this time.
Oh, Steve. So sorry to hear this. And on top of that, your mom needs you. I understand that because my mom is 87, and wheelchair-bound. She does have an aide, but me and my sister are very involved in making sure she gets the care she needs. You must have seen the doctor by now. Why do you think your platelets were so high? How are you feeling now? Please take care of yourself. Thinking of you,
Cindy
Hey Cindy...
Thanks, yes I couldn't leave her at that point, although I did come very close to calling an ambulance for myself. Still going through testing and seeking answers. However, they are much harder to discover so long after the event window, I am told...
As Hunter speculates, it can be a blood factor relating to my Von Willebrands Syndrome, however, I doubt that there will be much in that test either because I waited so long... we shall see I guess...
Steve
ox
Very sorry to hear of your recent problems. Wishing you a full and speedy recovery. We need you back on that bike and riding, riding, riding. Get well quickly.
Hey Meatloaf...
Thanks, & I am working on it... Slowly rebuilding my weakened left leg, and restarting my training program for my upcoming FR Ride from Sydney to Coffs Harbour, which will commence from April 10, 2021
Hopefully, all augurs well...
Best wishes
Steve
Ditto, my platelets were way above the 1000 mark. Above a Million( Aus/ us) when I had my 2 strokes. Tina.🤗
You have, as usual, accurately outlined key issues in dealing with MPNs. 👍👍
1. We are each different in how our MPNs present. We each need a treatment protocol that is tailored to how our MPN presents. The "Standard Protocol" does not apply to all people with MPNs because they are NOT monolithic diseases.
2. We each need to track how our bodies respond and become aware of what our individual risk factors are, how our bodies respond to treatment options, and do what we can to manage the MPN variant we each have.
3. We need to understand some basic science to manage our MPNs effectively. One element of this is to understand the difference between correlation and causation. Two factors can vary simultaneously (correlation) due to the action of another factor (causation). Correlation/causation get confused a lot, even by researchers (and doctors) who should know better.
4. Platelets in and of themselves do not stick together. Thrombosis requires that something trigger the coagulation cascade, which is not driven by the number of platelets. My doc explained it to me this way – platelets are like bricks, they do not stick together no matter how many there are. Building a wall (clot) requires mortar (coagulation factors).
5. We know that some people tolerate platelet levels over a million and do not experience symptoms of thrombosis or hemorrhage. Other people experience significant issues at levels considerably lower. If platelet levels were a direct causal factor for thrombosis, then this pattern would not be evident.
6. Hypothesis – something else is going on in regard to thrombocytosis and the symptoms we experience. There is some good evidence that this is so. It is more about how the blood cells behave than about the absolute number of platelets. It appears that our blood cells can be “extra sticky” – this some of the symptoms we experience.
longdom.org/proceedings/sti...
ashpublications.org/blood/a...
I would expect that other factors play a role too. The increased production of inflammatory cytokines driven by deregulation of the JAK-STAT pathway also plays a significant role in disease burden. There is some emerging thinking that inflammation plays a significant role in disease progression as well. Yet another factor for us to stay aware of. I would also expect there are yet to be discovered factors that impact how our MPNs present.
To conclude – I absolutely agree that platelet levels will correlate with MPN symptom presentation for some of us. I also believe that controlling for other factors that are the causative factor driving symptoms will often reduce platelet levels. The distinction is important since it influences how we think about the goals of treatment and how best to achieve them. I favor an approach that is to reduce symptoms, improve quality of life, and prevent disease progression. That is a different goal than to simply “sanitize” blood cell numbers.
Thanks Steve. Your comment prompted me to think this all the way through. That really is one of the things I love about our discussions on this forum. Now I want to look closely at the distinction Dr. Spivak made about the difference between thrombosis and microvascular events. Another opportunity to learn and get a better understanding of MPNs!!
Thanks Steve, agreed...
It will be interesting to see if my Von Willebrands Sydnrome shows any thing out of the ordinary, (so long now after the event really...)
I'll keep you Posted buddy...
Steve
Perhaps the known inflammation from the disease, rather than platelet counts (under a million) is more causal that the count, for ET at least. Also, clearly the type of mutation, plays a role in clot risk.
Also, I think the damage that stress can do, especially on the cardisovascular system is not fully understood or quantified.
I do believe what we experience is a complex array if interacting factors. Inflammation is most certainly a major part of what we experience in terms of symptoms a very likely in terms of disease progression. How the blood cells behave (quality) is just as important as how many of them there are (quantity). Which is not to say quantality does not matter, just that there is so much more to consider.
Stress absolutely plays a major role. Stress contributes to inflammation and vice versa. The body is an integrated system not just a collection of independent parts.
Very sorry to hear that this event was worse than the previous two. That is really concerning. I hope you have been able to connect with a good neurologist to follow you now. It takes an integrated team to manage the things we face with MPNs. I learned that lesson in spades with the PV progression, brain tumor/surgery and aftermath. I am very fortunate to have a fabulous care team at Johns Hopkins and in my local community. I hope you have the same opportunity to create an integrated care team.
I appreciate the issues with managing your own health while caring for an elderly parent. Over the Holidays, in the middle of the surging pandemic, we had to get my 85 year-old Mother and her husband placed in Assisted Living. Between the two of them, we are dealing with Dementia (Alzheimer's and vascular), progressive peripheral neuropathy, and a host of other age-related conditions. Due to where they were living and their decline they have not been receiving needed medical care for several years now. The situation was quite grim. It is looking better now.
I hope your Mum is doing better now. Also hope you are recovering and doing better too. You and MPN-Mate are terrific resources and a vitally needed voice of reason and knowledge in the MPN world.
All the best my friend.
Thanks Steve...
Mum's on the mend for the moment although she still requires close monitoring because of her very stubborn nature that one must contend with at times...
Hope you & your parents are happier now with their recent move to assisted care etc.
I am looking into who else I can add to my medical team... There are such a myriad of ways that having an MPN can bring one down, fascinating really!
Best wishes buddy
Steve
Boy could I ever tell you some stories about "Stubborn Mums" 😲!
We are hopeful that the Assisted Living facility will work out for them. They are at least finally getting the medical care they need to live more comfortably. We are very hopeful that they will be able to have a more vital social life as well as they were totally isolated where they were.
You know my mantra about "Assertive patients..." That certainly applies to creating your care team. Having a good neurologist is vital for both of us, though for different reasons. My Neurologist is also the director of the Neurofibromatosis Clinic at Johns Hopkins and one of the world-class NF researchers/experts. She is also a fabulous doctor, both a a scientist and a healer.
One silver lining to this whole pandemic is how it has opened up the options for telemedicine. Tele-vid appointments are so readily available now that distance is not nearly the problem it used to be. It really opens up options for people not near major hospital centers.
All the best my friend.
Yes, balancing their needs of wanting to maintain some level of independence against requiring assistance can be a balancing act, and one that requires a heightened sense of empathy. It is also something we might all learn from too, as all of us continue to age, (w/out intervention of course...
Thanks Steve
Totally agree with ‘no one size fits all’ Steve.
Really sorry to hear that you’re struggling at the moment, bless you. I can totally relate to the care duties too.
On a positive note, you seem to have Covid controlled pretty well in Australia now I believe.
Sending good thoughts your way. Xx
Thanks Mary...
Yes, many years ago an Australian author Robert Hughes wrote about Australia's natural disadvantage referring to it as the "Tyranny of distance..."
Today, our natural geography coupled with that distance is largely responsible for our success in staving off CV–19, in my view...
Human nature is the same everywhere in my view...
Stay safe & well Mary...
Steve
Totally agree with the negativity surrounding HU.There are far better treatments such as peg Ifn. The only reason that they still push HU, in my opinion, is that it is cheap.
I am only on aspirin and phlebotomy even though my platelets registered 923 last month. I have a new hematologist who seems to listen well with the patient. However, he seems to be suggesting a treatment using hydroxyurea and not Jakafi or Ruxolitinib because the latter are not "tolerable" based on his experience. Any comments about the side effects of Jakafi or Ruxolitinib or their "tolerability"? Thank you.
Interestingly enough, I am on phlebotomy-only for PV tx and my platelet levels are up by about 200K. Used to cycle between 500s - 700s, but am now cycling 700s to 900s. We ruled out progression of the PV. It appears to be the result of the chronic iron deficiency caused by the phlebotomies. I am having other issues due to the iron deficiency so I am considering other options.
I am HU-intolerant. Have been on it. Experienced toxicity even at very low doses. While others have more favorable experiences with HU, I do not favor its risk/benefit profile compared to the other options (just my own opinion).
Jakafi (Ruxolitinib) has a reasonable risk/benefit profile for people with PV. It is especially effective for pruritis and other PV-related symptoms. I would definitely consider it, but favor one of two other options. I hope others who have actually been on Jakafi will weigh in on this.
Besremi (ropegylated interferon) has one of the better risk/benefit profiles for PV in my opinion. Basing that on reading the research and hearing from others on the forum about their experiences with PEG-IFN. The chance for hematologic and molecular remission is very exciting. if you can tolerate it (not everyone can) then it may offer the best chance for dealing with the symptoms and preventing disease progression. (Note: currently under FDA review in the USA - approval coming soon).
PTG-300 (hepcidin mimetic) - currently in clinical trials here in the USA. Another very exciting possibility. This med alters iron metabolism and allows your iron levels to be higher while controlling erythropoiesis. I am considering participating in the clinical trial for this because the research is so promising.
The bottom line is that ALL of the meds we use to treat PV have a risk/benefit profile. The decision should be based on what you are willing to risk/tolerate and what benefit you are making the priority in your own treatment. It is not about what your doctor can tolerate - it is about what you can tolerate - and what you prefer.
I hope some others with experience with Jakafi will weigh in and give you feedback based on their own experience.
Just watched this in its entirety and sure to watch again later although I have to admit i'm not entirely convinced by their being no correlation of platelets numbers and thrombotic events. The 2 strokes I suffered, the first being small and no long term effects but subsequently a more significant stroke,on both occasions my platelets were way over the 1000 mark while Hb, Hct and other counts in range. The juries out on Hydrea but in my view should never be given in a first line of treatment in someone with an Mpn who is low risk or most certainly not in younger patients when other treatment is available especially now more is known about Mpn's. Unfortunately at some hospitals, I believe cost can play a significant part in what treatment you recieve in the UK, thats view anyway. As you say a video were the basic science is easy to understand. Tina.🤗
Did you have any symptoms , however slight , before strokes ?
Hope you are ok at present . X
As I said no long term effects with the first but because of it, it finally led to my diagnosis after a lot of tests and Bmb. The second was more significant and like the first their was signs and symptoms, ie, visual impairment, coordination problems. It took a lot of physio at home when I came out of hospital to get movement back in my arm. It left me with epilepsy, numbness in some of my left hand fingers and some memory problems but very much alive and kicking. Thanks. Tina.🤗
You accurately used a key word - correlation as opposed to causation. The distinction matters. The issue lies in how the blood cells behave, not their absolute number. You may in fact be more prone to symptoms when thrombocytosis is higher due to the the underlying cause of the increased platelets as opposed to the number of platelets per se. That is some of what Dr. Spivak is getting at in the video.
BTW - he is just as entertaining and pithy in person. Dr. Spivak used to be my MPN consultant prior to his semi-retirement. I really do like him a lot. He is a great doc and a brilliant mind in the MPN world.
Yes, I do like the way Dr.Spivak presented it, and as you say entertaining, even if I didn't agree on some points. I certainly agree with a previous point you made in a previous reply that the Haematologists should put more emphasis on controlling symptoms and dealing with disease progression and wellbeing of patients instead of just being obsessed in 'sanitizing' the bloods. Haematologists are blinded with numbers and not the actual wellbeing of patients. You are so right in your assertion regarding assertive patients over passive patients getting better care. Take care Hunter. Tina.🤗
The whole thing about symptoms and actual risks being more important than sanitizing lab values is the whole point per Dr. Spivak. It certainly makes sense to me. He is not the only MPN specialist who looks at it this way. He does do some things differently than others, but is well reasoned. Case-in-point - he recommended against my being on aspirin since I was over age 60 due to the age-related increased risk of hemorrhage. He said "You really do not want to get a brain bleed." Two weeks later I was diagnosed with a hemorrhagic brain tumor that was surrounded by edema. Too late! Already bled in my brain. I also had a history of excessive bleeding/bruising for the last 10 years or so. Going off aspirin is the right choice for me. I would NOT advocate that it is the right choice for everyone. That is the whole point - Individualized treatment plans based on the individual's presentation of the MPN, risk tolerance, and patient preferences.
I really do prefer to see a range of opinions about the issues we face with MPNs. These are not monolithic disorders and there is still a lot to be learned. Understanding why the experts disagree is very informative and enhances out ability to make good decisions for ourselves.
All the best my friend.
Yes I agree. HU is always pushed at you first, reason being it's cheap, not because it's more effective.
Well HU does "work" but at a cost in terms of the toxicity/potential adverse effects. With the other options we now have available, what was once considered the "gold standard" for MPN treatment no longer has that status. It is a sad statement that financial cost overrides the human cost in what drives so many formularies.
What I find most disturbing is when providers do not review all of the options and their risks/benefits with patients. It should be up to patients to decide which risks/benefits they wish to assume. Doctors advise. Patients decide. That is what the ethical practice of medicine requires. It is really wonderful to hear about providers who practice medicine this way. They are out there and we are fortunate when we have them on our care team.
Maybe in the states , but from my experience in the UK they are sadly lacking.
• in reply tohunter5582
My latest Haematolgist wanted me to start HU, especially now I am 69!! But I have no symptoms of ET. Platelets came down from 970 to 645 last month. Only taking aspirin daily. So I said no I don't want to take it. She wasn't happy. Looking for a new Haematolgist. I am certainly interested in your link. I will listen to it again. Thankyou Hunter.
hunter5582• in reply to
It is ultimately your decision. There other options if you do need to initiate medication to treat your ET. You will certainly hear from others on the forum that they have benefited from HU and did not have problems with it. That is not the case for all of us and many of us do have concerns about the inherent risks of HU. What we all deserve is doctors who respect us and our right to make our own decisions.
I hope you find a MPN Specialist who will work with you and treat you the way you expect. Just in case you have not seen it, here is a list pf patient-recommended docs.
• in reply tohunter5582
Hunter i am hoping to move near Hamilton so I see there is a couple of MNP specialists there. Only 55mins away from where I could be living. Thanks so much for the list.
I’ve had ET for about 7 years now. Everything was stable till this year. They were around 700 then went up to the million. My hematologist had me on aspirin all these years. I work at a dermatology and vein clinic and was having mild pain in my leg. It was a superficial clot. My hematologist increased the aspirin to 325mg. I also had Covid this past year to make things worse. My cough and shortness of breath lingered months. When I went in and still had a cough and pain in my chest, he ordered a CT scan because he was concerned about PE. It was normal, thankfully. I haven’t had any major health issues other than asthma my life and this ET. He has started me on Hydrea and I’m 35. There aren’t other options at this point when my platelets keep increasing.
At this moment in time the options are very limited especially with Covid. I think most Consultants would be reluctant to change medications because of the extra monitering in the beginning to see if its suitable, something they don't want to do when the Hospitals are overrun with Covid cases. I had the same problem at the beginning when I was diagnosed when I was put on Hydrea, the more my Platelets went up the higher the Hydrea dose was raised. 12 years on when the drug Peg Interferon was raised as a possability as now gone by the book because of the Covid situation. Tina.🤗
I haven’t had any issues making appointments and seeing doctors for treatments. I specifically go to an oncology clinic for treatment not a hospital. I’m not familiar with the protocols and differences between US and UK. From my understanding most people here are based in UK? I heard there was a large MPN group here so thought to join converse and get more information.
Here in the UK, the majority of patients see an Haematologist at a hospital and because of Covid cases in the hospital it is impacting on other services with operations being cancelled and outpatients appointments being put back, the difference being many patients here are treated on the NHS which is still overwhelmed at the moment and will be for a very long time after with long waiting lists even when the Covid cases start falling. Tina.🤗
That’s very unfortunate. They should have all specialists in separate facilities. Having one facility obviously leads to increased contact with the virus. It makes no sense to group infectious patients with chronically ill patients who are immunocompromised or at that, healthy patients who need to be seen for acute injuries etc. This doesn’t contain the virus at all and increases the spread. I hope they will find a solution for this problem so that people can receive the proper care.
Sorry to hear you have such a rough go of it in the last year. Certainly do know what that is like, as many of us on the forum do. Since you are symptomatic with thrombosis and the platelet levels are going up, it makes sense that your doc would suggest initiating cytoreduction. Given your age of 35, it seems a bit surprising that your hematologist would not have discussed the other options for treating the PV. Pegasys (PEGylated interferon) and Jakafi (ruxolitinib) are also options for treating ET. There is also anagrelide as a second tier option. Do note that each one of these medications has a risk/benefit profile. None are without risk. Continuing to treat the ET with aspirin-only would also have risks.
Do note that all these meds are off-label for treating ET in the USA since they are not FDA approved for this purpose. They are certainly in common use for ET anyway. There may be an issue with the formulary from your health insurance. HU is much cheaper than PEG-IFN and RUX. In some health insurance programs doctors are essentially required to start with the cheapest med. My daughter (who also has ET) is in this situation. Her hematologist may start her on HU if needed, then switch her to PEG-IFN as soon as she experiences any adverse effects. She is also considering the clinical trial for Besremi.
Ultimately it is up to you to decide which of the treatment options you want to pursue. It is very important to understand what all of your options are then review the risk/benefit profile of each choice. It may also be helpful to get a second opinion from a MPN specialist. Most hematologists do not have the KSAs to provide optimal treatment for MPNs. Here is a list mpnforum.com/list-hem./ . It can be very helpful to consult with a specialist who sees MPN patients on a regular basis and has the experience to individualize treatment.
This is an older article (2012) by Dr. Claire Harrison. Note how she describes how to present treatment options for ET to the patient. There are more options now than in 2012.
mpnjournal.org/how-i-treat-...
This is an 2017 article on MPNs by Dr. Jerry Spivak. There is a lot of good information here, but it is heavy reading. If you look on page 2177 in the section on Treatment you will note a specific recommendation for ET patients under the age of 65 who need reduction in platelet levels.
legeforeningen.no/contentas...
NOTE: There is a newer form of IFN - Besremi (ropegylated interferon) that is soon to be FDA approved for PV. It is in clinical trials for ET. You may want to learn more about this option as well.
businesswire.com/news/home/...
Hope that helps. Do stay in touch and let us know how things go.
Thank you so much for the information. I will see if there is a doctor I can see that that specializes in MPN. From my understanding the physician I am currently seeing is also an oncologist so I’m not sure how knowledgeable he about MPN. I never thought there was even a specialist until now. I always thought it was a hematologist who was the one who treated it and since he’s also an oncologist, I thought I was in the right place. I’m unfamiliar with the details about the other medications. Sounds like one is more appropriate for someone positive for JAK2, which I was not surprisingly. I could be completely wrong. I’m learning a lot here. Years ago, back in 2014, my bone marrow biopsy showed that I was negative for JAK2 and still diagnosed it at ET but without an explanation other than my platelets proliferating so rapidly. I’m wondering if another bone marrow biopsy should be done. It was never suggested again and I’m wondering if it’s even necessary.
Completely off topic but my grandfather had leukemia when he became older. He actually died from heart attack before the leukemia. I sometimes wonder the genetic link and if ET was a precursor. I’ve know based on things I was reading that it can lead to acute leukemia, but the thing is, he was never diagnosed with ET. I have so many questions.
Many docs are board certified in Oncology/Hematology since they are the same board. However, some specialize more in blood disorders than others. MPNs are very rare disorders. Most hematologists rarely if ever see MPNs on their caseload. That is why it is so important to consult with a MPN Specialist. Do whatever it takes to find one. I learned the hard way about this. My old hematologist had me misdiagnosed as ET, when I actually had PV. When it became clear that he had missed the diagnosis to his credit he fired himself. He said "Your health is more important than my ego." I now see a local hematologist who specializes in blood disorders but who is not by his own definition a MPN expert. I am only his second PV patient (6th MPN patient). I also consult with a MPN Specialist at the Johns Hopkins MPN Center. This doc consults with me and my ongoing hematologist regarding my care plan. This arrangement works great. NOTE: ethical doctors will ALWAYS welcome input from a more knowledgeable colleague.
There are three driver mutations that can cause ET (JAK2, CALR, and MPL). Exclusion of JAK2 does not rule out the other two. There are also people who are ET - triple negative. This diagnosis is made when you meet criteria for ET in the absence of a driver mutation. Note - your Bone marrow biopsy would have looked at bone marrow morphology, including fibrosis and the appearance of megakaryocytes. This is part of how the diagnosis is made. This is the WHO dx criteria for MPNs
mpnconnect.com/pdf/who-diag... .
Your grandfather's leukemia is not off topic. Acute Myeloid Leukemia is the the ultimate end point for some people with MPNS. While most people with ET do not progress to AML, it is a risk. Progression to AML is usually through Myelofibrosis, which is what they would have been checking for when they did the BMB. You did not note which form of leukemia your grandfather had, but it is potentially relevant. This is definitely something to discuss with a MPN Specialist. NOTE: There is some additional genetic testing for non-driver mutations that can be done to help shed light on the level of risk of progression. The test is called a MPN Myeloid panel.
files.labcorp.com/labcorp-d...
Educated and assertive patients receive higher quality care. Passive patients do not. This would be another lesson I learned the hard way. Here are a few more links that may help you build a knowledge base.
Be sure to watch the MPN Molecular Biology
mpninfo.org/conferences/201...
Nice explanation by my former MPN Consultant. He is just as entertaining and pithy in person. He is one of the leading MPN experts, but not everyone agrees with all of his views.
youtube.com/watch?v=hbVr9u3...
I have done research on my physician and he in face specializes in MPN as well. He specializes in hematology/oncology relating to MPN as well as other areas of oncology and internal medicine. So I guess I am in the right place. Question is, why do I still feel so clueless about my disease. I feel like I lack so much in knowledge.
You do often have to educate yourself. Given the time constraints the docs have to work under, they rarely have time to spend with patients to provide them a vase of knowledge they need. That is why organizations like Voices of MPN are so important. There are excellent organization available to us to provide the information we need to make good decisions.
Speaking of which, there is a patient education webinar on ET coming up on March 11 presented by MPN Advocacy and Education International (another great organization). register.gotowebinar.com/re...
You still may want to get a second opinion. Depending on where you live and what health insurance you have, this may be very easy to do.
I am actually not sure what kind of leukemia he had just know he had it. He also had melanoma. I have to dig a bit to get some more information. Back in the day they blamed in on his exposure to radiation in ww2 but with my experience in all this, sound like this could be a genetic thing
Familial MPNs are known to exist. It is an expanding area of knowledge. I have the JAK2 mutation with PV. Used to be ET. I am 65 now and was diagnosed with ET in my 30s. My daughter (age 35) is also JAK2 positive and presents with ET. It looks like it may actually be PV. My son (age 38) is presenting with idiopathic erythrocytosis. He is JAK2 negative. My brother was recently treated for non-Hodgkin's B-Cell lymphoma. We are all also positive for Neurofibromatosis Type 1. Genetics matters! We are participating in a Familial MPN study.
Very interesting. I hope the studies done will help expand our knowledge of this. That’s a great thing you get to be a part of.
I’m very curious to know the impacts Covid had on this condition. My platelets were at a stable 700 then increased to over a million. Maybe it’s coincidental but I have been cough and short of breath so much for months. I even had a superficial clot in my leg. I work at a dermatology and vein clinic and had one of the RVTs scan my leg and it showed up in the ultrasound. I was having mild pain in my calf. I have been having throbbing pains in my knees and legs for years but it has become more severe. I’m just puzzled. I messaged my doctor from my portal to get a more detailed explanation since I won’t return for another month.
The question about how COVID will affect people with MPNs is one that many of us have. So far, no evidence has been presented that we necessarily react differently that I have seen; however, the database is just to small to know for sure. Given the impact COVID has on producing a cytokine storm, many in the MPN world have had concerns. The is no question that inflammation and infection can increase thombocytosis. Reactive thrombocytosis is a well known issue.
The risk of thrombosis and microvascular issues is one of the key components of managing ET. The odd thing is that the higher when your platelets get higher, you are also a risk for hemorrhage. There is a condition called Acquired von Willebrand Disease that is know to occur when you are upwards of 1 million. Some research indicates it can happen as low as 800K. You can also experience a decline in von Willebrand factors that does not meet criteria for the disease. Given your platelet levels, you hematologist should have already run a von Willebrand Panel along with checking your prothrombin times (ptt/aptt - INR).
Regarding the leg pains, there are various types of leg pains people with MPN report. Some are related to inflammation. Some to bone pain which is associated with MPNs. Paresthesia is also fairly common. You may also hear about people getting leg cramps, particularly at night. There are various reasons for this. In my case, it appears related to magnesium deficiency. (comes from the Nexium I have to take for GERD). Erythromelalgia is also associated with MPNs. You may be experiencing any of those. Or not. Perhaps it is unrelated. You know what you are experiencing the best,
Hope that helps answer your question
Very interesting. So many ifs and buts .
As with all conditions , but problems more so with MPNs not one size fits all..
One thing we can control is our mind .
More attention needs to be given to how we react to life’s events . And not make an already bad situation worse . !!!
Always good to chew things over with you and others ..
Thanks for posting. Very interesting
Excellent, thank you so much. They really need to customize treatment based on the mutation and factors like smoking, especially as it relates to thrombosis risk. As an ET patient with MPL, my risk for myelofibrosis is higher than a clot. And interestingly the newer anti-coagulants can raise and lower platelets, causing clots and hemorrhage!! I have seen two cardiologists who did not know this!!!
Being informed can save your life!
Being informed can absolutely save your life. So can being an assertive patient. So can involving a MPN Specialist in our care for these rare disorders. Many doctors, even hematologists, do not have the KSAs to provide optimal treatment. Finding a care team with MPN expertise who will actually listen to you, respect you and work with you collaboratively to create a care plan makes all the difference in the world. It can in fact save your life.
Yes, yes, yes having an MPN specialist is critical. It has made a huge difference for me.
• in reply toIndigo42916
You are lucky, New Zealand is real short of MPN specialists. I see by Hunters list, three in NZ way up north to where I live.
hunter5582• in reply to
It is worth doing whatever it takes to engage a MPN specialist on your care team. With tele-video medicine so widely expanded, this is easier than ever. The way I handle this is that I have a local hematologist who handles my on-going care. He is a wonderful doctor, but not by his own definition a MPN Specialist. My MPN specialist is about 2 hours ways. I see him approx. annually. He consults with me and my on-going hematologist about my MPN care. This arrangement works great!
Me too. The docs at the Johns Hopkins MPN Clinic are terrific. I started with Dr. Spivak. When he semi-retired, I was shifted to Dr. Braunstein, one of the docs he trained. It made a huge difference in my quality of care. My Neurofibromatosis care and neurosurgery team is also based out of Johns Hopkins. I am truly fortunate to have such a fantastic care team.
Hi! I'm new to this forum, and also first time I have been to site with real life experience. I see an Onco, supposed to be best in my county, but I realize also has only treated a minimum #'s of mpns.Interesting to read about others, as I am on Hydrea, and suffured 5th stroke in Nov. Apparently from the brain tumor, of chronic nature, a Raspberry or more known as a hemangioma, in R frontal.
I realize I need a MPN specialist, as well as a stroke specialist, as any Neuro in my area is unable to treat, or advise. I was told I have probably had ET for at least 15 yrs. Have not had normal CBC since 2004, was always told, dehydrated! My platelets were only in 5-600 range when any of my stroke occurred. I can drive short distances, but fam doesn't like, hard to get rides to monthly tests, much less to get to major institution, which Family Dr's now recommends. I was already a hermit, b4 Covid, so at least I am use to it.
Very hard to get my Dr's to work 2gether, have asked 5 times, 2 to Fam Drs, 3 as of today for med for ulcers in my throat that occurs every 6 weekso or so. Each says ask other Dr. I am my own advocate, been very hard. Was told to try Hopkins, but rather lengthy process to apply. And then I found this forum. Sorry if posted on wrong place! Ty
Read your background. It is similar to my own. I have a JAK2 + PV. Was diagnosed with ET about 30 years ago. "Progressed to PV" about 7 years ago. May have been masked PV all along. I also have Neurofibromatosis Type 1, which lead to a brain tumor (adult pilocytic astrocytoma) that required brain surgery last year. The year before, I had to have heart surgery for paroxysmal atrial tachycardia. There is more in my health history, but the key point here is that MPN-expert consultation is absolutely critical to care for everyone with a MPN, but especially when you have a complex case.
I saw your note about Johns Hopkins, which is where I see a MPN Specialist, A NF Specialist, and had my neurosurgery I have to travel 2 hours to get there. It is totally worth it! The care at Johns Hopkins is amazing. I cannot imagine a better pace to go, particularly when you have a complex case. I had not trouble at all accessing services there. You may have to wait a bit for a specialist appointment as MPN Specialists are pretty booked, but they have a world-class MPN clinic with multiple providers. Here is the link to get in touch with the MPN Center. hopkinsmedicine.org/hematol... .
Contact the medical concierge. They are very helpful and will get you connected as soon as possible. They are doing a lot of tele-video medicine currently.
The approach I use is that I have a local hematologist that handles my ongoing care who is a wonderful doctor, but not by his own definition a MPN expert. The MPN specialist consults with me and my care team about my care. This arrangement works great.
Also note that the neurology team at Johns Hopkins is also amazing. Having a neurologist and a MPN Specialist both in the same institution when you have a complex case makes a huge difference. It is not exaggeration to say that it could save your life. It will certainly improve your quality of care.
Do whatever it takes to get yourself to Johns Hopkins. It could be one of the best things you have ever done for yourself.
Thanks for posting this. I have found Dr. Spivak's work to be very insightful. He also was very kind and helpful when I emailed him with some questions even though I never was a patient of his.
Dr. Spivak really is a terrific doc. Gifted both in the science of medicine and the art of healing. He is also a genuinely nice person and quite engaging. I love his presentations. He can be just as pithy in person as he is in public, which I really enjoy.
For anyone close enough to Johns Hopkins to consider it, the MPN Center is a great place to get MPN care or at least find a consultant to work with your existing care team. I am not being seen by Dr. Braunstein, who is also a great doc. Very similar views as a MPN Specialist to Dr. Spivak.
Hi, I’m new here and just came across this post and was wondering if you still find it valid? I ask because I need to do so much research, I’m ET jac2+. Thanks, I have been appreciating your comments very much and I got practically nuthin’ from my haemo.
Yes I do find this view valid. Note: While Dr. Spivak is one of the leading experts in the MPN world, not everyone agrees with all of his views. There are a range of views regarding MPNs and how they should be treated. The answers are not black and white. MPNs are not monolithic disorders and one size does not fit all. It is very important to consider a range of opinions on the treatment of ET in order to have enough information to make a valid decision for yourself.
Here are a couple of more resources to see some additional viewpoints.mpninfo.org/conferences/202...
Included the 2019 version as the MPN Molecular Biology presentation is worth watching.
mpninfo.org/conferences/201...
Hope that helps
Thanks for the reply and links, h. I think its time I put myself out there so I’m going to post. If you see it, any thoughts of yours would be much appreciated!
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