Progression to MF

[UKZA]

Hi All,

I had a BMB in early January and the results show level 1 Post ET MF . The results of the genetic tests have apparently “failed “ but it is my understanding that they need these to correctly diagnose my MF? I have been on peg interferon for the past 4 weeks (45mg) running alongside my HU . I had bloods yesterday and there has been no change. Platelets at 800+ and neutrophils at 1.3 . They have decided to continue as is for another month. I also asked if I am type 1 or 2 CARL and I am apparently neither? I am feeling a bit frustrated and the past month was a rollercoaster of acceptance. They initially did their number cruncher prognosis that gave me 80 months, or 35 depending on the genetic result. So after a month I am no further along with personal decisions., as still no results and starting to doubt I will get them. I understand that this prognosis is dependent on each individual and I do lead a healthy lifestyle. The consultant yesterday mentioned 10 to 15 years but said I can go to telephone consultations. Am I wrong to feel confused , has anyone else experienced something similar, what should I do next? Thanks and have a good weekend. Penny

[hunter5582]

I would be very confused too. What does "failed" genetic tests mean? Are you testing triple-negative? Did they do an entire MPN NGS genetic panel? Have you had a previous CALR positive finding? What stage of MF are you at? When managing a MF case like your with the questions you most certainly need answered, why would you go to telephone consultations? That is a much less adequate level of care than in-person appointments.

Suggest that it is time to get a second opinion from a MPN Specialist on your case. This is even more important given the number of unanswered questions you have. Just in case you have not seen it, here is a list. mpnforum.com/list-hem./

All the best moving forward.

[UKZA in reply to hunter5582]

thank you for your response. It is the high risk mutations that they were testing for in the bone marrow aspiration. The bone marrow segment has shown level 1 fibrosis. I tested ET CALR positive in 2017. I do feel concerned with the telephone consultation as I had this for two years and my symptoms were worsening and HU flattened my neutrophils to 0.8 at one stage. The problem is the reduction of HU has increased my platelets.but not my neutrophils, hence me insisting on answers or a change in meds. Now I still have no definitive answers and am only part of the way through the process of a new drug. I do feel the lack of care. I am with the northern cancer care unit at Freeman’s Hospital in Newcastle. They do have consultants that are listed as MPN specialists, but the trouble with the NHS is that you never see the same person. I will consider another move. Thanks again.

High risk mutations

[Rachelthepotter in reply to UKZA]

Hi. I saw Dr Mark Drummond- an MPN NHS specialist based in Glasgow who also sees patients for private consultations in Edinburgh. Was a very useful one off consult with an expert. At that time, I thought I might be moving to Edinburgh to live, and I wanted to check out the MPN care there first. However, life intervened and I don’t now think I’ll be moving permanently to Edinburgh.

He needs a referral from your GP, which all good specialists do. And, of course, the English and the Scottish NHS systems are differently funded. However, he seemed very used to having people who were being treated under the English system come to him for a second opinion.

Good luck – it’s tricky when you’re trying to make complicated decisions about practical matters and it’s so hard to get a realistic estimate of prognosis. Complete with a neasure of its error

[hunter5582 in reply to UKZA]

It sounds like someone messed up with the labs. The good news is that the NGS panel can be done with a blood sample. There are various panels that can be done. This is the panel I did with a blood sample. labcorp.com/tests/451953/in....

I have heard that there are some practices that cycle patients through a series of providers. This is quite unfortunate as it reduces the quality of care. The individual doctor-patient relationship is a vital element of medical care, building trust in an ongoing professional relationship. Seeing the same provider over time allows the provider to know your individual case is a way that a team of providers can never match.

If there are good MPN Specialists at your hospital, suggest insisting on seeing one of them, the same one, every time. You may have to push to make this happen and wait longer for an appointment. Given what you have going on, suggest you also insist on in-person visits. Fight for what you need and deserve if you must. Assertive patients receive higher quality care. Passive patients do not.

Given what you have going on at this stage, you need and deserve to have a relationship with a specific MPN Specialist. The best physicians are those who combine advanced knowledge in the science of medicine with being gifted in the art of healing.

I expect you are quite correct that it is time for a change in treatment plan. There are other options to treat MF, but treatment needs to match your individual case presentation. The good news is that MF treatment options are improving. You need and deserve to have a relationship with a MPN Specialist who can help you select the optimal treatment plan.

just in case you have not seen it, here is a really good presentation on current MF treatment options.

youtube.com/watch?v=vZsXbWr...(Not working as expected?)

[MFBMT2011]

I was primary Myelofibrosis (Intermediate risk 2) aged 58 and my first prognosis from a general haematologist was 8 months; an error. It was extended to 2-8 years, median five years, about a month later. The latter prognosis was later confirmed after I sought a second opinion from an MPN specialist at Guys hospital. From there I went the stem cell transplant (SCT) route, the only cure, twelve years ago this month.

Once you have a firm Mpn diagnosis you can decide your way forward which could be watch and wait for a while. There are various tablets that can help with symptoms but not cure.

SCT is tough and risky, but there are several here who have been that route and can help with information. Not everyone can have an SCT, might not be a match or not healthy enough, but it is worth exploring in case it is in your future.

Best wishes

Chris H

[beetle]

as usual Hunter’s comments are spot on! I would just caution against putting any store by prognoses which by their very nature are historical. You already know that our paths through this group of diseases vary enormously. I was diagnosed with ET in 1994 and post ET MF in 2012 so I am now in my 28th year of living with an MPN. When I was initially diagnosed the medication options were extremely limited but there is now much more choice with much research and clinical trials happening. Who knows what potential there is in discoveries yet to be made?

I was told probably 2 years in 2012 right before my oldest son’s wedding. My youngest son is getting married next Saturday and I’m still here to join in the celebrations

If you read my last post you will be aware of the potential pitfalls of telephone appointments but there is no reason if you prefer it not to ask for face to face consultations

Good luck with establishing your particular facts and ongoing care. Very best wishes. Jan

[UKZA]

Thank you to you all for your support and advise. It is so comforting to hear from others in a similar situation. I have been told that I am a possible candidate for SCT. I am a fit and generally healthy 53 year old. I love travel and sports and have not enjoyed the decline in my energies, but am determined to beat this. I will be seeking a once of second opinion with a specialist. Penny

[ainslie]

you’ve had some good answers already, it sounds as if a visit to a MPN expert would be ideal, quoting you from 35 months and then 15 years is frankly ridiculous and unkind.

I wonder how without a proper diagnosis anyone can give to a prognosis, well they can’t.

Prognosis is very difficult at best but without proper testing and diagnosis it’s irresponsible.

I don’t know how easy it is for you to see a MPN expert to help put you in the right direction and give a better more accurate diagnosis and to keep your local Haem on the right track, someone else mentioned Mark Drummond in Edinburgh, I’ve seen him for years and he is very good and well respected by other haems, he is also passionate about his work and a nice person. There will be other experts maybe Maz has a list.

Something to bear in mind that somebody else on here asked, a Haem who specialises in MPN is not necessarily a MPN expert. That comment may ruffle a few feathers but it’s a important point to bear in mind.

You deserve to be looked after properly , it’s not easy sometimes to get that , I urge people to think positively, be very very persistent until you find a doc or docs you feel comfortable with, they are out there for sure.

Wishing you good luck 😀