Just diagnosed with Polycythemia Vera

[Dolman]

Hi I have just joined this group for hopefully advice,answers etc as I have just been diagnosed with pv as my red blood cell count was high . I am awaiting for a bone marrow biopsy to confirm it . After reading on the internet I as so very worried about the outcome and life expectancy etc as I am 49 yrs old . Does it get a lot worse very quickly and also worried about having a bone marrow biopsy again after reading reviews . I’m going to stop googling as it’s scaring me to death but I did come across this group which looks very good . Many thanks Shaun

[Ebot]

Welcome to our exclusive club! KEEP AWAY FROM DR GOOGLE!!! If you want some sensible, up to date and reliable info check out the MPN Voice website. You’ll see that you have every chance of living a normal life for a normal life span. The key thing is that your PV is being monitored and managed. The problems tend to occur when MPNS are not detected and not managed / treated.

As for the BMB you’ll see from previous posts that I’ve had a couple and whilst they are not my leisure activity of choice, they are more than do-able. It just sounds altogether more invasive and slightly ick than it actually is. Just take it easy afterwards - don’t set off on a hike through town, take a cab! (Lesson learnt!!). Any questions, just ask on this Forum. Wishing you all the best.

[Dolman in reply to Ebot]

Many thanks , yes not looking forward to it by any means but needs to be done . Not been on google since and will just get information from all the kind and lovely people on here

[mark382 in reply to Ebot]

As above keep away from Google. Use the MPN voice for information. I've had pv for 11 years. Blood tests every 3 months. Sometimes a venesection and sometimes 2, with a week between. Low dose aspirin once a day. Fortunately didn't have a bone marrow biopsy. I was diagnosed at 52. As someone once said providing I'm being monitored and managed I'm more likely to die with pv than from it.

[Dolman in reply to mark382]

Thank you Mark , still makes me wonder why I have got got have s biopsy as had so many blood tests . Anyway I will certainly do as the heamo doc says ,

Thanks again Shaun

[mark382 in reply to Dolman]

Hi Shaun, I had a lot of blood tests and ultrasound scans. I can only assume my haematoligist had enough information to make a diagnosis without a bmb.

[hunter5582]

I was diagnosed with Essential Thrombocythemia over 30 years ago. About 6 years ago, the ET progressed into Polycythemia. I have been almost symptom free for all of this time. I am one of the lucky ones who is mildly effected. It is actually the secondary symptoms that have been more bothersome. Please be assured that while there are quality of life issues you will likely have to manage - your length of life will likely be quite long.

It is a really good idea to educate yourself about MPNs and the on-line resources can be very helpful. But be careful what resources you tap into. You can scare yourself silly with some of what is out there, particularly if you do not yet have the background and context to evaluate what you are reading. You have plenty of time to learn about all of this. For all of us on this MPN journey - there is lots to learn, ups and downs, and a real need to mutual support.

This site is a great place to start. One of the most important things you can do is to find a MPN Specialist. Most docs, even hematologists, lack expertise in MPNs due to their rarity. Whatever it takes, access a doc who really does have the KSAs you need. Here is a list of patient-recommended MPN expert docs mpnforum.com/list-hem./ . I had to travel to find a true expert. My approach is to use the expert doc as a consultant who works with me and my local hematologist regarding my care.

Do not be afraid to ask your local docs about their experience treating MPNs. Is a doc cops an attitude about your asking - fire that doc. Your health is more important than their ego. The doc works for you - not the other way around. Assertive patients receive higher quality care. Passive patients do not. You will also want to find a doc who will actually spend time with you, listen, and answer questions.

Hope we keep hearing from you. Hang in there and try not to worry too much.

[Dolman in reply to hunter5582]

Hi hunter and thanks for the reply , I actually did another post on here as I thought I posted it in the wrong section so apologies to anybody who is reading .

You say that your was a et and progressed to pv . Does that mean that pv is s lot worse than a et . Not sure why I have to have a bnb as I have had numerous blood tests now which I had to wait 3 weeks for the results . Maybe she needs a bmb from myself to see how bad it is but thought she could get that from my results.

Yes complete learning curve for me as never heard of this before.

Many thanks again for your reply

[hunter5582 in reply to Dolman]

With Essential Thrombocythemia only platelets are elevated. With Polycythemia Vera there are multiple blood cell types elevated (platelets, whites, reds). So PV is "worse" in that there are more blood cell types abnormally high. Whether you have ET or PV does not always predict how significantly the MPN will effect you. Blood cell numbers do not tell the whole story.

Many of us find the secondary symptoms more troublesome than the issues caused by thrombocytosis, erythrocytosis, or leukocytosis. Some experience chronic fatigue, pruritis, night sweats, etc. The JAK2 mutation can also trigger your body to create an excess of inflammatory cytokines. This can lead to a variety of inflammation related problems. I experience: GERD, eczema, insomnia, osteoarthritis, fasciitis - all of which is more troublesome than any of the other PV issues.

The direct PV blood cell number issues I have experienced include: hypertension, mild splenomegaly (enlarged spleen), and a tendency to bleed/bruise to much. I was in stage 1 hypertension (135/95 resting BP) until the Hyperviscosity of my blood was controlled by phlebotomy. Now my resting BP is in the normal range. My spleen size has reduced (was never large enough to be a problem). I am one of those whose risk for hemorrhage (bleeding) is worse than my risk for thrombosis (clotting) when my platelet levels get too high. I have never had a DVT or any other form of inappropriate clotting in over 30 years with a MPN.

Regarding the BMB - it is not necessary for a diagnosis of PV. That can be done with blood tests alone. There is a difference between the WHO criteria/diagnostic process and the European/British hematological association approach. Different docs have different takes on whether a BMB is worth doing. All the docs I see say "not worth it." The real value to a BMB is to look at the level of fibrosis present in the marrow. My docs feel that if you are not symptomatic for fibrosis, there is no real value to doing the biopsy.

There are some tests that are worth doing. If you are JAK2 positive, then a JAK2 Mutant Allele Quantitative Analysis should be done. This will tell you what percentage of the JAK2 gene is mutated. Less than 50% is indicative of a milder course of the MPN (my JAK2 = 25%). You may also want to see about a von Willebrand panel and what your prothrombin times (ptt/apt) look like. This assesses your clotting factors/rate.

While staying away from scaring yourself with all the Dr. Google info is a good idea, it is important to learn the basics of what these blood based disorders are. It is a bit like going back to school. Key terms to know/understand are: leukocytes, erythrocytes, platelets, hemopoietic stem cells, hematopoiesis, megakaryocytes, coagulation cascade, von Willbebrand Factors, thrombosis, hemorrhage, JAK/STAT Pathway, inflammatory cytokine. It helps to have a basic understanding of the terminology when talking to the docs. An educated patient will receive better care.

Do be sure to consult with a MPN-Specialist/expert doc. Most hematologists rarely see MPN patients and really do not have the KSAs to provide optimal treatment. These docs will default to the "standard protocol" in providing your treatment, which may or may not be what you need. The best approach is to find a doc who has the expertise to evaluate your individual case then create an individualized treatment plan based on your presentation of the MPN.

No matter what - feel empowered to take charge of your own treatment and insist on the quality of care you deserve. Get all of your questions answered and all of your concerns addressed. Assertive patients receive higher quality care. Passive patients do not.

[Blonde25]

Hi Dolman

Wishing you all the best with your BMB they measure percentage in the bone marrow , My PV is 20% so they can decide what treatment is needed and they also look for scarring?

Ask who completes the BMB how long they have been doing it for , it helps if they are experienced, my first one went wrong so needed repeating .

It's not a pleasant procedure but it's definitely worth getting the correct diagnosis. Just use the gas and air and try and relax it's over sooner than you think

Life expectancy can be good because it progresses slowly

Good luck