Essential Thrombocythemia: Looking for information and support

Posted by shenriq @shenriq, Jun 4, 2018

I was recently diagnosed with Essential Thrombocythemia, a rare incurable blood cancer. Platelet count aside, I am asymptotic. This current condition morphed from (constitutional) thrombcytosis, something I’ve lived with for 25+ years. While the new diagnosis was the result of a bone marrow aspiration and biopsy, my age was an additional factor, which was completely disarming, having been walking around unwittingly for the past 8 years! While at the low end of risk for clots, heart-attacks and stroke, nothing has truly changed - except the “C” word. No chemo yet, but active discussion about hydroxyurea. Uncertainty about ET is anxiety provoking and swoethatl, but I’m feeling betrayed by my blood. I’m looking for all information about ET, the chemo and support.
Thanks!

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1995victoria | @1995victoria | Jun 18 9:42am

In reply to @et1055 "My platelet counts have gone up over the last two years. It is presently at 1054...." + (show)

I wonder what stats are for ET Jak2, if taking HU+baby aspirin, and still get a stroke? Anybody know?
I didn't know a blood test could be done in place of a BMB. I've been tired all my life, so don't know if ET or HU is making me more so.

richie123 | @richie123 | Jun 18 9:08am

In reply to @nohrt4me "MPN mutations are not inherited, but researchers think a predisposition to mutate might be. Family MPN..." + (show)

I was diagnosed with ET in February of this year. I did have a bone marrow biopsy. I have a CALR mutation. Hydroxyurea is what I am taking. Still in process of titrating the drug to try and keep me in a normal range of platelets. That being said, I have periods of extreme tiredness. Sometimes the top of my head feels "light". If I rest for a while that really seems to help. I'm just wondering what other ET patients have experienced. You also mention sugar free snacks etc.,. I am also type 2 diabetic, non insulin. Can this make my diabetes worse? I have also experienced more neuropathy over the last few months since starting hydroxyurea. I don't know what to think. My doctor doesn't seem to think the ET or hydroxyurea is a cause. Any opinions? Anyone? Thanks

nohrt4me | @nohrt4me | Jun 18 8:04am

In reply to @et1055 "My platelet counts have gone up over the last two years. It is presently at 1054...." + (show)

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.

maryandbud | @maryandbud | Jun 18 7:07am

In reply to @et1055 "My platelet counts have gone up over the last two years. It is presently at 1054...." + (show)

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!!!!

et1055 | @et1055 | Jun 17 9:25pm

In reply to @et1055 "My platelet counts have gone up over the last two years. It is presently at 1054...." + (show)

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

janemc | @janemc | Jun 17 4:28pm

In reply to @et1055 "Thank you for responding – – all of you – – very much appreciated. I found..." + (show)

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.

et1055 | @et1055 | Jun 17 4:08pm

In reply to @janemc "Diagnosis is an awful shock -- please know your friends here are sending love and support...." + (show)

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.

docoltun | @docoltun | Jun 17 12:00pm

In reply to @et1055 "My platelet counts have gone up over the last two years. It is presently at 1054...." + (show)

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.

nohrt4me | @nohrt4me | Jun 17 9:59am

In reply to @et1055 "My platelet counts have gone up over the last two years. It is presently at 1054...." + (show)

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.

nohrt4me | @nohrt4me | Jun 17 9:52am

In reply to @et1055 "My platelet counts have gone up over the last two years. It is presently at 1054...." + (show)

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."

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