As of today I have been diagnosed with Post ET Myleofibrosis intermediate Phase 2, JAK 2 Negative, CAL R Positive. The probability is that I was at the Pre Fibrotic stage of MF when I was diagnosed with ET 10 years ago, 2008.
It is a very sad day but I am compelled to not let this disorder get the better of me. I have too much to live for.
My Haematologist was very kind and spent a lot of time discussing my many questions, many of which I have learnt to ask through this forum and MPN voice website.
I have also been granted a Second opinion and for that I will be seeing a Professor Adam Mead at Oxford University. In the mean Time I would be very interested to hear from anyone that could suggest any questions I should have for him regarding my new challenge in life - Myleofibrosis. I also would like to know whether I should start with Interferon Alpha or Hydroxycarbamide.
I would welcome any support, guidance and advice in my next chapter of my MPN journey.
Wishing wellness to all
Paul
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SP-2008
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Hi Paul I have ET and at this stage no experience of what you are going through, so cannot offer any advice. However, I just wanted to say how sorry I am to hear your news - it’s what we all dread hearing. I’m sure others on here will be able to help and it sounds like you have a good haematologist. I hope your appointment for the second opinion goes well. Keep us updated. Best wishes, Karen
I am so sorry to hear your news, it is a great worry for you. I can’t offer any advice, I currently have ET diagnosed 2 years ago, but I am under Adam Mead at Oxford and he is an excellent consultant who is an MPN specialist who has spoken at our conferences, so you will be in good hands. Good luck! Tessa
Type 1 means higher risk of early progression to MF but prognosis then much better. Type 1 a good mutation to have.
I’ve seen Prof Mead, I assume he will give you Myeloid Panel Test to check whether any ‘bad’ mutations such as ASLX1 which could require earlier SCT.
I posted yesterday re PV treatment options with link to Ropeginterferon. Suggest you watch since Interferon has been shown to help (ie possibly slow down) progression in early MF. You could start Pegasys now and hope to switch to Ropeg next year.
The alternative is Ruxo. I posted a couple of days ago about how this can slow progression. However Ruxo is normally used for symptom control and for some, only effective for several years. Hence since your prognosis is hopefully good (CALR1), you might want to hold this back until you need it. Obviously Prof Mead and your Hem will advise.
Do you have any symptoms - spleen, WBC, bone pain?
Hopefully you sound early stage? Hence have lots of time to wait for new drugs that are coming over next five years....... I’ve seen several presentations which say within five years there will be new drugs to delay progression another 5 - 7 years and so on. Hence can hope to live near normal life span.
Hi Paul, thank you for your informative details, I will look up some of your posts with great interest. I know I need to become my advocate and hope to understand my condition as well as many of you do in this forum. I do have an array of symptoms that are more frustrating than debilitating. I normally see improvements in these when I exercise, which I do 3 times a week when I can. I will be very interested in your exercise and diet regime.
I will update the forum once I have any further updates.
Are you taking any drugs? Just read your Summary and hadn’t realised that you are suffering from a significant symptom burden. Ruxo has proven a wonder drug for many other MF sufferers and should reduce your spleen/abdominal discomfort. Has your regular Hem not suggested starting this?
You are an interesting mix, JAK2 -ve and CALR+ve. Do you know whether CALR Type 1 or 2?
As I said earlier, there is a low chance that you have additional ‘bad’ mutations which may persuade you to consider early STC. Hopefully you should not have any (I assume Prof Mead will test you, I think he does 31 panel Myeloid Test) and then it’s a question of to what extent your CALR mutation, assuming Type 1 improves your prognosis.
Prof Mead is a MF expert so you are in good hands.
My regular Hem is a SCT specialist (by chance) and the impression I get from him is if you need a STC, don’t wait too long. Survival odds are pretty good, he quotes 80 - 85% if unrelated donor and 90% related. However I think the more advanced your MF, the greater the risk of relapse.
Normally patients at Int 2 would be pondering when to bite the bullet but as I said, I don’t know to what extent your CALR slows progression to next stage. Prof Mead should earn his money here!
SCT is a daunting prospect hanging over all of us but it does offer a decent chance of a symptom free cure, although there is the hurdle of GVHD to overcome.
Hi Paul, I did mention both Ruxo and Interferon, as my haematologist offered Hydroxycarbamide. My haematologist felt that we did not need to start Ruxo just yet, but was happy to offer interferon Alpha as I am young. I will discuss this again with Prof Mead when I meet him. I did forget to ask if I am Cal r 1 or 2, so I’ve put a call in to find out.
The interesting thing is when my case was discussed between specialist and Haematologist, at their weekly meeting, they were divided on whether to start me on medication or not as I am border line level 1/ level 2 scarring. Professor Mead voted for starting medication along with my Haematologist. I will look for more clarification around this when I meet with Prof Mead. I definitely have symptoms but most of them are manageable if I pace myself and have regular low impact exercise. At the moment I take 75 mg aspirin only and I am leaning towards interferon Alpha as it is replicates what we natural make in our bodies.
I’ve been told the appointment with Prof Mead should be within 4 weeks, and I should start to take medication in approximately 6 weeks. As far as I am aware all my bloods are ok bar my haemoglobin which has been stubborn and not wanting to move from 117. I should receive my haematologist letter in the post in the next few days confirming my diagnosis and hopefully that may put more light on where I am with my new condition.
Pegasys is latest version of Interferon, a weekly injection versus every couple of days for Interferon. It appears to be a bit more refined than Interferon, hence more easily tolerated and fewer side effects.
Ropeginteterferon is the next improved version, currently in trials. Injects every two weeks and better tolerated than Pegasys. Should be available next year. Could be new front line treatment.......
Intereferons offer your best chance of slowing progression but only for a sub set of users. However is can take a long time to work, perhaps up to two years to have a good molecular response (ie reduced fibrosity and Allele Burden).
I think Hems like HU because quicker working.
Your dilemma is, according to various trial results I’ve posted links to, Interferon works better for Int 1 MF (rather than Int 2) whereas Ruxo other way round! You have additional impact of (hopefully) CALR 1 which could mean slower progression and hence more time to wait for Pegasys to work.
I’m so pleased you are seeing Prof Mead, he will sort you out.
I’m about to post a chart of drug comparisons, it’s a bit old and I’ve posted before but worth a look.
Thanks Paul, your support is invaluable and gives me some good food for thought and things to discuss with Prof Mead and my Hem. I will be watching out for your posts we much interest. Atb Paul
Sorry to learn about your news of progression w/ MF.
Try not to be too sad... There are many of us here w/ MF, & no doubt we are all at slightly different places from each other, just because of our own individual uniqueness... Some people do still live long lives w/ MF...
However, there are a host of differing variables that one might have to consider...
As it happens, I am also Post ET/MF CALR+Type2, & I also possess another mutation known as ASXL1, which is supposed to suggest a more adverse outcome, (my understanding is that when one has CALR then JAK2 is negative).
Generally speaking too, a CALR mutation is usually viewed as less of a threat than most other mutation types...
When I first learned of the ASXL1 mutation's presence, I was also a tad down...
However, these days, I have determined to be far more positive about my own MPN condition, & as a result I have created my own cycling exercise/diet/ & intermittent fasting regime...
Although my doctors might disagree w/ my sentiments because they believe that it was the Ruxolitinib that has seen a significant improvement in my fibrosis level, (however, I believe that the regrading of my last BMB from Grade2 to Grade1 level of bone marrow scarring), is largely due to my new diet & exercise regime. Of course, I might be mistaken, but that is what I choose to believe...
Of course, there is a third possibility too, and that is that we are both wrong and both correct, in that both the med's & the exercise/diet are contributing factors... (?)
In any event, I am 59, was originally diagnosed in May 2016 with ET. I have had at least two (2) TIAs (minor brain strokes) that I know of (making me high risk). My platelets are still stubbornly high, (c. mid 700s-1M), and apparently that is in keeping with the CALR Type2 mutation...
Age can also play a role here...
Due to my Very higher platelet counts, I am generally more apprehensive about strokes than progression to AML, at this juncture. However, when & if a Allogenic Stem Cell Transplant (ASCT), becomes required, the fitter I am, the better my body will be to withstand the rigours of such a procedure etc...
As our bodies age, the state and condition of all our vital organs diminishes, and especially so the more sedentary we become...
Paul, I am guessing that you might have already had a Bone Marrow Biopsy (BMB), is that correct?
And you say that you are Intermediate2, and that you were originally diagnosed w/ ET in 2008...
Can they also tell you which CALR Type you are: 1 or 2?
Are your doctors aware as to whether or not you possess any other mutations? Question worth asking, in my view...
I guess I am curious to learn from you about how you might have been treated thus far w/ your medications etc?
Which ones have you been using all this time?
Will they now be starting you on Ruxolitinib?
Have you been able/unable to have a fitness regime due to your symptoms?
What does your diet consist of, and do you eat well?
How are you blood counts?
What is happening with your White blood counts, Red blood counts?
Are you platelets High or Low?
All of this can take some time to get one's head around it but it is worth asking the questions, in my view...
Personally, I feel better about my MPN, just by knowing a little more about what it all means. Hey, but we are all a tad different, and what works for one might not for another of course...
There are many fine people here w/ MF and I am sure that all will offer you their support, and answer your questions as best they may...
Have just read your reply to Paul’s blog, I have just been diagnosed with post ET/MF. Very down at the moment and it seems there is a minefield of information out there. I am currently on no meds as am waiting for BMB to find out extent of scarring. I was very interested to read about your exercise/diet regime. Are you able to give me any details of the type of exercise you do, also what type of diet is best for this condition. Also how long you would fast for. I am CALR mutation. I will continue to read your blogs with interest.
Thankyou so much for your reply, I am 45 years old with two young children, was originally diagnosed with ET in 2011. I am attending Guys and St Thomas Hospital in London which I understand is an excellent centre for this condition. I do not have any significant symptoms at the moment other than general lethargy, tiredness but with now the added burden of worry, but it has been good to find this site and read blogs like yours to give us all inspiration.
Thank you for your post, it’s very thoughtful of you to make the time for a detailed reply.
As far as I am aware my bloods are ok bar my haemoglobin, which is why I had a BMB. It was a good spot from my Haematologist, which will hopefully put me in good stead for managing symptoms and progression.
I will ask Prof Mead more questions with relation to other markers of my condition and update the forum. I’m hoping to see him within four weeks. You have done so well to reverse the scarring of your bone marrow, I would be very interested in your fitness and anti inflammatory diet regime, as I struggle with post exercise fatigue and headaches and have not come across an anti inflammatory diet before. Thank you again for you post. It means a lot. Paul
Sorry Paul, missed the part where you said Int 2 MF. As Steve says, Ruxo could be best treatment but Prof Mead will be able to decide this. As my link from several days ago says, it can slow progression and improve MF grading. CALR1 is still a good mutation to have re prognosis.
I fully endorse Steve’s logic re diet/exercise and am doing the same. Our MPNs appear to feed off inflammation so anti inflammatory diet must be helpful. There are studies ongoing into this and my bet is that both Steve and his Hem are correct!
Sorry to hear that your ET has progressed to MF as mine has. I don’t think I really have any advice to give in addition to the sound advice of previous replies. I would just say write down all your questions before you see Prof Mead otherwise you will not remember all you wanted to ask. I often jot brief notes as to the replies too so I don’t forget those! I have never had a doctor object to this procedure and when I get home I can digest all that was said and start writing down questions when I think of them for the next appointment. I hope you get some answers and as you see you will get plenty of support here although we are only participants in similar journeys, not doctors!
Thank you Jan, your advice for making notes as and when I think of questions is a valid one which I will follow, as I am quite good at forgetting! 🙃 I find this forum amazing, people are so friendly and happy to help. ATB, Paul
It sounds as though you have a great, supportive haematologist. I wish you all the very best. Good luck with your second opinion. All good wishes Penelope
Thanks Penelope, I recently moved hospitals so I was nearer to home. I was a bit worried about changing my haematologist but I’m so pleased I have now, Dr Akkani has been really good 😊ATB, Paul
You're so lucky. I feel worse rather than better when I visit mine. He seems determined not to help me. What were your symptom that made the haematologist investigate further? Love and Hugs P
Hi Penelope, I’m so sorry your not finding your Hem helpful, that’s just not what you need. Is there not another Hem you could be transferred to ?
The main reason for my Hem investigating was down to my Haemoglobin not reaching the reference range for several months, it was staying at 117 ish. My last Hem just told me to supplement with iron tablets and eat red meat.
Mine was very suspicious when I asked this time if I could have copies of my bloods and of the report concerning my diagnosis. He told me I know them. Not sure how he deduced that. I haven't even been given a report on my existing condition. In March, having been sent an appointment to the MacMillan Windmill ward, my follow up haem said I have ET and JAK2 mutation and I would be transferred to another. All he has done is prescribe hydroxy and be objectionable when I had the audacity to stop taking it when I had a bad reaction to it. Thank you. All the best and I hope you find some well earned support yourself, which results in an upturn in your own health. Sending you lots of love. P
Hi Wyebird, it is difficult and I’m still telling family, so far everyone has taken it well, they just want to help with whatever I need. I hope your MPN is being good to you. Paul
Hi SP-2008,try to keep positive & strong. I know its easier said than done but as a lot of people have said on here already,more & more knowledge on this terrible condition is being discovered all the time. I wish i could just send you & your family a fraction of the inner strengh i have gained over the years but that is impossible,the only thing i can send you are my very best wishes & wish you well on your mpn journey paul. Atb tina🤗
Thank you Tina, I do feel compelled to not let this condition get the better of me. Just your kind words and good wishes make me feel better. Thank you, Paul 😊x
Hi Paul, I’m sorry to hear about your change in diagnosis but you have an admirable approach to this. One suggestion I have is you try & take someone along to your appointment who can make sure you’ve asked all your questions (I give my husband a copy) & more importantly is to take notes. Such great news about your meeting with Dr Mead. Good luck & keep us posted please. Katie
Thanks Katie, your spot on with taking someone with you and letting them have a list of the questions. I missed a few questions at my last appointment. So great advice. Thank you, Paul
Thoughts are with you Paul. I can’t imagine what it’s like to hear this news. Progression is what we’re all concerned about.
I do hope that when you begin treatment you are able to regain a better quality of life. It must be comforting for you to know that you’re in the hand of an MPN expert who will strive to achieve this.
Hi Mary, the original Trainee Hem whom carried out my BMB said to me that I probably had MF as she was unable to get a BM Sample, and then due to holidays etc, I had 6 weeks to understand my symptoms better, and once I had I had resigned myself to having MF, so no shock when I received my diagnosis, just sadness but very quickly followed by a desire to understand my new condition and give myself the best chance to slow it’s progress. Unfortunately every now and then the grey cloud does come out, but I am determined not to let it get me down. Thank you for your kind thoughts and I wish well. Paul
I too have just be diagnosed Post ET/MF JAK2 negative CAL R positive. I am 45 yeats of age with two young children. Am waiting for BMB to find out the extent of scarring. I am being treated at Guys and St Thomas hospital London and so far am not on any meds. Apart from severe tiredness I have few symptoms as yet but I am finding it hard to accept my diagnosis and carry on with life as normal. Whilst I realise we are all at different stages of this very cruel disease, any information you could pass onto me would be appreciated.
That is the news we all dread to receive. You must be so worried.
It sounds as though you are in good hands, with a specialists who is keen to think and learn and find the best treatment for you, even sending you to another doctor who may know more.
You do your bit by knowing as much as you can about your illness and how to live as well as you can with it.
From the separate link I posted last night re impact of Interferon on MF:
‘The type of driver mutation had a statistically significant impact on survival: the median overall survival was 13.5 years for CALR-mutated patients compared to 7 years for JAK2-mutated patients (P<0.0001)‘
Just wanted to say that Adam Mead and his team are amazing. He was my mum’s consultant. (She had PRV and then MF). My mum was in hydroxycarbonide for 10 years with her PRV but then had to come off it when her diagnosis changed to MF. She was then put on Ruxilitinib.
Thank you Hope47. I’m so sorry to read you lost your mum to MF. I hope you will find some peace in knowing she is now at peace. Stay well and thank you for your kind words. Paul x
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