1. < Blood Cancers & Disorders

Starting 17th year with ET. What's ahead?

Posted by nohrt4me @nohrt4me, Dec 29, 2024

I developed ET-CALR in my early 50s, diagnosed at 60, on hydroxyurea and aspirin at 64, now 70. HU has kept platelets stable in 400s with occasional spikes to 500s.

Conventional wisdom is that "many people live for 20 years with ET just fine." But what happens after that?

I'd be interested in hearing from other longtime ET patients. Did your docs do more tests at the 20 year mark? Was progression to MF or AML more of a concern? Did HU start to lose its punch for you? Were you advised to do anything different?

We're all different, and those of us with CALR mutations are pioneers (or guinea pigs, depending on how you look at it) because that mutation was only discovered within the last decade or so. How ET plays out for us may be different.

I'm not a big worrier, but I do like to get a gander at what's around the next bend, even if the answer is "nobody knows." Thanks!

Interested in more discussions like this? Go to the Blood Cancers & Disorders Support Group.

Mentor
Lori, Volunteer Mentor | @loribmt | Jan 14 1:24pm
In reply to @1pearl "Hi, I am just curious how those of you with diagnosis of essential thrombocytosis had diagnosis..." + (show)
@1pearl

Hi,
I am just curious how those of you with diagnosis of essential thrombocytosis had diagnosis confirmed. Is just high platelets enough? What if platelets are decreasing with no treatment, but white blood cells are high, and neutrophils are increasing? I ask because I went to doctors for four years about my very enlarged right ring finger proximal phalange joint. MRI from two years ago was unable to determine problem and that radiologist recommended needle aspiration to rule out infection. Rheumatologist I saw through difference insurance now since I became Medicare age recently ordered ultrasound guided needle aspiration about two weeks ago but hasn’t been able to find where to schedule me for that. I think infection should be ruled out before I take Hydrea which is a serious cytotoxic chemo med.
In summary, how were those in this group with ET have their diagnosis confirmed? If you are comfortable sharing, please comment.
Thanks

Jump to this post

@1pearl Since you’re new to the diagnosis of ET, you might want to look through some of the other discussion by members with ET.

This would be a good place to begin: Essential Thrombocythemia: Looking for information and support https://connect.mayoclinic.org/discussion/essential-thrombocythemia-1/

You’ll be able to speak with other members, such as @huronshores who was diagnosed with ET with no assignable cause.
They write in this comment:
https://connect.mayoclinic.org/comment/203937/
You can also find a lengthy list of other discussions regarding ET by typing Essential Thrombocythemia in the upper search bar.

I’m also posting a few more articles to read through since you’re concerned about ruling out an infection before starting HU to treat your condition. These talk about differentiating between essential (primary) thrombocythemia and thrombocytosis https://emedicine.medscape.com/article/206811-workup?form=fpf
https://www.nhlbi.nih.gov/health/thrombocythemia-thrombocytosis
I hope you’re able to get your test run soon by the Rheumatologist. At least you’ll feel like you’re moving forward.

REPLY
nohrt4me | @nohrt4me | Jan 14 3:41pm
In reply to @loribmt "Good morning, @nohrt4me I hope you won’t take too long of a break! ☺️ I think..." + (show)
@loribmt

Good morning, @nohrt4me I hope you won’t take too long of a break! ☺️ I think I can speak for all of us in the group with how much we truly value you sharing your life experiences with ET and well, just life!

I’ve said it before here in Connect, but I like to think of us all sitting around a kitchen table having our morning beverages, snacking on coffee cake, hashing out the good, the bad and the ugly of whatever life is tossing at us. I grew up with my mom, auntie and grammy talking about everything under the sun around that table... Some days it was nothing but whining and blue smoke. LOL. Other days, there’d be resolution or commiseration or a 3 tissue box discussion over loss. And just like the kitchen table the conversations in some of the discussions do veer off topic.

I know you are hoping to get responses from other ‘members who have ET with the CALR mutations and the impact long term. To get a better understanding on the potential progression of the disease. Give it time, they will find you.
In the meantime, this discussion, even though it zigs and zags, is valuable to the other members who maybe are new to the site or to ET and will appreciate the openness, honesty and vulnerability of the conversations here.

I hope you’ll rejoin the table soon…I’ll bring the coffee cake. ☺️

Jump to this post

Yes, fine that comments zig-zag in the ways most people need/want them to. I know there is an intense need for info when people are first diagnosed.

But I think there is also a need for sharing among patients aging with chronic cancers that were diagnosed years ago. I have a host of thyroid, skin rash, erythema, and dental issues that have worsened in the last couple of years, and accelerated in just a few months. I suspect that the ET and possibly the meds play a part, but when you ask the specialists about it, most have never even heard of ET, and they are VERY quick to dismiss it as a factor and just start piling on more meds.

And the time and expense of seeing umpteen specialists. Ye gods! When do I get my hair done much less afford coffee and chocolate!

I need to get some answers for myself. If I ever find them, I will report back.

REPLY
1 reply
cariadk | @cariadk | Jan 14 5:35pm

Diagnosed at 60 now almost 65 CALR on HU 1000 5x/wk 500 2x/wk and aspirin daily also HRT an unpopular choice according to my last Hematologist that Im not going to drop. My levels started at 1237k. I thought I had Covid and went in to ER. Was told I had a panic attack. now fairly stable at 600k. Anecdotally my levels seem to get higher when I am physically or mentally stressed. I’m working on stress management

REPLY
1 reply
janemc | @janemc | Jan 15 7:52am
In reply to @1pearl "Hi, I am just curious how those of you with diagnosis of essential thrombocytosis had diagnosis..." + (show)
@1pearl

Hi,
I am just curious how those of you with diagnosis of essential thrombocytosis had diagnosis confirmed. Is just high platelets enough? What if platelets are decreasing with no treatment, but white blood cells are high, and neutrophils are increasing? I ask because I went to doctors for four years about my very enlarged right ring finger proximal phalange joint. MRI from two years ago was unable to determine problem and that radiologist recommended needle aspiration to rule out infection. Rheumatologist I saw through difference insurance now since I became Medicare age recently ordered ultrasound guided needle aspiration about two weeks ago but hasn’t been able to find where to schedule me for that. I think infection should be ruled out before I take Hydrea which is a serious cytotoxic chemo med.
In summary, how were those in this group with ET have their diagnosis confirmed? If you are comfortable sharing, please comment.
Thanks

Jump to this post

A bone marrow biopsy has long been the gold standard for diagnosing ET.

Happily, now diagnosis is possible from just a blood draw, as more sophisticated analysis has evolved.

First: do you have a level of platelets above the normal range?

Second: Do you have one of the three "driver" mutations (JAK, CAL, MPL) that cause overproduction of platelets?

If both boxes are checked -- ET is the diagnosis.

It is also possible to have ET and NO driver mutation.

And it is also possible to have high platelets for numerous other causes than ET.

There is a lot to process. Click on the links wonderful Lori has provided, and soon you'll feel much better informed.

Your concern about Hydrea if you have an infection is interesting. Hydrea (or HU) does lower our immune response, so it might mean antibiotics would be less effective.

I hope you will share with us what you learn on this topic.

REPLY
janemc | @janemc | Jan 15 7:55am
In reply to @cariadk "Diagnosed at 60 now almost 65 CALR on HU 1000 5x/wk 500 2x/wk and aspirin daily..." + (show)
@cariadk

Diagnosed at 60 now almost 65 CALR on HU 1000 5x/wk 500 2x/wk and aspirin daily also HRT an unpopular choice according to my last Hematologist that Im not going to drop. My levels started at 1237k. I thought I had Covid and went in to ER. Was told I had a panic attack. now fairly stable at 600k. Anecdotally my levels seem to get higher when I am physically or mentally stressed. I’m working on stress management

Jump to this post

Great work, lowering your platelets so dramatically.

Keep taking good care of yourself and please don't be shy about sharing your stress management secrets!

REPLY
janemc | @janemc | Jan 15 8:07am
In reply to @nohrt4me "Yes, fine that comments zig-zag in the ways most people need/want them to. I know there..." + (show)
@nohrt4me

Yes, fine that comments zig-zag in the ways most people need/want them to. I know there is an intense need for info when people are first diagnosed.

But I think there is also a need for sharing among patients aging with chronic cancers that were diagnosed years ago. I have a host of thyroid, skin rash, erythema, and dental issues that have worsened in the last couple of years, and accelerated in just a few months. I suspect that the ET and possibly the meds play a part, but when you ask the specialists about it, most have never even heard of ET, and they are VERY quick to dismiss it as a factor and just start piling on more meds.

And the time and expense of seeing umpteen specialists. Ye gods! When do I get my hair done much less afford coffee and chocolate!

I need to get some answers for myself. If I ever find them, I will report back.

Jump to this post

It is doubly unfair to get cancer and then to have to fight for the care we need.

Would his medical license be revoked if a doctor said: I don't know, but I'll educate myself about this because I want to help you?

Instead they brush aside our questions.

It makes me want to scream.

Anything you learn and wish to share will be greatly appreciated, nohrt4me.

REPLY
eloise999 | @eloise999 | Jan 18 8:16am

I am new to my diagnosis. I am triple negative ET. It looks like that is a small percentage of ET patients. Does anyone here have it? What are the differences? What should I be aware of or look out for. Are treatments the same or do I have a more limited set of drugs?

REPLY
3 replies
janemc | @janemc | Jan 19 6:59am
In reply to @eloise999 "I am new to my diagnosis. I am triple negative ET. It looks like that is..." + (show)
@eloise999

I am new to my diagnosis. I am triple negative ET. It looks like that is a small percentage of ET patients. Does anyone here have it? What are the differences? What should I be aware of or look out for. Are treatments the same or do I have a more limited set of drugs?

Jump to this post

Triple negative! You are a unicorn among unicorns, eloise999.

You are asking great questions.

My ET is MPL-driven, so unfortunately I have no personal experience to share. Hope better informed voices will chime in soon!

REPLY
janemc | @janemc | Jan 19 7:03am
In reply to @eloise999 "I am new to my diagnosis. I am triple negative ET. It looks like that is..." + (show)
@eloise999

I am new to my diagnosis. I am triple negative ET. It looks like that is a small percentage of ET patients. Does anyone here have it? What are the differences? What should I be aware of or look out for. Are treatments the same or do I have a more limited set of drugs?

Jump to this post

Did you know you can search for topics on this forum?

Try entering "triple negative" or "triple negative ET" in the search bar at the top of the page.

REPLY
eloise999 | @eloise999 | Jan 19 9:31am

Thanks. I will try that. I did not notice the search bar

REPLY
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