I’ve been on this forum a few months now but this is my first post. After a routine blood test last October I found out my platelets were just over 500, then after a subsequent jak2 test I can confirm I have it at a percentage of 2%. I’ve now just had a bone marrow biopsy to hopefully get more answers but it looks like I probably have ET.
I’ve been on a bit of a rollercoaster with all this as I’m still quite young at 36 and healthy with no symptoms yet anyway.
The one question I forgot to ask the doctor which has now been on my mind is will that 2% inevitably go up regardless as the disease progresses? Or could I live a relatively normal life with my platelet numbers and the jak2 percentage staying where they are longer term?
thavks in advance, after having a disastrous time on google like I’m sure many others have, this forum has made me feel more positive about the whole situation.
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Joshy87
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Welcome Joshy. Your numbers are low so try not to panic. There are others on here who have more knowledge on progression. What I will say is there are already drugs that are showing they can stop it even reverse progression. Progress is being made all of the time. I would bet by time you get to my age this will be curable.
Hello and welcome to the forum. Glad you found your way here. This is a great place to be when managing an MPN.
I was diagnosed with ET about the same age as you. That was over 30 years ago. The ET progressed to PV about 11 years ago. I have lived a good life while managing the MPN and at age 68 plan to continue doing so. I am currently using Besremi to successfully manage the PV. I feel better now than I did 10 years ago. My JAK2 allele burden has been reduced from 38% to 10%. I am very pleased with my response to this treatment.
One of the important things to know about ET is that it is a condition that can be managed. Most people with ET can live a normal lifespan. You are more likely to die with ET than from it. You will at some point see reference to ET being a "blood cancer." It is important to take this information in context. ET is a chronic condition, not an acute condition. You can look at ET as being cancer with a little "c" not cancer with a big "C".
Your hematologist will likely be unable to give you a definitive answer to the question about progression. There are multiple factors involved in progression. The presence of non-driver mutations, systemic inflammation, environmental exposures are among the factors. The JAK2 mutated hematopoietic stem cells have a clonal advantage over wild type (normal) HSCs. There is a tendency for the JAK2 allele burden to gradually increase over time. This is an area of active research as well as preventing progression from occurring. Progress is being made in this area.
Random Googling has a tendency to draw up less than ideal information. It is often out-of-date and does not reflect the progress that has been made in managing MPNs. It is important to educate yourself about MPNs, but using reliable sources is essential. Starting with MPN Voice, here are some reliable sources of information.
There are some excellent patient education forums hosted by MPN Voice in the UK and by other organizations in the USA. This set of recording trainings is quite good. Suggest starting with MPN Molecular Biology.
Thanks for your reply, very informative! I’ve had the tests for other mutations which has comeback negative on all fronts which I think is good news? I’m over the initial shock and now just keen to move forward with as much info as possible. Like many others I’m sure you hear the words blood cancer and can’t help but think of the worst. One minute I’m a healthy youngish person with a young family and the next I’m questioning everything. This forum has been great though I check it daily and I’m looking forward to being part of the community
The standard first test that many do is to check for the three driver mutations, JAK2, CALR, MPL. JAK2 is by far the most common of the three. While it is theoretically possible to have more than one driver mutation, it is so extraordinarily unlikely that it is extraordinarily unlikely. What is possible is to have a non-driver mutation that can increase risk of progression, such as ASXL1, TET2, NF1, and more. These gene mutations are involved in other cancers as well.
Note that I also have one of these non driver mutations, NF1. In my case, the NF1 mutation is a germline variant that causes another condition, Neurofibromatosis Type 1. The NF1 mutation causes a brain tumor that I had resected in 2019. That experience was an interesting health learning opportunity.
Adopting a good attitude and equipping yourself to thrive makes a huge difference. I would suggest that you are still "a healthy youngish person with a young family" - just one who has a manageable chronic condition. Have confidence that you will be one of many people with a MPN who will succeed in managing the condition.
Welcome Joshy87! Your feelings are totally understandable. I am still getting my head around being diagnosed with ET in Nov. 2021. I cannot add anything to DougyW and Hunter except that I hope they have given you loads of reassurance as they certainly have to me! All the best!
Don't worry!! My wife was diagnosed at 60 years old nearly 6 years ago, and never thought anything much was wrong with her for all her life. At diagnosis her Allele burden was over 70% and was jak 2 positive with MF. Her enlarged spleen was clearly visible in photos from 20 years prior.
You're a young MPN patient, i.e., under 40. I'm sure there's a vibrant sub-group for young patients and there's an in-person forum coming up next month in Cambridge. Although you've been given wise counsel from others, it would be a good idea to check these out.
I was in my mid 30s when i was diagnosed with ET. At the time my platelets were 1300. I am now 50yo and platelets hover around 400. Im on 1500mg Hydroxy. The biggest factor for my health has been in order of importance
1. Eat healthy. Lots of veg and fruit. I dont exclude meat but have sensible portions. Minimal white carbs-none if possible.
2. Sleep. To be guarded like your life depends on it.
3. Keep active.
4. Dont stress or worry. A calm heart is life to the body, as the scripture goes. At first i didnt know how. So i make it a priority to learn this skill.
Thanks for the reply, I keep healthy, swim outdoors, run marathons etc so felt fairly young and invincible but it’s nice to know I can hopefully manage this and live a hopefully relatively normal life. It’s still a bit of a shock though
hello Joshy87, welcome to our forum. It can be very daunting when you are newly diagnosed with a MPN, especially when you are young. I hope that the information on our website is helping you to understand more about ET.
I would also recommend that you have a look at the young people and MPNs blogs, written by Alice, specifically for young people, in the blogs she speaks with other young patients and health care professionals. mpnvoice.org.uk/about-us/yo...
You will also find in one of the blogs, information about the young people and MPNs network, this is a group for people with MPNs, aged 40 and under, they have a very active and informative private Facebook group facebook.com/groups/mpnvoic...
We held a virtual forum in Oct focussing on young people and MPNs, you can view the video on our YouTube channel, where you will also find some vlogcasts with young people youtube.com/channel/UC-S_Ic...
I do hope that this all helps you, and if you feel you would like to have a buddy for some one-to-one support from another young person then please email me at buddies@mpnvoice.org.uk
My first heam said confidently my mutation (VAF) would go up with time. (14% at that time) This was a very long 3-1/2 years ago. I showed him the fresh data on Besremi that showed otherwise. Current practice now accepts that it can go down for many or even most patients. This reduction through long times is specific to interferon therapy, and more recently revealed, Jakafi. I'm seeing reductions from both, now at 5%. But most current data is for starting VAFs higher than 2%, and for PV where higher values are common.
Is your Dr planning to check for other (non-driver) mutations? This test is often done in the last few years and good to have esp for future reference.
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