Thoughts on PV/ET

[Paul123456]

There are a lot of new posters here (I’ve been here 3 months and feel like an old hand!) who are understandably nervous and worried. The dreaded cancer word and for many of us, our first real brush with our own mortality.

However, there are two really important factors we need to focus on.

PV and especially ET are chronic, they usually develop very slowly as in decades.

Medical research is advancing rapidly. Amazingly, JAK2+ was only identified about 10 years ago. We now have impending trials of drugs which focus of the specific JAK mutation and imo more importantly, we have drugs such as RG7388 (in Phase 2 Trials) which target other mutations. RG7388 looks very exciting but we have to be realistic and wait for Phase 2 results, a high % of early starters usually fail before the finishing line. There are now trials using combinations of existing drugs which again appear exciting.

Nevertheless, everyone I’ve spoken to or read about reckons that we have have a drug that pretty much ‘stops’ progression within 5 - 7 years. It seems to me we have have multiple mutations, incl say JAK2+ and MDM2 (which RG7388 targets). The boffins are working on identifying other mutations which is tricky because we may have different basket of mutations. The big breakthrough will be identifying the prime driver.

I suspect that many new posters here can reasonably expect 20 - 30 years before serious progression so plenty of time.

And if we progress to the dreaded MF, there is currently a cure but not an ideal one! Stem Cell Transplant involves using radiation/chemo to kill all the mutations which are then replaced with new genetically matched cells. The two great risks are the radiation/chemo wipes out immune system and your body repels the new cells. You need to be reasonably fit and healthy to reduce risks. However there are recent advances re both risks (see my other posts) and it’s possible that SCT outcomes could be dramatically improved in say 5 years time, opening this treatment to the more frail and elderly. Whatever, SCT prognosis is on an improving curve.

Forgive this waffly post but the point I’m making is that, imo our MPNs will not be a death sentence. We just need to stay fit and healthy for next few years (as in eat well and exercise) and wait for imminent medical advances to transform our prospects.

Stay +ve and view your Burden symptoms as a challenge! I’ve transformed my calf/shin cramps by wearing loose fitting thermal socks in bed. Not a great look but does the job!

Best Paul

[Kelly2]

Thank you for informing us. Please continue with any news.

[mikerodol]

Really enjoyed your post. My ET transformed to MF after 4 years so perhaps not so lucky but have been on ruxolitinib for about a year and a half and it has improved how I felt massively. You are correct - very encouraging research going on at the moment.

[Lab-Rat in reply to mikerodol]

Hi Mikerodol

I am sorry your ET transformed. Glad that Rux is helping. How was your progression picked up? Bloods, BMB, did you experience a worsening of symptoms? All the best to you.

[mikerodol in reply to Lab-Rat]

Hi Yvettenaude

Initial signs that disease was changing was decreasing platelet levels resulting in decreasing hydroxycarbamide doses. Physical signs were loss of appetite and generally feeling yuk together with feeling more tired. BMB confirmed MF and ultrasound scan showed spleen double normal size which constricts stomach hence appetite loss. Hydroxy stopped and ruxo started. Spleen shrank to normal size in a couple of weeks. Anaemia a problem at first but now much improved. Hg was 77 at lowest point but now up to 114 at latest blood test.

Sorry if reply a bit waffly but good to chat. Thank you for your enquiry

Regards

[Dianne-Guisborough]

What a fantastic and very informative post Paul . Thank you so much for taking the time to explain everything so well about our at times, complex and rare conditions which can have differing symptom burdens to each of us .

Hope you and all the lovely pepes on MPN Voice keep well , positive and happy .

Love Dianne xxxx

[Betsywest]

Well said!! All the best to you Paul x

[Ebot]

Yeah. I spent the first few weeks after initial diagnosis thinking I was going to drop dead. I then realised that that hadn’t happened and so I better get on with living. I also realised how thankful I was to have been diagnosed (quite by chance) and therefore that my chances of leading a long and active life were greatly enhanced. 😁

[hall2]

What a lovely positive post! Thanks so much!

[Paul123456]

Thank you for all your kind comments.

Those of us still at ET/PV Stage always need to remember that this is one of the ‘better’ cancers to have. A good friend of mine was recently diagnosed Stage 4 lung cancer and immunotherapy not yet sufficiently advanced to target her specific genetic mutations. There are many others who are enduring awful chemo and poor short term prognosis.

I hesitate to say we are ‘lucky’ but those of us over 60 must be half expecting something to start going wrong with our aging bodies. I think we are lucky because we still have that critical element - time. You only have to pick up a newspaper to see the amazing advances being made. And because we are a small group, we get orphan status - ie new drug trials rolled out much quicker, for better or worse!

For the younger members of our community, I can understand your worries and shock. However, progression is very much age related so the younger you are, the longer you can wait for the big breakthroughs. Still doesn’t help with the symptom burden I know, especially if working long hours. It will not be a life time curse though, hopefully no more than a few years.

Even those with MF could still have significant time, some posters have been around over 20 years. If I had MF and was contemplating timing for a SCT, I’d be pressing for genetic testing to determine my prognosis. Not sure whether this is offered to all SCT eligible MF patients as standard treatment?

I forgot to mention Ropeg before, just finished year 2 trials (versus HU) and hopefully will be available to all of us next year. Looks (to me!) like a good chance of being our first line drug of choice.

Finally there is an upside. We have so many check ups and access to great doctors that any thing else going wrong should get caught early. Plus I think most of us have made healthier lifestyle changes which will benefit us as we get older regardless of our MPNs.

Best wishes Paul

[JaynieQ]

Great post! Thank you Paul 😊

[Skyehope]

Thanks Paul - always nice to hear some positivity

[eire]

What a great post Paul very informative and put in a way everyone would understand. Many thanks for your positivity.

[Paul123456]

Ref Ropeg. Hopefully will be available next year and looks drug of choice

onclive.com/web-exclusives/...

[christiev]

Yes - so much good work and real progress happening - its fantastic, and so important to keep reminding all about that - thank you.

Please also can we all remember that some here do have MF already or indeed have Primary Myelofibrosis (so never had ET or PV at all). It seems often be be called 'The Dreaded MF' etc and seen as the one thing that everyone is so fearful of. This can be disheartening for those who are carrying on their lives with MF - some very easily, happily and symptom free! Of course we all hope that what ever conditions we have don't progress, but do be assured that there is life with and after MF!

[Meatloaf9]

Hey Paul, thanks for the great post. Please continue to keep us all informed of new and promising treatments and trials, and may you and everyone else here - live long and prosper.