hi i am still very worried about et diagnosed in younger people for example age 30. They say life expectancy is normal etc but this seems based on older people being diagnosed so wondering where that leaves people being diagnosed younger? In terms of life expectancy and progression does anyone have this experience
Et and younger adults : hi i am still very worried... - MPN Voice
Et and younger adults
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Irishgal12
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As long as you are monitored regularly and your platelet levels are well controlled, to avoid blood clots, there is no reason for your life to be any shorter than anyone else's.
Thank you
I've had an appointment with a very well known MPN specialist today and I - of course - asked the same question about life expectancy. I'm 36. I wanted to know if I can grow old with this and he answered: Sure, why not? But it has to be managed properly.
However every case is different, of course. He refered to my special situation.
Thank u. Wonder what managed properly means
Did you meet a specialist? What's his position? I for myself made up a plan after I've read tons of papers and patient reports. I asked my Doc about my plan today and he seems to agree with me, so atm I feel he will manage my case properly.
Yes atm am just on aspirin
"Managed properly" just means controlling platelet levels and taking precautions to avoid blood clots (eg your daily aspirin). I promise you that life can be perfectly normal with ET. I was diagnosed at age 39. I took care of myself, went to my appointments and took the recommended medication. Apart from that, I rarely thought about it. Even if you turn out to be one of the few unlucky ones like me and progress to MF, life can still be pretty normal apart from greatly increased fatigue. But bear in mind that progression is not inevitable.
Hi Jane, I remember your last post when the dr called you to go to his office, hopefully things are better now after you saw the specialist. give us some update when you can. Best wishes.
You actually asked a rather complex statistical question. You are correct that life expectancy figures are projected based on data compiled on heterogeneous groups. Age at diagnosis is only one factor. You also have to looks at co-occurring medical conditions, the presence of non-driver mutations and other risk factors. Some of the better studies do some risk-stratification, but still are clustering people into low-medium-high risk categories.
It is important to understand what the longevity studies actually show. These are statistical projections for large number of people. These projections have nothing to do with your individual prognosis. They cannot tell you whether you will fall within the statistical mean or be three standard deviations out from the mean. The studies are only useful for looking at groups of people, not individuals.
There are things known to correlate with disease burden and course. Type of mutation, mutant allele burden, the presence of non-driver mutations and cooccurring medical conditions do relate to your individual prognosis. This is something you can review with your MPN Specialist if you wish.
I think the conventional wisdom regarding ET is correct. "You are more likely to die with ET than from it. " Sure there are risks, but more likely that you will need to deal with quality if life issues than end of life for quite a long time. I am speaking from the perspective of someone 30 years in the future of where you are at now, I was diagnosed in my 30s too. Now at age 65 - still chugging along. Plan to keep chugging for some time to come despite my ET progressing into PV and few other interesting and unique health learning opportunities.
My approach is to control what I can and let go of the rest. It is normal to worry about this stuff, but do not let it consume you. You will have a long time to embrace life an enjoy it. Plan to make it so.
All the best.
Thank you so much for this postI am 36 diagnosed at 35 so I often wonder this question about health longevity lifespan etc
My main issue is not lifespan but quality of life and how long will I be healthy for? I guess no one can answer this question
Most of the general population will probably get health problems by the time they are 60 and they lived their 30s 40s 50s in ignorant bliss. For many of us we’ve been told we have a condition at a young age so it feels like it’ll be years of worrying about what will happen in the future even though we may not be that different from others around us. Although of course we need careful monitoring to even get to that stage!
Yes and at the min thankfully my counts are not too high am on aspirin
That is right about when I was diagnosed with ET. I lived most of the time in relative ignorance because my MPN was relatively indolent. Never any incidents of thrombosis, tending more towards hemorrhage while I was on aspirin as I aged. Turns out I am at higher risk for progression to AML due to the NF1 mutation, which I knew I had but did not know the implications. In hindsight, there are some things I would d differently. I would not have waited so long to really educate myself about the MPN and the NF1 and how they interface. I was unaware that many of the quality of life issues I dealt with as I aged were in fact related to JAK2-related systemic inflammation. Now that I understand it I have found better ways to deal with it. I wish I had learned all that years ago. There are number of things I would have handled differently had I known better. For those getting diagnosed with MPNs now there are way better treatment options than when I was diagnosed 30 years ago. The understanding of MPNs is now far more advanced. The future for people with MPNs really is improving.
Hi Hunter
So wish I had been diagnosed in my late 20’s than mud 50’s post a stroke type event perhaps the scary few months would not have happened my lifetime of anxiety whoosh gone anyhow it is what it is this site is awesome I too have ET and JAK2 and probably haemorrhage risk taking aspirin frightens me as if I take the full 100mg I can feel the changes in my head so I am learning to listen to my body I know I wasn’t paranoid but it’s hard when Dr’s in hospital make you feel you are paranoid and out of it for thinking cancer then I get a haematologist and the light gets cleare
I need to get the old or rather new biochemistry books out and understand kinase pathways
Thanks for your insights
Is there a Harrogate risk taking aspirin
I think spellcheck helped your post!
The answer to your question is that yes, hemorrhage (bleeding/bruising) is a risk of aspirin. It reduces your risk for thrombosis (clotting) by interfering with coagulation. That is its benefit. That is also its risk. We each respond differently to aspirin, in part based on age. That is why the American Heart Association changed its recommendation for routine use of aspirin. People over age 60 are at higher risk for hemorrhage. Our body's response to aspirin does change as we age. I found this out from personal experience. In my 30s- 40s I tolerated aspirin with no apparent effect. As I got closer to my 60s, I noted a significant change. Unexplained bruising and really severe hematomas from injuries, along with excessive bleeding. Even a minor shaving nick would take 30 minutes to stop bleeding.
My MPN specialist took me off aspirin when I was 63. He said "You really do not want to get a brain bleed." Two weeks later I was diagnosed with a hemorrhagic brain tumor surrounded by significant edema. Too late - already bled in my brain!
In telling that story, do let me be clear that I am NOT saying people with ET/PV should not be taking aspirin. Only that you have to look at your individual profile and how you react to aspirin (or any other medication). It is about weighing the risks/benefits of each choice. It is also about working with your care team to make the best choices you can.
Thank you they just put me on aspirin as standard procedure but now am worried
I would not sweat it. Aspirin + monitor is the standard protocol for low-risk ET. It worked for me for well over 20 years. It is like absolutely anything else you take. There are always risks and benefits. Not treating the ET would also have risks. Use your best judgment and monitor how you respond. You will definitely notice if you have a particularly strong reaction to the aspirin.
You may have seen this before, but it is always worth a watch,
youtube.com/watch?v=d-diB65...
Thank u
It is a problem when providers think you are "paranoid" when in fact something is going on inside your body. Paying attention to what your body is telling you is very important. We just need to distinguish fear of what is happening from what actually is happening. It does not help when providers do not listen and just blow you off rather helping you sort it out.
Do dust off the text books. If you really want to understand kinase systems, there is a LOT of studying to do. Understanding biochemistry, genetics and proteomics are all required. I am only dimly beginning to understand. It is highly relevant to me as I have two mutations that cause issues in kinase systems, JAK2 and NF1. The JAK-STAT and RAS-MAPK pathway interact in some rather complex ways. Lots to learn on this topic.
Regarding aspirin, that one is a judgement call. You have had a stroke-type event. If it was a thrombotic event then it is pretty clear that blood thinning is indicated. If it was a hemorrhagic event, then that is another story. It is true that our bodies response to aspirin changes as we age. Past age 60, we tend to be more prone to hemorrhage while on it. Not sure about your dose. Standard low-dose aspirin in the States is 81mg. Regardless, most MPN Specialist still recommend aspirin for most ET/PV patients. It is all about weighing the risks/benefits.
Hope you enjoy dusting off the old textbooks.
Thank u unfortunately here they won't test the allelle burden
How very odd. Will they run a MPN Myeloid Panel to check for non-driver mutations? maybe not this exact on, but something similar?files.labcorp.com/labcorp-d...
Hi! What does non-driver mean? I dont really get that part.
There are three known driver mutations that cause MPNs. (JAK2, CALR, MPL) There are also non-driver mutations that do nor cause the MPN but can affect the course of the disease (e.g. TPt3, NF1, ASXL1, etc.) These non-driver mutations are typically associated with other cancers as well.
Ok! Thank you i did not know. I have some, and I have AXL1. Is that bad?
The ASXL1 gene provides instructions to proteins and plays a role in the expression of many other genes. It is one of the non-driver mutations known to be associated with increased risk of disease progression with MPNs. This does not mean that your MPN will progress, but that you are more likely to progress than if you did not have the additional mutation.
Like you, I have an additional non-driver mutation, NF1. It causes another condition called Neurofibromatosis Type 1 that causes its own issues. NF1 also makes it more likely that I will progress into AML. Having said that, my history is that I was diagnosed with ET 30 years ago. It progressed to PV 7 years ago. There is no sign of progression to MF/AML at this point. I may well never progress beyond PV.
This is about probability, not certainty. It does inform your treatment decisions. You may opt for one of the medications more likely to slow or prevent disease progression as opposed to medications that remediate symptoms but do not impact disease progression. You may opt to treat sooner than you would otherwise do. The answer to your question is complex and involves more than just the presence of ASXL1. You also have to consider your driver mutation allele burden, cooccurring medical conditions, and other risk factors. You definitely want to review all of this with a MPN Specialist (not just a regular hematologist).
If you want to get a start on understanding what you are asking about the presentation on MPN Molecular Biology will be very helpful.
mpninfo.org/conferences/201...
Here is some more about ASXL1 and non-driver mutations. I will caution you to be careful if you decide to delve into this. It is very important to take what you learn in context. What you are reading about is probability as applied to large numbers of people. It is NOT your individual prognosis. To really understand what you are reading will require a basic understanding of physiology, genetics, cellular biology, and proteomics. If you decide to dive into this kind of material, pace yourself and be ready to do a LOT of extra reading to understand what you are looking at.
"ASXL1 is altered in 4.42% of all cancers with colon adenocarcinoma, lung adenocarcinoma, breast invasive ductal carcinoma, acute myeloid leukemia, and myelodysplastic syndromes having the greatest prevalence of alterations"
mycancergenome.org/content/....
medlineplus.gov/genetics/ge...
ashpublications.org/blood/a...
We are each different in how we cope with our MPNs. I find knowledge to be empowering. The better I understand things, the better I can make informed choices. Even when I find that my risk is higher due to something like having both JAK2v617f (26%) and NF1:c5425C>T (germline mutation)- understanding what each of these things means makes it easier for me to cope with the implications. Not everyone finds delving into these matters to be helpful. We each need to cope in the way that works best for each of us. We each need to focus our energies where it will do the most good in creating a high quality life for ourselves.
I hope that all helps. All the best to you.
Thank you! You help so many of is in this forum. We are all glad that you are here.
Thank you Johan. We are all here for each other. Folks on the forum have been there for me too during some rather interesting times in the last couple of years. We truly are stronger together.
I was diagnosed with primary mylefibrosis at 24 but it’s rare find really young patients
I was diagnosed with ET in my early 20's after recurrent miscarriages. I'm now 61, have 3 healthy adult children and platelet levels remain around 600 where they always have been. Have been told I'll need to start treatment with hydroxy once pandemic is over but so far I've been able to live a very normal life. It obviously affects people in different ways but fingers crossed you will be as lucky as me. All the best.
Have you symptoms? How are other bloods . Sounds like you are doing well .
Is it cos platelets are a bit high ?
Brilliant
65 is the new 60. Some docs have shifted to the new standard in the UK (and elsewhere). I would note that "need" to start hydroxy is a relative term. Suggest "advised" to start is a better term. You can do your own risk/benefit analysis and decide for yourself what is in your best interests. Not all 60-something-year-olds are the same. We all age differently. Individualized treatment is best. You also have choices if you do need meds to treat the MPN. Bit of a challenge to get the more expensive meds approved since hydroxy is so much cheaper, but you can always advocate for what you think best. That includes starting hydroxy if that is what you think is best.
Thanks for your interesting comments hunter 5582. I've been told at every haem appointment for the last 20 years that I will need to start treatment at age 60 so I guess I've never thought to question it. Obviously the main risk factor seems to be age but there must be plenty of other factors which affect risk such as obesity, smoking, general fitness, other health conditions etc. Are you aware of any studies that have been done on this which could enable us all (and our haematologists) to make more individually based decisions? Certainly as someone who is lucky enough to be slim, a non-smoker, very active and with none of the usual ET symptoms other than what could be expected in a "normal" person of my age, I would be very interested to see it. Obviously statistics are all we can go on because nobody can tell what is round the corner for us each individually, but looking at age alone does seem to be a bit of a blunt instrument. Have a nice weekend.
The WHO still recommends using age 60 as the starting point for the definition of "high risk." The British Society for Haematology and others have moved that start point to 65. (I originally saw the reference to that on the forum, but now can't find it. Perhaps the lovely Mazcd knows where it is). You will find other references to the start point for high risk for ET/PV being 65. Here is one of them from the article on Myeloproliferative Neoplasms by Dr. Jerry Spivak.legeforeningen.no/contentas...
My own thoughts on using age as a criteria for high risk mirror precisely what you said. Age is a blunt instrument. We do not all age age the same rate. We do not all have the same number of cooccurring medical conditions. It is certainly true that as we age, we tend to develop more medical problems. It is also true that we form addition mutations in every year of life. Aging also has impact on all of every cell in our bodies. When you look collectively at risk across large numbers of people then age is a valid predictor of risk in groups of people. The same thing is true for predictions of longevity. This does not mean that these statistics can predict your individual level of risk, the course of your MPN, nor your individual prognosis. I am not saying that age is not a factor, only that it needs to be considered in the context of your unique presentation of the MPN and your overall presentation of health conditions.
I do get concerned when people with MPNs hear from their doctors that the "have to" - "need to" - "must" - "insist that you" start a medication in your 60th year of life as though somehow you are now at much higher risk than when you were 59. There is no sudden surge in risk that happens on your 60th birthday. It just does not work that way. There is also an underlying issue about the role pf physicians in providing care. Physicians advise, consult and write orders. They do not give them. Physicians recommend. Patients decide. The patient is the only person who has the right to make a decision about what the treatment plan will be.
It is important to understand things from the perspective of a physician. The protocols do define either age 60/65 is the "high risk" point. Even when this high risk does not appear to apply to an individual patient, if the physician uses valid medical judgement in holding off on recommending the use of medication there is an exposure to liability. Even if that recommendation is what is right for the patient. Defensive medicine is a reality in the practice of medicine. When an individualized treatment plan that is in fact what is best for the patient is not in synch with the standard protocol, the physician faces a very real dilemma. Add to this another factor. Most hematologists are not MPN-experts, they do not have the KSAs to individualize a treatment plan that is not in synch with standard protocols. It is quite understandable that they would be very uncomfortable doing so.
Sometimes all physicians can do is to calculate the relative risks of the medications used to treat MPNs vs the risk of the MPN itself. The answer is not always clear. Medicine is not an exact science. As patients we each face the same dilemma. We have to make decision about how to treat our MPNs when the answers are not always clear. It is about judging relative risk based on the best information at our disposal. We have to consider our own treatment priorities and goals, our risk tolerance, and our own assessment of health status. Age certainly is a factor in this decision. We each have to decide how to factor all of that in to making a decision. That is both our right and our responsibility.
Hi Irishgal. I have found this forum to be very helpful in normalizing symptoms that I have experienced for years, however, I have never posted. Since your question was regarding diagnosis at a young age, I wanted to offer up my experience. I was diagnosed with ET Jak2+ when I was 24. I am now 46, and my bloodwork progressed to PV one year ago. Early on, my platelets were mildly elevated, eventually progressing and sustaining at 1.2 million over the past 10 years. I was managed with baby aspirin for years with no issues. Once progressing to PV, I required intermittent phlebotomies and was started on Pegasys approximately 3 months ago. In hindsight, I was mildly symptomatic for years (migraines and ischemic foot pain in my 20's, hypertension, mild to moderate fatigue and occasionally what I call "mixing up my words/saying the opposite word" in my 30's and 40's. I was never aware that these were symptoms of ET until in my latter years. I would say the fatigue significantly worsened with progression to PV, although I was still very active and in general, living a healthy, active lifestyle. In the last 2 weeks, I have finally started to respond to the Pegasus and I would say that for the first time in a long time, I do not feel as fatigued. I am an active individual and have lived a normal life not limited by ET/PV. I hope this helps you feel more at ease about diagnosis at a young age.
Thank you. My worry is progression is it hard to have both?
I am not considered to have both, ET and PV. My initial diagnosis of ET progressed or maybe a better word is transformed to PV (my platelets, RBC and WBC all became elevated vs just the platelets, previously). PV is my current diagnosis (no longer ET). I do not find that there is much difference in my clinical signs between the ET and PV.
And what is your treatment
Baby aspirin, started Pegasys 14 weeks ago and phlebotomy as needed. Although, I have just started to respond to the Pegasys, which will hopefully decrease frequency of phlebotomy (which was every 3 to 4 months)
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