Progression question

[WileyFrench]

So, if the hydroxyurea keeps platelets in check, and the clopidogrel (and oodles of water) reduce platelet “stickiness”, and since the vast majority of ET doesn’t become fibrosis or leukaemia, does most ET just stay as it is - hence the “normal life span” prognosis? I know ET is considered a non-aggressive MPN, so am I understanding the future correctly? (Barring great research and a “cure” of course!)

[mhos61]

Great question. Something I’d like clarification on too.

Maybe you could pose this question to ‘MPN, Ask the Expert Live,’ Patient Power, Tuesday August 28. Dr Naveen Pemmaraju.

[WileyFrench in reply to mhos61]

Possibly. I will be travelling so I may not be able to. I’m hoping someone weighs in meanwhile.

[Ramy22]

Yes!

[PhilReade]

Hi I’m on 1500 mg a day of hydroxycarbamide I’ve still had two Haematomas two DVTS recurrent TIAs I’m now off Clopidogrel and on Apixiban daily I’ve had a splenectomy prior to the hydroxycarbamide

No one is the same with Essential thrombocythemia

[WileyFrench in reply to PhilReade]

Thanks Phil. You’re right in that we’re all different - our health, background, response to treatment etc will all produce different outcomes. I’m almost 63 and take no meds other than the hydroxy and clopidogrel, am very active, exercise a lot, no fatigue or pains and I’m hoping things stay at least roughly that way for a long time.

[Timjonze]

It seems to progress at different speeds with different people. As I understand the chance of ET becoming MF is 10% over ten years (20% over 20 years) and the chance of it becoming AML is 1% over ten years. Therefore the majority of ET cases won’t progress into those worse diseases any time soon. However they might still get a bit worse over time (ie progress towards them without arriving there) and you may need to up your dosage. Hope that helps.

[WileyFrench in reply to Timjonze]

That makes sense to me. I’m in early days of meds right now. I’ve never had any of the symptoms people describe with ET - fatigue, pain, etc. As you say, that may change - but of course I hope not!

[mhos61 in reply to Timjonze]

I know ET is not static. We are told it’s mainly indolent and progresses slowly in most cases. Hopefully I will be one of them 🤔

The bit I don’t understand, is for those of us with ET who never get MF or AML - what does that progression look like? Do we inevitably get some form of fibrosis, or do other blood cells eventually begin to elevate?

I know there are people on this forum who have had ET for 20+ years.

[Timjonze in reply to mhos61]

I think progression within ET would be platelet production rising over time but never scarring the marrow (and the marrow never producing leukaemia cells). It’s different for each person. For me I’ve got high red blood cells (still within normal range but borderline) so that looks like a potential progression to PV in the future.

[WileyFrench in reply to Timjonze]

Let’s hope your red cells just stay where they are! If hydroxyurea can lower platelet counts, is there something that can also keep red cells in the normal range?

[WileyFrench in reply to mhos61]

Yes I’ve seen posts from those with ET (and no MF or leukaemia) for 20+ years too. The stats would indicate the non-progression is the majority. Like you, I’m not sure what other “progression” there could be. Another question for our doctors!

[JackLina]

Doesn't it depend on age, TIA or stroke history etc. I'm 66, taking Pegasys and Clopidogrel and although I'm carrying on with Pegasys because it is doing the job it's meant to do, my side effects are getting more pronounced. Some weeks I'm tolerating well but towards the day I usually inject, my pain increases as does the sleeplessness and agitation. None of these cn beat the immense fatigue. It's a constant battle but when I'm woken by my quickened heartbeat (this is a new one last night) and pain in my calves, I'm wondering if this is a need for my medication or an indication that I should not be taking it today????

Any ideas? Anyone?

I hope you get the answer you want but as your many replies state, we're all different.

All the best. Penelope (Many hugsxxx )

[Wyebird in reply to JackLina]

At the moment Im on hydroxicarbomide 17x500 mg a week and 1 tablet of anagrelide a day. Im feel I’ve got more tired( lethargic) as my dose has increased. Or is it in my head and the fact I ve gone back to the gym after a 3 month break has had a negative affect on me because now I don’t want to be there.

[WileyFrench in reply to Wyebird]

I’d think that exercise would help, but maybe it should be found elsewhere than in the gym - if your head is saying you’d rather be somewhere else!

[Wyebird in reply to WileyFrench]

Yep think you are right thank you

[JackLina in reply to Wyebird]

I cannot do the gym, booooring. I love walking, cycling, yoga and squash. I have yet to get off my backside to cycle, even though I love it and I can find no-one but my grandchildren to play squash with. that's great but I rarely see them so I can't arrange regular sessions. I love yoga but am too distracted to remember to do it......... That is what this has done to my brain. Not happy with this lack of concentration but trying. Still trying to get it right.

[WileyFrench in reply to JackLina]

Thanks Penelope. Have you posed your question to your doc re your body’s reactions as your injection day approaches?

[JackLina in reply to WileyFrench]

I will have that opportunity on Tuesday, which will be my first appointment in 7 weeks.

I'll let you know his response. After the 3rd week on Pegasys I was 380 and he said stay on the same dose, See you in 7 weeks!!!!! I feel I will be 300 or less by now.

ATB Penelope xox

[PhilReade in reply to JackLina]

Hi I’m 63 I’m on hydroxycarbamide 1500 mg daily and Apixiban plus Amlodipine Allopurinol Irbesarten statin and Zapain x8 and pantroprazole and VK penicillin x 4

I to get angitated have sleepless nights joint pain lack of concentration

I also had a heart monitor recently after a TIA which shown heart fibrillation

My blood pressure up and down and my platelets are always 450 plus most recent 470 I’m back on Wednesday to see the professor Sommervaille at Manchester Christie hospital

I used to train squash gym 5 a side but don’t do anything now as my fatigue doesn’t allow me to plus joint pain

I’m worried it turning to MF or AML I see people at my clinic being treated for those it’s frightening

[JackLina in reply to PhilReade]

Walk every morning! That's how I started and I intend to keep kidnapping my grandchildren who play really basic racket ball and squash with me. It's just so kiddie fashion that it just raises my spirits enough without me feeling completely worn out. I find it hard to believe that it actually combats the fatigue. Adrenalin is a great thing.

The fact that i can only do that about twice a month is annoying but I keep up the walks. From 30 minutes to 1 hr. 20. It's up to the dog! I miss my squash so much that it hurts but cannot find a partner to play regularly and I'm not the 'squash ladder' type!

ATB. P.x

[JackLina in reply to PhilReade]

Also I just discovered the sublingual B complex from Holland and Barrett really help with the aches and fatigue.

If anyone is interested, I take Starflower, Co enzyme Q10, and vit. E as well and really notice the difference if I miss any of them. Oh, and also Vitamin D3 sublingual (DLUX). All those on hydroxy should be using this to give them the D they miss from the sun. Hope any or all of these help. ATB.

[PhilReade in reply to JackLina]

Interesting I’ll look up sublingual B I take vitamin B complex and folic acid and omega 3 6 & 9

Also Evening Primrose oil

If I need a pick me up I take malt and cod liver oil.

I miss squash I played on the ladder at my local club but cycling really helps now as I’ve stopped the squash

They always say you get fit to play squash not play to get fit

Sounds like your doing good on your keeping fit well done

[JackLina in reply to PhilReade]

I'm so not. My bike is still awaiting fresh air!!!

[JackLina in reply to PhilReade]

Hi, just a warning about evening primrose. I used to feed these to my husband until my doctor told me not to because they can cause hormonal changes. they should be taken by females only.

Just thought i should tell you! P.x

[Grumpyfairy in reply to JackLina]

Hi Penelope just catching up on posts, and I’ve been thinking of taking some extra vitamins and was looking at Vitamin B for aches and pains. Di you find there ok with your meds? I take 500g Hydroxycarbamide and aspirin, and when I mentioned to my clinical nurse I was taking Tumeric tablets she told me to stop them as they don’t know how they would effect my platelets count! Which Vitamin B do you take? I went into Holland and Barrett today and so many to choose from. Jackie

[JackLina in reply to Grumpyfairy]

They've stopped doing their B complex sublingual and keep B12, Better You instead. Very annoying because it was brilliant.

I now use Puritans Pride from puritanspride.com and sometimes just the blast of Better You B12. My doctor said it shouldn't be a problem. I did ask. Asked if it caused high blood pressure and she said it shouldn't.

I'm on Pegasys and clopidogrel. Hope this helps you as much as it helps me. I don't take the complex every day, just when I feel I need it.

All the best. Penelope

[Grumpyfairy in reply to JackLina]

Thank you Penelope, will have a look, I’ll try anything really Jackie

[JackLina in reply to PhilReade]

I doubt I would be able to do any of this if I was still on hydroxy. Pegasys has so many side effects including fatigue and joint and muscle/bone pain but for some reason, it allows me to overcome a little and get started as long as I do it first thing. The latest sublingual B complex discovery is definitely making it more possible.

[Angelinagaffer in reply to JackLina]

I think it is more the illness than the medication because I have alot of the same symptoms and 63-64 but on different medication xx😎

[Indigo42916]

It can depend on your driver and secondary mutations. I have mpl and some other secondary mutations that can accelerate progression to myelofibrosis or AML.

Best to you.

[WileyFrench in reply to Indigo42916]

Good point. What is a “driver” regarding MPN? And secondary mutations - like not JAK etc?

[mhos61]

As far as I know the ‘driver’ mutations are the Jak 2+, CALr, and MPL. They ‘drive’ the disease so to speak. I don’t know what ‘drives’ the disease in triple negative MPN patients, maybe an undiscovered mutation?

‘Passenger’ mutations acquired such as TET2 or ASXL1 for example are significant in disease prognosis; the latter mutation adversely affecting prognosis. That’s how I understand it. I’m sure someone will correct me if I’m wrong.

[WileyFrench in reply to mhos61]

Most interesting. Thanks a lot for this.

[ourlife]

Hi WileyFrench,

I have not been on the site for a long time and the progression questions are now a burning issue for me. I started with ET 19yrs ago aged 30.

And to cut a long story short, after 15yrs I progressed to PV with signs of fibrosis, but not enough for a MF diagnosis. I have become intolerant to most medications and am now on a cocktail of Rux & HU and will be having a BMB in two weeks to see what is going on. We will all progress differently if at all, and it is this side of our condition where mental health becomes important, although concerned I am not worried or anxious , I hope your question was born out of curiosity and you are coping well. I struggled in the beginning and needed a lot of help and support.

Good luck and have fun,

Ourlife

[WileyFrench in reply to ourlife]

Hi and thanks a lot for your reply. Yes, at this point my question was just curiosity. Your thinking of being “concerned but not worried” seems to me to be very smart (even if not always easy). I’m going to try to stay with that, and I really hope your BMB goes well. 👍

[Joe1117 in reply to ourlife]

Hi, i also have been diagnosed pretty early. I wanted to ask you if HU has affecting your fertility in anyway? The Docs want to to start treatment but i am very worried. Have you been through HU treatment?

[WileyFrench in reply to Joe1117]

I’m 63 so fertility isn’t an issue for me but I understand that hydroxyurea can affect a foetus. Maz (this site administrator) has posted on this subject and if you do a search of “hydroxyurea and fertility” on this site you should find her post right away.