Et and mf2

[Charm33]

Hey all. I don't know if I'm asking a question or just venting so bare with me.Brief history- 31yr old female. Diagnosed Jak2+ ET start of this year but going by bloodwork, I have had it since 2019. Had a BMB in the summer which showed "mild fibrosis" and "hypercellular marrow" but diagnosis remained ET. On aspirin, hydrea, allopurinol, folic acid & bisop.

(In Ireland we dont have access to our results)

Had appointment today and I asked for more clarification on the "mild fibrosis" as its been bothering me, as they told me months ago that "it's not a matter of if you will need a stem cell transplant, it's a matter of when". I questioned how mild was it as I know stem cell transplants arnt done on patients with ET usually. So the doctor showed me the report from the BMB and it reads MF2. I was pretty shocked to be honest as I fully expected to see mf0-1. I'm still pretty taking aback but at least my questions are finally answered.

Il get another BMB next summer to check progression.

I guess if any others who also have ET and MF2 I would be grateful to hear your stories 🙏

Sorry for the long post. TIA.

[evalaz]

Hi, i just got the same diagnose today. ET but with moderate reticulin fibrosis MF-2 . When i got diagnosed 10 years ago it was 0. My hematologist insists on continuing interferon to stop further progression.No talks yet for sct.

btw we are about the same age and i understand how scary this is!

[Charm33 in reply to evalaz]

Sorry to hear of your progression. My hematologist would prefer for me to be on interferon but unfortunately unavailable in Ireland until mid 2025 so the plan is to switch me to that once it's available. Talk about bad timing. Here's hoping it doesn't progress in that time.Wish you all the best.

[evalaz in reply to Charm33]

How about besremi? There is shortage of pegasys in Greece and i will continue with besremi.

[Charm33 in reply to evalaz]

Wasn't approved for use in Ireland. Just a few days though it has been approved for use for only certain patients that have already been using pegasys but can't get it. So hopefully that's a step in the direction of being approved for all others.

[evalaz in reply to Charm33]

Hopefully you'll be able to get on it and not wait till 2025..

[Audo_tt_boy]

hi charm33

Sorry to hear the troubles you are going through. I was diagnosed with et 8 years ago after having MI and 3xcabg. What meds are you taking . I was on hydroxy for first 9 months or so and then onto peg for the last 7 years and have no seen progression on the last Bmb which is great for me at this stage. Peg has hopefully held off any progression to the fibrosis, but moving to jakavi shortly as I have come to the end of my stock of peg and there is none available to the pharmacies

In relation to you results in ireland I am also in ireland and treated in Beaumount, while you don’t have electronic access to results drs and nurses are more than helpful to print out jest every results you want to have copies of , so just make sure you ask your team next time if there is anything you want to have access to (they are your results ) and they will get you what you neeed just ask ..

Hope you get the answers you are looking for from your team

Take care

[Charm33 in reply to Audo_tt_boy]

I have listed my meds in the post. They hope to put me on interferon once it's available again. Only patients who have already been on it are priority not new patients due to the shortage, which is understandable. See that was my impression also, so I asked if they could print me a copy and they said no its not allowed but she showed me hers and that's when she showed me the MF2. Finally seeing something with my own eyes finally helped. They did say they sent a copy to my GP so I may try get my GP to send me a copy.

Glad to hear you have had no progression since. Hopefully that last 🙏

All the best.

[ainslie]

I’m maybe a twinge sceptical about what your doc said about needing a SCT sooner or later. It seems a big and quick jump from ET to SCT. Even MF to SCT is a big jump and quite rare. I would have thought there are other treatment options for you. If it were me I would definitely think about seeing a MPN expert soonish and show them the BMB results and even better the slides. You’re young and the MPN journey can be for many decades and if possible it’s important to have access from time to time to good experts. They can set you and your local Haem in the right direction and keep keep you going in the right direction. I see an expert once a year or two years.

[Charm33 in reply to ainslie]

I was and am sceptical too, hence me practically begging for them to show me what they are seeing on my bmb results. Maybe they are just preparing me for worst case scenario but why even bring it up I suppose ? They told me yesterday they sent a letter to a specialist (not many in Ireland at all maybe 2/3) this week and consult with them, so i must find out the name.

Thank you for your reply.

[TLJ-1 in reply to Charm33]

I'm very sorry to hear you young people have to go through this! I suggest that the next opportunity, if they are willing to show you the results but not give you a copy, just ask if you can take photos.

[Charm33 in reply to TLJ-1]

I will, thank you.

[ainslie in reply to Charm33]

There is a specialist Haem in Ireland called Frances Macmullen (not sure of spelling), I have heard her at conferences and read papers she has written, I have met her or heard anything about her from patients, I think she has some dealings with Clair Harrison at Guys, so likely to be credible.

I wonder if you get an opinion from Clair Harrison at Guys, some on here write to her or get their Haem to contact her, she is one of the best and is well known and respected by most haems. She might give you her opinion and advise your local team.

Reading and making sense of BMB results isn’t easy for us, I asked my expert Haem if I should try to learn how to understand mine and he said no, they are quite tricky and best left to experts, one pathologist or Haem can look at a BMB slide and have a different opinion to another Haem or pathologist, I had that experience last year.

Try to keep calm but very persistent until you get all the answers and advice to satisfy you re the diagnosis and best treatment plan, I know it can feel uphill but what has to be done has to be done. Of course one has to consider the worst scenario in certain situations but we must of course consider the best scenario too.

[Charm33 in reply to ainslie]

I have never heard of her, thank you, I will look into them.

[ainslie in reply to Charm33]

that should have read I have NOT met her as opposed to I have met her, good luck and it will be interesting to hear how you get on 🙂

[Cja1956]

I was diagnosed with post ET MF intermediate 1 in 2019. I was told I would need a transplant within 3-5 years. It was a very scary time and I was an emotional wreck for months. I’m now at MF 3/high risk. I’ve had three doctors tell me it’s time to do a stem cell transplant since the beginning of 2023. But I just can’t bring myself to do it. I’ve been taking Jakafi for a year and a half and my numbers have improved and I’m feeling a lot better. But, they are saying that eventually the medication will fail and I could lose my window of opportunity. So I guess our stories are pretty similar except you’re pretty young. You will get through it. Make sure you’re seeing an mpn specialist and get a second opinion and if necessary, a third.

I wish you well.

[Charm33 in reply to Cja1956]

Sorry to hear that, how scary and uncertain. Thank you for your reply and I wish you all the best whatever you decide.

[Hydranga18]

Dr. Eibhlin Conneally St James Hospital Dublin . MPN Specialist

[Charm33 in reply to Hydranga18]

Ah yes that's who my hematologist is going to consult with and have sent a letter to about me this week. Thank you. Happy to hear they are an MPN specialist 🙌