ET Progression?: Are younger patients at higher... - MPN Voice

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ET Progression?

peach98
peach98

Are younger patients at higher risk of progressing to MF since they are living with the cancer for longer? Anyone have information regarding this? Having a hard time finding out my life expectancy. My platelets have been elevated since I was a kid. They go up VERY SLOWLY every year. Just turned 22 and my platelets are 502. Two years ago 499. 5 years ago 437. I always ranged in the high 400s but finally hit 500+ this year. I’m doing jak2 testing in 6 months but my hematologist is already sure I have ET.

He doesn’t use the C word. He calls it “disease of the bone marrow”. He warned me of bone marrow scarring. Worried about progression. If I do have ET that means I’ve had it for 10+ years now and I’ve seen online progression percentage increases every 10 years. I just want to make it to at least 60 without progression.

25 Replies
oldestnewest

It’s good you are being monitored so closely now . Consider looking in to anti inflammatory diet . Have you had any other tests done to rule out steady rise ?

I’m 64 and only taking aspirin . Feel better than I did when I was your age as always had something wrong .. fainted from very young , tired etc .

But years of doing things like yoga , using natural treatments and support from great practitioners have paid off

DESPITE being diagnosed withJAK2 a year ago

I really am feeling both emotionally and physically better

Stay positive x

peach98
peach98 in reply to Mostew

I haven’t had any other tests done to rule out reactive thrombocytosis. But along with the jak2 testing, my hematologist will also be testing my iron and ferritin levels. I think he’s testing iron because my hemoglobin is always on the lower end of normal. I think hemoglobin and iron are the same thing but I’m not sure... my hem is 11.8. I don’t think it’s ever been past this number. It dropped to 10.3 two years ago when my platelets were at 499. But now that’s it’s back to my “normal” which is 11.8, my platelets still went up to 502. He doesn’t think I have reactive thrombocythemia and said I fit more into the primary category... these other tests are just to be 100% sure. I am looking into a better diet and foods that lower platelets.. trying to hold off from taking the chemo drug for as long as possible. I am trying to stay positive but scared for my future... Thank you.

Wyebird
Wyebird in reply to peach98

Lol iron and heamoglobin are linked but there again so is ferritin. I have problems understanding . I take my hat off to those who do. Take care try not to worry . Easier said than done. You are being monitored that’s the good thing

I'm surprised they've diagnosed ET on the basis of slightly high platelets. Also why are they waiting 6 months to do the genetic mutation tests?

My Platelets have been slightly high for 10 years...negative for the 3 main mutations. Had BMB but waiting for results. I've also had low iron levels in the past but they're fine now but haematologist still can't rule out secondary thrombocytosis.

Try not to worry about prognosis etc until you have a definite diagnosis. I would be pushing for further testing if I were you...especially before contemplating medication.

I wish you well x

peach98
peach98 in reply to Jessiexx

I’m not sure but he told me he’s put me in the “primary thrombocytosis” category simply because of the information I gave him. Which is having high platelets since I was a kid. Not sure what age exactly (9-11?) but my mom mentioned the doctors would always tell her I had high platelets. I guess it wasn’t high enough to investigate so they just let me be for 10+ years until my new practitioner came along and my rising platelets worried her enough to refer me to a hematologist. I don’t know why he’s waiting 6 months either. He told me he’s sure nothing will change and still believes it’s a problem with my bone marrow but then told me to go home and not to worry about it or google anything. I wish he would test me sooner I can’t go on for 6 more months googling like crazy trying to find out how many years I have left before progressing.. especially if I’ve had it for this long.. I can only imagine how little time I have left before MF. I’ve looked at the list of secondary causes and none of them apply to me sadly so I’m not sure what other tests I can push for. I feel fine generally and have had no health issues. Please let me know the outcome of your BMB. I’m wishing you all the best.

Jessiexx
Jessiexx in reply to peach98

Aww bless you I'm not surprised you're going crazy with it all. My advice would be to write/email your haematologist telling them how anxious you are feeling and request the testing to be done now for a definite diagnosis (genetic mutations and if appropriate a bone marrow biopsy)

Apart from occasional low iron but never anaemic none of the secondary causes apply to me either but haematologist still believes unlikely primary. Maybe some people have raised platelets with no real cause. I'm not sure. Luckily I will find out in a few weeks. Please consider writing that email, sometimes you have to push a little for things to happen. It's your health after all. Good luck x

peach98
peach98 in reply to Jessiexx

Haha our hematologists are complete opposites! Yours is optimistic and thinks it isn’t primary while mine is basically giving me a death sentence. Praying yours is secondary/reactive. Good luck to you as well

I'm so sorry you have to go through all this stress at such a young age. If you do have ET, please remember that many, many people never progress to MF. It would be a tragedy to spend your life worrying about this. My sister saw a psychologist after she had breast cancer, the psychologist told her that she had seen far more lives ruined by fear than by cancer.

I had ET for 19 years, it progressed to MF this year. I was terrified as I used to think MF was an automatic death sentence but it turns out that there are effective treatments (including stem cell transplants) and even more will be discovered before you reach that stage IF you do.

The main thing for you is to get a proper clear diagnosis and be monitored. When that's sorted, try to enjoy your (long) life. Best wishes to you, Jennie

peach98
peach98 in reply to Otterfield

Oh boy. 19 years.. I bet you did everything right and it still progressed. I’m sorry about your progression. How are you holding up? Are you on any medication or planning on getting a bone marrow transplant?

Also, isn’t ET a chronic cancer that will get worse over time and bone marrow will scar overtime so progression is inevitable? My fear is the percentage applies to the more prominent age group of >60. I would assume most die of old age before it even gets to progress which is why the percentage of progression is low. I wish there were more information about my age group. I have not come across anyone online who was diagnosed in their 20s and lived until old age. I wish I could. That would ease my worries but so far none..

Otterfield
Otterfield in reply to peach98

Weirdly, having MF is not nearly as bad as I had always assumed. I'm on Ruxolitinib which is a very effective drug for me and my energy levels are pretty good. I am also having red cell transfusions but my bone marrow seems to be doing a reasonable job of making platelets. I was only diagnosed in August and although a stem cell transplant has been mentioned, it's still very early days.

I don't know about any research about the relative probability of progression in young people. However my ET was stable for many years and I lived a normal life, with no changes in my blood. And, as I said, progression doesn't happen to everyone and even if it happens to you, there's no reason to assume you will die from it. Suppose, for example, that you had ET for 30 years - imagine how many new treatments will have been researched by then.

I think you need full and clear information from your haematologist and a discussion about your fears.

peach98
peach98 in reply to Otterfield

Thank you Otterfield 🦦 I’m actually thinking of getting a second opinion. My current hematologist seems very pessimistic and he doesn’t say it’s a cancer which worries me he won’t take my problems seriously.

I can completely empathise with you, I was diagnosed 3 years ago and had the same frustrations, everything you read is focused on people being over 65 unfortunately, which adds a worrying edge in terms of life expectancy for obvious reasons. There are more of us on this group and MPN Voice FB page who are younger. Much like yourself my high platelet count has been ignored since I was 22 (this is as far back as my medical records go, due to moving) but it’s likely they’ve been higher prior to that. I moved GP surgeries due to not being happy with the care I received and the new GP instantly looked at my blood results and referred me to a haematologist that day, I was seen the following week and given a preliminary diagnosis of ET pending further blood tests, he took bloods at my first appointment for genetic testing, these did take about 8 weeks for a result and to be discussed by a team of consultants. It’s certainly worth pushing for the testing to be done sooner.

I should say I’ve just turned 40 and had two kids in my 20’s. My platelets sit between 450 - 600, I started hydroxy last year with no real side effects. ET is a chronic cancer in the sense that we can live with it, I’ve been told to expect a “normal” life expectancy, none of us know how long we have left so I try not to worry about it now. My mantra is ‘life is for living not worrying about dying’. it took me a little while to get to this headspace though! Take care of yourself and keep using groups like this to ask questions and seek support, it really does help xx

peach98
peach98 in reply to Trocken

Thank you. This comment made me feel better. I am trying so hard to not let ET take over my life but knowing how anxious I can get it’s been hard. You got diagnosed so long ago and still living a normal life without progressing gives me hope!

You seem very bright and informed for someone so young. It’s good that your doctor is covering all the bases. I was diagnosed with ET in 2008 at age 52, now progressed to MF. Since my original diagnosis, there has been tons of research and you will benefit from that. This site will also give you lots of support, as you can see. Hang in there and try not to focus on this too much.

I wish you all the best. Keep us up to date on your progress.

peach98
peach98 in reply to Cja1956

Thank you :). I will update you all of you once I get my diagnosis. I’m also going to be experimenting with natural supplements and foods that lower platelet counts (even though mine aren’t THAT high) i’d still like to try so I can let everyone know. Currently eating crushed raw garlic everyday.

Pediatric MPNs are very rare and there is even less known about them than the MPNs that occur later in life. This reference covers childhood - young adults with a MPN. Here is a link to a very good resource on this topic.

mpnadvocacy.com/pediatric-mpn/

The short version is that you will likely live a normal to near-normal lifespan. If you do have ET, the bigger issue is managing symptoms that occur. Preventing thrombosis and hemorrhage (there is risk of both) is important. There are also secondary symptoms that can be bothersome. This is related to the impact of the deregulation of the JAK-STAT pathway that is involved in MPNs. At the core, MPNs are inflammatory disorders and there are other issues we can experience beyond elevated blood cell levels.

FYI - I was diagnosed with ET in my 30s. It progressed to PV about 7 years ago. Now at age 65 I am still alive and kicking. I have had a good life and despite a few challenges of late, I continue to live a good life. I think you should plan to do the same.

Assuming you do have a MPN, you have entered this journey at an early age. There are a few others on this forum in a similar circumstance. One of our MPN brothers just turned 18 and has platelet levels in the low 1 million range. he is doing fine despite the ET and some other health issues. Your level of thrombocytosis is very low. It is likely your level of risk is also rather low. There is not straight line correlation between risk and platelet levels when you are below 1 million. It is common for everyone to have their platelet levels vary by as much as 100K in a matter of days. This is a normal reaction in the body to a variety of things (inflammation, bleeding, injury, illness, etc.).

The next step for you is to get a clear diagnosis. That would involve checking for the common MPN Driver mutations (JAK2, CALR, MPL). I am not sure why you are waiting for 6 months to do this as it is a simple blood test. If you are JAK2 positive, you will also want to know what your JAK2 Mutant Allele Burden is (% of hemopoietic stem cells with the JAK2 mutation).

It is very important to consult with a MPN Specialist rather than a regular hematologist. Most hematologists have little to no experience treating MPNs. Here is a list of MPN Specialists. mpnforum.com/list-hem./ .

One of the bigger challenges all MPN patients face is managing their care effectively. Assertive patients receive higher quality care. Passive patients do not. It is your responsibility to ensure that you receive the high quality care you deserve. Educating yourself about MPNs and then advocating for yourself is the key to getting the kind of care you need.

All the best to you on this journey.

peach98
peach98 in reply to hunter5582

Thank you for all the informative links! I will check them out. I actually don’t want to wait 6 months. That was my hematologist who scheduled it that late. I do have problems with being assertive as I’ve always had an anxious and passive personality so I’m still trying to find the courage to ask him if I can do it earlier.

hunter5582
hunter5582 in reply to peach98

Being a passive recipient of medicine is all too common, even with older patients. Many people have been programmed not to challenge a paternalistic authoritarian relationship with doctors. This is just bad medicine. You are in control of your own medical care. The doctor works for you, not the other way around. You want to seek a positive collaborative relationship with your doctors, not an obedient one. Doctors make recommendations. Patients make decisions. Doctors write orders for patients. They do not give orders to patients. If you are not happy with the current treatment plan, change it. Definitely follow up with a MPN Specialist ASAP. Do not wait to do a driver mutation test unless you really want to.

If you are anywhere near Baltimore, I highly recommend the Johns Hopkins MPN Clinic. One of the MPN Specialists who is a presenter on the Pediatric-Young Adult videos I sent, Dr. Linda Smith-Resar , is based out of Johns Hopkins. This is where I see a MPN Specialist. It is a great care facility.

Saw your question about Pegasys, which is the brand name for peginterferon alfa 2a. It is one of the PEGylated interferons. PEG-IFN is easier to tolerate than the older versions. The is an even newer version called Besremi (ROPEGylated interferon) that is awaiting FDA approval in the USA. It is supposed to be even easier to tolerate. If you need cytoreduction, then this would be the first-line choice for someone your age. The other common choice, hydroxyurea, is contraindicated for younger patients (particularly those of child-bearing age).

It is actually a moot point at this juncture. Your platelet levels are so low that in the absence of symptoms at most the recommendation would be for aspirin only and monitor. I was on aspirin only for most of my 30 years with a MPN with platelets cycling between low 500s to mid 700s. I have never had a single incident of thrombosis, tending more towards hemorrhage when on aspirin. Do be aware that your risk can go either way with ET, thrombosis or hemorrhage.

Do please stay in touch and let us know how things go.

I agree with Hunter that you should see a MPN specialist. At such a young age I think it would benefit you to have a hematologist who specializes in these diseases and keeps up on current treatments. I think most hematologists/oncologists rarely see MPN patients and are busy with the more common diseases. For instance, when I asked my local hematologist about using Pegasys for my ET he laughed and said that interferon is something that was used for Hepatitis C in the past. Luckily I have a Specialist who is up to date on current therapies. I am 71 with ET and currently take only aspirin, my ET probably started in 2003 per old blood tests but was only diagnosed 3 years ago. Good luck to you, the state of the art in treatment is changing constantly, live your life as if you did not have this disease while always keeping up with it.

peach98
peach98 in reply to Meatloaf9

Thank you. I will try to get into contact with an MPN specialist from Memorial Sloan Kettering. Also, Is pegasys and interferon alpha the same thing? I’m keeping track of all medications that will be beneficial for the future in my notes. I’ve seen a lot of great things about interferon. Then I saw the price and almost fell out my chair lol

Pegasys is a newer form of interferon that you take less often usually once a week, I think interferon alpha is injected nearly every day. It is supposed to have less side effects and is better for young people especially women who may become pregnant At 22 years old, you may not need any cytoreduction at this time. Some doctors believe that starting treatment earlier is better and some take a watch and wait approach. There is even a newer form of interferon, I think it is called ropeginterferon, it may not be approved in the US yet and maybe not is some European countries, I don't know. I would just try to find a MPN specialist hematologist and follow their instructions, There will be so many advances during your life that MPN's may even be cured. Live long and prosper, I'm sure you will.

Dovme
Dovme in reply to Meatloaf9

The newer form l think is called Besremi.

I checked here on the German MPN forum and It seems to have actually replaced Pegasys in Germany.

aoporphan.com/global_en/our...

No. Being young when you get ET doesn't necessarily mean progression is more likely. I was diagnosed at 17 and am now 50. This is a question I have asked many times over the years. I had increasing platelets for a while after diagnosis but eventually oved the years they have dropped without treatment. This doesn't mean that my symptoms are less though. I was actually more symptom free when my platelets were very high, 1.5million at diagnosis and now that they are only around 400-500 I have more symptoms. The number is not everything.

I have asked about progression and it seems it has more to do with the driver mutations you have rather than the age you get them. In fact knowing about your disease early means that you can look out for signs of clotting and get treated to prevent anything serious happening. You can also choose to live a healthy, active life to maximise your chance of living well.

There are other reasons to get high platelets so without the test for the mutation you won't know for sure but don't get too stressed. I lived most of my life with very few problems from my ET and although I am starting to get some now I am still going pretty well. A lot of the stats for progression etc is done on people who were first diagnosed in their 50's and 60's so read the literature with care as it may not always be the same for you. Question your specialist about things that worry you and try to stay positive.

Regards,

SliverET

peach98
peach98 in reply to SilverET

Thank you so much! I’ve stopped googling anything relating to progression it’s driving me insane and turning me into a crazy person! Trying to be more positive. Can I ask what is your mutation or are you triple negative?

Good idea. You can get stuck in the Google rabbit hole guessing what might happen to you and it all seems overwhelming. Your platelet levels are only just above normal and the body is an amazing system that can cope with a lot before it has issues. In fact stress is probably a bigger risk factor right now for you than ET. So try your best not to go crazy with thoughts of 'what if?' Easier said than done, I know.

I'm Jak2+ but they didn't know about any of the mutations when I was diagnosed. They only discovered them years afterwards. The good news about all this is that while you are young the body seems to cope with a lot. Even with a very high platelet level my body just functioned like normal. I went untreated by anything (not even aspirin) until I was probably mid 30's and had very few issues. I have never had to have cytoreduction (chemo) and have had very few patches where things went a little haywire. I was just monitored occasionally and lived my life exactly as I would have had I not had ET apart from extra monthly blood tests. I don't seem to suffer the fatigue that people with MPN's often do (or if I do I don't know any difference as I have nothing to compare it to). I find that exercise gives me more energy. Even if I don't feel up to it at the time, after I run I feel more alive and in balance. As I am getting older it seems I am having more symptoms but most of them are manageable. I remember my very first haematologist told me that ET is something most people die WITH but not OF. I have used that as my mantra over the years.

A lot of doctors know very little about MPNs so if you can find a good specialist to help you monitor and manage your health that is the best thing you can do.

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