ET

Diagnosis is an awful shock -- please know your friends here are sending love and support.

Who has ever even heard of "ET"??? And then to learn that you have something totally different from what you had first been told -- that's an awful lot to deal with.

Yes, ET is a chronic blood cancer. It happens when the "drivers" that regulate platelet production mutate, causing our blood marrow to churn out platelets like mad.

Having extra platelets makes us prone to blood clots, and this manic over-product also depletes our bone marrow.

But don't be discouraged! The low-dose aspirin you've been taking is already protecting you from blood clots. And there are effective medications for ET.

HU is what most of us take. Some ETrs have been on it for decades. It's cheap and it works.

Should you have adverse side effects, some other options exist too.

Finding your effective dose may take a while. Frequent blood draws will be part of the process.

You can learn SO MUCH on this forum. Whatever concerns or issues you may have -- someone else here will share them.

You are not alone!

I would suggest to get a bone marrow biopsy/aspiration to confirm your diagnosis. With best wishes

Might be good to find out what driver mutation you have--JAK2, MPL, or CALR. It makes a difference in treatment protocol. However, if you are over 1,000, you may be at risk for acquired von Willebrand's, a complication that can cause bleeding, so that may be why your doc wants the platelets brought down. Maybe ask doc about this?

My dad had ET. I have ET-CALR. It is not curable, but it is manageable. I have had it for 15 years. Main complication is clots and, if platelets get high enough, bleeding. (Yes, seems contradictory, but everything about ET is kinda weird.)

Yes, most docs consider ET a chronic cancer, but if you take care of yourself, prognosis is pretty good. Dad lived to be 82 and died of something else. Just let all your docs know what you have and be prepared for a lot of blank looks and people telling you, "Well, you don't look sick."

PS, do ask your doc about your immunity levels. All of us have some lessened immunity on hydroxyurea. If you had yr spleen removed that could increase susceptibility to infection? This came up a lot during Covid: just how immunity suppressed are ET patients and what factors make a difference? That seemed to be kind of an unknown.

I've had ET since annual blood work maybe 20 years ago noted platelet count over 1800....my doctor was aghast and immediately sent me to a hematology oncologist. I was offered HU or Angrylin (newer then, now Anagrelide) without a bone marrow test. I recall taking the newer drug as side effects of HU seemed more severe. As long as I take the meds, see my doctor when asked (started with monthly, went to quarterly, then bi-annual, annual, back to twice a year), remember medication when traveling (forgot once, overseas and didn't have enough so appointment the week of return showed increased platelet count), platelet count runs in an acceptable range. Changing to my husband's oncologist in two months (closer, simpler).....hope at 73 he doesn't ask for a bone marrow test! For me, this has been a very liveable condition, not death-defying cancer management.

Thank you for responding – – all of you – – very much appreciated. I found out today that I have the jak 2 mutation from Genetic bloodwork. Do I need to have a bone marrow biopsy too?
With this diagnosis, it is not quite as mind blowing as the ovarian cancer was Because this has a long life expectancy with treatment. However, my worst fear in life is having a stroke. I would rather die of cancer than have a stroke.😕 So wouldn’t you know it? I get a disease with the high probability of stroke.!!!

So with the Jak 2 genetic mutation, should my family members be tested?

I tested positive for the mutationBRCA 1 years ago, —all my family members could be tested as well.

I am so sorry you've already been through another cancer.

I will let others respond to your bone marrow biopsy question. I am grateful my oncologist did not suggest that for me. My bloodwork indicated the MPL "driver."

Most cases of ET are "spontaneous," not inherited. Your question about whether family members should be tested is a good one, though.

There is so much that's not understood about ET. That's frustrating! But we are very lucky there are tests to identify the condition, and medicines to treat it. ET wasn't even recognized until the 1920s.

Another question… Have any of you with ET ever had any problems with flying? I have a trip booked for Europe.
now I’m a little nervous.🫤 With the new diagnosis.
The haematologist said to go for it and just take two aspirin instead of one.
I’ve decided this will be my last big trip😕 And even question if I should go on this one

I have had ET for 10 years and avoided a bone marrow biopsy completely. The fact that I have the JAK 2 mutation was enough to make the diagnosis and medicate. I also have been told that the 450 limit for a normal platelet count may, in fact, be low if other factors such as diet, exercise, and a healthy life style are maintained. My Droxia dosage has been at 600mg and 800 mg alternately daily. I am trying to keep my level below 700. Recently, my levels have been fluctuating and currently I have increased my Droxia to 800 mg per day and having bloodwork next week. My goal is to take as little Droxia as possible. Although I don't seem to have any side affects yet, it is a chemo pill and may affect other parts of the body at some point. You are correct, there is not a great deal of information on ET and you really have to work with your doctor and do your research. I am currently trying to research the effects of vitamins and nutrition in relation to ET. If that would be helpful, we may be able to limit the Droxia. Wouldn't that be nice!!!!

MPN mutations are not inherited, but researchers think a predisposition to mutate might be. Family MPN clusters are not unheard of. I told my son and brother to mention my diagnosis as part of family history and to keep an eye on routine CBCs. Testing was not deemed necessary.

I wear compression stockings on long travel trips.

Be VERY wary of "research" that alternative practitioners make about platelet reducing treatments. It's not just the number of platelets we have but the stickiness. Best lifestyle changes I've found in 15 years with ET and talking to others:

1. Get daily mild to moderate exercise; whatever nudges you out of your comfort zone just a bit.

2. Quit drinking or smoking.

3. Strive for a heart healthy diet that may help reduce stroke risk.

4. Do not obsess over 25 point fluctuations in blood counts; it's the rise or fall over a year that is significant.

5. Drink 64 ounces of water on top of juice, tea, coffee, etc.

6. Sugar and carbs may increase fatigue, so try to look for other options. Snacks are a good place to make changes.