Progression

[JAKboy]

I am 23 years old & have had PV for 6 months & haven’t had any problems other than body aches maybe? I know this can progress into serious other cancers, but my question is that since I am so young at diagnosis is it pretty much guaranteed that I will transition to MF or AML at some point? Also, kinda worried about the huge financial burden this may cause in the future.

[hunter5582]

The short answer is NO - it is not guaranteed that you will progress to MF or AML. Most people with PV will not progress to MF/AML, including people diagnosed with PV at a younger age. From a purely statistical basis, you could make the case that the longer you have PV the more likely you are to progress at some point; however, that has nothing to do with your individual prognosis. It is also not valid to try to predict your individual prognosis based on the past research on PV progression. The newer treatments for PV are changing the MPN landscape. Your potential prognosis is much better than people with PV had even 10 years ago.

Suggest that rather than focus on what your risk for progression might be, you focu on doing what you can to reduce your risk. The steps you can take include:

1. Reducing your exposure to toxins and carcinogens in the environment. For the love of Peter, do not smoke. Limit alcohol consumption as well.

2. Limit exposure to toxins in the foodstream. Avoid toxins like pesticides, hormones, antibiotics, xenoestrogens, and other contaminants.

3. Control the systemic inflammation that is part of the JAK2 mutation. Inflammation causes many of the secondary symptoms we experience (e.g., body aches) and increases our risk of progression. Inflammation can be reduced through diet (e.g., Mediterranean Diet) and the use of some supplements. Some of us have had success with complimentary health interventions liks curcumin and NAC. Note that you should consult with a provider who is knowledgeable about complimentary health if you wish to pursue that option.

4. Maintain good cardiovascular health. Exercise and keep off the excess weight.

5. Manage stress effectively. Unmanaged stress and worry damages the mind and body. We each have to find out own way to do this.

6. Stay on top of the emerging research on preventing progression. There is research underway looking at starting the interfons sooner with low-risk PV. There is also some interesting research that is looking at targeting the JAK2 allele in a different way that is hopeful for a different outcome. You may be a good candidate for a clinical trial if you are so disposed.

The future is quite hopeful for people with MPNs. The landscape may look quite better 10 years from now. You can improve your own odds for a positive outcome. Have confidence that you will live a long rich life managing PV successfully. Note that I say this as someone diagnosed with a MPN 31 years ago. I have had a great lifer and at age 67 continue to do so. You should plan to do the same or even better.

All the best.

[Steve_Essex in reply to hunter5582]

Couldnt have said it better myself!… I was 35 when diagnosed just over 4 years ago… I had / still do have all the concerns that you raise and currently pushing consideration for starting interferons sooner, as currently on aspirin, venesection and ‘watch and wait’ . research has shown interferons can reduce risk of progression through deeper molecular changes at the Haem Stem Cell level.

I’m also hopeful for all the research that’s going on, lots of potential in the pipeline, CRISPR, simulated bone marrow that represents human bone marrow architecture to rapidly test new techniques / ideas ( this is huge) it’ll still take time for a real game changer to become available, but we do have time on our side!

In the meantime, control what’s in your control… health.. diet.. mindset… making sure you have a great mpn team.. etc

If I can ever offer any individual support let me know!

Best wishes

Steve

[ainslie in reply to hunter5582]

Hunter

I agree with what you have written here as a good formula for a healthy life generally , I follow most of that myself and then some. However points 1-5 as good as they are for general health advice , to the best of my knowledge I don’t think there is any evidence they affect MPN progression. I hope they do and there is probably evidence they may affect solid cancers but with MPN do we know that for sure?

I mention this as when I was diag I spent a few years and much money on detoxing , alternative treatments , removing amalgams, optimising nutrition and supplements etc etc, some may say obcessionally. I still do most of it but not to the extreme, it should keep us healthy generally but I’m not sure we can say it will affect MPN progression, I hope it does though.

[hunter5582 in reply to ainslie]

It is really in two parts. We need to reduce our health risks and generally stay healthy to manage the risks of MPNs. I would agree that there is not irrefutable evidence that MPNs behave like other cancers; however, it is reasonable to think that the factors that produce cancers or accelerate them are likely to have an impact on MPN progression. The current research on the role of inflammation is based in part on the concern that inflammation promotes MPN progression. None of the interventions mentioned are a sure and certain formula to prevent progression. It is about doing what we can to improve our odds, feel better and stay healthy.

[ainslie in reply to hunter5582]

great, I think that clarifies it

[Mostew in reply to ainslie]

meant ‘to do’

[Mostew in reply to ainslie]

I would say anything done in extreme can lead to stress.

Balance is the key

Nothing is sure . but it makes sense to Hunters ‘ 1 to 5 . Certainly can do no harm . Wether we have cancer or not they are good guidelines..

[monarch5000]

youtu.be/3e2A61g6-UE(Not working as expected?)

3:21 mark: "disease progression to myelofibrosis was inevitable in about 50% of patients after 30 years of followup. This led to our 3rd key finding which was the excess deaths from progression of disease to myelofibrosis can be prevented with interferon-alfa. In fact, patients treated with interferon alfa had a normal life expectancy across a 30 year duration of follow up. Their survival was comparable to the age matched population without PV."

With regard to financial burden, depends on the country you reside in to obtain the drugs, lab tests, doctor visits, etc. The best outcome, if you end up being prescribed with Pegasys interferon, is that it could induce a stable and durable state of "Minimal Residual Disease" where far less frequent dosing would be needed with much longer intervals between routine blood tests and doctor visits.

[ainslie in reply to monarch5000]

well, sorry to have to disagree with this yet again but the reality is that the interferons may slow or stop progression in a minority of those you can tolerate it. The drop out rate at 6 years is in the order of 60% and it’s a minority if I recall something 20 % of the remaining 40% or so that may slow or stop progression. It’s still worth taking for some as it’s probably the best drug we have currently but we have to be realistic about what it can and can’t do. That is the purpose of my reply.

Re the 50% to MF at 30 years , I have not heard that from other docs , Gisslinger reckons 6% at 15 years so quite a jump to 50% at 30 years?, I am a bit sceptical.

Of course these are always historical stats (including Gisslinger) and hopefully the future will be much better.

[Anag in reply to ainslie]

I was with Gisslinger in Vienna for a few years. Average life expectancy for ETers from day of diagnosis in Vienna 22 years. Why? Average age of diagnosis is mid 60s, therefore it’s a normal life expectancy. Of course, the younger we are diagnosed, the longer we live with the illness. I’ve had ET since 2009, I was 44. I am 58 now and healthier now than then because I do all that Hunter listed above and also prayer and trauma therapy from my childhood (do it on myself). 😉

So dear Jakboy! Welcome to the club and be prepared to have a good life! I hope this forum will become your greatest friend and supporter.

Regarding the Interferon Alpha, the new one Besremi has been great for many, me included. It’s still new so horrifically expensive. Prices will be coming down. Yes, you’ll need insurance that will pay. In Austria I pay €6,60 per 250mcg. Normal price 5000€ for one pen. Much more expensive in the States. It actually acts on the root of the problem. One can start on one medication and switch I just switched after 5 years on Anagrelide.

Please educate yourself and find an MPN SPECIALIST! Few Normal haematologists know enough for proper care of MPN patients. The specialist is for the best guidance and haemotologists for normal check ups. 🙂

Have a good weekend!

Anag

[ainslie]

sorry to hear your in the MPN club, however the good news is that treatments and research is way ahead of where it was when I was diag 12 years ago. Also the stats you read about progression are historical , some dating back 50 years, so in your life it is likely to be very different. There are lots of different stats on progression but if we look at a recent one by Dr Gisslinger he reckons 6% convert to MF at 15 years, that also reads 94% dont. Also these stats are mainly in us older people ie in our 60’s or thereabouts, I would speculate it is likely different with younger people. The number one advice I can give anyone from experience is not to waste valuable life time worrying about something you can’t change ie having the MPN. What you can do is educate yourself on MPN and treatments. Keep up to date with the forums and possibly attend conferences , try to have a good Haem, and if possible see a expert MPN doc or two to point you in the right direction re a plan and see that expert or experts periodically. What might be good for us older patients may not necessarily be good for you so bear that in mind when reading. I would strongly recommend seeing a MPN expert , ideally one experienced with younger patients. And above all enjoy your life, if I could change one thing looking back is not to have spent so much time worrying over something I can’t change, it did zero good , just wasted valuable life time. My philosophy is learn all I can, find what I think is the right treatment for me, keep fit and healthy generally , monitor all my counts on a spreadsheet and if that’s all done it’s all I can do so make sure to enjoy every day,don’t waste them worrying.

[Mostew]

glad you have found this forum . It’s such a good place to turn to .

[monarch5000]

If you end up desiring interferon and are considered medically suited to take it, then the next question for you and your hematologist is when to start it? The world's leading interferon experts, like the one in the brief video below, believe it should be started as early as possible, while the malignancy is still in it's infancy, because that's when low, easily tolerated doses are most likely to be effective in "burning out" the Jak2 mutation to an undetectable or nearly undetectable level. If and when that happens, the PV patient is considered to have attained "Minimal Residual Disease". youtu.be/TIlzFKLtj0k(Not working as expected?)

[MAP44]

Hi JAKboy,

Sorry you received such a crazy diagnosis at such a young age. I am 54 with PV and my 22 yr old daughter has ET. We are both doing very well. Once I was diagnosed and seen all the symptoms I knew my daughter’s headaches and stiff neck was connected to what I had. We are both Jak2+.

We started by flattening all our anxiety. This is super hard but a must. Getting and staying out of flight or fight and believing that you can still lead a long normal life is important. Next inflammation is your enemy. Adjusting your diet to help you look and feel great is important. Whatever types of exercise you do whether walking or visiting a gym, enjoy it because it should be looked at as part of your stress reduction. Next resting and sleep are also very very important.

Find as much support as you can and lean on it when you need to. Get an MPN specialist in your corner also.

I have been on Pegasys for 12 months and my daughter is watch and wait. I tell her often that for sure in her lifetime they will figure out the disease. Exciting the changes in the last 5 -10 years.

Continue to make plans, take trips, live your life as normal as you can. They are trialing new meds, combining others and , tweaking other meds hoping to find the right combination to solve our issues.

Hope you find lots of support here. There is also a young MPN chat on this sight too. You should definely stay connected with them. All the best in the future. ❤️❤️

[Mazcd Partner MPNVoice]

hi Jakboy and welcome to our forum. I can see that the lovely people on this forum have given you lots of advice, so all I will add is, have you see the blogs for young people with MPNs on our website, they are written by Alice, who was diagnosed with ET when she was 26 mpnvoice.org.uk/about-us/yo...

You will read in the current blog about the Young Patient Network which has recently been started, including a Facebook group you can join facebook.com/groups/mpnvoic...

best wishes, Maz