Dear friends, I have a ‘what would you do’ scenario that I’d really love your help with, please.

[Magentas]

A short update for those following -

When I saw my haematologist last week it was quite unpleasant, I think her impatience was due to her ridiculous workload or her frustration that she can’t really guide me. I dared not question her words or ask too many Q’s, I left it at a couple, one of them was re my feet, she doesn’t think I have Erythromelalgia because I don’t get the red rash, I have so many unanswered questions.

The consultation ended with the suggestion that I may like to see a more local haematologist? Maybe this is more practical at this stage, if there’s nothing left to learn, but the timing wasn’t too kosher if you ask me.

Anyway, down to facts, It’s been confirmed that I’m ‘unclassifiable’ due to not reaching the criteria for PMF. I have all the major criteria but none of the minor criteria. She implied I will have no trouble getting Ruxolitinib but first some antibody, I think, tests need to be done.

She suggested I start Ruxolitinib now because I am at the ‘early stage’ (huh) and it most likely extends life expectancy….but only at the early stage. I’m not so sure about the ‘most likely’ bit as this information comes from the COMFORT study which appears to be a total mess to me, lol.

She told me 50% of patients at my stage of the game live another 8 years.

I was told I’ve probably had MPN for 5 years. After the recent bone marrow test, which revealed Grade 2 fibrosis, I was told I don’t have ET but what I don’t know is, am I post ET, did I ever have ET? Does any of that even matter?

I’ve been trying to pick up whatever I can regarding Ruxo because I feel need to weigh up the pros and cons of taking this drug and this is really where I’d appreciate anyones perspective, as mine is all a bit skew-whiff currently, maybe….see? 😊

So far I’ve got this-

Pros

Will extend life expectancy!! (or will it?)

Will alleviate my fatigue. (I can handle the fatigue, it’s only the post viral fatigue that’s killer but that passes)

It will lower my platelets (been 400-700 past 5yrs…meh?)

Does it alleviate irritability, hot flushes, hot feet and slow cognition? Maybe these things are irreversible and just part of the aging process 😊.

Cons

It’s a drug.

More drugs may be needed, such as, controversial, Statins to reduce cholesterol and anitvirals to reduce infection due to compromised immune system and even Imodium for diarrhoea, although, I would definitely try Slippery Elm first.

I’ve heard first hand that shingles can be really nasty (especially if in your eye!) and I don’t know if antivirals can stop shingles.

I have had chickenpox when I was young and I have also had the shingles vaccine which is available in Australia but it is only 50% affective as opposed to the American one which is 90%…..ha, sounds familiar.

And who knows what other dominant viruses may come to the surface again!

My body will most likely become unrecognizable due to weight gain, a most depressing and uncomfortable prospect.

Anemia

And most importantly it may lose it’s efficacy after a few years.

I’d like to think I’m a bit of a unicorn because I really do feel good…apart from some fatigue and when I’m not being tortured mentally and emotionally post haematology appointments, haha….but maybe I have my head in the sand? Ugh.

Am I mad? I’m feeling a bit mad, maybe some of you lovely sane people could help me out?

Please feel completely free to say anything, whether it’s off the top of your head, an intuitive response, a bit considered or well considered, all thoughts welcome and thank you!

Warm regards to you all, Jacqui

P.S If anyone can send me a link explaining allelle burden with MF I’d appreciate it.

And, not my dog or neighbourhood, just had to share because I’m crazy about this pic from a lady who posts in fb page Unique Trees

[hunter5582]

As you likely know I will say, assertive patients receive higher quality care. Passive patients do not. You have legitimate questions and concerns that need to be addressed. It does not matter how busy or frustrated the doc is. Doctors have a core ethical obligation to provide the best care possible for patients. It does not sound like this happened. I thought you were already seeing a MPN Specialist. If not, you need to see one ASAP. Or see a different one. Having a local hematologist to provide some of the on-going care can be very helpful, but you need MPN-expert consultation. (This is the approach I use).

Regrading Jakafi, it is a reasonable thing to consider. I know you have considered Pegasys, but have some understandable concerns based on your own history. One benefit to Jakafi is that it is known to do a good job controlling at least some of the secondary symptoms. There is some evidence that it can reduce allele burden (see below). As with all of our decisions, you will have to analyze the risk/benefit profile of each option. Continuing on your current path is also an option that also has its own issues.

Regarding the foot pain, I have been experiencing something similar. Maybe mild erythromelalgia, Maybe mild gout. Maybe something else. My MPN Specialist also said it could just be the PV (e.g. a microvascular issues). We are still figuring it out for me. Ultimately the Pegasys may help with this. It is possible the Jakafi could help as well.

The other option is to look into what is in clinical trials in Australia. If there is nothing else available, then you have some choices to make. You have to choose between trying to make things better or letting them stay the same. There is not a right or wrong answer. It is about what you prefer to do in regards to the choices available to you. From my perspective it makes sense to try Jakafi. It may help. You may tolerate it well. If it does not work out for you then you can discontinue it.

Here are some links you may find of interest.

ncbi.nlm.nih.gov/pmc/articl...

ashpublications.org/blood/a...

ncbi.nlm.nih.gov/pmc/articl...

link.springer.com/article/1...

I know you have looked in detail at your choices. Perhaps it would help if you could talk everything through with a hematologist who has the patience and caring to spend the time with you that you need to process your decision. I hope you will be able to do this so you make peace with whatever you decide to do.

All the best my friend.

[Magentas in reply to hunter5582]

Sweet friend, thank you for your thoughts and time. All this rings true and as my other new friends @Cja1966 and @Pontygirl say, there is no magic bullet for us folk. You three have all brought up very valid and pertinent views to issues that affect us all.Just wish I had a together and trusted doc on board. No, no MPN specialists in this part of the west, so, as was brought to my attention early in the game, it really is about case managing and informing ones self…harumph. Meanwhile I DO find myself thoroughly astounded that so many human health issues are dealt with these days. Truly astonishing! And I like that my husband calls me a unicorn ☺️.

Any feedback, no matter how uncommitted (fully understand why!) or just a shared acknowledgment of the difficulties we all face is welcome.

Big hug, Hunter, and best of luck dropping the dose of those phenomenal tasting animal fats. I don’t know if this will help but I like the idea of envisioning the meat hunter gatherers ate. It makes me wonder how they could’ve ever been satiated!

And, gosh, thanks for the links, feel free to repost and repost and repost if ever I repeat myself. It’s kind of hard to take a break from all this and also investigate at the same time. Time is so short for all of us, be us unwell or well these days.

[MCW22]

I've been taking Ruxolitinib for my pv for about 2 years now. It reduced my spleen and stopped the itchy skin. The only side effect I had was slight dizziness for a couple of days at the beginning, nothing since. My advice is to give it a go.

[Magentas in reply to MCW22]

Glad to hear it is agreeing with you so well, thanks for the input, M.

[Hidden]

the Erythromelalgia:

I went to EVERY foot specialist in my city - Wellington, capital of NZ resulting in the final one saying - your old (59 at that point), expect pain.

What helped, purely by accident, was bone broth. I was looking around generally at 'healthy' eating which helps, and not for the feet at all, one of the Drs (maybe Dr Jockers) gave a general suggestion for stuff (whatever it may have been) was drink 3 - 4 cups of bone broth a day for 3 to 4 weeks and see what happens. I gave it a go and it was a real shock to find that my feet were sooo much better after just over a week (I had not been able to walk more than a few steps, pain so bad, could not get into any of my usual shoes). So - now I regularly consume 1 mug a day as part of my maintenance plan. It worked for me - maybe for you. That got me back 3/4 of the way to normal again. The last wonderful bit has been achieved by Kefir, Kombucha and fermented veges (not sure which, maybe them all - who is to tell??)

Maybe you are up to giving it a go - maybe not, but because I was so debilitated with no advice how to fix it and this is what did work for me, I thought I would write.

All the best, and hopefully you will find a solution which works for you. (bone broth recipe at the bottom of my profile, should you want it.)

[Magentas in reply to Hidden]

Thanks for that. yes I’m starting to suspect that it is age related pain, on top of planter fasciitis, well, thickened plantar really, on top of platelets making my feet hot when the weather is warm. Did you know that the padding on our soles dwindles as we age?I’ve actually made my own bone broth once, some years ago, I would not like to have to live with that smell again. It’s available in a couple of stores I frequent, it’s pricey but I will certainly give it a go! Thanks, Max.

[Hidden in reply to Magentas]

Cooking it at that lower temperature (80C) means there is very, very little smell. I am very sensitive to smells and fragrances.And yes - it is jolly annoying that the padding goes - mine has to some extent - but that is a way different issue to the hot pain.

Anyway - good luck with the bone broth. It is still working for me some years later.

[jillydabrat]

I am sorry but I wouldn’t have been able to hold back.

If it happens again maybe start the conversation with “I am really sorry that you must be so overworked at present which is obviously very frustrating to you as I can hear it in your voice but I am very worried and I need to talk to you or a member of your team that can answer my questions.

Maybe they need to realise how they are coming across. Someone pulling them up about their attitude might make them think twice. You are there for medical assistance, not to be talked down to.

[Magentas in reply to jillydabrat]

Hi, jilly, I am sure I would’ve replied the same way as you, had I read this on someone else’s post. I certainly don’t shrink back from giving people a piece of mind if I find something unfair! lol. But in this case I am reticent as she is the only doctor in Sydney that I can see, that I trust with the most expertise re MPN’s. Garrrr, I dunno, this place is a far away island and prior to globalization it was very parochial.

But I like your suggestion, it helps to have a line up my sleeve, so thanks!

[Hidden in reply to jillydabrat]

You know - I went (with my husband) to a private consultant to try and get some of my issues addressed. It was worse than hopeless. All the guy did was talk to my husband as if I was not there. Husband realised what was happening and lowered his head so that the consultant would have to talk to the top of it --- which he did. I ended up in tears, asking the consultant 'did he not know I was in the room?' 'did he not know it was me actually verbalising and asking the questions?' 'did he not know that my ears were working just fine'?' and left. It is a pain in the butt not being able to get answers to your questions - even from those who set themselves up in the field as 'medical experts.

[Magentas in reply to Hidden]

So true, near impossible to depend on any of them down here, god knows how they got their degrees, let alone how they became so completely socially inept, or stupidly egoistical What a world.

[papakura55 in reply to Hidden]

A car dealer did that to me..talked to the male friend I had taken along ( who was visiting from the USA, so NO help at all for Oz vehicles) . Doctors need to lift their game if they don't want to be thought of as on the same social level as car salesmen ( second hand ones!). Or just stop being misogynists. That'd be nice.

[givingITMybest in reply to jillydabrat]

I'd agree with your suggestion and think the words you choose convey the right tone. It is important to speak up, using I messages as opposed to starting with you this and you that. I would hope the doctor might respond positively as you are not saying 'you are a bad doctor", you are simply asking to get your needs met as the patient. Tone of voice is criticle. Doctors have bad days, they also have health issues etc. and therefore can be curt or less than thoughtful on a particular day. Good luck

[Magentas in reply to givingITMybest]

Tis true, I get wrapped up in wanting to be cured but there is no cure nor is there any release from the torment this particular disease presents. Sometimes I just can’t help feeling like a child.

[Wyebird]

Hi Magenta, I have messaged you on the past and I think they’d is one there for you to answer . Your head is in turmoil. When I read your post I immediately thought of my friend. She analyses everything when I see it straight. So here is my view. You’ve had a BMB. It shows fibrosis. What do you have to lose by taking the meds? If she is an MPN specialist then I would stay with her. Lots of love xxxxx

[Magentas in reply to Wyebird]

Hi my friend, you are correct, it’s been on my mind, I owe you a private message. I guess the main thing I have to lose is that it’s great for reducing symptoms but I really don’t have any unmanageable symptoms and if I start taking it now it may not work in three years when I really need it!

There’s a lot going on for that scenario but whether these alternatives reach Australia or would even be affordable is very doubtful indeed.

targetedonc.com/view/addres...