Recently diagnosed with MF

[Tdg73]

After about 5 years of changing diagnoses, CML, PV, ET, my husband has been told his condition has progressed to MF, based on rapidly enlarged spleen and latest BMB. Our appointment with the consultant was a bit of a blur after the shock of her saying his life expectancy is approx 80 months, so we have a lot of unanswered questions and no follow up appointment for another 3 weeks. He has been given a form for his brother to see if he would be a match for STC so I guess they are going down that road already.

The ruxolitinib appears to have not worked for him so she wants to wean him off that and onto a new drug (name forgotten) and he has been sent info about joining a clinical trial. We are both a bit overwhelmed. I cannot get the Tim Jonze Guardian article from last year out of my mind where one minute he was being told he needed an urgent STC and the next he is back to just taking aspirin.

Any advice or shared similar experiences would be welcome.

[Cja1956]

Nobody has a crystal ball with this disease and a SCT decision is one of the hardest things to do. When I was first diagnosed with post ET MF in 2019, my doctor told me I would probably need a sct in 3-5 years. I was 63 at the time. I’m 68 now and I have consulted with 2 different transplant specialists and they both said I might miss my window of opportunity. I feel pretty good at the moment but I decided to go ahead with it. They are searching for a donor now.

My advice to you is not to panic. I was terrified to do a SCT but I also want to have a chance to see my adult kids get married and possibly have a grandchild. Also, there are more treatments available and you certainly have time before you need to make that decision.

[Tdg73 in reply to Cja1956]

Thank you for replying and good luck with your SCT.

[hunter5582]

Sorry to hear about the progression to MF. It would be a shock for anyone to hear. It is not surprising that you would both be feeling overwhelmed.

It is important to know that some people manage MF for extended periods of time. The projected lifespan for someone is a statistical projection that is based on the assessed level of risk. This varies quite widely. It can range for 15+ years for Low Risk to 2-3 years for High Risk. Usually, SCT is not considered until Intermediate 2 or High Risk. There are other factors that com into play beyond the assessed risk level. It would be best to ask detailed questions about this the next time he sees his MPN care team.

It will be important to gather more information about MF and his treatment options. This will help you both feel more confident in the treatment decisions. SCT is the only potentially curative treatment for MF. You will read that it comes with significant risks, but it also offers a potential cure. If he opts for SCT, he may well have the same outcome as Tim Jonze.

It will be important to find out what other treatment options the hematologist is considering. There are three other JAK inhibitors, Fedratinib, Pacritinib, and Momelotinib. There are also drugs in clinical trial that are not JAK inhibitors.

Wishing you both all the best.

[Tdg73 in reply to hunter5582]

Thanks hunter, our consultant did mention Intermediate / High Risk in leading to the calculation of 80 months but to be honest, I didn't really take it all in because I didn't appreciate what it meant. She did say that SCT is normally considered when life expectancy is 60 months or lower, but she would recommend my husband for consideration because he is comparatively young and healthy.

We will be better armed for our next meeting, but any more advice you can give is welcome. Thank you, I have learned so much from this forum in the last few days.

[Scaredy_cat]

Not the greatest diagnosis but having a label helps. You don't say how old your husband is or what his health is like apart from the mpn. You can read my posts from 2022 when I was in a similar position and had to make a decision about a sct. The sct MAY cure the MF but it has lots of risks and is a horrible process for several months

[Tdg73]

Hi Scaredy_cat, he's 62 and otherwise fit and healthy. It's the weighing up the risk process that I find so daunting, but hope we will be hand held by the specialists in that.

[MFBMT2011 in reply to Tdg73]

Hi Tdg73. I was 58 when I had mine and considered ‘young’ and fit. I was IR2 (intermediate risk 2) with heavy scarring in my bone marrow. It was 14 years ago and very few of the various medications that manage (don’t cure) Myelofibrosis existed. They can complicate the decision. The risk at the time was 60% ok, 20% relapse and 20% don’t survive the treatment. I chose to go ahead and was treated in the excellent UCH in London

It is worth investigating the SCT option as it is not available to all sometimes there is no match and somone might fail the required medical checks - I had a 70 year old cousin who could not proceed as tests showed that his heart and kidneys were not up to scratch.

My story from diagnosis to three months post is online if that helps. I have had lots of buddies in the past, and am always willing to chat if it helps.

Best wishes

Chris (Princess Leia version)

[Tdg73 in reply to MFBMT2011]

Thanks so much, I have read your story and it gave me hope - although those odds are scary, but I have no idea how much they've improved in the interim 14 years.