Rollercoaster Ride - MPN

[FAM_KT]

Hi Everyone,

I know I don’t post on here all the time but I do think it helps me to rationalise my thoughts

My story - April 2020 I found out:

- I had Varices Grade 4

- Portal Vein Thrombosis and collaterals

- Jax2 Positive

I was then treated for ET MPN as my platelet count was high.

After over 12 banding procedures of the varices and a bleed in August 2021 the Hepatology doctor, that dealt with me during my bleed, took on my case and wanted further test as he believe we are dealing with more than ET .

6 weeks ago I had a bone marrow biopsy - first lot of results back within a few days - everything looked great - the doctor said looking at the results he would classify me as MPN - ET U because it was not significant enough at this point.

Yesterdays consultation - my Tibia results were back - turns out there is scar tissue and I have Primary MF - I feel like I have gone from one end of the spectrum to the other - even the consultant said he had to double check the results.

I feel a bit out of sorts, with my old consultant I requested bone morrow biopsy’s and he would say everything is under control we do not need to do one.

Although I know my treatment wouldn’t differ at this point - (I’m on peg interferon) - I do feel slightly let down mentally - for over 2 years I believed I was dealing with ET - and to be honest I think most doctors can’t believe my results - or how I present - but I constantly feel like I am given with one hand and taken with the next!

I know I will be ok, I’m too head strong to let it get on top of me but I just need to process it all - I do feel there should be strategy plan the word go for people with an MPN and everyone should receive the same care plan - I know the timing for myself was is in the hight of covid and many have suffered worse but never the less I have seen multiply post about people not been offered a BMB and worried it could be something worse!

[EPguy]

I agree there is differing thoughts on BMB both from Drs and patients. My 1st Hem ordered one at the start and I feel it was the right idea.

How are you doing on PEG? Are your blood counts all good? Are your other conditions doing ok? I believe the Portal Vein Thrombosis can be associated with MPN, did your Dr discuss that?

From an old report:

pubmed.ncbi.nlm.nih.gov/220...

<<Myeloproliferative neoplasms (MPNs) are frequently identified as an underlying cause in patients with non-cirrhotic portal vein thrombosis (PVT)>>

I understand the Covid timing, I got it early Mar '20 and Long Covid overlapped with my MPN Dx to add to and muddy the symptoms.

BMB:

-INF has the possibility to reverse or at least stabilize marrow conditions (morphology), fibrosis, celluarity... The reports vary but there is a pattern of this possibility. But you need a starting BMB to compare.

-BMB can add info to a Dx, as you have found. In my case it points to ET while some other factors point to PV.

-Knowledge is increasing quickly for MPN and there should be increasing use for our BMB info with this knowledge.

Do you have your allele burden? This is generally the % of your marrow cells that are mutated. Also you should ask Dr about Nex Gen sequencing. This looks for any other mutations you might have. These items are also likely to be increasingly useful as the MPN field quickly gains more knowledge.

[hunter5582]

Sorry to hear about the struggle and the ups and downs. It sounds like you have the right attitude to deal with this situation. Do please keep posting about how things are going. You are right that it does help to crystalize your thoughts. No matter what, know that you are not alone. We are stronger together.

[FAM_KT in reply to hunter5582]

Thank you Hunter!

[Cja1956]

I went through the same thing a few years ago with my long term hematologist. He had originally diagnosed me with ET in 2008. Around 2016, I felt that my symptoms were getting a lot worse and I asked for a BMB, but he said I didn’t need one. Finally, in 2019, I switched to an MPN specialist and she did a BMB and did tons of bloodwork and said I had post Et Mf intermediate 1. So it definitely helps to listen to your body and find a doctor who will listen.

[FAM_KT in reply to Cja1956]

Yes I believe that 100% - someone can tell you there is nothing wrong - but your body tells you otherwise!

I think the whole experience from being diagnosed to now has been very full of ups and downs - I’m a very positive person - I know I can’t change my prognosis but I can’t help but feel that I should have had a BMB with everything else I have going off it was picked up straight away with the new consultants just from the symptoms. I do think it’s down to money in the end!

[Cja1956]

I get it. When my new doctor told me I had progressed to MF, I was on an emotional roller coaster for months. It was a tough time. But I’ve been mostly stable now for about 2 years and I feel there are many treatments in clinical trials and a medication called Fedratinib that is already on the market in the states and that there is hope for us.

[Pte82 in reply to Cja1956]

Cja1956, my wife has moved from Jakafi to Fedratinib then pacritinib. Pacritinib was the only one to shrink her spleen allowing her to go from a wheel chair to walking significant distances. It also did not cause a decrease in platelets and hemoglobin like Jakafi and Fedratinib. If Fedratinib is working for you stay with it but at least there are options and more coming in the pipeline.

[Cja1956 in reply to Pte82]

I’m glad your wife is doing better. I was on Fedratinib for about 2 years along with Hydroxyurea. My hematologist sent me to another doctor for a second opinion and she took me off of the Fedratinib since my spleen was only slightly enlarged. I’m an unusual case in that my platelets are high, but my red blood counts are low. Right now I take 15 hu/ week and low dose aspirin. So far, it’s kept me stable.Thanks so much for the information. I hope your wife continues to do well.