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.

1pearl | @1pearl | 5 days ago
In reply to @anavleek "What is your mutation ? It changed so quickly ?" + (show)
@anavleek

What is your mutation ? It changed so quickly ?

Jump to this post

CALR

REPLY
janemc | @janemc | 5 days ago
In reply to @lynns51 "I have recently been diagnosed with ET. I am currently at 601,000, but my platelets have..." + (show)
@lynns51

I have recently been diagnosed with ET. I am currently at 601,000, but my platelets have been rising steadily for two years. There is a mutation on the CALR gene, so this won’t go away. When I hit 750,000, my doctor will prescribe Hydrea. I have had headaches and am very tired most of the time. I thought this was caused by having had COVID19 in 2020, but what I read here is it might be the ET and Hydrea will help eliminate the fatigue and headaches. Has anyone experienced this?

Jump to this post

Hello, lynns51. Fatigue and headaches are the two most frequent symptoms of ET. Overproduction of platelets burns a lot of energy.

Everyone's experience is different. For me, as HU brought down my platelet count, it also gave me more energy, and relieved those headaches.

Also really helpful was my oncologist's staring me out on HU very slowly! For several months, I just took one 500mg capsule. This gave my body time to adjust to the medication, sparing me from any side effects.

With ET, you are experiencing something serious. Be kind and patient with yourself.

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lynns51 | @lynns51 | 5 days ago
In reply to @anavleek "What is your mutation ? It changed so quickly ?" + (show)
@anavleek

What is your mutation ? It changed so quickly ?

Jump to this post

Type1 CALR in exon9. Everything looked good until 2023.It went up fast after that. It was 328,000 and started the uphill climb to 601,000 it is today.

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nohrt4me | @nohrt4me | 5 days ago
In reply to @lynns51 "I have recently been diagnosed with ET. I am currently at 601,000, but my platelets have..." + (show)
@lynns51

I have recently been diagnosed with ET. I am currently at 601,000, but my platelets have been rising steadily for two years. There is a mutation on the CALR gene, so this won’t go away. When I hit 750,000, my doctor will prescribe Hydrea. I have had headaches and am very tired most of the time. I thought this was caused by having had COVID19 in 2020, but what I read here is it might be the ET and Hydrea will help eliminate the fatigue and headaches. Has anyone experienced this?

Jump to this post

Welcome to Club CALR. Me, too x 17 years.

HU did make me feel better. Less tired fewer headaches. And I've never had a clot. Ask yr doc, but I've read that CALRs are a bit less likely to clot.

Fwiw, my dad also had ET. Died in 2010 at age 82 of unrelated COPD.

Your doc follows same protocol as mine.

Tip: When you have to go on HU, ask to start on low dose, no more than 500 mg/day. Guessing your doc will do this anyway, but the lower dose gives you a chance to ease into it.

I went down to 600s pretty fast, then stalled there for a year. Dose was increased to an extra 500 mg M-W-F and I've been in 400s x 5 years now.

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nancyra | @nancyra | 4 days ago

27 years with ET
Platelets always in the 500-600 range which was fine with my hems. Was on HU 18 years but got bad skin cancer on head so switched to Anagrelide. Recently had to up my dose to 3 -.05 daily. Now numbers under 400.
Fatigue and headaches but at 67 no different than anyone else my age.
Also CalR. Numbers will always spike after vaccines of any kind so don’t be so quick to up medication.
And NO live vaccines!

One hem thinks I should have another BMB at this stage, but the other does not think it necessary.
Always good to have 2 opinions on everything with this disorder because it is rare and very few hems know enough about it.
I’m always reading up on the latest and follow threads here.
I read that it’s important to have a stroke assessment test to see how your arteries are doing. I had to insist on one with my dr. As they don’t do it which made no sense to me since that is what we are at risk for! Look into…
Coronary calcium score
Carotid artery test
Good luck!

REPLY
1 reply
gset | @gset | 17 hours ago
In reply to @nancyra "27 years with ET Platelets always in the 500-600 range which was fine with my hems...." + (show)
@nancyra

27 years with ET
Platelets always in the 500-600 range which was fine with my hems. Was on HU 18 years but got bad skin cancer on head so switched to Anagrelide. Recently had to up my dose to 3 -.05 daily. Now numbers under 400.
Fatigue and headaches but at 67 no different than anyone else my age.
Also CalR. Numbers will always spike after vaccines of any kind so don’t be so quick to up medication.
And NO live vaccines!

One hem thinks I should have another BMB at this stage, but the other does not think it necessary.
Always good to have 2 opinions on everything with this disorder because it is rare and very few hems know enough about it.
I’m always reading up on the latest and follow threads here.
I read that it’s important to have a stroke assessment test to see how your arteries are doing. I had to insist on one with my dr. As they don’t do it which made no sense to me since that is what we are at risk for! Look into…
Coronary calcium score
Carotid artery test
Good luck!

Jump to this post

Hi, I too am ET CALR diagnosed in 2004, switched to anagrelide following 3 years watch and wait, 10 years HU ( then ulcers) and interferon not working. After that anagrelide has controlled counts well. Am monitored for spleen size through annual ultrasound and heart for any damage from drugs. In 2020 had a new BMB that caught me on the turn to MF. I had the heart CT and have calcium score of zero and Doppler artery scan too, all good. However although I have post ET MF nothing has changed in my treatment as everything has stayed stable and the progression is very slow for me. The main thing is I had lower hemagloblin ( 9.6) so I had one transfusion last year and then felt normal again. It’s no harm to know where you are as there are lots of new treatments coming on line and you may qualify. I feel no different once I got over the shock of progression but the disease is actually a continual line of progression so we are all changing all the time. My spleen is still normal 4 years on too, blasts are low ( tear shaped cells) . Good luck, I hope you feel able to make the decision once way or another.

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eloise999 | @eloise999 | 10 hours ago
In reply to @lynnevb "Hi eloise999, I also have ET and I am triple negative. I take hydrea since age..." + (show)
@lynnevb

Hi eloise999,
I also have ET and I am triple negative. I take hydrea since age 60 and aspirin since diagnosis. I have had this since 2002, that would be 23 years now.
Here is a website I fine very helpful. It takes several readings to start to comprehend.
I am doing well. I hope you are doing well too. Hope you have a good hematologist/oncologist.
Let me know if you want me to share more about living with ET (triple negative).
https://www.nccn.org/patients/guidelines/content/PDF/mpn-patient.pdf

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Lynnvb, did you ever figure out what mutation was driving your ET?

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1 reply
lynnevb | @lynnevb | 3 hours ago
In reply to @eloise999 "Lynnvb, did you ever figure out what mutation was driving your ET?" + (show)
@eloise999

Lynnvb, did you ever figure out what mutation was driving your ET?

Jump to this post

Yes I am triple negative.

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