Is a normal lifespan possible for PV patients?

[hunter5582]

From our friends at MPN Forum Magazine. mpnforum.com/

The short answer is YES - with proper management people with PV can live a normal lifespan.

Richard Silver et al have previously presented research indicating improved survival for people with PV when they receive appropriate treatment nature.com/articles/s41375-...

Silver et al recently published an updated letter with some very good data.

"To compare PV survival to the general population, they used US Census Data to determine the life expectancy for people of matching age, sex, race and year of birth.

Their conclusions: Overall survival was significantly higher (26.6 vs 15.8 years) for the PV-Cornell group. The authors attribute these results to “current standards for attentive management with aspirin, targeted phlebotomy and cytoreductive therapy at an academic medical center..

And about that normal longevity issue? There was no statistically significant difference in Overall Survival between the PV-Cornell group and the matched US population.” ergo, a normal lifespan."

You can read the letter here: nature.com/articles/s41375-...

[friendofpiglet]

Encouraging stuff.

But I for one have no idea of what my 'normal life expectancy' is.

So, since I am still alive and kicking, I'm doing my best to enjoy what remaining life expectancy I might have and would advise everybody to do likewise and.... keep taking the tablets (as the idiom has it).

[hunter5582 in reply to friendofpiglet]

I would agree 100% with that. My primary treatment goal is to extend my quality of life. Extending length of life is secondary and subordinate to quality of life. That being said, I would rather stick around longer provided my QOL is intact. I have a lot left to do on my bucket list. Plenty of fun left to have a time with the people who I love.

Wishing everyone lets of fun and time with loved ones.

[Scaredy_cat in reply to friendofpiglet]

For the UK the following link gives you life expectancy at different ages. Remember life expectancy is where about 50% of pe will live to or beyond ons.gov.uk/peoplepopulation...

[sbs_patient]

Thanks for sharing this very interesting paper. However, the discussion of age at onset still seems a bit limited. Both populations (WCM, SEER) seemed to be relatively young. I'd like to see the OS for those first diagnosed with PCV at age 70 under various treatment modalities. That being said, I am aware that the numbers of such cases are likely to be too small to support statistical analysis.

[Kelly2 in reply to sbs_patient]

one in 100.000!!!

[hunter5582]

Per the CDC in the USA.Life expectancy at birth for males was 75.1 years in the first half of 2020, representing a decline of 1.2 years from 76.3 years in 2019. For females, life expectancy declined to 80.5 years, decreasing 0.9 year from 81.4 years in 2019.

cdc.gov/nchs/data/vsrr/VSRR...

I expect you are correct about the difficulty in getting statistical valid numbers on OS for those diagnosed much later in life. Not only due to smaller numbers but also because the closer one is to the average life span the harder it would be to demonstrate efficacy in treatment.

According to the Sanger MPN Risk calculator my predicted life expectancy would be to about age 74. That would give me a predicted life span of another 7 years. I do not pay any attention to this at all nor worry about it a single bit. I have no plan to die on a schedule. There is no timer ticking away inside of me. I have moved on to a plan that maximizes my quality of life while at the same time giving the best chance to extend it based on the current research.

I think the main takeaway from the Silver et al research is that there is treatment that can extend OS. It is up to each of us to seek out the plan than best meets our needs. We are blessed that the understanding and options are so much better now.

[Aime]

Thank you, Hunter for great information as always. My motto is make the most of every day because I might get run over by a bus tomorrow! Enjoy the precious time we have with our families and friends, make the most of every opportunity, there is always someone worse off, who needs the sympathy and care. Kindest regards to all, Aime x😻😻

[Steve_Essex]

26.6 years OS is encouraging but there’s still questions about probability of younger patients achieving a ‘normal lifespan’ … appreciate the data doesn’t go back further than 25-30 yrs and we’re now more accurately diagnosing younger patients who would previously have not been detected / expected OS is now much better than a decade ago…

I was diagnosed 4 yrs ago with ET jak 2, but being treated as PV due to marginal HCT at 45.5 - I’ve now just turned 40. but I still can’t gain comfort that if I look after myself, have effective management of my condition etc that I’ll live to see my winter years through… I’m holding hope that medical advances continue either through some form of gene therapy or through more focused studies on besremi usage as a first line for younger low risk patients to reduce risk of progression etc

.

[hunter5582 in reply to Steve_Essex]

That is the problem with the longitudinal data for people diagnosed at a younger age. Applying the 26.6 year OS is not really an accurate measure since that number includes people diagnosed at an older age.

To take my own case as a heuristic example, I was diagnosed with ET around age 36. It progressed to PV around age 58. Now age 67 I am 31 years OS and counting. And doing fine. Not looking to exit the world anytime soon.

I would suggest definitely taking comfort in know that if you take care of yourself you can expect to live a long lifespan. One of the big changes is the better understanding of the importance of earlier intervention in MPNs and the potential impact on OS. The existing research on the use of the PEGylated interferons in lower risk patients looks promising. The possibility of someday finding a gene therapy does exist. Meanwhile, treatment options for MPNs are expanding, LSD1 inhibitors, MDM2 inhibitors, hepcidin mimetics, and more.

It is really important to work with your care team regarding your treatment goals. If you wish to move more aggressively to treat a lower risk MPN based on your treatment priorities, that is your prerogative.

Wishing you all the best moving forward.

[Steve_Essex in reply to hunter5582]

Thanks Hunter! I do take comfort from your many posts and your journey, I have a great team at Guys looking after me.. just every so often, as many people on here likely experience, you get niggles creeping in now and then where you overthink things. However I will discuss options going forward in more detail at my next consultation.

My second child has also just arrived into the world and that definitely makes you reflect - however I feel the future outlook is positive.. and there’s more research in younger patients than ever before..

Irrespective, my symptom burden is still very low, I’m fit, active, and I’m determined to be the best version of myself that I can.. controlling what is within my gift to do so…

Thank you for your many valuable contributions to this forum! Long may they continue!

Steve

[hunter5582 in reply to Steve_Essex]

That is a great attitude to have. With your second child recently arrived (congratulations) you can now look forward to seeing your grandchildren. My granddaughter turned 12 yesterday, Being a grandparent is great!

Glad to hear you are at Guys. It is one of the best places to be for MPN care. good to know you have a great MPN care team when needing to squash the niggles.

All the best my friend.

[Aime in reply to Steve_Essex]

Congratulations! Daddy again. Every best wish to you and your wife. Enjoy your family. X😻😻

[Steve_Essex in reply to Aime]

Ahh thank you!! The little man is settling in perfectly!! ❤️

[EPguy]

Thanks for the discovery. Is the report behind a paywall? It does look neat. This Cornell study I believe is one reason for the "rediscovery" of INF we are seeing recently.

But the message is broader than that, they say all of these help (as in your quote) <<attentive management with aspirin, targeted phlebotomy and cytoreductive therapy>> So the comparison is more to those without basic modern MPN care. This is consistent with the older papers on this study. But INF did get the best responses.

There is some older info on this study in a post I've often referenced, with some full articles, but not the new one you found.

healthunlocked.com/mpnvoice...

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The MPNForum page has some other nuggets from the author that are interesting.

-The author is not impressed with Rux. This viewpoint seems severe and I don't agree with this level of negative: <<Jakafi — unable to stop progression of MPNs, bringing with it serious side effects, triggering new mutations, and by masking symptoms contributing to delay in alternatives like stem cell transplant — has sucked up the vast majority of funds and brainpower devoted to MPN research in the past decade.>>

The issue of SCT timing was in a recent post on a report (key point was hi risk MF should get it while your Rux response is still good)

-Two INF experts recommend INF for reducing Covid severity. But this was 2021, a long time ago in Covid years. I posted on this recently, Besremi had a small Covid trial and an inhaled INF is in a large trial.

-<<Jakafi, a potent antiinflammatory JAK2 inhibitor, might have some stiff competition from a humble source (Curcumin)>>

[hunter5582 in reply to EPguy]

That one study is behinds a paywall.

Like many things MPN, there is not universal agreement on treatment strategies. Silver et al are well known for their preference for the IFNs over other treatment options for many people with PV. While I tend to agree with that opinions, all opinions are best consumed with a grain of salt. Healthy skepticism = healthier body.