In a few days I will see my specialist for the first time since my ET diagnosis(And APC-resistency).
I am really nervous to be honest. I would like to ask you all if anyone has any advice what to ask/discuss. I only get to see her once a year and I want to make the most of it. (And if you could send my some emotional support as well it would be greatly appreciated)
Hi Bohus.
I’m surprised you only have yearly appointments... !! All the more reason to have the appropriate questions ready for this appointment. Obviously this has to be relevant to you, so consider what has been bothering you. For example, have you had any symptoms or worries since you were last at clinic that you would like to discuss? You could ask are there any additional risk factors for APC resistance that you should be made aware of.
I’ve just briefly read a couple of your old posts, you mentioned you had a clot, but also said you’re in the ‘low risk’ group. Maybe you could get clarification on that decision?
Write down all your questions, and if possible take somebody with you for support.
Good luck
Hi,I too have an appointment coming up and I would like to be prepared. I am watching the links so kindly posted here recently by hunter5582. I am planning to ask for a bone marrow biopsy (had last one 14 years ago) and to ask for allele burden test. I would also like to know if I am a high or low risk patient. I am still researching. Any tip welcome.
That sounds like a wise decision Mairead.
Hope you have better luck than me on the question of Allele Burden. My original lab report states ‘presence of the V617f has been detected at greater than 1%’. I’d like to know how much more! I’ve asked on numerous occasions, but get deflected. I think that they don’t know either and are unwilling to offer another test due to ‘budget watching.’ This is where hunter5582 would tell me to be more assertive🤣
You got that right! 😁
Below is the IPSET thrombosis risk table for ET. You can type in your age, any previous thrombotic events, mutation status etc, and it will give you your thrombotic risk group status.
siematologia.it/LG/IPSET%20...
Wow. For ET. I will check it out. And see if any clues for PV. As I watch the links given by hunter I listened to Dr Sliver from the Scotsdale Conference last November and he was quite clear on regular testing for the allele burden and less regular bmb checks. But if you say that having got the precious allele test and you didn't get definite results ... well. How disappointing. Sometimes I think we take a step forward with MPNS and two steps back. Anyway thanks for these comments. I will keep at it and report my experience. Many thanks. Mairead
There are several types of JAK2 tests. Broadly separated into Qualitative (YES/NO) or Quantitative (%). The quantitative tells you more, but costs more.
Is the Jak2 quantitieve test different to the allele burden test? I know I am Jak2 positive I have now got the allele one booked in for 7 October.
They are the same thing. Quantitative = % of JAK2 mutated hemopoietic stem cells present. aka mutant allele burden
You can try this risk calculator for all MPNs
sanger.ac.uk/science/tools/...
One word of caution, this is not going to tell you your individual prognosis. It is a statistical projection of probable outcome based on a number of variables. You can't know whether you will fall within the statistical mean for your risk group or be 1-2-3 standard deviations out from the mean on either side.
Hi will you have the results of a recent blood test available as part of the discussion. On my last telephone appointment I had got one from local health centre. I got the hospital to send through the forms beforehand. I have done the same this time. Otherwise my precious consultation would have been a polite social call. I have listed the questions I hope to ask below. And i have 4 appointments a year. 1 would give a very long gap and things coukd change. Maybe you could ask for more crequent ones. Good luck.
Hi Bohus, as per the advice below, write down your questions because it is easy to get side tracked and then forget to ask something, and if possible take someone with you, someone else listening will often remember something that maybe you forget, and can also ask questions. The main questions are:
what are you current blood counts and how do they compare to the previous readings?
if you are currently taking medication, will the dose stay the same?
if you are not on any medication, are there any plans to start medication, if so, when and what medication?
when will you next be reviewed?
if your next review won't be for another year, what should you do if you have any concerns or are worried about any symptoms, i.e. if they get worse or you feel unwell, will you be able to contact the haematologist about any concerns, or is there a haematology nurse specialist in the department that you can contact at any time for advice?
Good luck, Maz
Thank you so much for your answers! This morning I have put a list of questions together.
I guess the main thing will be the risk assessment, I am on aspirin only, but last year I had a blood clot in my left leg. I am also apc-resistant, does that matter in the risk analysis?
I take a complete blood count every six weeks and I would like to get my numbers analysed. However the platelets are between 495 and 680.
Last time, when I got my diagnosis she told me not to worry and just live my life as normal as possible. However the more I learn about Mpn:s the more I want to learn about how to life my life in a way that minimizes the risk of progression. But my hopes of getting any help with that isn’t too high....
I am also really curious to know whether my et has anything to do with my other health issues. I have a lot of gastrointestinal issues, I have got some sort of what I suspect is an inflammatory back pain. And some skin issues. And a numbness in my left foot and the occasional pain in my left big toe.
Well thanks for your responses and wish me luck
I wish you all a really nice day!!!!
The fact that you have a history of thrombosis, ET, and APC-resistance (a clotting disorder) absolutely impacts your level of risk. Your platelet levels are relatively low and in and of themselves do not predict your level of risk. Below 1 million, there is no linear relationship between platelet numbers and risk of thrombosis. The coagulation cascade is more complex than that. This is definitely something you need to get addressed with the hematologist.
MPNs are at the core inflammatory disorders. The secondary symptoms we experience are often related to this issue. Systemic inflammation is real and often ignored by treating physicians. There is now a large body of research into this issue with MPNs that you can easily access. FYI - inflammatory issues I experience include: osteoarthritis, plantar fascitis, GERD, insomnia, eczema, and more. Here is a great presentation on this topic just in case you have note seen it.
youtube.com/watch?v=FzyoPAG...
Regarding you questions, it looks like you have a good list going. The only thing I would add is to be sure you list ALL of the symptoms you are experiencing. Insist the doc hear it all. Insist that you be treated in a holistic fashion. Do not ask for a Mutant allele quantitative analysis - assert your right to have one done. Nicely, but assertively. You are describing a set of symptoms known to be associated with MPNs. Suggest you be aware of what the definition of pruritis, paresthesia, peripheral neuropathy, and neuropathic pain. Describe what you are experiencing in concise, accurate terms so the doc will hear you and make the correct associations. If you feel a BMB is warranted, say so and cite the relevant protocols.
I always go to my appointments with a written list of information and questions. I give the doc a copy. It helps me stay on track and cover everything. hope that all helps.
My HCT was low for me..help. 
All about me and my Myelofibrosis
Having my first BMB next Tuesday! 
Please please help Lump on the front of my ear **** 
First month of the rest of my life done!
