ET progression

[Camelian]

Does progression of ET come with the length of time you've had it or is it related to the platelets going higher? I'm trying to fit all the pieces together to understand the condition. The hematology nurse told me the risk of developing leukemia is a little higher because I'm Jak2+ but reassuringly said it only happens to a very low percentage of people- she didn't say if risk of leukemia comes from the medication or the condition itself I've heard both?

By progression I guess they mean more symptoms associated with the condition like fatigue, leg pain, hot flushes headaches etc. My thoughts initially was these symptoms were side effects of the medications. But I have all of these symptoms and bad hip and lower back pain and at the moment I'm only taking aspirin? Maybe my aches and pains are related to other things.

[WileyFrench]

I have ET too and the Canadian Lymphoma Society (I’m in Canada) says progression to leukaemia is very low percentage. My haemo says 3 to 5% possible. I think, as with all diseases, the longer it’s around the higher the likelihood of change. There are people on this forum who’ve had it for decades with no progression and they seem to represent the statistical majority. The idea of progression is possibly the most worrisome part of having this condition but I’m trying very hard to assume we’ll be part of the non-progressing majority. And I’m betting that we will be!

[Camelian in reply to WileyFrench]

That's a very positive response and just what I needed to hear so thank you for that. Living with the condition is scary but your right the possibility of progression is the most scary.

my uncle had leukemia so I think that's why I've latched onto that element of the condition but there's probably no connection?.

[WileyFrench in reply to Camelian]

Nope. My haemo said that family history doesn’t play into this. I have zero family history of TIA or any blood disorder and yet, here I am - with both!

[JackLina in reply to WileyFrench]

Ditto. I would agree with your very positive outlook. It's the way I do things. It's almost impossible some days as I have daily, those symptoms Camelian describes and more. That message tells me they are the condition rather than the medication. I'm on Pegasys and Clopidogrel. Next blood test results are Feb 13th. but on the last one, by platelets were i normal range, yet still those symptoms continue. I find it rather confusing.

[Camelian in reply to JackLina]

Me too jacklina I'm very confused.

I was diagnosed last year but was told my platelets have been up and down since 2015. Could be longer. Maybe the platelets shoot up and that causes the symptoms?? As I said I've attributed theses symptoms to menopause. Stress and genes as my mum and her mum suffered the same things. My mum did have 2 clots and phlebitis when she was pregnant with me??

I did however start having bouts of vertigo a couple of years ago but that seems to have settled down I get it occasionally when I have have an episodes of migraine- and again migraine is a family thing linked to hormonal changes??? So yes it's all very confusing.

[JackLina in reply to Camelian]

I have had (over many years) TIAs and a minor stroke in 2012, since which I have been taking clopidogrel due to an allergic reaction to aspirin.

I am well past menopause and the 'inner heat' we (ETs) experience is different from that which I experienced when going through that. I AM a stress-head! Always have been. I never suffered migraines but a number of times (first in around 2005) I did suffer what I now understand to be 'silent migraines' when I lost one side of my sight, my speech and understanding of words and total confusion. I had no headache at all and recovery was anything from 20 minutes to 2 days. These became more debilitating as years went by with weakness in legs and arms and other symptoms. I was treated as though these were TIA but it transpires they were another symptom of the MPN. I was only diagnosed in March 2018, when I was suffering these weekly. Thank goodness I was because I am convinced I would have suffered a major stroke had I not taken myself into hospital and for once, told someone I had suffered a TIA This got me all the tests you can imagine. I am ET & JAK2+. No idea when that mutation occurred or how they deduced this. I had CT & MRI scan numerous phials of blood taken for goodness knows how many different tests. I also had a lumbar puncture. Since changing from hydroxy to Pegasys I have had no TIAs or similar episodes. I do however continue to have many side effects. There are more people who do not have any at all, so don't take that as an indication of what you may experience. I a notoriously intolerant to anything that isn't made my my own body. Most foreign bodies are rejected!!!

I do hope this helps. All the very best.

[Wyebird in reply to JackLina]

Hi I’ve just read your reply. I’m curious about ‘inner heat’. I’m Calr Et. The other night I experienced an unbearable hotness all over my body. Removing bed covers did little to help so I put a fan on which in summer I always found relief from. In the end I took sleeping tablets. I didn’t sweat. Could this be what is meant from ET night sweats?

[JackLina in reply to Wyebird]

Hi Wyebird. That is as I understand it. I eventually purchased an under mattress protector that professes to be cooling. They were selling a lot of versions of these when we had that very hot spell last year. I tried it for a while and it did make a bit of difference, I thought. It was only when I removed it when stripping the bed and decided to leave it off because the weather had cooled down a lot that I discovered how effective it was. There were my night sweats, straight back.

I tried sleeping without it again two nights ago because the weather is really cold....not for me without it. It's back on there again and I look forward to a comfortable night sleep tonight, with perfect temperature. These are nothing like those I had with the menopause. The only similarity is the heat.

[Wyebird in reply to JackLina]

Thank you if it happens often I’ll bare that in mind.

[Scottishterrier]

Hi i have had it since 1994 and it is still ET yes i have had 3 clots to the liver one of my old consultant's told me this is very rare for it to change to Leukemia this is because it is an older persons disease i have a very close friend his Mother is 90 and just went down with it so i say do not worry till they tell you have it and that may be many years way of

best wishes

ScottishTerrier

[Camelian in reply to Scottishterrier]

Thank you

[Searcher56 in reply to Scottishterrier]

Can you explain to me what these "clots to the liver" are? I have only had one ultrasound scan on my liver when I was first investigated for ET and the radiologist told me I had a cyst on my liver.

[Scottishterrier in reply to Searcher56]

the three clots were at different times and it was in the arteries that led to the liver but the lucky thing about it being the liver is that it is the only organ in body that heals itself

[Deefen]

I have ET - JAK2 positive - only on aspirin. I don’t have any symptoms- No headaches, muscle pain etc. I thought I was perhaps getting some hot flushes, but it’s hard to tell when the temps have been soaring into the high 30’s - and hubby says he’s feeling abnormally hot too.

I was only diagnosed a few mths ago - but it looks like I’ve unknowingly had this for many years. So as far as progressing? Mine definitely hasn’t, I was only picked up via a routine blood test. I’m fighting hard to stay off harsher meds by trying to keep exercising and eating a ketogenic diet.

[Camelian in reply to Deefen]

What's a ketogenic diet?

[Deefen in reply to Camelian]

Very low in carbohydrates, very high in good fats. I must admit I feel pretty good when I stick to it - lots of energy and mental clarity, zero hunger. Bonus is I’ve lost about 12kg.

[wotan10]

Good morning Camelian.Great user name.

Et is a complex issue but it is a condition that is managed with sensitivity by your team.

By now you will have realised that there are lots of fellow surfers like yourself who are still enjoying life and have had ET for a significant number of years.

The key here if you can see is to learn to adapt to the changes needed by You to find your own safe space listen to your body,sip water through out the day, take your medication consistently.

Remain positive.

Making sense of the big beauty is quiet normal at this stage however for me it was about accepting this changed situation and moving on.

I am two years into this new adventure and I will do everything in my power to carry on surfing.

Hope this helps.

[Camelian in reply to wotan10]

Good advice woton

[westlieght in reply to wotan10]

Yep me to with respect felt and still keep positive then -all going along thinking ok can live with this -wops another bonemarrow by-op diagnose post ET/MF watch and wait aspirin depended 4 monthly transfusions!

It’s a changing blood disease !

Best wishes

[Bigmann1 in reply to westlieght]

Your story sounds just like mine. Dr told me don’t worry everything is fine. Your going to live a long time. Bmb results oh ya btw you have to change your meds and take jackafi now. Wow.

[beetle]

In this day and age with so much research going into MPNs around the world and a far better understanding of the significance of genetic mutations there is little point in worrying about statistical progression for these diseases as by their very nature the statistics are historical and the holy grail cure might be just around the corner😀. I am still hoping for a cure for my post ET MF. I was diagnosed with ET in 1994 and MF nearly 7 years ago. I am still going (I won’t add ‘strong’ to that though). I still believe a cure could come along even in my lifetime and by that I mean a cure that does not involve the risks of stem cell transplant. Have faith and try not to worry about the future. Live life to the full each day - it’s the only way!

[Bigmann1 in reply to beetle]

Hi thank you for your post. What medications are you taking?

[beetle in reply to Bigmann1]

Main one is Jakafi but it’s starting to fail. I seem to have accumulated other drugs to counteract side effects along the way too! I am also transfusion dependent so I’m using desferioxamine to counteract too much iron.

[Bigmann1 in reply to beetle]

How long are you on jakafi? I was just diagnosed with et mf and put on jakafi. Only two days now. I hope this works. I’m very scared after reading and researching. Thank you for disclosing your thoughts.

[beetle in reply to Bigmann1]

I’ve been on Jakafi for over five years and it is now starting to fail. I’m seeing London specialist next week so hoping for alternative suggestions. I was also very scared when I got MF diagnosis but I’m still here nearly 7 years later!

[Bigmann1 in reply to beetle]

I appreciate the private information you are sharing with me. We only can be positive with respect to this diagnosis. I realize now that everyone’s situation is different. Did you have any side effects on jakafi? Was your life normal while you started to take it? Do you exercise? I have so many questions. I hope you don’t mind me asking.

[beetle in reply to Bigmann1]

No I don’t mind. Bear in mind that we are all different though. Jakafi brought down my blood counts and now, more likely as a result of disease progression, they are all low. I became transfusion dependent after a while. I have also developed high blood pressure and cholesterol. To begin with I was fairly ok but I take life a lot slower now. My brain still says I can conquer the world but my body does not really agree😂. Although Jakafi appears to give a survival advantage it is not a cure and the disease progresses in the background. The only cure at the moment is Stem cell transplant which for various reasons I decided against. For some though it has been a spectacular success and others have not fared so well. It might be something you consider?

[beetle in reply to beetle]

I forgot to say that I do gentle exercise in the form of walking daily, weather permitting. I also do housework and some gardening but nowadays I find breathlessness limits my activity

[Bigmann1 in reply to beetle]

I’m going to pray for you. I’m glad you do things you like to. As for me, struggling with my rbc numbers this year and recently getting really bad night sweats. My rbc count was 8.9 and my bmb resulted in scarring. I look on internet and see the Mayo Clinic talking so highly of jakafi slowing progression. I hold on too any positive I read as I read horrific stories. My oncologist suggested I wouldn’t be a candidate for bmt. I have to get a second opinion. I do like my dr but feel as though he could have diagnosed me sooner. He kept me alive for 15 years and I never really had such problems. I’ve been on hydrea for more than 10 years. I just had two test done last month. Bmb and liver biopsy. My liver doc said liver has nothing to do with the liver enzymes escalating. She said I have to go back to oncologist and see what he can do. When I went into his office he very calmly told me that my dresses had progressed and that he was changing my medication to jakafi. I was devastated when I got home and started to read the statistics etc. do you have any suggestions for me and as you been through this in an early phase what should I do? Do you take supplements? I’m 65 years old I hope and pray I can get in too my 80’ to see my grand children grow. Thank you soo much for your time. May god bless you!!!!

[beetle in reply to Bigmann1]

My grandchildren are part of my motivation too. My liver enzymes were high but have come down since stopping a statin. I have read of good results combining Jakafi and interferon in early progression. Might be worth you looking into. Try not to read and scare yourself with other people’s stories. I try to latch on to the positive ones and forget the others. I think a positive attitude is very important. I take a vitD supplement which has helped considerably with bone pain. I am considering going onto plant based diet and if so will take B12 too. My diet is mostly fruit, vegs, fish and chicken but I think I could happily cut down/out the fish and chicken. I have always liked pulses, legumes and nuts so it wouldn’t be a hardship for me. I too took HU for many years before Jakafi

[Bigmann1 in reply to beetle]

Having problems with urine retention. I read jakafi causes bloating but not being able to urinate is not good. Did you experience this?

[beetle in reply to Bigmann1]

Not actual retention but countless UTIs!

[sugarbear56 in reply to beetle]

Hello beetle, in reference to your uti's...consider trying cranberry vitamin...I've been taking it since diagnosed with ET in 2007. I have not had one since. Was always prone to uti's. Hope it helps you!!

[beetle in reply to sugarbear56]

Thank you. I’m not keen on cranberries and they didn’t seem to do much for me but I have found an apple cider vinegar that is very tasty and easy to take even without honey. It appears to be helping and got the thumbs up from a urologist I saw recently.

[Bigmann1 in reply to beetle]

Hi beetle! How are you feeling lately? I hope your doing well. Just wanted to let you know I started jakafi and I acquired strep throat after 8 days. I’m disappointed. Hemo suggested stop jakafi till Tuesday. Hope you are ok

Ttys

[beetle in reply to Bigmann1]

Hi Bigmann1. Thank you for asking after me. I am not doing too badly. I saw Prof Harrison today and she is suggesting winding down Ruxolitinib have a break and maybe go back to it. She made one or two other suggestions including drug trials but they would be logistically quite difficult for me. At the moment my platelet count is too low to qualify for the trials but the break in Ruxolitinib might improve the count. A lot to think about

Take care and hope your throat is soon better

[Bigmann1 in reply to beetle]

Hi Beetle

Well I hope that works. Seems like it would. I did read that eating sweet patatoes raise platelets. No dietician but maybe you can check that out. I k ow you eat very clean so just in case. I started jakafi for 8 days and night sweats stopped. I was so happy! Finally, I thought I was making progress then boom got hit with an infection. Step throat! Called my hemo he instructed to stop jackafi for a couple of days. What do you think? I little scared my hemo text me asking me if I had any more fevers after going home from er. I wonder if he’s suspicious thinking this might turn into something worse? I do know that my bmb showed negative for aml and that was in December. Anyway, wondering if you heard of any anti-fibrotic agents that might help you? I read about prm151 trials. They sounded very promising!!!

[Bigmann1 in reply to beetle]

Hey how are you? Have you gone to see the dr lately?

[beetle in reply to Bigmann1]

Hi. Transfusion yesterday and now urinary tract infection. Feeling not quite on top of the game today. How are you?

[Bigmann1 in reply to beetle]

Ok still have the night sweats been three weeks now on jackafi waiting to see if it kicks in. Sorry to hear that your not feeling well. Did they take you of jackafi yet? I hope your counts go up Quickly and you feel better soon. They will! Have you needed many transfusions while on jackafi? I also have low energy. I heard recently good things with aaranesp and rux combo for anemia. Also azetezkne and rux combo. Have you heard about these?

[Deefen in reply to beetle]

Wouldn’t that be nice!

[ccsial]

I was diagnosed with ET JAK positive about 16 years ago. Last year it progressed and I had PV also, A few months ago it progressed to MF. I am now TP53 positive and an AML progression is likely.

Mayo clinic said average life expectancy of ET is 10-15 years. Some go a lot longer. This disease is like a time bomb without knowing the time.

I was and am still on two 500mg of hydrea since the beginning. My doc has now added two 20mg of Jakafi daily.

[Camelian in reply to ccsial]

Wow that's scary although I've neither been told or read this. Some people on this site have had the condition for decades so I'm holding on to that and trying to stay positive.

[JackLina in reply to Camelian]

Don't be scared. Check the information. I'm sure Maz will reply to this for you. Her experiences far outlive the timeline suggested above. ATB.

[Camelian in reply to JackLina]

Thank you jacklina I will certainly try hard.

[Mazcd Partner MPNVoice in reply to Camelian]

Hi Camelian, we can all understand how scared you are feeling about your diagnosis and how it will affect you, but try to calm down, easy to say I know, but there are a lot of people around the world with ET who have had it for years and years and are still going strong, this is what we have on our website: Prognosis

-

If you have ET, your prognosis depends on many factors, including your age and whether you have other illnesses or complications.

ET patients who do not suffer from severe thrombotic (clotting) or haemorrhagic (bleeding) complications can probably expect a near-to-normal lifespan.

I myself was diagnosed with ET in 2003 and I'm still going strong and been on Hydroxycarbamide for 11 years. Some patients with ET develop acute myeloid leukaemia (AML) or myelofibrosis (MF) at a late stage of the illness. But this is very rare, and again very rare is the possibility that Hydroxycarbamide may increase the tendency of MPNs to change to a form of leukaemia, this is very uncommon and it has not been proven that this is a result of treatment with Hydroxycarbamide. So try not to worry too much, my advice would be to read about it on our website, mpnvoice.org.uk, and look at some of the videos. I am sure this will help you. Best wishes, Maz

[Camelian in reply to Mazcd]

Thank you your words are reassuring and I will have a look at the videos.

[Cja1956 in reply to ccsial]

I was diagnosed with ET Jak2 positive 11 years ago and it progressed to PV about 2 years ago. I also read that average life expectancy is 10-20 years. I’ve had a very difficult time dealing with this emotionally since I’m only 63. But I am finally decided to face this head on. I started seeing a therapist, going to yoga, and walking more for exercise. I take 2 hydroxy and jakafi per day and of course, 1 baby aspirin.

[Camelian in reply to Cja1956]

That heamotologist telling you things like that is not very helpful. People on here have had the condition for decades so I'm trying to stay focused on that. I always read they've made great progress in treatment over the last 5 years so hopefully the next five years will bring a cute.

[Cja1956 in reply to Camelian]

No, my hematologist never told me that. I read it on Google when I was researching PV life expectancy. He told not to look online for answers years ago when I got ET. But when it progressed, I got worried.

[Meatloaf9]

Hi Camelian,

I am 69 and ET Jak2+ (28%AB). Diagnosed at end of 2017 but likely had it a lot longer. I was told by a specialist at the cleveland clinic that the risk of AML is 3% per year (does not increase with years, stays at 3% each year). Also, the risk of progression to MF is about 10% over 10 years. They said the drugs do not cause the progression it is just the disease that does it. He said that most people live a near normal lifespan, he could not say what a near normal lifespan would mean. How old are you and how long have you had ET if I may ask? Best wishes always.

[Camelian in reply to Meatloaf9]

Hi meatloaf no I don't mind you asking at all. I'm 57 and was diagnosed last year but the Dr said my platelet count has been up and down since 2015 so I guess I've had it longer. I'm really trying hard to hold onto the positive stuff I'm a worrier by nature so I'm finding this diagnosed a real challenge I'm buying s many positive thinking books as I can find so fingers crossed.

[Meatloaf9]

Hi Again, I think I am a worrier also. It is hard to go very long without thinking about this thing we have. I have decided that the best thing for me to do is find a good doctor or doctors , which I think I have done, and then do whatever they think is the best thing to do, then after I leave their office I want to go out and live my life as if I didn't have this thing at all and not worry about it at all. Can't always do that, but it seems to work better for me in dealing with this thing (my name for it). Whatever works for you is best. Live long and prosper.

[Camelian in reply to Meatloaf9]

Thanks for your words of wisdom meatloaf your coping strategy sounds good to me I'm going to try and apply the same principles.

[JackLina]

I don't think anyone has said this to you yet but if you can get into walking daily and yoga, you will immediately feel the benefits, mentally and physically. x

[Camelian in reply to JackLina]

Funnily enough jacklina I was speaking to my sister earlier about starting a yoga class and we agreed we'd do more walking. As I mentioned my job involves a lot of computer work so quite often I'm sitting for hours this definitely has to change. I will start the new me next week. I'm getting there now with drinking water which has been a task in itself. I've drank more water in the last 6 months of my life than I have in the other 56.5 years of it but I do feel better for it so it's all good.

[JackLina in reply to Camelian]

Hi Camelian. I can identify with your work situation. I too spend a lot of time at the computer for work, which annoyingly continues at home!

Thankfully I have a dog who walks ultra-high speed and this is what I make myself do every morning. Prior to diagnosis I regularly did yoga and squash! I no longer play squash but only because my husband will no longer play for his own reasons! It's taking a lot of effort to keep up with the other two and I feel guilty every day that I don't do my yoga every day but it will come, I'm sure. Fingers crossed.