Kia Ora Koutou!

[KyleeR]

Kia Ora MPN whanau 😊it’s taken me a little while to write this post but I’d first like to introduce myself - hi 👋🏼 my name is Kylee and I am a 39 yr old New Zealand Māori.  I have 4 children; 14 yrs, 11 yrs, 6 yrs & 3 yrs.  I was diagnosed with ET in September 2022 following a routine pre-surgery blood test which showed platelets in the 600s followed by a BMB which confirmed JAK+To say this diagnosis came as a shock is an understatement.  I don’t have any symptoms, am relatively healthy and  exercise regularly albeit that I love a glass or two of wine.Alot of the info online I found was extremely scary and for the first time in my life I suffered from extreme anxiety.  I have come to hugely value this page for information and other members experiences.  I am currently on watch-and-wait and taking baby Asprin daily but the results from my BMB showed a very slight increase of reticulin.  This has weighed heavily on my mind and I have requested an earlier appt with my specialist which is next Tuesday.I would love to know if you have any suggestions for questions I should have for her.  I’m particularly concerned about the reticulin level which I think this points to definite MF. But any suggestions are hugely valued.  Appreciate your timeNgā Mihi

[Mostew]

nau mai

Hope that's right greeting

Good news is small amount of wine , red is good for us ! Also good no symptoms. But makes it more of a shock to be diagnosed

I have only JAK 2 POS. SO not able to advise on questions.

No question to small or silly. If you are made to feel it is don't be afraid to ask to see someone else next time.

I always get a print out of my results and keep for reference .

Hope appointment goes well

Carolyn

[KyleeR in reply to Mostew]

Hi Carolyn, thanks for your reply. Great idea to have my results all printed, will add that to my new found health journal x

[Mazcd Partner MPNVoice]

hello KyleeR, what a lovely greeting from you, thank you, and welcome to our forum. It can be very daunting when you are newly diagnosed with ET, there is such a lot to understand. I hope that the information on our website is helping you, there is lots of information on there about ET, the different medications used to treat it, ways to cope with symptoms and lots more. mpnvoice.org.uk

It might help you to have a buddy to talk to, a buddy is someone who has the same MPN as you and takes the same medication, and can give you support and advice from the perspective of someone who really understands what it is like to live with ET on a daily basis. Contact is by email, phone or a combination of both, whichever suits you and the buddy, and how often you contact each other can be sorted out between you, some people find that they feel better after speaking/emailing with their buddy a couple of times, others need more contact, it is all up to you and how you are feeling, if you feel you would like to have a buddy then please let me know and I can send you the buddy guidelines which set out the boundaries of how a buddy can help and support you, and also a request a buddy form. Please note that in order to be put in touch with a buddy you will need to be registered on our mailing list, this is part of GDPR and means that you will also receive our news and update emails we send out about patients' forums; drug trials/surveys; fundraising news and general updates. Email me at [e-mail]

Our patients’ forums are a great way to hear from experts and patients, we have been holding quite a few online forums, you can watch them and lots of other useful videos on our YouTube channel youtube.com/channel/UC-S_Ic...

With regards to talking to your haematologist, this information on our website might help you mpnvoice.org.uk/living-with.... It's a good idea to start writing down your questions and take them with you, that will keep you focussed on what you want to know, and if possible, take someone with you, this can help as someone else will often remember something you don't, and can ask questions as well.

Good luck with it. Best wishes, Maz

[KyleeR in reply to Mazcd]

Hey Mazcd,

Thanks for adding the links - they are super helpful.

I will be in touch in regards to a buddy, it sounds like a great idea.

Thanks for your time x

[Solyesh]

Welcome Kylee and glad you found this page. I know with 4 kids that age (just a little past that myself with our 4) you are kept plenty busy which is a great thing.

The important thing to remember is that ET is manageable and as my MPN specialist keeps reminding me we are more likely to die with ET than from/due to ET. We are seeing new therapies and treatments all the time! Staying active also helps both mentally and physically.

On your upcoming visit with your specialist (is it an MPN specialist?) - my biggest piece of advice would be to write down every question you have - big or small. I personally find it easier to make sure that I get all things I think important covered if I have them written down beforehand. It is quite easy to get overwhelmed in appointments especially when presented with new information from the doctors/medical team. I would echo Maz's suggestion as well in that if you can take someone with you even better. For my "major" appointments, my wife comes along and we each have a copy of the questions and she follows along to also make sure that I don't skip any and that the doctor answers all. I sometimes send my specialist the questions beforehand so they both have an idea of how many I have and also might have an opportunity to answer some prior to our meeting or at the very least be prepared/knowledgeable that I am coming in "hot" (with a lot of questions).

While understandably worrisome a slight increase in reticulin does not necessarily point to MF. I have spent a lot of time asking my specialist about the line between ET and pre-MF given my BMB results; my allele burden and some of my blood work. It is not a definite science (the line between them) and my MPN specialist (who believes all MPNs lie on a continuum and have characteristics of each other) has constantly reminded me that: 1) my treatment plan/goals (maximizing quality of life and symptom free living) would not change either way; 2) our approach to managing the condition would not change (although I do think I might have pushed to start on Peg vs Hydroxyurea given the potential to slow disease progression and we might have more BMB than I would have otherwise (to check on the reticulin).

As for questions:

1) Do you know your allele burden? If not, ask to have it tested

2) Do they see any signs of pre-MF in the BMB? If so, would that modify their treatment plan/approach (i.e. would they move from watch and wait to perhaps interferons)?

3) What level of platelets do they find acceptable? (many doctors, mine included, for example are fine with allowing platelets to be up to about 600 (vs the 450 reference range) if no history of thrombosis

4) How often do they plan to test your bloods? How often do they want to see you in person?

5) Do they see any other questionable readings in any of your blood work? (also do they do a complete work up of all bloods and a liver and thyroid panel)?

6) Confirm a glass or two of wine is OK (my MPN specialist highly encouraged it..until I had questionable liver readings due to the interferon and then asked me to be extra kind to my liver)

7) Confirm that they are aligned with YOUR treatment goals (mine were quality of life goals)

There are other questions - I will go back and look at some of my earlier notes and see what I had asked at some of the initial appointments.

A diagnosis can be a shock..our kids were at about the same age of yours when it was first suggested that I might have an MPN - and I remember turning to my doctor and saying Ok we can deal with this but I need to make sure I am around to dance at my grandchildren's weddings (a tall ask under normal circumstances) - he said our goal is to keep me as healthy and symptom free as possible for as long as possible and I said I would take that.

Good luck!

[KyleeR in reply to Solyesh]

Hey!

Thank you so much for your reply, your suggested questions are fantastic. With my first meeting with the specialist I found myself walking out wishing I had asked certain questions. Its been a huge shock and a lot to wrap my head around. Mostly now that I feel so much of my on-going health is out of my control.

Its so good to chat to someone who has had a similar experience as mine. Its been difficult navigating what and how much to tell the children, especially as I knew the older girls would have lots of questions. But for the most part I'm still the same old mum >.< as no symptoms as of yet.

I will definitely be asking about the allele burden % - it doesn't seem to be something that was tested at the previous BMB?

Where in the world are you based?

Chat soon

[Solyesh in reply to KyleeR]

Kylee - hope it helps! It is definitely a lot to wrap our minds around. We actually decided not to say anything to the kids..largely because my wife had a very difficult battle with Stage 3 breast cancer about 8 years ago so any mention of the "c" word would just be too hard for them - also, so far, no real symptoms and no change to me as their dad as far as they can see so didn't want to put that in the back of their minds for now...if things change we will reassess.

The Jak2 allele burden can be tested via BMB or bloodwork. If at all possible it would be best to have a complete NGS (next generation sequencing) panel done which targets not only the "Big 3" for MPNs/ET (Jak2, CARL, MPL) but a number of additional driver and non-drive mutations. This could be important as more information becomes available as to how the various mutations interact and influence one another and MPNs. This usually is not standard practice and can be expensive but might be covered depending on doctor/MPN specialist/insurance.

I am based in Israel - it looks like the expanded gene panel will become standard of care here for MPNs (part of the national insurance basket).

Best of luck in the appointment and keep us updated!

[saltmarsh]

Hi Kylee. You've found this group and that will be very helpful to you going forward. Congrats. Everyone's reply to your post here is spot on. Trust me, you will do fine. Just make sure you have the right doctor and be your best advocate. And post any and all questions. This forum has been invaluable to me and it will be for you. Saltmarsh

[KyleeR]

Thanks Saltmarsh This group is a huge help, I would be really lost without it.

K