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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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.

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 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

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!!!!

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.

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

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.

nohrt4me | @nohrt4me | Jun 18 9:55am

In reply to @richie123 "I was diagnosed with ET in February of this year. I did have a bone marrow..." + (show)

I'm CALR, too. Been on HU for 6 years and bloodwork very stable in 400s once they got the right dose.

I think there is a tendency for docs to say "no" rather than "I don't know" when you ask if ET affects or causes other ailments.

It might help to get a second opinion, as managing two chronic illnesses puts a lot on your plate. But remember that ET-CALR is a relatively new discovery, and how it's different from the JAK2 "flavor" isn't well understood.

I can say that my glucose levels often run high on my quarterly blood counts. When I pointed this out to the GP, she ordered A1C tests at intervals, and they are always well within limits.

I also developed an asymptomatic goiter, what the dermatologist calls erythromelalgia (so?) and rosacea (periodic burning and flushing of hands, face and feet), itching and constipation. All of these things cropped about the same time as the ET diagnosis or after starting HU. All are nuisances, but can be irritating when they flare up. Docs like to blame menopause and age (I'm a 70 yo female). I wonder if they are related. I try to stay informed, but I don't expect to have answers in this lifetime.

Hoping you can keep the Type 2 managed thru diet and not feel too deprived. My husband struggles with his at times.

christinele | @christinele | Jun 18 11:05am

In reply to @et1055 "Another question… Have any of you with ET ever had any problems with flying? I have..." + (show)

I’m leaving for Europe in July. My platelets are 600 and I take 2 aspirin a day. My doctor wan me to go on hydro,, but I am hesitant to do so. As far as flying, she told me to wear compression socks, and stay hydrated. I have taken a few 3 hour flights and haven’t had an issue.

sregiani | @sregiani | Jun 19 6:20am

In reply to @et1055 "Another question… Have any of you with ET ever had any problems with flying? I have..." + (show)

No travel problems here. We fly extensively every year, even since I was diagnosed, ET w/JAK2.

From Michigan, I’ve flown all over the US, and to Alaska, Africa/Tanzania & Zanzibar, all over Europe (Poland, France, Italy, Spain, Portugal) - get the idea?

This fall we’re going to meetings in Wash DC, Vegas, then vacation in Italy and Slovenia.

What we have is an inconvenience. Always carry any meds and necessary supplements in a carry-on bag. Don’t let it stop you from living your life!

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