PV question

[MJoye]

Hi, I’m new to this site and would like to talk to others in a similar situation to myself. I’m 55 and was diagnosed with PV JAK2 genetic fault in August. I’ve had several venesections and my rbc has now stabilised. I’ve always had a high wbc (20) and an enlarged spleen. Today the dr has decided to refer me for a bone marrow biopsy and I’m now terrified they’re looking for myelofibrosis. Has anyone else been in this position? I’ve only just come to terms with PV. Otherwise I feel ok....any advise appreciated

[Aneliv9]

Didn't your doctor mentioned the reason for biopsy?? Will this be your first biopsy?

[MJoye in reply to Aneliv9]

Yes, my first biopsy. It’s because my white blood count hasn’t gone down

[Aneliv9 in reply to MJoye]

I don't know what it may mean if whites are high, but did your doctor had a suspicion of MF? Or is it just your worry?

[MJoye in reply to Aneliv9]

The appointment was a bit of a blur, I might call them tomorrow, I am a worrier!

[Aneliv9 in reply to MJoye]

Yes i think you should ask straight if biopsy is for MF suspicions . Let us know tomorrow!

[tracey13]

Hi, I feel your pain please try to stay positive.

My husband was 41 diagnosed with PV that's knocked us for 6 he had venesections to start with , but after 3 years he decided to go on hydroxy yeah he was so scared as it's a chemo tablet. He's had no side effects and it really helped his symptoms.

Last October his spleen was 21.2cm this lead his consultant to check out his bone marrow which revealed early stages of MF .

This time last year we got this result he's had to see the bone marrow transplant team they agreed to start him on ruxolitanib which has been brilliant.

His spleen last month was 15cm all his bloods are in normal range.

Yes he still gets tired .

He still works full time and we've now put the transplant to the back of our minds as he may never need one .

There's no way he wants a transplant unless it progresses to AML.

His consultant said last week there's better medication coming out and he's sure there will be a cure one day.

Try not to worry too much as it took over our life and now we kind of relaxed about it.

I joined lots of forums and there's people had MF for many years.

This site is amazing there's so much support for people who have the same illness.

Googling information scares the hell out of you tells you life expectancy 4 years that's all nonsense.

There's no cure as yet but these blood cancers can be managed which is a massive plus.

We are thankful my husband's cancer isn't terminal and that it can be managed.

You take care

Tracey

[Aneliv9 in reply to tracey13]

How many years after PV your husband progressed to MF? Is it true that your doctor told he may never need a transplant???? Tell me please more details for the reason that believes that. I wish you all the best.

[tracey13 in reply to Aneliv9]

My husband had PV for 5 years before he progressed to MF.

Yes his consultant said only a certain amount of people progress to AML and need a transplant.

He's had all the testing done for a donor match . He's got 9 unrelated matches on the donor register.

As long as his medication keeps working he should be ok.

Tracey

[Aneliv9 in reply to tracey13]

I see..so may never someone with myelofibrosis need a transplant? Very interesting

[tracey13 in reply to Aneliv9]

Not everyone progresses to AML.

We just praying my husband's MF doesn't progress x

[Maisie10]

Hello I can fully understand where your coming from. I was diagnosed with PV Jak2 positive August 4 years ago. I had a bone marrow biopsy to confirm that it was PV. My red blood counts were very high and so was my white cells. At first I was just having regular venesections but as these were not enough I was put on Hydroxycarbamide and as you was very frightened as they are a chemo tablet. It has kept my red blood counts in the safe zone but although my white cells have come down they are still high. I still have venesections as well when my Hematocrit creeps up. I have been lucky not to have side effects only fatigue which I think is part of the condition anyway. I used to have before being diagnosed very bad headaches, which now have gone. So, I feel so much better now I am being looked after by a very good team at the hospital. Try not to worry to much, I know it’s difficult not too. It’s good to have a site like this as you realise you are not alone. Keep your chin up. You will be good. I am now 59 years old so was diagnosed at 55 also. Take care x

[AndW65]

Hi MJoye, I like many others here know exactly what you are going through. It’s a bit of an emotional roller coaster coupled with confusion. Maybe even bewilderment as to why you have this illness. The BM biopsy is usually more of a confirmation test to see if you are Jak2+ as well as insight to the current condition of your bone marrow. I’m 54 and was diagnosed 17 months ago with PV Jak2+. I’m currently taking 75mg of Aspirin and having occasional venesections. I was taking tablets to try and do something to combat the tiredness and fatigue but although it worked at the time, as soon as I stopped, I’m back to square one.

The bone marrow biopsy can be a bit uncomfortable but it’s not the most painful thing I’ve been through, and it’ll be over quite quickly. I suppose the worst part is the anxiety of waiting for the results even if your blood results are not that high.

Try to take it slow, and try not to think of the worst and remember there are people here that have had our condition for many, many years. I suppose it’s about accepting you have an illness, managing it and remaining as positive as you possibly can without letting things get you down. I know it sounds difficult, I too get very anxious whenever I have a doctors appointment or a venesection which currently number 11 venesections and 21 blood tests since my diagnosis.

Keep strong and I hope that your BMB goes well.

Remember your not alone!

Best regards

Andy

[Mazcd Partner MPNVoice]

Hello MJoye, welcome to our forum. Good idea to call your haematologist, or do you have a haematology nurse specialist you can call for some advice, most haematology departments do have a specialist nurse you can call. Try not to worry too much, easy to say I know, but it may be that your consultant just wants to do the bone marrow biopsy just to check what you bone marrow is looking like now and to use this in the future to compare for any changes, this is often done. Let us know how you get on. Best wishes, Maz

[hunter5582]

I also have JAK2+ PV. Actually diagnosed with ET over 30 years ago, which progressed to PV about 6 years ago. Still alive and kicking! Many of the challenges we face in managing PV are not life-threatening in the short-term and have more to do with quality of life issues.

The bone marrow biopsy is part of how some docs make the initial diagnosis of PV. Not all docs agree on the need for it. The MPN specialist I consult with does not feel it is necessary in my case. however, other docs routinely do it on all patients. For sure ask your doc why the BMB is being recommended and whether it is really necessary.

It sounds like you are in both leukocytosis and erythrocytosis. What about thrombocytosis? What do your platelet levels look like? Are there any indications that you have entered into myelofibrosis based on your actual symptoms? Some with PV go many years and never go into MF. Suggest you not stress over this unless there really is a specific reason to be concerned.

A few suggestions for you as you are new to the PV world.

1. MPNs are rare disorders. Many hematologists have limited experience and knowledge with MPNs. Do not be afraid to ask your provider how much experience he/she has with MPNs.

2. Seek consultation with a doc who is a MPN expert, especially if your current hematologist is not a MPN-expert. Here is a list of patient-recommended MPN docs mpnforum.com/list-hem/ . it is worth travelling to see one.

3. Be aware that there are different treatment approaches to PV. It is important to find a doc that will individualize your treatment based on your unique presentation of the PV and how you respond to treatment interventions. Here are two different approaches

mpnforum.com/spivak-how-i-t... Dr. Spvak also has a published article on this recommended in this interview. Dr. Spivak is the consulting MPN expert I use and it is the tx he recommended that I have chosen for myself. Not all docs agree on this approach.

ashpublications.org/blood/a... . Dr Vannucchi takes a different approach, one that is more common in the hematology world. This more conventional approach is another of your options.

4. Please do remember that it is your choice how to approach the diagnosis and treatment of your MPN. It is also your responsibility to educate yourself so you can meaningfully participate in your treatment decisions. Assertive patients receive higher quality care. Passive patients do not. It is important to be empowered to ensure that you receive the best care possible.

All the best

[MJoye in reply to hunter5582]

Thank you for your response, my platelet levels are also ok. It just seems to be the WBC that’s a problem at the moment. After talking to the Dr I don’t think there’s any indications I’ve entered Myelofibrosis, they’re after further information and a base line. I’ll read the information you sent and try not to stress. Thanks again

[Mpncoda in reply to hunter5582]

Thanks Hunter for your background and knowledge. I was just diagnosed last year with PV and no BMB is suggested cause my Hemo said my white cell count is normal. For now, it’s only baby aspirin. But I’m bruising so I take it 4-5x week instead of every day. Also, I found it comforting when he said he has patients in their 80’s and generally speaking PV doesn’t shorten life span as long as it is managed. Meaning I Might have to go on HU in a couple of years. He did suggest phlebotomy due to HCT at 51. And I’ve read they can give us more energy as the body replaces the blood donated with fresh new blood. Interesting...

[hunter5582 in reply to Mpncoda]

The MPN Specialist had me discontinue aspirin altogether. At age 64 and with my profile (30+years of MPN and no thrombosis + excessive bleeding/bruising on aspirin + von Willebrand factors that drop below norm with increased platelets), he said my risk of hemorrhage was greater that my risk of thrombosis. He rather prophetically stated "you really do not want to get a brain bleed." A couple of weeks after he told me this, I was diagnosed with a hemorrhagic brain tumor. I already had a brain bleed! Aspirin was not such a good idea for me. That was back in April of this year. As of June 17 - the tumor is gone (craniotomy/resection). Thankfully, it was a Grade 1 (benign) tumor - a Pilocytic Astrocytoma. While it does appear to be all the way gone thanks to the surgery, there is a very real risk that it will recur. I am thinking aspirin will not be in my future.

it just all goes to show that one size does not fit all. We each have unique MPN profiles and need individualized treatment. Hope you find the right answers for yourself ASAP.

[MJoye]

Thank you all for your responses to my post. I’m overwhelmed that so many have taken the time to reply and I no longer feel alone dealing with this condition. I called the hospital today and spoke to the consultant, I think I was in shock yesterday and didn’t take everything in. He has requested the bone marrow test because my white blood count hasn’t gone down (it hasn’t gone up either) he isn’t testing specifically for MF but is getting an indication of what my BM looks like at the moment. I think it’s the waiting and worrying that’s affecting me the most, I’ve only just come to terms with having PV. Many of you have said try not to worry so I’m going to try! I don’t know where this disease will take me but I’m glad I made the move to write my first post here. I think I may need medication to control the WBC but will keep reading and talking, thank you again. Michelle

[BlushNoisette]

Hi, I am 68 and was diagnosed with PV in 2006. Back then there was little support or info readily available for new patients.

The science and the treatments available have advanced hugely in the last 10 years. If you can attend a patient forum sometime, I'd really recommend it.

I participate in a research study and as a result I am treated with ruxolitinib. Previously, for about 10 years I was treated with aspirin 75mgs and venesections. My quality of life has improved hugely since being on the rux and I'm very well cared for. Also, the rux does a v good job controlling my blood counts and reducing spleen size back to normal.

If you are having a bone marrow biopsy (and I have had 5 of them , one every year for the last 5 years. I would recommend that you ask for gas and air to help with the pain of the procedure. Some people say that it doesn't hurt but that has never been my experience. So for first timers I'd suggest that you have gas and air and then if you don't have pain then next time you won't need it. My experience has been that it is not a god idea to start the procedure and then try to start using the gas and air when you are in real pain because it is very difficult to get your breathing right after you are in pain and v anxious.

With good management, you should be able to enjoy a pretty good quality of life. Fatigue is a bugbear for most of us but with some of the new treatments you should be OK. Try not to worry. Very few people with PV progress to MF and very few of them go on to get AML.

So, all being well you should be fine once your treatment is sorted and your symptoms are under control.

I wish you improving health and lots of blessings. Lyn

[MJoye in reply to BlushNoisette]

Thank you for your reply. Can I ask whether your WBC was high before you started your medication? From my limited understanding I may need medication to reduce the size of my spleen and also the WBC

[BlushNoisette in reply to MJoye]

Hi, I'm sorry but I don't remember. I was just so relieved to know that I would be receiving ruxolitinib, and I felt so fortunate to be being treated by this particular specialist at Oxford that I just put my trust in him and his advice. I was really quite poorly at the time and I had read good things about ruxolitinib that I couldn't wait to start the medication. And my belief was rewarded as I started to feel improvement very quickly.

Best wishes, Lyn