I'm a young person with mpn. Some days are good some days aren't. I keep thinking abt the risk of progression of the disease. As a person who is a medicine and surgery graduate and for better understanding of the disease i have asked around quite a bit, especially to people who have had longer periods of myelofibrosis free years, and it did seem like a symptomatic disease especially pruritus seemed to be almost absent in those who had slower disease progression which is also marked by only phlebotomies as treatment unlike those where disease burden is higher with symptoms treated with rux. I found someone posting an article here that someone posted with doctors finally piecing this as a possible link too. Leucocytosis is always known a risk for progression.
I'm very scared because i have both pruritus and leucocytosis. I know it's all numbers and no one really knows how it exactly is for each and every person. But it's very scary sometimes even if i just keep going about my life i dread these things oftentimes.
Written by
Xuzy
To view profiles and participate in discussions please or .
Edit: mhos61 has the right point, I see in your old reply post that you are negative for all mutations and marrow. By the 2016 WHO criteria, table 1 here, you don't have MPN type PV. If so it leads to secondary PV or other non-MPN type.
I think a Dr did say you have MPN, did Dr give specific reasons for this opinion? You're right a top MPN doctor would be great to consult to confirm either way.
--
This report has some interesting guidance on Jak2 negative, providing some correlations, see the table here. If you in fact do not have MPN type PV, the prognoses that have been developed for MPN types may not be as relevant for you.
Yes i visited on mpn specialist and he said maybe I'm very early in disease pathogenesis that findings are not clear. He did say that symptoms usually pruritus is very specific to pv. So at the present a wait and watch policy is the best and to do phlebotomies and asprin. I know I'm worried about statistic which may or may not be applicable to everyone but somedays it keeps plaguing me about what ifs i had a different curable disease. Thanks for the info EPguy I'll check it out
I believe you were found to be negative for the Jak 2 mutation including Exon 12-15. Wouldn’t this then rule out MPN and be more a case of ‘Secondary/Idiopathic Polycythemia’?
I did visit one doctor who said he's an active reaserch oriented hematoncologist in mpns. He said my symptoms match very to a typical mpn and classically when there was never a criteria they always differentiated secondary vs primary by means of high hemoglobin and symptoms. He says it's a wait and watch game as i must've been very early into disease pathogenesis that i might not have had typical bone marrow findings. In the meantime I'm prescribed to undergo phlebotomy and take aspirin.
From your comments it seems your Dr prefers to use the pre-2016 methods, and in particular an emphasis on symptoms and a lessor consideration of mutation and biopsy results. This is unusual in current practice. But we may learn something from your Dr's approach, especially "He did say that he's seen some cases like this happen" (PV MPN arises from a fully negative marrow and mutation patient) If he's seen this more than once it's something the other specialists, and we, would like to know more about.
My 1st Hem said IFN cannot reduce the mutation, we now know that is not correct, I am proof myself. He was also using older thinking. So we all benefit from combining info from many places.
As the others indicated, the best answers will come from a consultation with a MPN Specialist who can review your case specifics. While there are people with a triple-negative PV, this is very rare. You really need to consult with a doc who has a high level of MPN expertise. A MPN Specialist can also help you to address better symptom control with issues like pruritis.
I would offer you a different observation as you identify as a young person with a MPN. I used to be a young person with a MPN. That was 31+ years ago. I am now an old person with a MPN. At age 67, I have lived great life while managing a MPN and continue to do so. You should plan to do the same. Your options are better than what I had 31 years ago. The understanding of MPNs has grown immensely. There are more treatment options now and more in development. Ten years from now, the treatment landscape will likely be transformed compared to what we have now. You have good reasons to be optimistic about your outcome. You can take action to make that positive outcome happen.
Hello hunter! Thanks for always being kind about these concerns. I'm ever so greatful for the support and assistance offered here.
So I'm living in India and i did consult one doctor who said in his profile he's a mpn research doctor in India. He said that my so called secondary causes of elevated hemoglobin didn't seem to prove the cause and effect. As he checked some parameters like methemoglobin levels which came out to be normal range. As such he said i cannot say this is secondary because most known causes including rare gene mutations have been ruled out. As for primary he said previously when they had no WHO criteria or gene mutations known it was always based on symptomatology especially pruritus. So he said accordingly i might have pv and very early in the disease that bone marrow findings aren't yet apparent on gross level. He did say that he's seen some cases like this happen and while i also asked if we can get one more biopsy he said let's just wait and watch and meanwhile just carry on with phlebotomies and asprin.
So while also I'm very hopeful for some advancements which may or may not be readily available in the Global south. It's just some days my anxiety goes haywire. Anyways thank you sm for reassuring. I will have another appointment next month around these dates. Thank you
It sounds like you are on a path to successfully dealing with your situation. The MPN Specialist assessment makes sense as does the treatment approach. About the only thing to add to it is to try the different strategies people have found helpful with pruritis and with systemic inflammation.
Identifying and avoiding triggers for pruritis is important. Some people find antihistamines help. Others report success with Beta Alanine . Maintaining a healthy skin moisture barrier can also help. I use CeraVe cream to keep eczema at bay.
It is also important to control inflammation. Anxiety can make inflammation worse, and inflammation can make anxiety worse. An anti-inflammatory diet can help. Some have reported success with using curcumin and N-Acetylcysteine. I have had particular success with curcumin.
The anxiety is important to manage as well. We each have to find our own way. Feeling empowered to manage the MPN can certainly help. Educating yourself about MPNs can help you to feel empowered and make better decisions.
I’m sorry that you are having such an anxious and difficult time. You are doing the right thing by gathering information and I hope that it helps alleviate some stress for you xx
Hi. I’m afraid I can’t be helpful with knowledge but want to say you’re not alone in worrying but most of us realize after a few years of consistent symptoms & labs that statistically we’re likely to die of something other than our MPN. That’s what my experts said almost 10 years ago & so far it’s true. There’s been little if any change. Please remember you have a fan club here that cares about you & vent whenever needed. Katie
Here is some very good news: A 2 minute summary about how a multi-decade retrospective study found the drug interferon-alfa enabled PV patients in the USA to usually live a normal and a myelofibrosis-free lifespan. youtu.be/JXI9FwRcauw
I was diagnosed with ET but it turned out to be masked PV in 2020. My platelets were high after my 3rd child was born, 17 years ago. My legs would be super itchy and turned blue in a hot shower for many many years before I was diagnosed. Jak2+
In the long run I am much happier knowing that I have this issue than not knowing. Specialist explained it to me as, your body has a secret, it can make blood clots, but now we know it too. Simply this is a great think to know. I am healthy in pretty much every other way ( I believe 🤨).
I also have the crazy itch, for 6 or 7 years now. Just trying on a new shirt at a store made me burst into tears and flee the store acting like a maniac. I tried everything over the counter, it did not help and I wasn’t interested in more drugs so I visited a health food store. I take 2 quercetin each day and turmeric. I started out taking stinging nettle also several time a day but really only needed it a few weeks. I believe the least amount of inflammation in your body is best.
I drink plenty of water, use various creams and oils on my arm and legs and wear only certain clothes that calm my skin. Sadly they are mostly legging and other smoother soft material ( mainly polyester) I discovered that a heating pad also helped so I bought a heating blanket and pulled that out on dry winter nights. I also put some oil in my tea in the morning- moisture inside and out. There are special diets to try for high histamine, but they did not do much in my case. A big practice is also meditations, podcasts and audiobooks. So many free app that can help, many have free versions. I love “ Insight Timer” , lots of relaxing, breathing, music and stress busting ideas. Try all these at night in bed, helps you relax while listening. Tip: Find topics that interest you, not this crazy disease. Learn, grow and thrive because you will have a long life ahead of you.
We are all here to support you in your journey, as ours will be similar but not exactly the same. So many wonderful folks on this site. I am hoping to meet and hug everyone when we take our MPN Successfully Cured - Cruise in the Caribbean some day in the future.
My now 22 year old daughter was diagnosed shortly after I was with ET. I have a good reason to take care of myself as I want to help find a positive outcome for both our health issues. The mind games that your brain will try to play with you are crazy. Stress is a problem for much of the world. Continually refocus and take one step forward.
Most days the itch is controlled. The reality is, many night I sleep in my day clothes because I am too afraid to change in case the itch gets activated. Once it starts it spreads! I happily take a super hot bath twice a week and soak with Epson salts. After the bath I use oil on my skin.
Slowly I have found my way to a much more normal life. The crazy kind of snuck up after many years and it was hard to remember what normal was. Please try and find a happy normal, whatever that looks like. You may have a bumpy road or hopefully you have passed the bumps and it is smooth sailing from now on.
It is late and I should be sleeping BUT I needed to offer you some love, some support and some of my crazy solutions.
I owe many thanks to other fine friends on here who helped me realize mine and my daughter’s life were not ending anytime soon. Also that worrying instead of living and thriving was not going to help flip the gene back or stop progression. Dr Bruce Lipton has a few good books to start with. ❤️❤️
Thank you for your kind words and support. We have wrapped our minds around our health issues and are taking them on head on. We are positive, happy and excited about the future. 🍻
Content on HealthUnlocked does not replace the relationship between you and doctors or other healthcare professionals nor the advice you receive from them.
Never delay seeking advice or dialling emergency services because of something that you have read on HealthUnlocked.
Annoyed and Need to Vent
Mental Tiredness and Anxiety
Post ET MF - Advice Please
Just starting out on hydroxy
Possible Future Treatment Involves Healing Your Gut
