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.
Your contributions are always informative, wise and uplifting, Hunter.
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
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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,
Please don't leave. You have echoed the thoughts I have (although I don't even have a diagnosis yet) and it has been a relief to know there is someone else who shares the worries, concerns and, yes, fears. I really thought I was alone with some negative feelings.
Thank you for the courage you have in sharing what you have, good and bad.
Hello Lynn. I am sorry to hear about your diagnosis. I was diagnosed with ET 6 years ago. I got picked up for high blood pressure several weeks ago, and I have high Iron levels in my blood. My sister says our mother has the gene for Haemochromotosis. I am seeing the doctor for high blood pressure. My Ramipril has been increased from 2.5Mg to 5Mg. It is still high. The Ramipril is having no effect whatsoever after taking it for a week and a half. I am getting another blood test on Friday, with a follow-up appointment with the GP in November. I have had another blood test to check my Iron levels, with a follow-up appointment with my Haemotologist on 29 October 2024. I am dreading the results. It could be Haemochromotosis as well as ET. I am also dreading a Mylofibrosis diagnosis. Good luck to you and your battle.
I had the same problem, b.p bring 283/104 on Ramipril...I accidentally took 20mg ( not a typo ) and ended up in a and e , and it shot up to neatly 300... which was unheard of apparently...I'm now on Doxazosin and Ramipril and glad to say, it's now come down to a more acceptable level, and I feel much better. E.T jak 2 +
Thank you. I hope it didn't do any damage to your organs. My blood pressure is still high after two weeks on 5Mg Ramipril. It is in the168/100 range. How long does it take to go down.
I’m in a similar position to you, I plodded along with ET for many years hoping there wouldn’t be a change but now have post ET MF, I had the next generation sequencing tests which showed high risk mutations, which I found VERY scary. That was about 18 months ago. I have been on various treatments since then, current Momelotinib plus hydroxy. I don’t have any particular MF symptoms, I’ve been very anaemic for a long time, which I hope the Momelotinib will help with. I am also anxious about how long I have left, and whether I will progress to leukaemia. It is a worrying disease and it’s hard not to dwell on it. Everyone seems to have a different journey so it’s hard to tell what will happen.
Having said that it’s been on the news this morning that one of our great Olympians, Sir Chris Hoy, has cancer and his prognosis is 2 to 3 years, you think wow if it can happen to an incredibly fit young man like that to get to your 70’s is an achievement. I do wonder why so many people develop cancers of varying sorts now, not long ago it was one in four people now it’s one in two. Is it something we’re eating/drinking/breathing?
Dear Lynn - I don't know what has happened but please don't leave.... I only found your post 10 minutes ago and followed through the thread only to find you are leaving the site..... very sorry to read your news and, as a fellow patient, sympathise and send you my most warm good wishes. Thinking of you - and looking out for 'Hidden', love, Sue
Think that Hidden pops up when someone has left the group. Think 2 people left the group but both posts now say Hidden as the author.. one person did not like the negativity in the post and caused them anxiety, Lynn put the original post on which was not meant to offend anyone but to get support.
sorry to hear about your diagnosis. I was 16 years CALR ET before being diagnosis with MF and that was 4 years ago. I too was in shock but actually I don’t feel much different today than I did 4 years ago and my treatment has remained the same. So I don’t think about the 80 months type statistics as they were based on people previously and I think we are doing better with specialists understanding more about our diseases today. Keeping well , active and looking after ourselves helps a lot and my specialist also spoke about very positive news on the treatment landscape for us in the next few years. Already we have two new treatments in the last 12 months alone. I suggest that if you want to speak to someone you look at MPN voice and their buddy scheme can be helpful during theses traumatic times.
Please don't leave. Many of us have bad experiences with the disease and should be able to describe those things frankly as it can help many people to not feel alone. I don't know what age you are as this is very relevant when thinking about prognosis and possible sct at some point. I progressed from ET to myelofibrosis about 3 years ago and had a prognosis of 14 years, then 9 then 3 from memory. At that point I decided on a sct but I was in my late 60s. I might have had different thoughts if I was younger
Dear Lynn. Please reinstate your account. The purpose of this forum is to allow us to freely share our fears and worries and acquire information and reassurance. I’m sorry to hear about your new diagnosis. Others here are better equipped than I am to impart information on new treatments etc. but I’m sending you a virtual hug. Judith
I totally understand why you feel a bit overwhelmed at the moment. I was diagnosed with primary Myelofibrosis 10 years ago at age 49. I did not have cll or ET previously. I have been on tablet form treatment the whole time on a drug called Ruxolitinib. This sadly has recently stopped working for me and I am on a new drug. I have been relatively lucky as I have been able to live a fairly full life, with obvious restrictions of course, since my diagnosis. There are lots of symptoms I have to manage but, if you have good support, you will cope I am sure. My advice is ask lots of questions about treatments available and what support you will receive from your medical team. No question is stupid. You can still enjoy your life with MF. You just have to be more careful, put up with certain restrictions to what you can cope doing and manage various symptoms. I wish you well.
It’s Lynn, I have rejoined the group, was just devastated that anything I had written would bring harm and anxiety to others.
All your kind words have made me realise more how delicate life and people are and that we all cope in very different ways
I will be glad to stay in the group under my new name but this experience has made me even more aware than I already was about how our words can be received.
I am glad you have rejoined. Your original post succinctly summed up how many of us feel when we progress from ET/PV to MF. It is scary and of course a shock but being part of this forum is a huge comfort so that we know we are not alone. We turn to the forum as progression is so rare thankfully - and I think that can't be emphasised too strongly to those recently diagnosed with ET/PV - that it is difficult to get accurate information. We must not though be frightened from asking for help. Naturally we all wonder about survival and you rightly note that the prognosis you were given is a median figure. I am post ET MF Calr Intermediate 2. I progressed 12 years ago and by that time had had ET since 1985 (and probably well before diagnosis). Drugs have changed immeasurably since then and now momelotinib has been added to the jak2 inhibitor family for people for whom ruxoltinib no longer works or causes too much anaemia. You are doing well on pegasus and your spleen is fine but if need be in the future good drugs are there with more in the pipeline.
Glad you made the decision to rejoin. As Grateful333 stated, you are not responsible for anybody else's feelings especially when you are struggling to come to terms with your own.
My experience with this disease is that there is a lot of generalized information out there that can be scary, but I've discovered two things with that......much of it is based on old data (we are living in the "dawn of discovery" with MPNs with so many advances that are changing previous understanding) and MPNs are essentially a spectrum disorder so we won't all have the same standardized journey & outcomes.
I've reread your original post several times and I'm not getting the same negative interpretation as was alluded to. You relayed what you were told, acknowledged that the prognosis piece was a standardized answer and asked your forum mates if any of us have specific experience in this area. You are in a period of uncertainty right now barring more information, and you are accessing your resources for support and to further your understanding. In my mind, that is what forums like this are for.
I hope you got what you were looking for thru Hunter and the others. Lots of wisdom and experience there. And I genuinely hope the other poster can find some support for themselves as well, as it seems like they are in a fearful place at the moment, which is a horrible place to be.
My dear Lynn. Please listen to all the posts and remain involved here. You are an important part of the forum and we all need to share and question as our journeys unfold. Glad to see you've rejoined. I was recently telling a patient at my oncologist's office about this website and she is going to join. Over the last few years this forum has been as important to me as my doctor. So post away - I'm a permanent fan. Wishing you good times moving forward.
Well, I wish I had help for you. This is our story
My husband was diagnosed with ET in 2013 and has been taking the hydroxeyurea and aspirin therapy. His platelets remain between 500-700. In 2021 the ET morphed into MF. He suffers with extreme anemia, no other symptoms. His weight has gone from 180 down to 158 in the past 5 years. He started getting Aranesp injections at 60 mcg monthly to every other week this year. Last week his dosage was increased to 150 mcg. His spleen has enlarged so his oncologist approved Jakafi 20 mg. Which we just started 10/17. We were so hoping for improvement. He feels even more fatigued and is starting to feel like his skeleton aches. I am assuming that is from the higher dose of Aranesp. I know it is early in seeing results. I am still hopeful. He was such a strong guy, when we moved to East Texas in 2018. He always looked so much younger than his age and now his 78 year old body appears to be 98.
Thankyou for you response, it’s a difficult journey we are on. I hope your husband has some better results going forward and gets relief from his symptoms. Sending you both the biggest hug and my best wishes. Lynn xx
Dear Lynn this is why we are all here to listen support share throw out our thoughts and get responses .
We need to hear other people’s experiences and they like life in general are good bad indifferent up and down .we need each other most when in turmoil and these thoughts wander around my head from time to time but there is no certainty about life .
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ET - Calr what is this? 
ET with CALR+ type 2
