Had appointment today with my consultant. I’ve had PV since 2014 and been on Peg since 2018 but over the last year my HGB has dropped to 100 and spleen increased to 18cm. Peg has therefore been stopped.
As a result, he told me he was pretty sure I now have MF and started to talk about Momelotinib and SCT. As you can imagine I’m absolutely devastated! I don’t actually have a BMB until Monday but he feels confident that the test will back this up.
I have very few symptoms and keep fit with 3 gym visits a week and a lot of walks. I feel incredibly overwhelmed and like I need to do something. I have a young family and that makes it even worse.
Sorry but I felt the need to offload the experience as I know how wonderfully supportive members are on this forum.
For those who have been through the same experience, if you have any advice it would be greatly appreciated.
Many thanks
Pete
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Sorry to hear the news that you may have progressed to MF. Once you have a clear diagnosis, you can make a good treatment decision. Given what you describe, momelotinib would be a logical starting point if the MF diagnosis is confirmed. It sounds like you are fortunate to have a hematologist who is familiar with MPNs. This will make a huge difference as you move forward.
It sounds like you are doing well with overall health and are in a good position to manage MF successfully should that be needed. We are fortunate that treatment options have improved and are continuing to improve.
The best advice I can give is to manage the stress of this situation effectively. We all need coping strategies that work for us. this is my list.
1. Support from my family, friends, and faith community.
2. The MPN forums (my friends and MPN Family)
3. Maintain your sense of humor and find ways to have fun no matter what.
4. Surround yourself with things that are positive and lift you up.
5. Mindfulness practices - I practice Qigong.
6. Say the Serenity Prayer every day and take it to heart!
7. Educate yourself about your condition(s). Knowledge is power.
8. Create a high-quality treatment team who you trust.
9. Advocate for yourself. Assertive patients receive higher quality care. Passive patients do not. Remember that you are in charge of your care. It is your goals, priorities and preferences that must drive your treatment. Empower yourself to deal with the MPN.
There have been some challenges with the MPN and the other related and unrelated medical issues I face. Sometimes you do, as the military folks say, have to "Embrace the Suck" or just "Suck It Up Buttercup." However - it is not all "Suck." There is plenty of good in life to enjoy and embrace. As the prayer says, I seek the serenity to accept what I cannot change, courage to change what I can, and wisdom to know the difference.
More than anything else at this point, know that you can manage this. Have confidence that you will be one of many who do manage the MPN successfully.
Please note that I have not always been so rational in managing my health issues. I learned the hard way by not managing so well at all times. After I got through a particularly nasty bout of medical issues, I swore never again. In 2018, I found out the ET had progressed to PV and over the next two years had four surgeries, including heart and brain surgery. My list of coping strategies was essential in maintaining my sanity.
Managing a MPN can certainly be a rollercoaster, and not the fun kind! We can, however, find ways to cope. Most importantly, we do not have to cope alone. Resilience is a choice as well as a set of attributes. It is a choice that we can all make. Fortunately, there is plenty of help available if we choose to access it.
Yes they are a rollercoaster, but I’ve decided to not ride it. The Besremi got me to middle of normal range. 250. I was always hovering between 650 and 900 thrombocytes before Besremi. Then the horrible debilitating side effects started. I eventually stopped in June. The thrombocytes are crawling up, ever so slowly. Watching and waiting. I knew that if I made it to the 3 years point, I might have gone into remission. It was not in my stars.
Anagrelide has done a number on my heart and still does. HU gave me devastating neurological symptoms right at the beginning. I’m trying to bridge over the time till the next med appears. Meanwhile, I’m doing most of the points that Hunter suggests above and alternative supplements. I’ve have started a charity which is giving me great joy and fun.
Don’t waste a single day worrying. It’s not worth it! 👍
Having just been diagnosed with MF I would just like to say Thankyou Hunter for your ongoing advice and contribution to this wonderful and supportive group. Thankyou. Lynn
(Obviously no need to apologise for offloading!). Sorry for the stress of the news of possible progression; but try not to assume the most extreme scenario (easy to say and harder to do I know). Wishing you all the best for Monday’s BMB.
After managing ET for 10 years I was diagnosed with post ET MF 2 years ago, I'm only 44.
My advice, learn all you can about your disease and how it effects you. Once you get your BMB results back, work with your specialist on what the best treatment options are.
For me that was trying a jak inhibitor (momelotinib, rux etc). Some people respond well to them and have many good years.
When the Jak inhibitor option fails, if suitable, try a clinical trial.
When those options are exhausted, prepare for a stem cell transplant.
I'm currently preparing for a transplant.
Research and learn as much as you are comfortable with. Some people need all the details others don't.
If possible see a psychologist with experience treating patients with blood cancers.
I’ve been told a lot of things, ie that my daughter had systemic lupus at 2.5 years. The doctor didn’t wait for the completed tests. After that, we knew she had Rheumatism and also in the eyes. I was devastated thinking 1/2 these kids don’t make it to puberty. 1,5 years of fear and instability until we changed her diet and all went away. 4 years later normal diet and Rheuma again then diet and nothing she’s now 20 🙂
Please wait til Monday. Do your wonderful gym days enjoy the weekend and Monday you’ll know more. Then you continue as till today, looking for answers, but with a good MPN specialist, who is a team player and will support you best. Most importantly is, you feel well.
You will learn so much getting support here from our friends! There are some who were able to turn second stage MF to fist stage. Anti inflammatory diet, exercise, etc Trust in the future. Don’t get stuck in the moment. You family will be fine.
This is a similar reply I have just posted for MadgeMc but it is equally relevant as you both seem to be at the same stage.
I was diagnosed with primary Myelofibrosis 14 years ago, aged 58. I was ir2 (intermediate risk 2) with level 4 marrow scarring. I wanted SCT asap, as I didn’t like what was going on inside of me. It was different then as Rux and other recently produced helpful drugs were not available to complicate the decision. Those available at the time were not highly rated.
Success rate then was 60% with a 20% chance of relapse and 20% mortality rate. Nearing what then was near the upper age limit I chose to go ahead.
Hunter and others make some good points. You need to do your research with the transplant team
It’s a tough ride and you need to be reasonably fit, without other ailments, going in. SCT is not suitable for all and indeed not all can find a suitable match.
Like many here, happy to chat or answer any questions as you move forward. Good luck.
I was in a similar position in 2021/2022. You need to find your DIPSS or similar score. This gives you an estimate of survival time. I was about 69 so a score of 14 more years was fine; not so when they calculated 3 years. If you read my posts from 2022 you'll see the turmoil I was in deciding whether to go for a sct or not. My 2 year sct anniversary is coming up in January. The first 6-8 months was hard but I'm fine now. Not everybody is and the mortality rate is high.
Dear Pete, first of all it’s OK to feel downhearted about this, I think we all try to maintain a positive and balanced attitude to our MPN. Sometimes we can just feel totally overwhelmed, and that’s not a ‘mortal sin’ in the grand scheme of things. I think you should allow yourself a day or so where you let those feelings out. Share how you feel with your partner, and have a cry about it, and then start planning for the future where your fitness will be such a boon. Every good wish for the future. Paul
I’m so sorry to hear this news, but you’ve got this. Lots of people - especially young people like you - get SCT’s and are successful. This will be you! So keep your head up, and good luck.
I’m so sorry to hear this possible progression. Was there any evidence that it may have progressed with your allele burden increasing despite the treatment? Did you need phlebotomies over the past 10 years? Best of luck with the BMB, I will say a prayer for you. Hunter’s advice is spot on, I wish I was better at following it myself.
I definitely understand how you feel. When I found out my ET had transitioned to MF in 2019, I was on an emotional roller coaster for months. At the time, my doctor told me I’d need a SCT in 3-5 years. Now, here I am 5 years later, scheduling it for the spring. The fact that you are in good physical shape will work to your advantage. Keep it up.
TRUST that All Things are Possible through GOD. Let your FAITH be bigger than your fears. Worry not. Now is the time to live your Truth, your Faith. GOD knows your needs and hears you. Believe. There is only Love and You my Dears are Deserving of every moment of Great Love, Peace and Joy. Breathe and be kind to yourselves. Know that ALL is well and Good in this world. Be at Peace. Know You were guided here to read this. God’s Blessings are with you right now in this very moment. Feel them as you read this and accept that this is so. It may have been and it may still be a rocky road right now you’re having to walk on but you are Never alone and are so Loved. As is your family, pets and friends. Peace be with you and in your home.
I want to say I didn’t write this. I found it when I was saying a prayer. Someone out there in the universe wrote this. And it helps me every time I say it. Hope it helps all of you! It just helped me not feel so alone. And I found in a particular moment of when I needed it most.❤️
Hi Pete, You've already had a lot of great advice; very importantly it's from those who've already found themselves on the path that may now lie ahead for you. For myself I clearly remember, upon being told I now had MF, that my haematologist straightaway referred me to an expert at a nearby regional hospital. That was so important, as was all the follow up care. As had been said, the feeling that you're in good hands is vital. Just knowing that there are some brilliant Drs wanting you to get well matters so much.
Also, you will, I am certain, have amazing love and support from family and friends, and I'm that light looking back to that period of my life, and this may sound a bit strange, I can truly say that there were many moments which I profoundly cherish. I feel sure you will find depths of will power and courage in yourself that will amaze you, and you will find that you are so much stronger than you may now think yourself to be.
Hi Pete, sorry to hear of your current situation you must have so many questions. This site provides so much support and advice but I must admit since being diagnosed five years ago with ET it alarms me that so many seem to progress to MF. I am an anxious sole which I have managed most of my adult life with exercise and during my MPN journey it has been the only thing to keep me sane. I agree with others who have responded, friends and family support are a must and can be really useful when I want to 'milk it' and gain a bit of sympathy. It can be a struggle sometimes when the fatigue sets in and I really cannot muster the energy to wheel my beloved bike out of the garage but then I give myself a nudge and a telling off then magic seems to happen .... off we go and all the anxiety and worry has gone. So if you can try to keep your exercise routine going it will help you through this difficult time and also keep your body in good shape for what lies ahead. Very best wishes to you.
Thank you all so much for taking the time to post a message with your kind wishes, personal experiences and words of wisdom. It means a lot and helps no end. Thank goodness for this forum and all that contribute!
Apparently it takes 6 weeks for the BMB results to come back but your comments and advice will see me through I’m sure.
Hopefully you got through the BMB in good shape today, now the really hard part comes- the waiting … So start by taking it pretty easy to allow your local insult to heal. Then return to your usual activities gently.
In the meantime, I don't have any spiritual insights or lifestyle guidance to off.er
What I do have is a certain set of skills honed over a virtual lifetime of practice- in researching medical information for myself, family, friends, and my patients, here is what I found for you, even though some of this may be premature given nothing concrete is yet known about the current status of your medical condition/diagnosis.
Momelotinib expands the therapeutic armamentarium for myelofibrosis: Impact on hierarchy of treatment choices.
Abstract
The primary objective of treatment in myelofibrosis (MF) is prolongation of life, which is currently accomplished only by allogeneic hematopoietic stem cell transplantation (AHSCT). Determination of optimal timing for AHSCT is facilitated by molecular risk stratification. Non-transplant treatment options in MF are palliative in scope and include Janus kinase 2 (JAK2) inhibitors (JAKi): momelotinib (FDA approved on September 15, 2023), ruxolitinib (November 16, 2011), fedratinib (August 16, 2019), and pacritinib (February 28, 2022); all four JAKi are effective in reducing spleen size and alleviating symptoms, considered a drug class effect and attributed to their canonical JAK-STAT inhibitory mechanism of action. In addition, momelotinib exhibits erythropoietic effect, attributed to alleviation of ineffective erythropoiesis through inhibition of activin A receptor type-I (ACVR1). In transplant-ineligible or deferred patients, the order of treatment preference is based on specific symptoms and individual assessment of risk tolerance. Because of drug-induced immunosuppression and other toxicities attributed to JAKi, we prefer non-JAKi drugs as initial treatment for MF-associated anemia that is not accompanied by treatment-requiring splenomegaly or constitutional symptoms. Otherwise, it is reasonable to consider momelotinib as the first-line JAKi treatment of choice, in order to target the triad of quality-of-life offenders in MF: anemia, splenomegaly, and constitutional symptoms/cachexia. For second-line therapy, we favor ruxolitinib, over fedratinib, based on toxicity profile. Pacritinib and fedratinib provide alternative options in the presence of severe thrombocytopenia or ruxolitinib-resistance/intolerance, respectively. Splenectomy remains a viable option for drug-resistant symptomatic splenomegaly and cytopenia.
Joseph G. Jurcic, MD, discusses the benefits and limitations of several JAK inhibitors for patients with myelofibrosis.
Newer-generation JAK inhibitors are increasingly adept at controlling symptoms in patients with myelofibrosis and may recapture treatment response in patients who have progressed on prior ruxolitinib (Jakafi), according to Joseph G. Jurcic, MD.
“Using drugs that target all these particular abnormalities can result in symptom and spleen improvement, and in some, a reduction in cytokines and allelic burden,” Jurcic said in an interview with OncLive®.
In the interview, Jurcic discussed the benefits and limitations of several JAK inhibitors for patients with myelofibrosis, highlighting the treatment advances that have been made since the introduction of ruxolitinib to the treatment paradigm, considerations for the use of fedratinib (Inrebic), and the potential advantages of pacritinib (Vonjo) for patients with anemia.
For instance, Jurcic explained the clinical implications of findings from the phase 2 JAKARTA-2 trial (NCT01523171), in which 31% of patients in the intent-to-treat population who received fedratinib (n = 97) achieved a spleen volume reduction (SVR) of at least 35%. In a cohort of patients who met more stringent definitions of prior progression on ruxolitinib (n = 79), 30% achieved a SVR of at least 35%.1
Jurcic also noted key findings with pacritinib from the phase 3 PERSIST-1 trial (NCT01773187) that laid the groundwork for further use of this agent in patients with myelofibrosis. Pacritinib received FDA accelerated approval in 2022 for the treatment of patients with intermediate- or high-risk myelofibrosis with a platelet count below 50 × 109/L based on findings from PERSIST-1, the pivotal phase 3 PERSIST-2 trial (NCT02055781), and the phase 2 PAC203 trial (NCT04884191), in which the agent generated a SVR of at least 35% in 29% of patients vs 3% of those who received best available therapy (BAT), including ruxolitinib.2
Jurcic is a professor of medicine at Columbia University Medical Center, as well as the director of the Hematologic Malignancies Section in the Division of Hematology/Oncology at the Columbia University Herbert Irving Comprehensive Cancer Center, both in New York, New York.
OncLive: How has the field of myelofibrosis treatment changed in recent years?
Jurcic: [Myelofibrosis] is a major field in malignant hematology. Myeloproliferative neoplasms [MPNs] are fairly common, [including] primary myelofibrosis and myelofibrosis arising from the other MPNs. It’s also an exciting time because several new therapeutics have been licensed in the past few years. Putting all these factors into perspective is important for oncologists.
What unmet needs exist for patients with myelofibrosis?
The major issue in treating myelofibrosis is symptom control. Patients will develop extreme fatigue due to cytokines. They’ll also develop significant splenomegaly, which can result in multiple symptoms, including anorexia. These are the major issues we’re dealing with on a day-to-day basis. However, we also have to think about the long term and how [to] improve survival of patients with myelofibrosis. Those are the major issues: symptom control, controlling splenomegaly, helping with anemia, and ultimately, prolonging survival.
How did findings from the phase 3 COMFORT-I trial (NCT00952289) of ruxolitinib in patients with myelofibrosis establish a foundation for further investigation of JAK inhibitors in this patient population?
Ruxolitinib was the first FDA-approved JAK inhibitor. [COMFORT-I] was a major study [that led to] a major breakthrough in the treatment of [patients with myelofibrosis], in that [it showed that ruxolitinib] could reduce spleen volume in a large number of patients and result in symptom control or improvement. However, one of the problems with [ruxolitinib] is that it causes significant myelosuppression, so it could worsen anemia, which is [a symptom] that many patients with myelofibrosis suffer from. It can also worsen thrombocytopenia. [Ruxolitinib] had some drawbacks, [and] there was a need for more agents.
What did the phase 3 JAKARTA (NCT01437787) and JAKARTA-2 trials elucidate about the use of fedratinib in patients with myelofibrosis?
[These trials evaluated] fedratinib [and had] similar findings [compared with COMFORT-I] in that [they found that this agent] could reduce spleen volume and symptoms. Where fedratinib fits best [into the myelofibrosis treatment paradigm] is for patients who have been on ruxolitinib and have lost their response, because fedratinib has also been shown to recapture those responses. Its major drawback [is its association with] myelosuppression. You need to be careful about this. I would be reluctant to use fedratinib in patients who have severe anemia and severe thrombocytopenia.
How does pacritinib fit into the myelofibrosis treatment paradigm, based on findings from the PERSIST-1 trial?
PERSIST-1 evaluated pacritinib vs BAT. [Similar to the other JAK inhibitors, pacritinib induced] SVR and improvement in symptoms. Pacritinib doesn’t seem to worsen thrombocytopenia, so for patients with significant thrombocytopenia, I reach for pacritinib. [Also, interestingly, pacritinib] inhibits the ACVR1 gene, which is involved in anemia, [indicating that pacritinib may] improve anemia. That may be a potential advantage of pacritinib as well.
You're totally welcome- I just hoped it hadn't been too overwhelming.
I'm glad the BMB wasn't too big of an ordeal for you.
People's reports of their experiences here w/ BMB have run the gamut.
As for mine, I didn't hate it and wouldn't hesitate to go through another one, but I will remember to take the following day off- the prone positioning was for me the worst part.
Once you start to get more concrete information from your BMB results, we/I might be able to give you some more individually-directed information.
I did have a question though- you said that your HGB had dropped to 100- did you perhaps mean your platelets?
Also, what was your spleen's size before it increased to 18 CM? ...and over what timeframe did the increase occur?
The nurse who did my BMB has been doing the procedure for 35 years and was understandably very confident, so I think that has a lot to do with a more comfortable experience.
I did mean HGB at 100, platelets are pretty constant at around 300. Spleen wise, it was around 17cm in December, so 1cm in just less than a year.
Wow, I must have been assuming wrong- if that's in units of gm/dl, your Hgb was terribly high.
My highest levels [before treatment] were in the upper 60's and the MD's were quite concerned even at that level. At last check they were lower normal on 500 mcg Besremi every 2 weeks [11.50 12.5 gm/dl, where normal is 12.0-16.9 gm/dl].
I am so sorry to read your post and have ET so can’t advise on your condition…. It is good to get it all out and know what, you may be overthinking and things may pan out in your favour… virtual hugs from Ireland…. Be strong…. Be YOU
Hi Pete, I'm so sorry that you may have MF. Some people find BMB are a piece cake. They don't experience any pain worth reporting. However, some of us do experience considerable pain. I am one of those and I've had 5 BMBs over the years. First time was horrible and the doctor had difficulty getting the samples required. After that I insist on gas. If you choose this then make sure the nurse starts you on the gas before the BMB is started. I just need a few breaths of the gas to settle into a normal breathing rhythm before the procedure is started. Otherwise it all goes pear shaped. The gas works very well for me and the effects wear off quite quickly. Just make sure that you take time to have a drink and a rest before you leave.
Hello Pete. Sorry about your situation. As your post covers a couple of facets of life i wanted to ask what sort of advice would help most for you? Practical advice, dealing with physicality of the disease. Emotional advice, how to manage uncomfortable feelings? Or advice on comming to terms with the whole scope of things. The 'why me' aspect. I have noticed that people have strengths and weaknesses in the way they cope. So it best i ask before just rattling away my thoughts. I dont have all the answers but im particularly interested in being a support for other men on the forum. Its easier to understand the troubling thoughts of each other.
well the b is done ( ouch but done) hope it went ok.
just a brief message to say spleen increasing / drugs not working doesnt always mean mf.
to my surprise this year a bmb done for a drug trial ( i failed badly) showed i am
not heading into mf when i had prepared myself for that news, despite spleen size increasing/ drugs not working so well. it all seemed so likely. but was not.
so try and hang on until the info is there .
these mpns are a rollercoaster , and always have been but the options re treatments etc is so so much better than 20 odd years ago.
Thank you. The BMB was fine and quick for me fortunately. It’s great to hear others experiences so thank you. I’ll prepare for the wait now and try and remain as positive as possible.
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Peginterferon /Besremi and Anxiety issues 
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Update on vitamin 12 and my treatment plan for PV diagnosed in December 2022
