Feeling a little overwhelmed just now, following my appointment with my haematologist yesterday.
I have very recently been diagnosed with Myelofibrosis, post ET, yesterday I was told that I am stage Intermediate 1 risk with a prognosis of 80 months. I am aware this is an average prognosis, could be less could be more scenario.
I am currently taking 45mg Pegasys monthly, blood’s are not affected as yet, spleen normal size, liver ok.
Awaiting gene testing result.
Does anyone have any advice or experience on how to deal with this with as far as treatment/ progression/ prognosis is concerned.
I love this group and the support it provides to us all.
I have plodded along quite nicely since ET diagnosis 17 years ago but struggling with this one.
Sorry to hear your ET has progressed myelofibrosis. 14 years in with ET myself it’s something that plays on my mind. Out of curiosity if your bloods and spleen are OK what pointed them to progression. Also what is the plan now. Are you continuing with Peg or changing treatment. My MPN specialist keeps saying there are new treatments for myelofibrosis coming down the line and that’s it’s exciting times in that field so hopefully this will be of benefit to you.
Hi, it’s early days yet as far as treatment is concerned, I was having lots of unexplained symptoms and had not had a BMB biopsy since diagnosed 17 years ago, my Haem thought we needed another biopsy to look at what’s occurring now.
It has shown some fibrosis so here we are.
For now I am continuing with Peg, albeit reduced due to shortages but so far so good with the reduction.
I am not at the stage to change to Rux or have SCT, this apparently is at stage Intermediate 2..
We are waiting on genetic results from the biopsy which may give a better picture of what is going on and how things may progress as well as a clearer treatment plan.
All new to me and I have to say rather scary.
Hopefully things will become clearer in the coming months.
I think I just plodded on with ET over the past 17 years and didn’t worry too much about progression. Looking back I feel this was the right thing to do. Don’t worry about stuff until it happens.
Thankyou for your response and hopefully new treatments will come to light.
Hi Lynn. I'm around the 20 year mark for CALR ET myself. May I ask what symptoms you experienced? I have read that CALR ET is more likely to progress to myelofibrosis than jak2 positive ET. The good news is when we do progress to myelofibrosis it tends to not be a very aggressive form of it.
None of my symptoms completely ticked the boxes for MF apart from itching.
Lately I have seen Gynaecology, Vascular surgeon, ENT, Rheumatology, Opthalmology and NHS dentist for various symptoms,
it is taking so long due to the pressures on the NHS to get any diagnosis and treatment that my Haem decided it was time to have a BMB, probably to rule out any changes just in case.
It would be wrong of me to say any symptoms were actually due to MF as it was and still is not clear of of what exactly the cause of any of my Symptoms.
Sorry to hear about the progression to MF. At this point you will need more information, including the pending genetic test results to better understand your prognosis. It is important to differentiate a statistical projection of average lifespan from your individual prognosis. They are not the same thing. Moreover, the statistical projections from the past may no longer apply with the newer treatment options for MF.
I have been living with a MPN for over 30 years, ET that progressed to PV. The statistical protection for my lifespan would project that I have about a 4-5 year lifespan at this point based on JAK2 + NF1 + other medical conditions. I do not worry about this at all. There is no timer ticking away in my body. I am doing everything I can to live a high quality life, which is also doing the things that give me the best chance for a longer life. While I do not fear death, I have plenty left to do in this plane of existence and would prefer to stick around a while longer than my statistical projection. I stay focussed on living a good life and take steps to ensure it is a longer life.
We each have to find our own path on the MPN journey. Fortunately, it is not a journey we have to travel alone.
Wishing you all the best as you sort out the next stage of your journey and success in managing your care.
Thankyou Hunter, your positive words mean a lot, I try not to dwell on things and understand that everyones journey is a different one, I too do not fear death just want to live as comfortable life as is possible.
Gathering all the information I can is my key to this and enables us all to make an informed choice.
Although I knew my MPN could change it seems to have taken me by surprise, it’s really good to get others views and experiences which also helps me to make what are hopefully wise choices.
Your words make complete sense.
Thankyou Lynn x
• in reply to
I have decided to deactivate my account due to such negative posts which can cause a great deal of anxiety when suffering with the same illness
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I am so sorry if I have caused you any distress or upset to anyone this was definitely not my intention. I felt I was reaching out for support and advice about my own situation. I will willing delete my account if need be or not add any more posts,
Apologies Lynn
I am so sorry if I have caused anyone any distress or upset to anyone this was definitely not my intention. I felt I was reaching out for support and advice about my own situation. I will willing delete my account if need be or not add any more posts,
I’ve just come on to read the posts. Clearly, something has upset you, I don’t know what that is, but please don’t leave the forum. As you say, ‘I love this group and the support it offers to us all’.
Your post undoubtedly indicates you need support and understanding right now. Please reconsider, we are here to support you. Xx
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Recently diagnosed with ET (CALR+)
CALR and DNMT3A 
ET - Calr what is this? 
ET with CALR+ type 2
