A recent Bone Marrow has shown what I believe to be early stage Myelofibrosis. Next appt in two weeks. Has anyone got any advice on any questions I should be asking re treatment etc. I have had ET Calr for 17 years. Not quite sure what to think or feel just now.
I am also being investigated for auto immune issues due to abnormal bloods, this is taking forever due to the difficulties within the NHS.
Currently on Pegasys fortnightly, due to shortages have agreed to take a larger dose monthly. Platelets well controlled on 45 fortnightly.
Ant advice or information would be greatly received.
Thankyou Lynn
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lindyloulou
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I am curious what age you were diagnosed? I too have ET CALR. 17 years is a good run with no progression!
At any rate, there are a lot of good treatment options for MF. Several jak inhibitors and even a couple clinical trials for the CALR antibodies which could potentially be curative. You will have options and hopefully they work well for many years. 🙏🏽
I was 50 when diagnosed, am lucky I have got this far without progression. Still comes as quite a shock, guess you always hope your the lucky one that does not progress. Not totally surprised but still shocked. Fingers crossed for good care and treatment.
I know where you’re coming from and every chance you will make 17 and more. I can almost guarantee when you get to 17 years you will hope for many more. Take care and think positive.
I am similar to you. I was diagnosed with ET when I was 52 in 2008. But I got diagnosed with myelofibrosis in 2019. I am now being considered for transplant.
Thankyou so much for your response, I hope all goes well for you, I was also born in 1956, found raised platelets on a routine blood test then ET following a biopsy. It is good to know that our age group are still considered for these treatments. Thankyou.
Thankyou for your response, I am so glad you are feeling better than you were and hope you continue to do so. It’s comforting to know about others journeys, makes the situation feel a little better. Thanks.
I was 40 when diagnosed with ET after a BMB. I was immediately put on HU as my platelets were so high. The consultant said I was a very lucky lady not to have had a stroke or HA. In those days Asprin wasn’t considered. I took HU well for 23 years then it stopped lowering my platelets in 2019. I was put on Anegralide and after 6 months had a HA as I was intolerant. The consultant at the time who was not an MPN specialist did not listen to me and told me to carry on taking it. After 6 months I had the HA. I then requested to move to an MPN specialist and since then I have been on Peg IFN and asprin. In May this year my condition progressed to PCV. I get terrible bone pain and fatigue- but am alive! I hope they can sort you out. I am now 67.
I forgot to add that I have Sjögrens Syndrome ( an auto immune disease) that also gives dry eyes and sight issues, also dry mouth for which I have been given sprays. My rheumatologist has now referred me back to GP as she has diagnosed SJ but GPs are not the experts sadly! Do you think I should get a referral to St Thomas’s? I live near Liverpool so it’s a sleep but would consider going if I could get any further help? My specialist is an MPN experts d at the Cancer Clatterbridge Centre in the city but I have a feeling St Thomas’s has more resources.
I am no expert on this but if you have any concerns ask questions, I think from what others say if your consultant is on board you can have shared care between Guys and your current consultant. May I ask what criteria your Rheum used to confirm you SJ diagnosis, I am struggling to get a diagnosis based on symptoms alone. Take care Lynn
Just blood tests from what I remember. I was diagnosed 5/6 years ago. I had been under an ophthalmologist who did some private work for me ( vari focal lens replacement) at a local private clinic in Liverpool.Dr Illango is an expert in his field and is also in charge of Wolverhampton eye Hospital. My eyes were so dry and after a lot of visits to his private clinic he decided to put me back to the NHS to keep my costs down- and he took me on in Wolverhampton- which was my suggestion to him as we used to live for a spell in the Midlands. After about 3 x 6 monthly visits , & numerous treatments to my eyes he decided to refer me to a rheumatologist in Liverpool be because he suspected SJ ! The specialist there suspected this initially and I saw her over a period of 2 years. Once this was finally confirmed I discussed with Dr Illango that I didn’t need to see him again - I left his care eternally grateful that he had been so good in looking outside the box.
Sorry to ask again but what criteria did your rheumatologist use to make your diagnosis ie tests etc, I live in the midlands. Thankyou again for your posts. Lynn
A referral from my ophthalmologist I presume? When I saw her she did bloods she told me she wasn’t sure if I had it but would know more when the blood results came back. When I returned some months later she confirmed the diagnosis. This is all I know .
Have they told you which bloods were abnormal and/or which autoimmune (A-I) issues may be of concern? Do you have any symptoms of note there? Symptoms are as important as blood tests. Have you scheduled a rheumatologist?
You've likely seen the posts on A-I with IFN. This can be time sensitive. Is there a way to get the result faster, private maybe?
It was actually for lindy. But I see here you have a Dx of Sjo, currently limited to dry eyes, is that right ? (no excess fatigue or pain, neurological, GI, dysautonomia, weakness, breathing trouble, cardio/kidney issues...) You are fortunate to have the formal Dx, Drs resist this Dx for mysterious reasons, sometimes for years.
In a recent post you were considering the risk trade off of IFN vs Rux. With Sjo, IFN is actually contra indicated for you. (Sec 4.3 of the Euro PEG label)
"History or presence of autoimmune diseases"
Also: "Patients with signs or symptoms compatible with autoimmune disorders should be evaluated carefully, and the benefit-risk of continued interferon therapy should be re-assessed"
Rux has well known risks, but IFN's worst case risks are more severe, and you're at enhanced risk of these. Moderate to severe Sjo is a dark Evil place you never want to increase the odds of entering.
You should discuss in detail this risk with both your Hem and your Rheum and re-asses as the label says. We see here the risk is real and it is happening. With the intro of Rux for PV there is an effective lower risk option for A-I pts.
Thankyou for replying, I am absolutely bogged down with symptoms and not knowing what’s causing what, I have been waiting for a lip biopsy since last November, Rheum tried to expedite but they were not having any of it. Ana and anti Ro 52 abnormal.
Currently have Opthalmology for dry eyes, eye plugs
Rheum for aches pains, dry mouth, difficulty swelling certain foods etc.
Gynae for ? Lichens,
ENT for ear pain and swollen salivary, scan shows no sign of Sjogrens, slight change in bloods to do with sarcoidosis.
Vascular surgeon, waiting for surgery on both lower legs, circulation issues.
Hospital Dentist waiting for extractions due to difficulty with anaesthetic.
Gastro for micro colitis.
Haematology who have at last organised a bone marrow biopsy which is showing Myelofibrosis. MDT next week then appt week after re treatment options.
No speciality looks at me asa whole rather than the bit they specialise in. All my consultants are now at the same hospital so hopefully this will help
Really not sure whether I am coming or going. Symptoms are too numerous to mention.
Latest Haem was first to say symptoms could be down to progression. This is after many years of me reporting different symptoms which were dismissed as anything linked with ET progression or treatment by every doctor I saw.
Not sure whether I am coming or going.
Now transferred to another hospital who run MPN clinics and have a MPN specialist, first visit last Friday, if no joy it really is time to go to Guys.
Sorry it’s lengthy but it is complex and lengthy.
My body is doing strange things in terms of pain, general weakness etc which I have no answers for as yet.
If you've not seen it yet, please read my post "Last Dose", you'll see why I try so to help others here to avoid my life splat.
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I see your post last year where you said "I am currently off Pegasys due to the pains I am experiencing which are actually worse than when I was taking it." I replied there and so some here may be redundant.
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You have all the signs of well established Sjogren's. Drs often resist this Dx for some reason and may pts have to fight for it, sometimes for years. But your case is not borderline. Your Rheum suspects it with the biopsy order, but a negative result may not negate all the other indicators. You do have other conditions but Sjo is almost certainly a top contributor to your troubles. The powerful indicators are: Ro52 (I have this too, vs Ro60) Ro52 without Ro60 puts us on a potentially more aggressive Sjo path, (I am) and we need to watch for lung involvement amongst all the other stuff. Dry mouth with difficulty swallowing. Dental complications to the point of extractions. Dry eyes at a stage requiring plugs. Swollen salivary. You note Gynae, dryness here is Sjo Sx. Less specific but still Sjo stuff: ANA+, Aches, vascular, gastro. Fatigue too is the top Sjo complaint, but MPN can have that too.
"scan shows no sign of Sjogrens" does not rule out Sjo at all, I had same. This can indicate early or advance Sjo, or nothing at all. In fact much of everything above can be negative in the presence of Sjo, but your results are a clear Dx in my opinion.
Your comment "I am absolutely bogged down with symptoms and not knowing what’s causing what" and "No speciality looks at me as a whole rather than the bit they specialise in" - doesn't Dx anything but is a classic Sjo experience. Most MPN pts don't go thru this extreme.
"My body is doing strange things in terms of pain, general weakness etc which I have no answers for as yet." Sjo fits it all too well.
I've found there are very few Sjo experts, far fewer than MPN experts, and most focus on just the dryness part. So we have trouble getting sympathetic care from the long list of Drs we require.
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IFN can cause or increase existing auto immune issues. This is one of the severe warnings on the label. And each dose after the autoimmune (A-I) presents increases the hazard. Mine was like an on-off switch, no last dose, no Sjo. Sjo is one of the worst case A-Is connected to IFN, and we are seeing it here. Many Drs are not looking for A-I emergence on IFN so we need to watch for each other.
Ask your Rheum about pioicarpine and Cevimeline. These help many Sjo pts with dry mouth. Also ask about HCQ, it is a mild drug that helps some Sjo pts with pain and fatigue. Also consider joining Smartpatients forum. You will find compassion and support just like here.
Some good news is there are effective treatments in late trials, after years of none. And promising cell therapies that are looking crazy good for certain A-Is that could be curative.
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Regarding cell therapies, have you inquired on any trials available for your CALR? There are some going on now.
Thankyou so much for your advice and time. You info is extremely helpful, I will be going through the replies to my post and writing notes for my next appt.
Thank you, thank you for such an informative reply. While I don't have any of the issues Lin is facing, your post is very helpful to all of us. Be well
Hi, sorry that you are having such a bad time , it seems we have to conduct our own orchestra. I feel very strongly about a point you made, know one seems to join things up, in haematology there is no holistic approach. At appointments, rattling off a load of side effects to drug produces no response. Like others on here I have been advised to double my dose of Pegasys monthly instead of every two weeks to avoid waste, after EP guys experience I am terrified to do this. We end up spending so much of our time trying to sort it all out that we struggle to enjoy normal life, I cycle a lot and it's the only activity that allows me to switch off for a while, heaven knows what I will do if that comes to an end. Good luck with everything...always best to have a plan.
Thankyou for your response, I used to work in an out patients department some years ago and at that time we had an integrated medicine consultant who did look at all aspects of a persons health, since having all theses specialities I guess they know more about one subject but next to nothing about others, this really does make us our own advocates on subjects beyond our own knowledge, it’s crazy. Thank you for your kind words and keep up the cycling. X
Have been on 45mcg Peg for almost a year and have noticed, low mood, increase in aches and pains, dizzy now and then increase in palpitations (get them on and off anyway), increase in dry eyes which I have suffered for a number of years. All of this is making me feel anxious which I think triggers the palpitations although the haematologists is writing to my GP to request and ECG...just to make sure. Oh yes and a bit more hair thinning, as if hydroxycarbomide didn't cause enough and I am still taking one of those every other day as well as the Peg and a few headaches now and then which I never used to get. Speaking to the specialist MPN nurse today so I hope this helps to reduce the anxiety. Thanks for asking .
You are having quite a time of it, Dearheart. Guys sounds like a great path forward. I pray that you get real answers soon and that they bring you peace.
I was diagnosed with ET at 57, then MF at 70, last year. I’m currently on Momelotinib, just over 2 weeks, I’ll find out if it’s helped with the anaemia this week. I was told the cut off age for transplant in the UK is 70, so if you’re considering that you need to get it done in the next couple of years.
You’ve got a few health issues which probably affect each other but you’re right that hospital doctors never consider anything except their own specialty.
It’s relatively easy to be referred to Guys, my local team have been more attentive since I have had advice/care from them.
Questions to ask at mpn meeting based on my experience of progressing to MF from ET about 4 years ago. They gave me life expectancy of 13 then 9 then 3 years. Eventually at 69 they really thought I should have a sct which I had aged 70. There are a number of tools online to help predict life expectancy, eg DIPSS. The next question is "what will my quality of life be as MF progresses" it may be with your mutations the outlook is not bad
I am interested in whether anyone has got a satisfactory answer to the question "what will my quality of life be as MF progresses"?
After diagnosis, getting a prognosis out of the health care professionals has been difficult - for me at least.
I finally got one number (x% chance of a 5 year survival) but my own research (using the on-line tools mentioned above for DIPSS - qxmd.com/calculate/calculat... and MIPSS70 - mipss70score.it/ as well as those summarized in "Primary myelofibrosis: 2023 update on diagnosis, risk-stratification, and management" Ayalew Tefferi - pubmed.ncbi.nlm.nih.gov/366.... This gave me a much better picture of the probabilities and the range of estimates from the different scoring systems.
But with this information, the quality of life question is one that I found doctors ducked entirely or prevaricated over. One specialist suggested that the answer was as random as predicting the stock market movements on a day to day basis. This I did not accept.
I read the rather limited literature on Myelofibrosis progression and only got the x% die from y (e.g. "Cause-specific mortality following polycythemia vera, essential thrombocythemia, and primary myelofibrosis in the U.S. population, 2001-2017" Graça M. Dores et. al. - ncbi.nlm.nih.gov/pmc/articl...
It was only when I asked a doctor what is likely to kill me? And quoted from a medical journal on how Myelofibrosis progresses to critical illnesses: “transformation to acute leukemia, thrombohemorrhagic events, organ failure, and infections”. When I asked what do each of these involve in terms of management/treatment, then I got a sense of why it was tough to get an answer. I was told that you can’t predict accurately because each possibility has such a range of outcomes.
For infections it could be a mild flu, sore throat or it could be sepsis or septic shock leading to death. Low red blood cells could cause you to feel tired or give you a heart attack. Low platelets could cause a nosebleed or mean that you bleed to death. Blood clots could cause pain or discomfort or a massive pulmonary clot and death. The only thing the medical profession seemed to agree on was that transition to AML was not good.
But I still don't understand "what will my quality of life be as MF progresses". I have been tracking my symptoms daily for four months but that does not seem to help doctors narrow things down for answering the quality of life question for me. Will I spend x years of my y years remaining in a hospital fighting a disease? Will my bone pain continue to increase or will the other symptoms currently suppressed by Jakavi return as Jakavi stops working in a year or so? Doctors shrug and say "I don't know".
Given the idiosyncrasies of the disease, I have looked and patient stories (thepatientstory.com/patient... which are helpful in understanding what the medical profession is facing in terms of the range of symptoms and experiences.
I think I have come to terms knowing when (or at least a statistical estimate of when) but not how. The doctors don't want to answer the quality of life question for Myelofibrosis because the range of outcomes is so large and there is so little information they can use to predict what we will experience. They can use our symptoms, blood counts, and mutations to help estimate how long we will live but there is little to help them predict how we will live.
Having said all that, I still think we should ask the question. And for those who have asked the question and got an answer, I'd be interested in the answers you got.
I wanted to know just that before I agreed to a sct. At that point I felt reasonably fine and wasn't having transfusions. I was on fedratinib. My life expectancy had dropped to about 3 years. I got a lot of pleasure taking my friend's dogs out for country walks of about 1 hr 30 mins. The transplant doc said probably only another 18 months of being fit enough to do that. That was the straw that pushed me towards a sct
Scaredy_cat perhaps your approach is the way to go. That is, to ask about a specific activity or two that add to your quality of life. Doctors may be better able to respond. I'll give it a go at my upcoming appointments.
In connection with EP Guys post, I had to stop the peg due to it affecting my mind. Blew it completely actually. Have been in a very bad place but coming out slowly now. I have never had mental health problems but it caused terrible anxiety and panic attacks.
The point I want to make is that I had shoulder, neck pain that went into the head if I coughed. Only after stopping the peg did we realise that this was connected to the peg. Thankfully, touch wood, that pain has gone. I consider the pain a symptom of what was brewing up.
Peg has been great in the past but it comes with a warning. I would speak to your haematologist and suggest stopping it for a while to see what happens, although I guess if you are going to take it once a month, in between you might notice if your symptoms are connected. I hope you get it all sorted. It sounds like you are going through a very difficult time. Let us know how you get on
Thankyou for your reply, I have stopped the peg twice before and the symptoms if anything got worse. May need to change to Rux now due to Myelofibrosis. I guess these meds don’t suit us all, at the moment I am not sure what’s causing what, aholefully the future will bring clearer answers. The time it takes to get the diagnosis is key now. Thanks again. I hope you continue to improve. Will keep you posted as and when I have any news. Thankyou
Hello Lindyloulou. I am so sorry you're having such a hard time. I just wanted to say that Rux was a complete game changer for me in terms of quality of life. It really dealt with all my MF symptoms without too many side effects. I was on different dosages for about 10 years to allow for the anaemia it could and did cause. It has now stopped working and I have been on Fedratinib for about 18 months but this has not worked so well so will be changing to Momelotinib in the near future. My quality of life is gradually less good than it was. I can do less but I am 71 so must expect some reduction in the stuff I can do without feeling very tired. I am also having 6 weekly transfusions but this may stop on Momelotinib. I was originally diagnosed in 1991, so have to say I have had a good innings. So please don't despair. Rux may work well for you. Saying that I know that our journeys are all different. All best wishes to you for the future. Hilary
Thankyou for those kind words of encouragement. Currently my bloods are all normal on Pegasys. It’s all the strange symptoms that led to this last biopsy. I should know more after the recent blood tests, MDT meeting and my next appt. Fingers crossed for both of us. Stay well X
Peg is contraindicated for people with auto diseases, and, as EP guys points out, it can also cause autoimmune issues such as Sjogrens. Autoimmune issues (Sjogrens, sarcoidosis and others) can also cause bone marrow fibrosis on their own without the presence ET or PV.
In my case the combination of peg with previously undiagnosed autoimmune disease led to symptoms like those EP guy and you describe, such as dry eyes, neuropathy etc. It even caused sarcoidosis. The result of this has been that I went from grade one bone marrow fibrosis before starting Peg to grade 3 with blasts in the space of two years. If properly managed such a change would take much longer. Now I need a SCT.
Higher doses of Peg, even if spread out, may make you AI symptoms worse. You should discuss the impact of the AI issues on bone marrow fibrosis with your Dr to make sure you do not make matters worse than they need to be.
Thankyou for your advice, I take on board what you’re saying, have a lot to try and make sense of and it’s so difficult to get anyone to commit to any cause of anything. Good point about the peg, I only have 8 weeks of this left so something has to be done re treatment anyway due to shortages, I will not take the bigger dose until I have that conversation. Thankyou, I had read about an Autoimmune Myelofibrosis,I am really trying to understand all of this and work out the best way forward. Just hope everyone involved starts to listen.
That is a jolt to see the correlation of A-I and MF. Your progression does seem unusually fast. Did your blood counts suggest another BMB check?
The article you cited a while ago had "Autoimmunity is involved in a small subset of patients with marrow fibrosis". It could be MPN pts are more likely to be in that subset, but the summary does not get to that detail.
Your comment there: "Those of us taking interferons are finding out that multiple rare events are not that rare." is looking too accurate. Infrequent but regular reports here are happening. Notably, my final blow up was triggered by a reduced IFN dose. Hence my message to act fast.
Problem in my case is I had no A-I history, and no family history at all so no reason to think about A-I.
Have you received a formal Sjo Dx? Have you had the SSa test? Ro52 vs Ro60? I'm wondering if IFN associated Sjo trends to Ro52 as lindy and I have. Lung involvement is typical in Sjo, Ro52 in particular, while RA like Sx also can be in the Sjo spectrum.
Drs tend to resist a Sjo Dx to the frustration of Sjo pts, while other A-I Dx's are routine. There are exciting agents in late trials, but we don't qualify for these with our MPNs.
If you happen to get a lupus Dx, lots of great trials are available, including curative cell therapies.
I've seen Rux proposed for Sjo, but this angle is not very active, other Jak'i's are favored that go after TYK2 (Jak4). Some are in ph 3 trials.
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