MF (an acronym I often used, It'll never mean the same again!).

[P-O-T-S]

So finally I have gotten round to writing this post, it's about the third attempt! the last one got most the way through and my finger twitched left on the magic mouse and poof it was all gone

Ok, so Monday was the results of my BMB. Unfortunately, they have confirmed that I have Myelofibrosis! "Low Risk" at present. Although I was Polycycthemic in Hospital, I am not anymore. In fact, for the last five weeks including Monday, my bloods were fairly standard. which is good right? Of course they won't stay that way but hopefully, they will for many years to come.

I guess this way I have time to re-adjust my way of thinking and prepare myself for what is to come later down the road. Something sadly not all of us has/had, and I'm sorry that you don't/didn't.

My Haemotologist who is just lovely by the way, says that whether it was sMF or MF she would treat it the same in this case.

I have around three weeks to learn as much as I can about hematopoiesis (Try saying that with a mouth full of ET Timjonze). and understand how this is going to affect me in the long run as I feel there are still many miles left in this old corpse!

I will be returning in three weeks to see if I can be started on HU to try and shrink my spleen and suppress progression.

If it helps others here are my workup...

I am JAK2 V617F+ and MPL, CALR, Exon12 Negative.

WBC = 13.38

RBC = 5.18

HGB = 164

HCT = 0.480

PLT = 385

NEUT = 10.04

All the best for now

Barry

[jane13]

What a well-balanced-bod you are! The good news is you have a haem that u trust, she has sensibly used the risk stratification and you've come out low. And your blood counts are fabulously good! Only comment from me is that I'm not sure HU will shrink your spleen, so if it's problematic u may want to think about another drug. Ruxo certainly shrunk my spleen but think you have to be higher risk to get it on the NHS......

[P-O-T-S in reply to jane13]

Thanks for your reply jane13,

Indeed, what an odd set of bloods for someone with an MPN, that was my thought! I agree from what I’ve read and what you say about hydra, but sadly Ruiz is not an option in the low risk factor, was hoping for it myself for its spleen reducing powers alone.

[Aime]

Hi Barry, at least you have the answers and know what you have got, great that it’s low risk. I’m sure some of the other forum friends who have had the same experience will be posting soon to support you.

I have PV which I hope stays as PV but that’s for the future to see. Keep posting to let us know how you are.

Thinking about you, kind regards Aime x😺😺

[Paul123456]

Barry

Sorry to hear this. Hopefully you are very early stage.

Has your Hem discussed SCT options. My normal Hem recently retired and replaced, by chance, by leading SCT specialist. I asked about odds of survival and he said 90% for a 100% related donor and 80-85% for unrelated. He’d just successfully transplanted a healthy 77 year old male.

Can I suggest you ask about getting genetically tested for adverse mutations which can affect timing for a SCT. Hopefully you are say at least five years away, subject no bad mutations. And five years is a long time in terms of medical advancements.

Also, I’ve posted a number of video presentations by Dr Silver re chance that Pegasys can slow progression in early MF. I’m sure you have followed the Interferon debate which splits opinion, both amounst patients and highly respected Hems!

Also, I believe there are several trials of Pegasys and Ruxo combined - again some Hems think very promising whereas others concerned about long term side effects. Might be worth asking if there is a UK trial.

Why is your Hem prescribing HU and not Ruxo or Peg? I didn’t know that HU could slow progression?

Best wishes Paul

[P-O-T-S in reply to Paul123456]

Thanks for your reply Paul,

I haven’t considered looking into an SCT as my haemnsaid it’s no where near that stage. The rates look very good. Didn’t know about genetic testing for adverse mutations do you have a link for documentation on the subject?

I have read a little about peg and the possibility of slowing if progressions and didn’t think about it at the time. I’ll have to note it down thank you.

She did mention about trials and said that there weren’t any running at the minute but when they do she would definitely put me forward for them if I wished.

Maybe I have it wrong about HU, and progression, she did that patients had good results with spleen size reduction.

Thanks again Paul you’ve given me lots to think about!

Much appreciated

Barry

[Paul123456 in reply to P-O-T-S]

Barry

Watch the link I posted re Asia Conference. This is one side of the argument but it left me pleased I’ve started Pegasys. However I’ve been lucky re side effects.

This link also interesting re pre MF (which hopefully you are). Survival rates same as PV

bloodjournal.org/content/12...

Think it’s not a case of exactly where we are but how fast we are getting there!

Sounds like you are too early to worry too much about genetics

Best Paul

[P-O-T-S in reply to Paul123456]

Hi Paul,

this is very helpful thank you for posting. I am definitely going to be spending a bit of time over these few weeks to gather as much information I can ready for my next appointment. I really appreciate these recommendations.

I guess I will only know where my MPN is going forward from this BMB baseline.

I hate to mess with karma but been feeling pretty good this week physically apart from the dodgy shoulder which I don't think is linked.

Mentally and psychologically its been a little harder for me and the misses!

All the best, have a great weekend.

Barry

[Paul123456]

Scroll down to the Panel Discussions

mpnasia.com/site/mypage.asp...

Panel discussion

Dr. Jean-Jacques Kiladjian & Dr. Srdan Verstovsek

(Includes Dr Silver)

[Timjonze]

Sorry to hear about the diagnosis Barry but sounds like you’re in good shape presently and have a good outlook on it all which helps. Did they mention what grade of marrow scarring you have? I’m assuming fairly low at present. Your bloods look very normal to me. I’d definitely look into NGS testing as Paul suggests - the science behind it isn’t completely understood but there seem to be certain other mutations that predict a faster course of the disease. Fingers crossed you won’t have any of those - odds are that you won’t - but good to know where you stand. As for drugs I believe HU can have an effect on spleen size as it’s been recommended to me as well but might be worth researching both peg and ruxo (which seems to be the go to spleen shrinker!) and discussing with your doctor. MF is scary of course but a lot of informed people tell me that the prognosis isn’t necessarily as bad as it seems from the Internet these days and you have age and good blood counts on your side. Good luck with everything and keep us posted!

[P-O-T-S in reply to Timjonze]

Hi Tim,

Thanks for your reply, they didn't mention the amount of scarring and I didn't ask. I am presuming its low with me being "low risk" but thanks for pointing that out I will note it down for my next visit!

I will look into NGS testing as it sounds interesting but hopefully not something I will have to concern myself in the too near future. always good to be prepared and do your research though.

Ruxo I believe is a NO for now while a low risk, Pegasys is definitely worth looking into and mentioning at my next meeting.

The thought I MF is scary but like you said just be careful of the content you read and make sure it's up to date and relevant to you!

Has HU helped to shrink your spleen?

All the best for now

Barry

[Timjonze in reply to P-O-T-S]

I’m not actually on HU - docs happy with watch and wait for now. My condition seems stable in general - just the big spleen they’re are concerned with. I’m leaning towards low dose peg if possible when the time for treatment comes but they don’t want to see me until September now so I’m going to try my best not to think about it all until then (hard when your spleen reminds you every now and then!). All the best!

[mhos61]

Hi Barry,

sorry to hear of your MF diagnosis - but good to know that you’re low risk. At least now you know what your dealing with. Great too that you have a good relationship with your haematologist. It can make all the difference.

Educate yourself as much as possible; although I think there’s no worries there!

Mary x

[P-O-T-S in reply to mhos61]

Thanks for your reply, Mary,

It's the only thing on my mind at the minute! I like to know everything that I can understand, I have an inquisitive nature, should come in handy for once.

To be honest, all those that I have dealt with to do with my MPN so far have been really helpful, let's hope it continues.

All the best

Barry

[pj1963]

Hello Barry

I should introduce myself. I am 54 and was diagnosed with MF, low-risk, at the beginning of 2017. I'd had ET for 10 years. My blood results are actually very similar to yours and my condition was described just yesterday at my appointment as being stable. I'd actually had another BMB a month or so ago (I am to have them annually) and received the results yesterday. They showed a slight increase in scarring but that I remain in the low risk category. Like you, I am only JAK 2 +. I've asked about genetic screening, but they'll only do that if - or rather, when - I move up a risk group.

It is all very alarming, isn't it? I'm sure you find yourself thinking about it an awful lot, but as Paul and others have said on this forum, there is so much research going on, that I think we can remain hopeful that a new treatment will emerge for us.

I was put on Rux straight away to reduce my spleen (it was about 9cm below my ribs) and it has shrunk it so much that my haematologist cannot feel it. However, I live in Scotland where I know Rux is prescribed for low-risk patients. I hadn't realised HU can have the same effect and wonder why I wasn't prescribed that? Nor did I realise it can slow progression. I worry that the effect of Rux will wear off - as it seems to do - and that there currently isn't anything else available (although I know that Pacritinib is in the pipeline).

Do get in touch, Barry, if you want to ask anything.

Best wishes

Paula

[P-O-T-S in reply to pj1963]

Hi Paula,

Thanks for your reply, Its good to hear that I am not the only one who is in this category especially with your fibrosis progressing from ET. Do you still feel well day to day? has your spleen shrunk right down? I hear Rux is the king of spleen reducers so its good you have been given it. sadly as far as I have understood so far they won't prescribe it in the down here with unless you get into the medium to high-risk category.

It is very alarming, oddly enough I think I am dealing with the news better than the misses and some of the other family members.

It does look like there is a lot to look forward to in the way of treatment for MF and who knows by the time we need it there may even be a cure or at least a way of slowing progress. I'm not sure that HU does, I think that may have been my misunderstanding, I'm sure there will be plenty more before I get my head around it all.

All the best

Barry

[pj1963 in reply to P-O-T-S]

Hi Barry

I must apologise that it's taken me 4 days to reply ...

I have to say that I feel absolutely fine. I have never had bone pain or night sweats. Before I went on Rux I used to get headaches (ET), but they have disappeared completely. And yes, my spleen has completely shrunk down. The consultant said the other day that it was 'amazing'! Are you bothered by your spleen? I'm assuming it's enlarged? As for the prescribing of Rux in England, I thought that everyone could get it now, regardless of risk category, but I'm clearly wrong. When do you start on HU?

I'm sorry your wife is so upset, but I can understand, especially if she's googling things. Has she read some of the posts here? My husband remains positive (which helps of course), and I have to say there is so much going on out there that I am somehow hopeful too that this can be managed, if not cured.

Like Paul, I follow an anti-inflammatory diet and exercise as much as I can (gym 4 times a week for half an hour). I'd read that every disease is driven by inflammation, so I've cut out inflammatory foods.

Sending you my best wishes

Paula

[P-O-T-S in reply to pj1963]

Hi Paula,

No worries, we all have busy lives whether it's holding down the day job or coping with our ill's and getting the chores done around the house. Its just nice that people do take the time to reply and I'm a little guilty of that myself but its difficult with work, then the little one then I'm tired etc.

My partner is much better now, as I have reassured her by taking it all in my stride or at least that's what she thinks and that's best for both of us. I did tell her now to google it as the info is so varied and some of it quite ancient unlike me!

Oddly enough I followed what could have been classed as an anti-inflammatory diet for several years before diagnosis as I ate mostly plant-based with a little fish here and there, no meat but since that dreaded day I have to say my diet has gone a little haywire I'm sure it will return once I get used to the idea again. It helps immensely especially with energy levels.

Best wishes received with thanks, and send right back out to not only you but all that are suffering at the minute.

(Must dash, time for a bedtime story)

Cheers

Barry

[fee13]

m.youtube.com/watch?v=JHyYQ...(Not working as expected?)

This is about the early use of LOW DOSE interferon and MF, only 2.5 minutes long. May be worth pushing for this if you can tolerate it.

[fee13 in reply to fee13]

It's mentioned that hydroxy can relieve symptoms of spleen enlargement but does not work on the cause of MF.

[Paul123456 in reply to fee13]

Thanks, not seen this clip before. I’d still advise watching the link I posted above since Kiladjian also an advocate of early use Interferon. They discuss early MF but also PV/venesections.

For me the bottom line is that these guys use Interferon as their first line attack, hence possibly may get different results from the Hems who use Inf after HU or post treatment via venesections and aspirin.

My understanding is that HU does not slow progression. Ruxo increases life expectancy but primarily via reducing symptom burden. It may slow progression but I’m not aware of any research that claims significant reduction.

The Interferon bulls claim this is the only drug that makes a real difference but this is clinically unproven. It does achieve molecular remission but only in a subset of users. Does this equate to significantly slowing progression? As a layperson I’d have thought yes but some very knowledgable Hems not yet convinced. I assume they argue that it could be that those in remission may have a more benign disease? Can’t compare like with like.

So they balance possible (clinically unproven) slowing of progression versus Inf side effects that cause high drop out rates and possible long term side effects re thyroid, autoimmune and depression.

So bit of a can of worms. I’ve gone for low dose Peg since should be low side effects. Re the long term risks, I figure my principle aim should be to try to slow the clock and hope new drug available within say 5 years.

[Timjonze in reply to Paul123456]

Have you noticed any effect on your mood at all Paul? My doc said a lot of people become more irritable and hard to live with (insert obvious joke here!) which did concern me a little.

[Paul123456 in reply to Timjonze]

Tim

No, not that I’m aware of but it is something I’m very conscious/self analytical about.

I think we have to be as proactive as possible and I’m happy that I’m doing everything I can to slow progression (Peg, diet, exercise) and as long as I can continue to consume my 25 units of red wine per week, life is great! (My principle worry is whether Peg + wine might overload liver/kidneys but so far so good!)

Best Paul

[Meatloaf9 in reply to Paul123456]

Hi Paul and all, I did see a mpn specialist at cleveland clinic about one month ago and his belief is that there is not any proof that any medication available today will slow progression of any of the mpn's which he said should all be lumped under the same umbrella. Let's hope he is wrong about that and that we just don't have the proof yet. Best wishes to all.

[Paul123456 in reply to Meatloaf9]

Agreed, it’s frustrating that opinion so split although I sense a slight mood swing post the end year 2 Ropeg versus HU trial, which indicate that Ropeginterferon delivers superior molecular response. Trial ongoing so end year 3 results could herald a new front line drug...

Will be fascinating - HU was better (quicker working) after 6 months, level pegging at one year and Ropeg pulling away on a molecular level at two years.

Ropeg is a more refined and therefore better tolerated version of Interferon. Easier to administer, every two weeks.

[P-O-T-S in reply to fee13]

Thank you fee13,

definitely worth noting and speaking to my Haem at our next appointment as its the only reason I would be taking it. I still have lots of research to do before I see her again.

All the best for now

Barry

[Simon96]

Hi Barry,

That is sad news. I hope a story from my travels may be a bit of a morale booster for you when you are reading up on myelofibrosis as the outcomes are so variable.

I have primary MF, JAK2+. My spleen was the same size as yours at diagnosis. While large compared to a normal spleen, that is actually pretty small in MF terms. My WBC was 6.6, RBC 5.27, HGB 139, PLT 553 and NEUT 5.2 at diagnosis.

My HGB level improved when I started on HU and stayed around 150 for a long time.

I did not care too much about life expectancy when I was diagnosed. I wanted to know what the quality of my life would be and how that would effect my family. Thus this story... fourteen years post my diagnosis, I made a 5 week trip to Europe with my main mode of transport being walking. it included a spur of the moment day trip to Gibraltar. On arrival I looked up and decided to climb as high as I could and be as close to Africa as I could. About half way up I realised that sensible people were being driven up in taxis (and were probably planning what wine to drink with their lunch on their way down). Still, I was very happy with my decision - I felt that I got the best views and experience.

I have been very lucky. Unfortunately many of the MF stories that you might come across on the internet are not that pleasant. There have been times when myelofibrosis has affected me too, but certainly not to the extent that you would think when you initially read up on myelofibrosis. It is important to take a bit of time to get to know the new you, learn what works for you and what doesn't. I am happy to answer any questions and wish you the best.

Cheers, Simon

[P-O-T-S in reply to Simon96]

Hi Simon,

Thanks for taking the time to reply, It is sad news but you already know that first hand. Our numbers and diagnosis are very similar. I have read some stories that I wish I hadn't but I'm sure the tellers wished the same that their story was more like yours. It sounds like it hasn't or doesn't at the minute stop you from achieving your goals and enjoying your life the way you want to and I'm really pleased for you!

How long have you been on HU? have you suffered any side effects?

You say you have had times when your MF has affected you do you mind if I ask how I completely understand if you don't want to answer.

I hope you not only enjoyed your trip but are planning many more if that's your thing!

I also feel very lucky that I am low risk with few side affects at the minute, I only I could reduce this spleen and sort this shoulder out I can get back to what I enjoy riding my bikes!

All the best, enjoy your weekend

barry

[Simon96 in reply to P-O-T-S]

Hi Barry,

Hopefully your low risk status will last for a very long time and it will be quite a different MPN world before your risk profile changes.

I have been taking HU for most of this century. I am not aware of having had any negative side effects from taking it.

Initially I was not really affected by my MF. I had not noticed my spleen until I was told at diagnosis time. As it grew I found that bending over in the same position for any length of time was not good for me. That may effect your cycling down the track.

After some years I did develop some of the fatigue issues that many other MPN'ers have described. I tend to think of it as being environmental fatigue. I was able to control it quite well by recognising my triggers and managing my environment. This topic is covered very well on the MPNVoice website.

This year I have been affected by anaemia, which is quite different from fatigue. I will cover that in separate posts as I learn more about my changing situation, though I suggest you just ignore those posts as the MPN world will be very different by the time you are considering those type of changes.

Best wishes, Simon

[P-O-T-S]

Thank you very much for all your replies and support and it’s much appreciated! I have a busy day at work today but will try and reply when I can.

Thanks again!

[S031251]

Good and bad news for you Barry but keep as positive as you are.

Good luck

Anna x

[P-O-T-S in reply to S031251]

I'm trying!