Hi everyone, I’m new here and live in Sydney.

[Jaqi]

Hi everyone, it’s Jacqui here. I’ve finally got around to introducing myself here!
Thanks to Steve for making this site available and I hope you are all doing as well as can be expected.


I’m 62 and was diagnosed with ET jak2 in early March ‘21. The haematologist at RPA in Sydney was very blasé and within three minutes he had me out the door with a prescription for Hydrea and told me not to be alarmed as “it is not chemotherapy”.
After coming home and doing a bit of research I was left reeling and feeling isolated but I found MPN voice and then heard about you guys and I’ve spoken to Steve a couple of times. So thanks to Steve for passing on Cecily Forsyth’s recommendation from fellow patients who have seen her. I’ve seen her twice now, she has thoroughly impressed me, it feels so good to be in the hands of someone I completely trust. Her nurse practitioner, Jacqui, that sat in at my appointment yesterday is lovely and excellent, too!
It took me seeing 3 haematologists to find someone I had faith in and who didn’t seem like some rote practitioner, so, obviously I can’t recommend her
highly enough.

As I was diagnosed with ET the first thing she did was have me get a BMB, just as well, as it turns out I have Myelofibrosis F not ET! I just found out yesterday and there I was just getting used to the ET. So that’s been a real blow. My fibrosis is 2 on a scale of 0-3 so not good. Geez, that was fast, how long have I had this I wonder!
As much as Cecily loves Pegasys she doesn’t feel it’s right for me, for 2 reasons, for one I am prone to depression and the best Interferon to counter that risk isn’t available here (U.S only) and as I may need a bone marrow transplant down the line if I’d been on Interferon it would mess with that. Sorry, not very technical but that’s all I’ve got at the moment.
So, she’s waiting on my chromosome results, due in a couple of weeks, and if they tell her enough then I won’t need the genetic sequencing test that will cost me $650. These are tests to see what other mutations I may have...important information for prescribing and prognosis.
From what I gather, I’m guessing I’ll shortly be on Ruxolitinib and need a bone marrow transplant down the line. Cecily said the JAK inhibitor Ruxolitinib makes your symptoms much better, so yay, that’s something.

FYI,
as I’d had an ET diagnosis, after my first appointment, she prescribed me 2 x Cartia a day and no meds until results were in. When I queried her about the fact that it should be 2x 100mg a day she cited this study and said it was the latest protocol. I am now back to one a day since the MF diagnosis.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5992153/

My platelets are still around the 650 mark.
My clotting factors are fine.
My red blood cells are low to normal **Hydrea lowers you red blood cells as well as your platelets.
White cells fine.
Large and abnormal shaped cells with some pockets of clumping in my bone marrow.
Increased Reticulum /fibrosis in marrow, graded as MF2.

The worst part is that my possible years on the planet have suddenly diminished and the only people my husband and I have are each other, we adore each other, and the idea of leaving him alone is killer.

Wishing you all the best new friends!

[MPN-MATE Admin]

Hey Jacqui...

Been a super busy day of catching up w/ so much since having returned from my SYD to COFFS MPN Awareness & Fundraiser RIDE...

My apologies for not being able to talk longer earlier... It's unbelievable how much I seem to have to wade through each day... But I am managing I guess...

Yes, it might seem like a truly quick progression for you from a recent ET diagnosis to one of Post ET /MF, which is by the way exactly what happened w/ myself too... Both you and I were quickly re-classified due to a Level 2 BMB result. However, you might hopefully also soon find out, (as have I), that the upgrade of diagnosis is not necessarily related to a shortening of one's longevity...

In my own case, (much of which is now known to you), I too was at first completely overwhelmed, but after a period I did the 'Taylor Swift' thing & 'Shook it all off' and decided to turn my Negative into a Positive, and so far so good I guess...

With my change of diet & exercise routines, I was soon to learn of a slight but significant 'molecular reversal' in my bone marrow fibrosis. My original grading was Level 2 like yours, but after less than a year of my 'Lifestyle' changes... It reverted to Level 1... YAY!!!

My DGS revealed that I do possess what is usually considered as an adverse high risk genetic mutation known as "ASXL1+" however... It's now 5 years since my diagnosis, and at times I feel great... Of course I still suffer from some symptoms, mainly overwhelming fatigue, and the side-effects that I now relate to very high Blood Platelets, i.e. Tinnitus, vision impairment, headaches, AF...

However, many of the worst symptoms like; bone & joint pain, Restless Leg Syndrome (RLS), and cold sweats were largely alleviated once I commenced taking Ruxolitinib (Jakafi), their incidence is much lower these days...

I do still suffer from some bone & joint pain, but I also believe that maintaining a consistent level of fitness helps me w/ my energy reserves and even though I often have to drag myself out of a warm bed... Everyday, I just tell myself that I am like that little engine who could...

"I think I can, I think I can... etc... " And after more than 18 months of waiting, I have just concluded one of my major goals, which was to RIDE c. 600km to raise MPN Awareness & a small amount of funding into MPN Research, and most importantly... Now that I did that RIDE, I am even more confident that I will keep going, and I shall never surrender easily... Much rather keep on fighting!

When it comes to discussions concerning all the pros' & cons' of Allogenic Stem Cell Transplants (ASCT), please remember that one of our own MATE'S Committee members, (Warwick Gosling), has just undergone this process and is now 62 Days into the first 100 Day window of his recovery, and Warwick will no doubt be along shortly to share with us much about his journey too...

Warwick will definitely be the best person to discuss this only currently known MPN curable option, (ASCT), and fingers crossed... his results thus far seem quite promising...

Best wishes Jacqui & I am sure that others will be along to say hi very soon... Keep smiling & keep that chin up girl...

Steve

[Jaqi]

Thanks so much for the encouragement and taking the time and energy from your demanding life to share, Steve.
And congrats on your bewildering accomplishment!
I am still overwhelmed by fatigue so that’s all I can say atm. Do take care.

[Lady in Red 01]

Hi Jaqi,
Firstly welcome to Mates & MPN Voice really glad you found us..

Like you I was very taken aback shocked when diagnosed with ET..
I had a BMB couple of weeks later and Lo & Behold I have MF too..
I am also JAK 2 + like you, I am classified as Intermediate 1 on the Scoring Schedule..

None of that actually matters in reality when first diagnosed, I believe...
My world had been rocked, I was both scared of the illness and my much decreased
Life Expectancy !

Why me ? I asked myself, I had no desire to discuss my issues with my husband and loved ones, simply too painful to say & hear the words!
I found MPN Voice the night I was diagnosed with just ET, & someone helped me immensely that first night..
Total stranger but she knew exactly what was going through my mind...
At that point I didn’t know that I would soon discover the MF..

Like you I needed to know more of this disease, thus I researched etc..

I met Steve on Voice 2018 a year seared into my soul, “ the year of change”...
Voice is marvellous as is Mates, so very much knowledge on these Forums..

Like you I needed to see an expert in MPN’s and that took me to Professor Claire Harrison st GUYS London...

The Proff put everything into perspective for me, I knew about Ruxolitinib but had to fight to get it..
I was on Hydroxycarbamide for almost two (2) years, I commenced Ruxolitinib in 2020..

I was suffering Bone Pain in Extreme, it took 6 wks of taking Rux when I realised that I was no longer living on Analgesics..
Ruxolitinib has changed my life for the better, during those two (2) years I was also diagnosed with PV

Thus I have all three (3) Flavours of MPN’s which is rare...


I am a Retired RN only just retired when first diagnosed...
Telling my Children was extremely hard & discussing it with my husband was & is very hard...

I feel for you completely, the best advice I can give you, is to make every day count..
Be positive in your thoughts with regards to leaving hubby alone...

Jaqi advances are always there in the pipeline, stay fit & healthy keep moving forward and remember to Enjoy Life with your Husband..

None of us really know what is around the corner, I feared for myself in 2018...
Come September 2019 my normally fit husband was first diagnosed with Bladder Cancer in the May, he had Surgery & Chaemotherapy for 10 weeks...

Come late September 2019 he had a routine Pre Op Assessment, the Docs needed to look at the Bladder to be sure the Chaemotherapy had done it’s job.
Only to discover my husband was a little breathless, on further investigation it was discovered that he had three (3) Arteries blocked, he needed a Triple Heart Bypass !

He was weeks in hospital hooked up to monitors etc 31 October 2019 he had his Bypass Surgery!

I had been terrified of leaving my family and in an instant things had changed..
I was terrified for my husband, it taught me a very valuable lesson, None of Us Know what is around that Corner..

Now I accept my lot with MPN, I have made wonderful friendships with folk on the same journey with MPN’s....

Life is for Living don’t waste time worrying about the unknown...

Things will settle down and you will settle within yourself...

Steven is a Prime Example of how to live a full life with MPN...
I will shortly be having another BMB & my Nurses Head tells me I have progressed to Level 2 on the DIPPS Scoring System..
Hey ho, I have just done three (3) years & I can do the next three years and so on, so forth.

Take Care, Stay Positive and if you should need support , it’s here on Mates & Voice...

All the Best to You
Maria

[Merry]

Hi Jaqi
Welcome to the forum and thank you for such an informative post. I live in WA and was diagnosed with ET JAK2+ back in 2015. I am under the care of a public health haematologist in Perth who I have a Telehealth appointment with every six months. I haven’t had a BMB as they have told me that would only happen if my blood test results changed. My platelets have only ever been up around 550 but I had a blood clot and since then have taken Apixaban (blood thinner). I was on Hydrea for a few years but stopped that around 18 months ago and my platelets have only reached 450 since then.
I’m so glad you have spoken to Steve and are now under the care of an MPN specialist. I wish there was one in WA!
Sending warm regards, Merry

[Alkemist]

Hi Jaqi
I'm pleased to see that you've received reassuring advice from Steve and Maria who both have a similar diagnosis to you, and I don't think I can add anything further as I still at the ET stage.
I was diagnosed with JAK neg ET back in 2006 following increasing platelets levels and a BMB. CALR mutation was confirmed a couple of years ago. I was on Hydrea for several years but this gave me numerous skin cancers and eventually stopped controlling my platelets. I started on Pegasys about 18m ago. Platelet control is improved but I have a number of side effects.
One thing I've learned from this and other support groups is that everyone seems to have a different experience of their MPN and their treatments. What works for one will not necessarily work for another. It is also important to keep up to date with developments (there's much more research going on in this area than their used to be) and it is good to question/challenge your doctor. It is good that you have found a specialist that you have confidence in and keeps up with the latest studies. Hope your test results allow the best course of treatment for you to be identified.
Best wishes
Allan

[Kinsale]

Hi Jaqi
As you can see we are a supportive and diverse group here who are all focussed on supporting each other as we learn to live and thrive with our MPN's. Re me I was diagnosed with ET in Sept 2014 and I was referred to Cecily immeadiately. Cecily is brilliant and has journeyed with me, discussing, guiding and assisting me. After initially just taking Asterix for 2 years (I'm still on it), we discovered that an MRI of my brain identified ischemic changes in the white matter part of my brain (ie minute patches of brain damage). So I've spent 2 years on Hydrea and am now into my third year of Pegasys. Cecily has been wonderful as I worked through the realisation that I was going on treatment for the rest of my life. Pegasys has been a miracle drug and I've achieved CHR (complete Haematalogical Response) where my haematology results are all within range as are my biochemistry results. I now inject 90 MCG every three weeks and whilst I've been determined and dogged in my approach to going on treatment, Cecily's guidance and navigation has been essential. You are in good hands with Cecily.
Regards
Mark

[Jaqi]

So lovely to meet you, Maria. Your message was so needed and welcomed this morning. Thank you!

The first day after my diagnosis it was quite surreal but by the third (yesterday) and having read about this hideous disease and it’s symptoms, well, it’s really hard to take and I feel so very sad and worried about future symptoms, the fact that I’m only debilitated with fatigue (mostly) atm is bad enough but a life of physical pain is not my idea of living. But, of course, thanks to Ruxolitinib and euthanasia for the terminally ill, which is now legal in some states here, I consider us relatively lucky.

It’s so nice to hear from someone who has gone through the same. Although, I really am so sorry to hear you have all 3, that’s really tough. And thank god you fought for and won your Rux. I think my Haematologist alluded to the fact that she may have to get something tweaked a little in order for me to get the Rux. She’s a good woman...and has at both visits impressed upon me the importance of finding and doing things I like and having a quality of life.
Hubby and I have always dreamt of moving to the seaside but it always seems to be one of those fleeting things that only happen once you come back from a break from the city, haha, but now we will do it. We rent so its just down to getting lucky and finding the right rental just 1 1/2 hours up the coast.

Your advice is very welcome and rings true of course so thanks Maria, I’ll take it on board. Both you and Katie came through for me today, which was wonderful as I was having a bad morning in bed with nothing but dark thoughts about all this. In fact, Katie just rang me now while I was writing this reply and we spoke for the first time ...she’s so lovely and she cheered me up, too! I’m much looking forward to visiting her in person after we move.

Steve is a force of nature (sometimes I wish had heaps of testosterone ) and he’s been very forthcoming with information and his desire to help is really something...so more to be grateful for.

I’ve always appreciated RN’s, such an honourable job. I guess for you more than most it must seem particularly disheartening to be dealing with this in your retirement, I’m so sorry...and then there’s the bloody pandemic, too. Have you had no trouble securing the Rux? I think I read that all your symptoms come back pretty much immediately if you stop taking it, a bit of worry given how precarious the world is these days....was thinking maybe I should stash my first months supply as a backup.

I sure look forward to the settling down period (as it did with the ET diagnosis) and hopefully some help with this godawful fatigue...half the time I don’t even know what time it is because everything is the same..nuthin’ but horizontal rest for so many days now! I did manage the seniors excercise class 3 days ago (I do these classes regularly) but now I have such bizarrely sore muscles lasting a second day now...makes me wonder if this is a new thing related to my blood.

I have my fingers and toes crossed for your marrow results, please let me know and I so hope your husband is doing well enough now after all those frightful health problems, thank god at least these things were discovered and dealt with pre covid...it’s amazing what surgeons can do these days!

Wishing all the best for you and yours, Maria. x

[Jaqi]

Hi Merry, lovely to meet you .
I have to say what you’ve told me worries me a little, its unfortunate that Perth is so remote in terms of specialists with knowledge in this feild but there has to be a way around it.
From what I have learnt recently it is even the criteria of WHO for anyone diagnosed with ET that they have a BMB as well as what any MPN specialist will order.
This is to diagnose for Masked PV or as I have just discovered MF. Treatment changes if these are present, as are/is , molecular genetic tests/sequencing/panel (many terms) to check on mutations, it seems there are quite a few not just these -
Jak2
CALR
MPL
ASXL1
These all impact on the disease and it’s treatment. I just came across this and scanned it, it appears to explain a lot re driver mutations, although they need a good editor, lol. Ugh, there is so much reading to do I find!

https://www.leukaemia.org.au/research/c ... s-for-mpn/

My platelets were just under 500 during 2018-19 but started rising further in late 2020, the last 3, all done over the past 3 months have been around 660. So you can see it didn’t take much for me to be diagnosed with the second to last, highest level of scarring- MF2. And the fact that you had a blood clot already is not good. If by any slim chance you did have MF (I don’t know too much about PV) your medication MAY change to incorporate the risk of bleeds rather than clots. I don’t mean to alarm you, you would most likely have symptoms but worth chasing up. And that’s fantastic that your platelets didn’t rise after dropping the HU poison, as I’d read that they usually climb right back up immediately, something that doesn’t happen with Pegasys, apparently.

You could start by sending your haem the literature on the importance of BMB’s and insist on having one done as this is no frivolous matter and will guide your outcome. If they refuse find another haematologist!
In NSW we have The Cancer Council, I don’t know what’s in Perth but it could be worth getting in touch with similar in your area if need be...actually, I imagine the cancer council is national.
The biopsy isn’t pleasant but it’s no big deal, really, in the scheme of things.

May I ask your age, Merry?

Please let me know what you think and how you go, Merry.
All the best to you.
Jaqi

[Lady in Red 01]

Evening Jacqui,
It’s lovely to meet you too, thanks for the kind reply..

Yes Fatigue is hard to adjust to, however you will get to know your limitations & the pre curser to a Bad Day .
You will however have so many more GOOD DAYS to come..

This illness just doesn’t respect our Feelings and Wants & Needs, You have to become the Ringmaster & take back control to suit your needs & lifestyle

Jacqui have faith that “ You Can Do This” yes it’s a challenge but I for one will definitely “Not Roll Over & Give In” to this MPN”...

We are not Terminal, we simply have a Life Shortening Illness...
As a friend and RN put aside your thoughts of Euthanasia, you have so much living to do with hubby...

None of us know when we will shuffle off this mortal coil of ours, in my view the Very Best we can do, is to make our journey on this earth as good and happy as possible, achieving our goals to the best of our abilities..

You clearly have dreams of Halcyon Days living by the Sea, with the man that loves you and you clearly adore him..
Go Do It, have fun when you can, rest when you need to. Get up refreshed and Enjoy the next part of the day doing whatever makes you both happy....

Yes we are all very different, but the common denominator we all experience is Fatigue!
I am a Nursing Sister that ran a 36 Bedded Ward, a Surgical Unit, it was truly alien to me when I realised I couldn’t undertake such a level of activity both mentally and physically anymore...

Thus I learnt very quickly how to manage my fatigue especially on the bad days, which still happen..
For me Activity from early morning absolutely suits me fine, by around 1 pm I am tired to exhausted..
If I need to, I retire to my bed for a couple/few hours and usually drift off listening to my music and awake refreshed for the evening to come.

I do everything I always did as routine prior to MPN, I simply pace myself and if it’s an “ Off Day” I accept it..
We are all on this journey together aboard the good ship MPN, a Cruise I never anticipated, between all of us Cruisers, we have a Wealth of Knowledge & Understanding....

You have already said it,” look at Steven” he will definitely “Not Surender to MPN”...

I have seen his injuries long ago from a Cycling Accident, he most definitely would not give in to his illness, he awaited recovery and doggedly set about training once again in anticipation of his mammoth ride around Oz completely, Steve & I are pretty much level on the MPN Stakes..

Ruxolitinib is for me and many others an absolute godsend...
When I referenced Bone Pain with you, for me it was Left Sided Pain only..
Once Rux got going and completely replaced Hydroxycarbamide, life got so much better...

I personally wouldn’t recommend,
“ Stashing your First Months Supply“ just in case scenario..
We have not experienced any shortage of supplies here in the UK, but that is not to say that we could experience it at some stage...
For me when I commenced Rux it was prescribed in just One (1) months supply at a time..
In order not to waste Medication, if and I mean a Very Large If, just in case Rux didn’t suit you...
Once it was established that I was responding & happy with Ruxolitinib I receive 12 weeks supply at a time..

Our respective Consultants will always ensure we have our medication..
With regards to stopping Rux and symptoms returning, again that would be a gradual titration of a drug to take over from Ruxolitinib..
No responsible Consultant would ever leave a patient without adequate Meds for their individual needs..
We simply cannot worry about every eventuality, life would become so very tedious for sure

Give yourself a break from worrying about Meds etc, concentrate on the here and now...
I know easy to say, I felt that too but quickly realised that I couldn’t worry about every single aspect of having my version of MPN’s..

In fact I actually thought that I must have done something wrong in life..
I was told on a Friday that I had ET & needed Chaemotherapy Drugs.
On the Saturday a letter dropped on the mat from the recent Breast Screening I had, saying that I had for the first time an Abnormal Screen !!!
I actually thought I would lose my mind worrying about all of it.
I had Breast Surgery which was absolutely fine as it turned out, just prior to the results of BMB...

They were hard days, weeks of worrying until my lovely lady Haematologist took my hand and said, “Maria you will undoubtedly worry yourself into an early grave, let us worry for you & get everything under control “......

I realised that Six (6) weeks had elapsed and I couldn’t tell you what I had done during those weeks other than withdraw into myself and worry...

You have a wonderful Physician in
Cecily Forsyth just as I have in Professor Harrison..
Put your trust in them, ask questions when you need to, push for answers if you need to, which I doubt...

Novartis Pharmaceuticals that make Ruxolitinib approached me last year for an in depth interview as such, as to how I manage having the three (3) flavours etc.

Also I had not commenced Rux at that stage, they hoped to interview me again once I had been taking Ruxolitinib for several months..
Purely to glean from the patient’s point of view exactly how much I was benefiting from taking Ruxolitinib...

I told them the truth on exactly how one feels once you hear those dreaded words, “Blood Cancer”
I was honest and very frank with them & detailed how I felt about my MPN’s and a certain level of distrust in Pharmaceuticals etc...

Apparently the interview was a success for both of us, many young minds logged in to hear about my battle to obtain Ruxolitinib...
Novartis feel Rux should be readily available to all that need it around the world etc...

Naturally I have never lost sight of the fact that Pharmaceutical Companies make Mega Bucks etc, etc...
I am however hugely grateful to have Ruxolitinib as my Drug of choice..

I was contacted few weeks ago to do another interview for a Symposium in the near future of Worldwide experts in MPN’s Haematologist to actually hear directly from a patient’s perspective where the Medical System let’s us down, as MPN’s are so rare...

I related how frightening it is and the Need for Clinicians to have some level of understanding of MPN’s...
All too frequently I get asked by various Physicians, “ what is an MPN “ ??
I hope I did all of us justice in the quest for Research, Patients given enough time at Consultation’s to ask questions, better understanding of newly diagnosed and the shock that the patient inevitably goes through...

My fabulous lady Haematologist was from Greece and sadly for me, she & her family eventually returned to her homeland..

I then came under the care of a new Haem a nice guy, Consultant level but not a huge experience with multiple Variants of MPN..
We took six (6) months to become accustomed to each other’s ways etc..
I quickly gleaned he was happy to listen to what I needed to say, the questions that still crop up and bless he has really researched the implications of multiple MPN’s etc..
He has seen me at my worst in tears etc, and he always helps if he can..

For me personally that part was hard, I trained at this Hospital and spent all of my working years there..
Walking the corridors as a patient was a huge change for me, I didn’t want former colleagues and friends to know my situation...
Someone very special on the Forum talked this over with me & slowly I decided to let folk know exactly why I was spending time at the Hospital...

Once I got my head around that, it became easier all round.
However our children know, but never question me on the subject personally, that suits me fine too...

On Forum’s one can be brutally honest about your private thoughts.
The anonymity it provides allows one to speak freely and that is a key ingredient, to talk...

Research on MF, there is plenty of it out there on various papers etc. Steve is the expert on what is going on in the world of MPN’s etc..
Professor Claire Harrison at Guy’s London is a world respected authority on all things MPN..
Ruben Messa is another that publishes papers on research into MPN’s...

At the end of the day, it is down to us the patient to navigate a way through the early day’s and live in relative harmony with MF...

When PV was diagnosed last year, I took it on the chin as such, until the day I had my first Venesection, I sat there and looked at the Blood being taken off & cried with a former colleague..
It didn’t hurt whatsoever, it simply made me sad.
I have only had two (2) Venesections last year, once I commenced Rux that issue hasn’t happened since...

If & when then I will deal with it not before, there is zero point in giving it air..

So Jacqui, I hope you can get yourself settled with your diagnosis, go live by the Sea, Enjoy Life as much as you possibly can, when you can..
I live on the coast and love walking by the sea Summer & Winter...

It’s coming up to 5am with you, have a lovely day as best you can, cheer up & look ahead to a fresh start on the coast..

I am currently staying with my daughter, we Sold our home late March this year, and have just purchased a Bungalow..

All I will say on that subject is , pace yourself with packing etc, I was totally absolutely exhausted and was horizontal for several days after the move.

But it’s exciting looking for a new home & in a sense a new chapter. 🥳

Stay Well & Safe Looking Forward....
All the Very Best to You
Maria

[Jaqi]

you are the loveliest, Maria!
Thank you so much for your kind words of wisdom and your generosity, that was a long message! All that time and effort, just know your sharing has not gone unappreciated, I am quite blown away.
It’s been a good day I got to read your message in full, it gladdened my heart and that bizzare fatigue that kept me horizontal for 8 full days seems to have vanished! I’m sure it’ll be back sometime but just to know there’s respite from it and that at times it wont be as severe, I like to think, gives me hope. Its also comforting, in a way, to hear how we are all in that tired boat...sailing the MPN waves together .

I have more to say but I’ve done my dash for today. My heartfelt thanks for being here for me, Maria. Big hug, Jaqi

[Jaqi]

.

[Jaqi]

Evening Jacqui,
It’s lovely to meet you too, thanks for the kind reply..

Yes Fatigue is hard to adjust to, however you will get to know your limitations & the pre curser to a Bad Day .
You will however have so many more GOOD DAYS to come..

This illness just doesn’t respect our Feelings and Wants & Needs, You have to become the Ringmaster & take back control to suit your needs & lifestyle

Jacqui have faith that “ You Can Do This” yes it’s a challenge but I for one will definitely “Not Roll Over & Give In” to this MPN”...

We are not Terminal, we simply have a Life Shortening Illness...
As a friend and RN put aside your thoughts of Euthanasia, you have so much living to do with hubby...

None of us know when we will shuffle off this mortal coil of ours, in my view the Very Best we can do, is to make our journey on this earth as good and happy as possible, achieving our goals to the best of our abilities..

You clearly have dreams of Halcyon Days living by the Sea, with the man that loves you and you clearly adore him..
Go Do It, have fun when you can, rest when you need to. Get up refreshed and Enjoy the next part of the day doing whatever makes you both happy....

Yes we are all very different, but the common denominator we all experience is Fatigue!
I am a Nursing Sister that ran a 36 Bedded Ward, a Surgical Unit, it was truly alien to me when I realised I couldn’t undertake such a level of activity both mentally and physically anymore...

Thus I learnt very quickly how to manage my fatigue especially on the bad days, which still happen..
For me Activity from early morning absolutely suits me fine, by around 1 pm I am tired to exhausted..
If I need to, I retire to my bed for a couple/few hours and usually drift off listening to my music and awake refreshed for the evening to come.

I do everything I always did as routine prior to MPN, I simply pace myself and if it’s an “ Off Day” I accept it..
We are all on this journey together aboard the good ship MPN, a Cruise I never anticipated, between all of us Cruisers, we have a Wealth of Knowledge & Understanding....

You have already said it,” look at Steven” he will definitely “Not Surender to MPN”...

I have seen his injuries long ago from a Cycling Accident, he most definitely would not give in to his illness, he awaited recovery and doggedly set about training once again in anticipation of his mammoth ride around Oz completely, Steve & I are pretty much level on the MPN Stakes..

Ruxolitinib is for me and many others an absolute godsend...
When I referenced Bone Pain with you, for me it was Left Sided Pain only..
Once Rux got going and completely replaced Hydroxycarbamide, life got so much better...

I personally wouldn’t recommend,
“ Stashing your First Months Supply“ just in case scenario..
We have not experienced any shortage of supplies here in the UK, but that is not to say that we could experience it at some stage...
For me when I commenced Rux it was prescribed in just One (1) months supply at a time..
In order not to waste Medication, if and I mean a Very Large If, just in case Rux didn’t suit you...
Once it was established that I was responding & happy with Ruxolitinib I receive 12 weeks supply at a time..

Our respective Consultants will always ensure we have our medication..
With regards to stopping Rux and symptoms returning, again that would be a gradual titration of a drug to take over from Ruxolitinib..
No responsible Consultant would ever leave a patient without adequate Meds for their individual needs..
We simply cannot worry about every eventuality, life would become so very tedious for sure

Give yourself a break from worrying about Meds etc, concentrate on the here and now...
I know easy to say, I felt that too but quickly realised that I couldn’t worry about every single aspect of having my version of MPN’s..

In fact I actually thought that I must have done something wrong in life..
I was told on a Friday that I had ET & needed Chaemotherapy Drugs.
On the Saturday a letter dropped on the mat from the recent Breast Screening I had, saying that I had for the first time an Abnormal Screen !!!
I actually thought I would lose my mind worrying about all of it.
I had Breast Surgery which was absolutely fine as it turned out, just prior to the results of BMB...

They were hard days, weeks of worrying until my lovely lady Haematologist took my hand and said, “Maria you will undoubtedly worry yourself into an early grave, let us worry for you & get everything under control “......

I realised that Six (6) weeks had elapsed and I couldn’t tell you what I had done during those weeks other than withdraw into myself and worry...

You have a wonderful Physician in
Cecily Forsyth just as I have in Professor Harrison..
Put your trust in them, ask questions when you need to, push for answers if you need to, which I doubt...

Novartis Pharmaceuticals that make Ruxolitinib approached me last year for an in depth interview as such, as to how I manage having the three (3) flavours etc.

Also I had not commenced Rux at that stage, they hoped to interview me again once I had been taking Ruxolitinib for several months..
Purely to glean from the patient’s point of view exactly how much I was benefiting from taking Ruxolitinib...

I told them the truth on exactly how one feels once you hear those dreaded words, “Blood Cancer”
I was honest and very frank with them & detailed how I felt about my MPN’s and a certain level of distrust in Pharmaceuticals etc...

Apparently the interview was a success for both of us, many young minds logged in to hear about my battle to obtain Ruxolitinib...
Novartis feel Rux should be readily available to all that need it around the world etc...

Naturally I have never lost sight of the fact that Pharmaceutical Companies make Mega Bucks etc, etc...
I am however hugely grateful to have Ruxolitinib as my Drug of choice..

I was contacted few weeks ago to do another interview for a Symposium in the near future of Worldwide experts in MPN’s Haematologist to actually hear directly from a patient’s perspective where the Medical System let’s us down, as MPN’s are so rare...

I related how frightening it is and the Need for Clinicians to have some level of understanding of MPN’s...
All too frequently I get asked by various Physicians, “ what is an MPN “ ??
I hope I did all of us justice in the quest for Research, Patients given enough time at Consultation’s to ask questions, better understanding of newly diagnosed and the shock that the patient inevitably goes through...

My fabulous lady Haematologist was from Greece and sadly for me, she & her family eventually returned to her homeland..

I then came under the care of a new Haem a nice guy, Consultant level but not a huge experience with multiple Variants of MPN..
We took six (6) months to become accustomed to each other’s ways etc..
I quickly gleaned he was happy to listen to what I needed to say, the questions that still crop up and bless he has really researched the implications of multiple MPN’s etc..
He has seen me at my worst in tears etc, and he always helps if he can..

For me personally that part was hard, I trained at this Hospital and spent all of my working years there..
Walking the corridors as a patient was a huge change for me, I didn’t want former colleagues and friends to know my situation...
Someone very special on the Forum talked this over with me & slowly I decided to let folk know exactly why I was spending time at the Hospital...

Once I got my head around that, it became easier all round.
However our children know, but never question me on the subject personally, that suits me fine too...

On Forum’s one can be brutally honest about your private thoughts.
The anonymity it provides allows one to speak freely and that is a key ingredient, to talk...

Research on MF, there is plenty of it out there on various papers etc. Steve is the expert on what is going on in the world of MPN’s etc..
Professor Claire Harrison at Guy’s London is a world respected authority on all things MPN..
Ruben Messa is another that publishes papers on research into MPN’s...

At the end of the day, it is down to us the patient to navigate a way through the early day’s and live in relative harmony with MF...

When PV was diagnosed last year, I took it on the chin as such, until the day I had my first Venesection, I sat there and looked at the Blood being taken off & cried with a former colleague..
It didn’t hurt whatsoever, it simply made me sad.
I have only had two (2) Venesections last year, once I commenced Rux that issue hasn’t happened since...

If & when then I will deal with it not before, there is zero point in giving it air..

So Jacqui, I hope you can get yourself settled with your diagnosis, go live by the Sea, Enjoy Life as much as you possibly can, when you can..
I live on the coast and love walking by the sea Summer & Winter...

It’s coming up to 5am with you, have a lovely day as best you can, cheer up & look ahead to a fresh start on the coast..

I am currently staying with my daughter, we Sold our home late March this year, and have just purchased a Bungalow..

All I will say on that subject is , pace yourself with packing etc, I was totally absolutely exhausted and was horizontal for several days after the move.

But it’s exciting looking for a new home & in a sense a new chapter. 🥳

Stay Well & Safe Looking Forward....
All the Very Best to You
Maria

you are the loveliest, Maria!
Thank you so much for your kind words of wisdom and your generosity, that was a long message! All that time and effort, just know your sharing has not gone unappreciated, I am quite blown away.
It’s been a good day I got to read your message in full, it gladdened my heart and that bizzare fatigue that kept me horizontal for 8 full days seems to have vanished! I’m sure it’ll be back sometime but just to know there’s respite from it and that at times it wont be as severe, I like to think, gives me hope. Its also comforting, in a way, to hear how we are all in that tired boat...sailing the MPN waves together .

I have more to say but I’ve done my dash for today. My heartfelt thanks for being here for me, Maria. Big hug, Jaqi

[Merry]

Hi Jacqui
Thank you so much for your reply. Sorry for my delay in replying -to you, it’s been a busy few weeks!
In answer to your query, I turned 57 a few days ago and was diagnosed at 50.
I agree with everything you have said and have only really not chased up a BMB due to all the other health issues I have been experiencing since entering my 50s. Kidney cancer and a nephrectomy, heavy bleeding and an endometrial ablation, breast biopsy, gallbladder pain and cholecystectomy, ongoing Graves’ disease and recently diagnosed Psoriatic Arthritis. These have all pushed to the fore following my blood clot episode so I’ve really concentrated on sorting out those problems and the ET has taken a back seat - luckily it’s been keeping fairly quiet back there!
I believe I have not been offered a BMB due to dealing with the public health system and also the lack of any major change in my blood test results. I think my next step is to search out a haematologist who is prepared to do it. There is only have one private haem where I live so I will probably see her, as my only option after that is to travel to Perth and as I still work full time, this usually means taking a whole day off.
Must away now to enjoy Mother’s Day breakfast! All the best and thank you so much for sharing and caring. Hugs, Merry

[Jaqi]

Hi Jaqi
I'm pleased to see that you've received reassuring advice from Steve and Maria who both have a similar diagnosis to you, and I don't think I can add anything further as I still at the ET stage.
I was diagnosed with JAK neg ET back in 2006 following increasing platelets levels and a BMB. CALR mutation was confirmed a couple of years ago. I was on Hydrea for several years but this gave me numerous skin cancers and eventually stopped controlling my platelets. I started on Pegasys about 18m ago. Platelet control is improved but I have a number of side effects.
One thing I've learned from this and other support groups is that everyone seems to have a different experience of their MPN and their treatments. What works for one will not necessarily work for another. It is also important to keep up to date with developments (there's much more research going on in this area than their used to be) and it is good to question/challenge your doctor. It is good that you have found a specialist that you have confidence in and keeps up with the latest studies. Hope your test results allow the best course of treatment for you to be identified.
Best wishes
Allan

Hi, nice to meet you and thanks for that, Allan, yes, I can see how it affects everyone differently now, I am actually unclassifiable, but its not ET or PV, go figure…. actually it’s just the criteria, I have all the major but not any of the minor but it seems like I’ll have no trouble being prescribed Ruxolitinib for my MF. Whether I take it is another story.
Glad to hear you are only at the ET stage wishing you all the best and that you stay there.

[Jaqi]

Hi Jacqui
Thank you so much for your reply. Sorry for my delay in replying -to you, it’s been a busy few weeks!
In answer to your query, I turned 57 a few days ago and was diagnosed at 50.
I agree with everything you have said and have only really not chased up a BMB due to all the other health issues I have been experiencing since entering my 50s. Kidney cancer and a nephrectomy, heavy bleeding and an endometrial ablation, breast biopsy, gallbladder pain and cholecystectomy, ongoing Graves’ disease and recently diagnosed Psoriatic Arthritis. These have all pushed to the fore following my blood clot episode so I’ve really concentrated on sorting out those problems and the ET has taken a back seat - luckily it’s been keeping fairly quiet back there!
I believe I have not been offered a BMB due to dealing with the public health system and also the lack of any major change in my blood test results. I think my next step is to search out a haematologist who is prepared to do it. There is only have one private haem where I live so I will probably see her, as my only option after that is to travel to Perth and as I still work full time, this usually means taking a whole day off.
Must away now to enjoy Mother’s Day breakfast! All the best and thank you so much for sharing and caring. Hugs, Merry

Oh, wow, I cant believe the harsh hand you’ve been dealt, Merry, so sorry to hear. So many challenges! And to take out the time to reply and be lovely and even cheerful, or should I say ‘merry’, is pretty amazing…you must be amazing!
Wishing you a happy belated birthday with many more to come and hope you had a lovely Mother’s day.
All the very best and good luck with everything, Merry. big hug