So here is an update following last week's consultant visit. First though thank you more than I can say to everyone who replied to me with information and such kindness - I could not have got through those few days without you.
Anyway to the point. So I have just written myself a timeline of my ET covering the dates / treatment and the numbers. I can see now that my ET has not progressed at all from 2012 to now really, my numbers remain stable. (yes I know how lucky I am.) I agreed to start Hydroxy in 2021 when I reached 60 and started off with 500mg a day 7 days a week. After being mildly neutropeanic, this was reduced to 1 x 500grm 5 days a week with the weekends off for good behaviour. I moved area and consultant at the start of this year. My new consultant carried out a blood test to check my JAK2 status which was found to be "suspicious". I asked what this meant ( not lurking in alleways apparently) and my allele burden was somewhere between 1 - 3 %. I have JAK2 and raised platelets (aroind 580 without medication) but my consultant questioned whether the two were linked as the JAK 2 allele buden had not progressed . So the raised platelets might be caused by some other mutation as other mutations were discovered in research in 2018. Or they might be part of a general ageing process. Or something else, interesting!
So what we agreed was that I should have a BM ( always fun) and an ultrasound of my spleen to aim to get a bit more information regarding the link or otherwise between my ET and JAK2. At which point we will be able to make a "grey zone" decision as to what to do about the medication. I really, really did not want to start Hydroxy and I am aware that it does reduce my neutrophils, albeit mildly. So coming off the medication might be a good thing. On the other hand it might allow the platelets to increase to unpleasant levels. For the time being, I am happy to wait for further information following the BM and carry on with the dose of Hydroxy which is a very small one appparently. It is planned I shall have anothe consultant appointment in about 8 weeks following the BM and I will post again then if there is further information that might be of any further use to you all. Below is a link to an excellent video that gives a bit more information on all of this in case you missed it - thank you so much Hunter for this and all the other resources.
Thanks so much everyone for your support and kindness - stay well.
Glad that you like the presentation by Dr. Nangalia. I think it is fascinating and very useful information.
It sounds like you have a very well thought out plan to move forward. With more information, you can decide whether you want to continue to pursue cytoreduction at this point. If you do wish to continue, you can review options and decide which agent to use. In the absence of symptoms, you can also discuss what your target should be. It is worth noting that there is no particular value to having the platelet level be "normal" for most people with ET. Each person needs an individualized goal. Some docs actually use 600 when a number is used. Others use the delta (degree of change). That is something you can decide with your MPN care team.
Wishing you all the best as you move forward. Please do let us know what you learn and how things turn out. Everyone can benefit from your experience and knowledge.
Thank you as ever, and I mean always, for your insight and wisdom. I think you are right regarding a discussion of treatment goals. There was never much discussion of my overall risk along with the healthy lifestyle that I really try to have, but don't always manage. I asked a lot of questions about starting the cytoreduction therapy but ultimately went along with my previous consultant knowing best. She is a excellent person and I trusted her so I don't think in the circumstances, we made the wrong decision to start cytoreduction at the time. I wonder also how much the focus on Covid interfered with the passing on of the research into other mutations . Now, it seems time to pause and look at any new information provided by the BM and then make a proper decision. I am so glad to feel more informed - thank you again.
I can see how much we all need this forum; not just when we are panicking in the middle of the night but also to learn from each other's experience of this condition that nobody has ever heard of! I will post again when there is news.
I must say, I’m very impressed with your consultant’s enquiring mind. Is he an MPN Specialist (if he isn’t he should be), and are you based in the UK?
As I have mentioned before, I’m very similar in disease presentation to yourself, platelets 500 at diagnosis in 2016, but unlike you, I was put on hydrea straight away, as I was aged 60.
My disease (ET) is indolent and has remained stable for seven years on low dose hydrea 500mg Monday to Friday, weekends free. My platelets now average 340. The only symptom I can honestly say I experience is some fatigue, which could be the disease, medication or even age related!
My allele percentage was taken last year for the first time when I consulted with Professor Claire Harrison, it is low at 3.69, which reflects my indolent disease and low symptom burden. A pretty good result after seven years, notably without interferon intervention. It’s just a shame I didn’t have it taken at diagnosis too to have clear comparisons.
Hiya, Thanks so much for replying and I am so glad you sound well and the ET does not sound to be progressing. You are right, your case seems exactly like mine. I would say all I have is fatigue but I think mine comes from pushing myself too much and generally liking to be busy. It is really helpful to hear that other people have the same presentation of the disease and I will post again when I know more. I have a BM followed by an appointment at the end of August. In the meantime, thanks so much for your reply and look after yourself.
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