So glad for this group. Have suffered with migraine since I was 8yo and have been treated for hypertension for over 13 years and I'm now 55. I have been diagnosed with JAK2-V617 and dNmt3A , just starting on the journey. Anyone have treatments that have been helpful? I think I'm waiting for a FISH result but my oncologist said it would take a bit. I don't see him until June. Thinking of getting a second opinion but need all results in. Open to any advice on how to decipher this process. Thanks, in advance!
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Getting a second opinion is always indicated for any serious medical condition. In the case of MPNs, it is very important that the second opinion be from a MPN Specialist. MPNs are rare disorders, and many doctors have very little experience with them. Here is a list of doctors with MPN expertise. mpnforum.com/tsr-the-list/
Headaches are a common symptom for people with MPNs; however, they can occur for other reasons. Since your migraines presumably predate your acquiring a MPN, the issue may be independent of the MPN. Migraine treatment has improved in recent years. There are more options now. I experience a different type of migraine due to the aftereffects of a brain surgery. I have had great success with a CGRP inhibitor, using Ubrelvy as a PRN when a migraine begins. The episodes are always gone within an hour. The CGRP inhibitors can be used as a PRN or on an ongoing basis for prophylaxis. If you wish to pursue this or any other option, suggest you consult a migraine/headache specialist who has this specific expertise.
You did not indicate whether the JAK2 mutation resulted in ET, PV, or MF. This does make a difference in your treatment. It is worth noting that if the JAK2 mutation is causing erythrocytosis (PV), this can cause hypertension. The hyperviscosity increases blood pressure. I was in Stage 1 hypertension until the erythrocytosis was under control. My BP returned to normal once my HCT < 45%,
Hope you get a clear diagnosis soon and treatment plan in place for all of the issues you are experiencing.
Thank you for the handy list of MPN specialists and I was able to locate someone in my area using it. I have an appointment next week on 5/15 with a specialist. That was very helpful as I navigate this care and my new life. My platelets are about 700 and JAK2 v617F positive with ET. Not sure what they will suggest and what I should be ready for? My headaches are regular as well as exhaustion. Although I'm getting over something viral. Do you find that when you get sick....that you are sicker than you used to be? I feel as though my immune system is more sensitive. Just trying to figure things out and adjust my expectations as needed. Are there typical questions that I should be ready to ask? My sister and husband will be there for moral support but I would like to be prepared. I will have some of these questions written down. Any other thoughts from your experience would be helpful!! Thank you!!
You are asking some really great questions with some fairly complicated answers. I am glad you found a MPN specialist who can give you the best case-specific insight.
Your immune system should not be compromised by ET. That may be seen with other MPNs, however. Immune compromise may also result from cytoreductive treatment.
Part of what you are experiencing may be the result of the systemic inflammation we experience with the JAK2 mutation. The JAK2 mutation does more than produce too many blood cells. It also causes the overproduction of inflammatory cytokines. This systemic inflammation is thought to be responsible for many of the secondary symptoms we experience. My own subjective experience is that this can exacerbate the symptoms of other inflammatory issues such as osteoarthritis, eczema, and GERD. Note that inflammation is normal part of the body's response to infections or injury. It is the overproduction of inflammatory agents that is the problem.
There is also an inverse issue. We can experience reactive thrombocytosis in response to inflammation, injury, bleeding, illness, iron deficiency and other causes. While this is normal bodily function, in the context of a MPN with thrombocytosis it can have a more significant impact.
As you already know, heachaches are a common MPN symptom. Typically, this is a microvascular issue. There are different treatment intervention that may work. We are all different in how we respond. Some find that bumping the low-dose aspirin up to twice per day works. Others find that they do need cytoreductive medications. Others find relief from other agents such as CGRP inhibitors. The latter has worked quite well for me though my migraines have nothing to do with the MPN, rather to a brain tumor surgery.
The other symptom you are experiencing, itching and fatigue are also very common MPN symptoms. Fortunately, there are interventions that can help with both of these issues. This is something to discuss with the MPN Specialist. there are also excellent resources at MPN Voice and other sources for this.
It is common to find hypertension in people with PV due to the hyperviscosity of the blood. Reducing the erythrocytosis can also reduce the BP. I went from Stage 1 hypertension to normal range when I controlled the erythrocytosis.
Regarding what questions to ask, here a a couple of resources you can consult.
What is my MPN diagnosis? Explain what supports this diagnosis.
What is my prognosis? What role does the JAK2 variant allele frequency play? What role does the dNmt3A mutation play? What role does the hypertension play in my risk profile?
What are the best interventions to prevent primary risk issues - thrombosis, hemorrhage, and microvascular symptoms? What are the best interventions to treat secondary/constitutional symptoms - fatigue, headache, itching?
What are ALL of my treatment options? Which do you recommend and why? What are the risks and benefits of each of my treatment options?
How will we monitor my response to treatment and potential for progression of the MPN?
I go to every appointment with a written agenda. This is the format.
Treatment goals:
Define for your doctor what your priorities are. Can be very specific at times.
Treatment Approach
Define what your approach to treatment will be. Make this specific to your treatment preferences. ALWAYs tell your providers about all the interventions you use.
Updates/Issues/Questions
Write out the symptoms and issues you are having and a list of your questions. Always include a holistic review of what is going on, particularly with specialists. Only give updates/changes to an existing provider. Do focus on what is relevant for this appointment.
Treatment
Review all treatment options – let the doc know what you are doing now and seek input about what to do next. Ask about ALL options, not just the one the doc recommends. Ask the doctor why the recommended option is better than other choices. Ensure that it is your goals and preferences that drive the decision about your treatment. This includes your risk tolerance and what risks you prefer to take to achieve your treatment goals.
I can't thank you enough for this information! This is super helpful as I prepare for my meeting on Wednesday. I've been going through everything, the reading and about the bloodwork/covid etc. and definitely getting a better understanding. Its a lot of information! Still digesting while prepping for the MPN specialist appointment. I will let you know what I find out! I'm so grateful for this group! I feel as though I'm learning so much, it gives me more control of the situation. Well, at least in my eyes and navigating how to get to the next steps. With my eyes wide open. I don't feel as overwhelmed and frightened. Its empowering.
Hello and welcome! How are you doing with all this? I remember when I got diagnosed, it was a lot to take in.
It sounds like you tested positive for the JAK2 driver mutation and a secondary mutation. There are different MPNs caused by JAK2, such as Essential Thrombocythemia (ET), Polycythemia Vera (PV), and Myleofibrosis (MF). Have you received a diagnosis yet?
You mentioned you also have a secondary mutation (dNmt3A). I also have one (BCORL1) too. It's really common for MPN patients to have secondary mutations. Certain secondary mutations, like ASXL1, carry a higher risk of progression. However most secondary mutations are of "unknown clinical significance" aka it's unclear how much they impact the disease (if at all). We just had someone else asking about that mutation and people shared a lot of good info, so I'm going to link that post: healthunlocked.com/mpnvoice...
Different MPNs can have different treatments, so it's hard to talk about treatments without knowing either the disease or the symptoms you're experiencing. There are better treatments available than they're used to be.
If you can, it would be best to get connected with an MPN specialist. These are rare diseases and the average hematologist isn't going to know the most recent research and treatments. Depending on what country you live in, we might be able to help you find a list of specialists near you.
Thank you, Brightlys! I appreciate your input and will research a bit more. I will admit it is "a lot to digest". High BP/Migraine/exhaustion and itchiness are my symptoms. I have a history of pretty bad Diverticulitis but now I'm even more nervous about a flare up for fear of bleeding. My hematologist/Oncologist, is really on the ball but I worry about waiting until June to see him. I am in Massachusetts/US and I'm thinking of reaching out to Dana Farber for a second opinion. Any information/recommendation is helpful! I'm so grateful!
I can see that Hunter has already shared the MPN specialist list. Definitely a good idea to get connected with a specialist if you can.
Itchiness is common among all the MPNs. I get migraines from ET (Essential Thrombocythemia) but I only started to have them once I developed ET and my platelets started to rise. My guess is if you've had migraines since childhood that is may not be related (though if you have high blood counts I could see how that my aggravate your existing migraines).
It is a lot to digest! Take your time and remember it's a marathon, not a sprint. I'm sorry you're in the MPN club, but the good news is you're not alone. There's a lot of knowledgeable people in these forums, so don't be afraid to ask questions.
Another good thing about the forum information (again and always, thank you) is that once you have digested the relevant areas that apply to yourself you will be fore armed with questions to ask at your next meeting.Might be an idea to write them down and also any symptom you also have but may be you thought trivial to your case.
I have Jak2 & ET and had dizziness, loss of eye focus, brain fog and heart rumbling. Prior to my diagnosis, when I also described pains (on a cramp level) in various muscles that occur randomly at any time, night or day, that triggered a response from my GP.
Thank you for your insite. I really appreciate everyone here and their experiences! You reminded me when I had COVID and actually thought I was going to die, with the fever of 105 for six days. My primary wouldn't put me on Paxlovid (before the MPN Dx) Now she sees it differently. I told her I can't go through that again. The dillarium and charlie horses were excrutiating and then it turned into pneumonia. I already feel as though I have some more answers on how to tackle this. Thank you!!
when I was diagnosed with ET Jak2 I already had Sjögren’s syndrome . In addition to ET I was told I had Antiphospholipid syndrome sometimes called Hughes syndrome after the doctor who recognised it as a syndrome .
The reason I mention this is because I had had symptoms , lots
visual disturbances loss of speech dizzyness brain fog etc etc that could have been attributed to migraines,but only in the years I now know I had raised platelets but also Hughes syndrome which is rarely looked for but can be responsible for many conditions associated with sticky blood .
All I know is that since starting Clopidigrel these symptoms are greatly improved or all but gone . I still occasionally get a sharp pain in my head and an ache behind my ears which seems to have gone along with visual disturbances in the past , Hughes syndrome is often treated with a warfarin type drug .
My thinking maybe sticky blood has caused the migraines could be a huge positive as treatment like blood thinners / anti platelet may resolve many issues for you . If this seems simplistic for a condition you have lived with so long I apologise but look up Hughes Syndrome very interesting L
I have to tell you, my migraines have been different. Of course was told the usual of hormone changes/menopause etc. So having more answers with a treatment plan would be so helpful. i think the doctors really have to be open and listening! I look forward to being able to manage this. How often are the bone marrow aspirations/biopsy done? Just trying to mentally prep for all of this! Wherever it takes us.
Morning Thanks for reply I still think you should read about Hughes Syndrome just from an interest point of view . If you feel inclined , my haematologist showed no interest at all so am not sure if that means many people are found to have it or having positive tests don’t necessarily indicate you have the syndrome . For me adjusting to actually having a diagnosis after years of symptoms I realise now has been difficult .
adjusting to fatigue now with a reason is all harder than I realised . I have always been good at working to get better after various health issues i am still finding that hard . Listening to very positive attitudes on this site and seeing the very healthy looking bunch at the forum in London my first last year was also a boost . L
Thanks for reaching out. I was only diagnosed a few months ago. I don't go for another blood test till the 29th. I will post how it goes. There aren't a lot of MPN specialists here in the Southern part of the US. The biopsy ( WHICH THEY BOTCHED) SHOWED it leaning toward MYLO Fibrosis and not Et. I don't have much time in as to treatment for this . I quit my hydroxurea several weeks ago and my platelets are ip to 650 thousand. Still it's down from over a million. It's a strange thing we have. I will let everyone know my progress
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