ET potentially becoming MF

[Glagirl]

Hi there,

I was diagnosed officially with ET when I was 20 although it was picked up when I was 15! I was a bit of a anomaly and so spent 18 months being a guinea pig. And then I moved from Scotland to Hong Kong and have spent the last 20 years pretty much ignoring my ET. Except during my pregnancies I should add!

When I was diagnosed, of course there was no internet as we have it now, and no way to connect with other my age who had ET therfore I felt quite alone so finding this forum is a bit of a treat!

My platelet have always stayed around 650. ( I have always had a couple of blood tests a year).Recently I’ve been pretty fatigued and had a few other aches and pains so finally went to see a heamotologist who because of lower count of 497 and a slightly enlarged spleen (16cm) now wants to do a BMB to test for MF. However he made the prognosis sound pretty bleak so I’m wondering if it’s even worth finding out right now!

Any advice?? 😬

[clubdino]

Hi. I was 26 when diagnosed and I'm now 41...and like you I probably had it longer. I worry about MF. I had scheduled a BMB a couple years ago to see if my ET was starting to transform to MF but I ended up cancelling the appointment. Since then I have gone back and forth between wishing I had gone through with the BMB to know the state of my disease to being glad that I didn't do it because it may be better that I don't know...as far as my mental state goes.

More and more lately I find myself wishing I did the BMB so I could better prepare myself for the future. And definitely if I was in your shoes with splenomegaly I would get the BMB. Are you anemic yet?

[Glagirl in reply to clubdino]

Hello!

Thanks for your response! I actually haven’t even spoken with anyone else with ET, never mind someone who was also diagnosed in their twenties!

My haemoglobin has dipped but wasn’t below a normal level. And at my last blood test it has gone back up and so had my platelets. (Though still below my average)

Not sure what it all means.

I have never had a great experiences with specialists as this one was no different. He wanted to do the BMB to verify which mutation and the MF then he “could give a time to bone marrow failure”. Errrrr.....maybe not!😆😱😬

[clubdino in reply to Glagirl]

Oh man, I understand why those words scared you. That's the opposite of what I have experienced. My doctor hasn't wanted to do any additional testing and I actually requested the BMB. He said it was the first time a patient of his requestd a BMB. But the reason I had requested it was because my last two bloodworks showed I had a higher amount of immature white blood cells than what is considered normal...and that's a sign that my ET may be starting to transform. He wasn't worried about it.

I guess one thing to take into consideration is your treatment plan. If treatment can extend your life expectancy than you would probably want to start sooner than later. I hope some people with MF can chime in on here to help you make the decision.

[Paul123456 in reply to Glagirl]

Please don’t worry unduly. The BMB is just a useful tool to judge where we all are on the progression scale. You are young and rate of progression should be very slow. My understanding is that ET patients who ‘progress’ to early MF often have the CALR Type 1 mutation in addition to JAK2. This is a good mutation to have with MF and dramatically improves prognosis. I would check to see if you have this mutation?

From what you have said, it is far too soon to start panicking. Are your white and red blood cells normal?

Best wishes Paul

[pbacon2549]

Hi, First dx at 42, although first symptoms I remember I was 35. This month I turned 70 and have been skipping along with occasional medication adjustments. Because there were so many distraction in my family and friends (cancers) I never read my notes, where I would have found my spleen was starting to enlarge. It was the right thing for I don't think at that time I could have handled it very well. This summer I had another BMB which show the start of the transition. I would encourage you to have one. they can identify more genes now (therefore maybe a med) and monitor you and discuss Stem Cell Transplant (SCT), currently the only cure.

I have opted to decline SCT. When the BMB was done, I was staying at one of the American Cancer Hope Lodges with my partner while he was receiving radiation. if I was younger I would consider it and who knows I could change my mind.

Knowledge is power but we all make the choices that are right for us.

Peggy Dx 1991, Hydrea, Baby ASA. Triple negative genes

[Tico]

Hi Glagirl, did your haemo specifically mention Mf? They can now find out if you have a mutation by special blood tests alone, years ago it was only by bmb only. When is your bmb? Sending hugs. tina x🤗

[beetle]

I was diagnosed with ET in 1994 when, as you say, the internet was in its infancy. It is good that you have been able to largely ignore your ET since your diagnosis. If you have a BMB and it does reveal progression please remember that the internet can now be a very scary place when looking up health issues. It is good to bear in mind that there is now a great deal of research happening in a MPNs and the statistics available to us now are by their very nature historical. As more and more is learned about MPNs there are several novel treatments under investigation which we might be able to benefit from in the not too distant future so the future does not look anywhere near as bleak as the past did. I progressed to MF nearly 7 years ago and I still hold out hope for my future.

You have found a good place here for support and advice. We are not doctors but there are many knowledgeable folk here that point us in the direction of new research etc. and all are always welcome

Very best wishes, Jan