SO MY JOURNEY BEGINS

[Chez1947]

when I was diagnosed 3 weeks ago for PV JAK2+ my readings wern't too bad to I wasn't put on any meds. I got my blood test results today although my blood went down my platelets were high so I have started taking 500 Hydroxy every other day and have to have blood test in 2 weeks, ( still trying to get my head around it all). x

[EleanorPV]

Welcome to the club. Have you found MPN voice?

I found the first 3 months, after diagnosis, I couldn’t make sense of any of it.

Ask any questions. Remember no question is stupid.

After more than 2 years, PV no longer defines me. I listen more to my body and rest when I need to. Look after yourself and drink water xx

[WileyFrench]

The first couple of months are definitely the hardest I think, in terms of just wrapping one's head around the fact that we have a "disease" and that we have to take medication for it, probably forever. I found it a real emotional roller-coaster. I'm nearly two years in since diagnosis, and one since starting HU, and it still is in the back of my mind, but not at the front. So I take that as progress! I'm sure it will be the same for you, and this forum is just great because its full of real people, many of whom have had an MPN for decades and are doing just fine, and all of whom I've found are willing to share their experiences, tips, advice, and listening ears.

[meaboutthis]

I like EleanorPV's suggestions. I also had a hard time getting my head around the idea of my body doing its own thing, and making me feel unwell in the process! No question is stupid, be an advocate for yourself, pay attention to your body and what it's telling you, rest when you need to and don't overheat. Good luck!

[hunter5582]

Have had an MPN for 30 years+. Started as ET and progressed about 6 years ago into PV. I am fortunate to have a relatively low JAK2 mutant allele burden at 25%, which is why my symptom load has always been low. If you have not had that checked yet, be sure to find out what your allele burden is (test called JAK2 Quantitative Analysis). Those of us with an allele burden less than 50% tend to have a milder coarse of the illness. Unfortunately, since PV and all MPNs are rare disorders, many docs, even hematologists, do not know much about treating them. They have a tendency to fall back into the "standard treatment" routine without having the knowledge to look at your individual case to see what approach is best for you specifically. I reached out to a true MPN-expert, who now serves as a consultant to me and my regular hemo. Found the expert on this list of patient recommended MPN docs mpnforum.com/list-hem/ . All the best to you on your journey.

[Cja1956 in reply to hunter5582]

Hunter, I always learn something new from you. I didn’t realize there were different levels of Jak2. Thanks for sharing your knowledge with us.

[hunter5582 in reply to Cja1956]

Well thanks. The thing I love about this forum is how we can learn from and get support from each other. If you are looking into the things that drive the course of MPNs, then also look at non-driver mutations (e.g. TP53, ASXL1, IDH2, etc). There is some really interesting research emerging on this. Not a lot of consensus on this or what to do with it - but it is of interest.

[Chez1947 in reply to hunter5582]

What do the tests involve? I will ask my nurse next time I call her. (Mutant Allele Burden ) x

[Chez1947 in reply to Chez1947]

I also looked up my nearest Hematologist and he is in Middlesex, unfortunately I am in Essex too far to go for me x

[hunter5582 in reply to Chez1947]

JAK2 Quantitative Analysis = standard blood test - same as you had when they did the JAK2 before. More involved in that they are countinh tje percentage of alleles on the JAK2 gene that are mutated.

[Chez1947 in reply to hunter5582]

Thankyou Hunter, I have an appointment with him in a few weeks I will be bombarding him with questions.

[hunter5582]

Bombarding is good! Hopefully the doc will have answers to your questions. FYI - the MPN expert I went to see is close to 2 hours away. He is not my regular treating doc, who is 30 min away. The "Expert" serves as a consultant to me and my regular hematologist. It was totally worth it. Many hemo-docs have little/no experience with MPNs and do not have the KSAs to assess your unique presentation of this rare disorder. I would have driven 3 times that far as the value of the consultation was worth it.

[Chez1947 in reply to hunter5582]

Can I ask what a normal stem cell count is ? mine was 520 and they have put me on hydroxy x

[hunter5582]

I am wondering if you are referring to hematopoietic stem cells (HSC)? Did you have a peripheral blood or bone marrow test specifically for this? This is not something that would come out in a routine CBC. A platelet count of 520 would be low-grade thrombocytosis. Unless you are in a high-risk group or are actively symptomatic (thrombosis, splenomegaly, etc) , most docs would not treat with HU for this level. I am not sure what the normal range for HSCs. Did a quick look and did not find anything. Here are a couple of good articles to look at moffitt.org/File%20Library/... , en.wikipedia.org/wiki/Hemat... . HSCs are the precursors for megakaryocytes en.wikipedia.org/wiki/Megak... , which are the cells in the marrow that make platelets. HU is a cytostatic agent that works by interfering with DNA activity and inhibiting the production of all types of blood cells. It does more than that in the body, which is why HU toxicity can be a problem. Suggest you make sure you understand all of the relevant terms that the docs are using. They are not always very good at explaining what they mean and all too often in too much of a hurry to explain. Hope this helps. BTW - I am not a medical professional (actually a MA psychologist). I am a MPNer with PV JAK2+. I have found it essential to my own mental health and vital to receiving good care to educate myself the best I can. Most docs, even hematologists, don't really know a lot about MPNs. I just try to do my best to be informed and be able to ask the right questions so we make good decisions regarding my treatment . All the best to you.