New member - Post ET MF (Brisbane)

[Hatchie]

I'm 42, married with six children.

In 2013, I was diagnosed with CAL-R positive ET. I started taking aspirin and managed pretty well.

After a spontaneous bleed in my right quadricep, it was discovered that I also had a genetic bleeding disorder, dysfibrinogenemia.

After that, I stopped the aspirin and was just watchful and waiting.

In 2017, after recurring migraines and burning in my feet, I started on pegylated interferon. It worked well and lowered my platelets.

In early 2022, after an increase in my platelets and symptoms (extreme fatigue, 15kg lean body weight loss, night sweats, bone pain, and abdominal pain), a repeat marrow test on August 18, 2022, identified Post ET myelofibrosis. Genetics were CAL-R type 2 with ASXL1.

I started Jakavi in September 2023, and due to poor response, I was also put on Hydrea and Pegasys to slow disease progression.

After my platelets dropped below 1000, I stopped the Pegasys.

My doctors have recommended that I start thinking about a stem cell transplant (SCT); however, the best donor they can find is a 9/10 unrelated donor.

I'm currently participating in the INCA033989 CAL-R antibody trial but also on Hydrea to try to control my platelets. My platelets are still around 1.8 million.

Symptom-wise, I still struggle with fatigue, constant nausea and abdominal discomfort, brain fog, and am battling to maintain my weight and trying not to lose any more muscle.

[MPN-MATE Admin]

Afternoon Craig...

Glad you found your way here okay...

Happy you've also managed to Post your first 'POST' ...

Just while talking ALL things 'POST'. I am including a small Picture below of the FORUM & your current Post, to highlight the "SUBSCRIBE" to TOPIC Radio Button, pls see pic below:

Craigs POST May 2024.png (155.48 KiB) Viewed 8045 times

You'll need to *Note that whenever you make a Post, pls 'Tick' the "SUBSCRIBE" Radio Button to be notified of ALL replies to your Post, okay? Hope all of that makes sense... If not pls do let me know...

Interesting, that you and I also share the HRM 'ASXL1'. Apart from our age, our illness seems to be very similar, (at least in the diagnostic sense).

Craig, may I ask why you stopped the Pegasys'? I mean, if your Platelets were dropping, that is the desired result is it not?

My go-to for 'Platelet Dropping' was Methotrexate (MTX), it's similar to HU but not quite as harsh, (well that was my experience in any event).

However, these days, I am utilising Nitric Oxide (NO), as naturally derived from slightly cooking Raw Beets, and creating a nectar style drink, that I take every day...

In my case, my very poor circulation is exacerbated by my (acquired VWS), that I mentioned to you earlier in my email. Nitric Oxide (NO), has the effect of opening one's vascular system (veins & arteries), providing me with stronger blood flow; and better energy, as a direct result of greater oxygenation of blood cells too... It really works!

I have been using this for some years now, and it works for me...

Not 100% sure that it's also keeping my Platelets lower, but it seems to be at the present ... (fingers crossed

Craig, our FORUM can be a tad slow in receiving replies at times... However, I am hopeful that you will manage to have a chat with a few of our members, and in particular, those who might share some of your experiences...

Anita, (who resides in Melbourne), has also been involved in the CALR Trials like yourself. It would be interesting to share what you have both learned from participating in those Trials, should you so desire...

Warwick, also went down the Allogenic Stem Cell Transplant (ASCT) path recently. He still has some struggles... However, he has a wealth of knowledge concerning that procedure 2+ years Post the event...

Craig, you have my number, should you wish to chat further...

Best wishes & welcome to MATES MPN Patient's Forum

Steve
MPN-MATE RF
Admin

[Hatchie]

Thanks for the warm welcome, Steve.

The reason the Pegasus was stopped is because I had neutropenia (too few neutrophils), along with feeling lightheaded and nearly passing out twice. It was hoped that rux and hydrea would be sufficient.

I find everyone's stories very fascinating. However, as you all know, while we may share the same disease, we each have unique journeys and respond differently to treatments.

Understanding the prognosis and disease progression is challenging, and even with various prognostic calculators, outcomes can vary. It's intriguing how people identify different indicators to monitor their own progression. For instance, in my first BMB, my marrow was grade 2, but recently, two samples collected on the same day showed one at grade 1 and the other at grade 2.

I'm young to have MF, which is why I'm considering when to undergo a transplant. My youngest is in grade 3—will I see her finish high school without a transplant? No one knows for sure. I have no comorbidities and am "fit and healthy," so theoretically, that should be beneficial for a transplant. If I were 20 years older, I don't think I would consider a transplant. It's a cruel disease, and half the battle is dealing with all the uncertainties.

[MPN-MATE Admin]

Evening again Craig,

Yes, you are speaking with the wisdom & knowledge of one who has been on this journey for a while...

Indeed, it is quite remarkable that many MPN patients can share such similar conditions, and yet react & respond in the most diverse trajectories, in my view...

Warwick is a tad younger, however had he not taken the ASCT adventure, he might not be here at all right now... (?)

It would be good for you to talk with Warwick if you are seriously considering the ASCT path... There is another girl Kathy, who has also gone down the ASCT road. However, we do not hear too much from her these days...

In the UK, Chris Harper, is one of the rare miracle cases of ASCT. He also did not have a 10/10 donor. And although it's generally advised that male patients should not take Stem Cells from a female donor, Chris did... And his success story is one that is nothing short of astounding! No levels of the JAK2 mutation exist in him any longer, he has no symptoms and back into his exercise regimes in a big way...

Chris turns up at our meetings, from time to time, (especially when he knows that someone is seeking ASCT advice...) I will ask him to attend our next Meeting if you like, (& he's available of course). In the event that he is not, I'll ask him if it would be okay for you to write to him via email, if you wish(?)

You may want to give Rux' & MTX a try too... It works quite well, and I found that I could regulate the taking of the Methotrexate (MTX) far more easily that having to take HU every day. It was working for me... However, I wanted to find a better substitute, and thus far my Raw Beetroot concoctions appear to be of great benefit to me.

It is a real science, (not hocus pocus). Nitric Oxide (NO) was originally discovered by a tripartite of Three (3) Nobel Prize winners. Prof. Lou Ignarro has written volumes on the subject. Quite a fascinating read if your interested, just Google Lou Ignarro & Nitric Oxide (NO) to learn more...

Anyways, I will send out a Notice to a few members to lookm in on you shortly, & provide you with some responses for your Post. They are a td tardy at times... but tell them I sadi so...

Best wishes Craig

Steve

PS. I have also added a Picture for your Avatar, hope its ok? You can choose from one of the others if you prefer...

[Warwick]

Gday Craig.

Sorry you are in this boat with us.

I had a stem cell transplant in 2021 after having ET since 2003. I'm 51 and have 3 kids. I feel your pain with the bone pain it was crippling for me in 2019 I had recurring bouts of it to the extent my core muscles would completely lock up and I could hardly breath and couldn't walk then it would gradually wain and I could walk again and by the next day I could play soccer again.

The Ruxalitinib stopped the bone pain about 4 months after taking it. I was on 2000mg of Hydroxurea for about 6 years. Tried interferon but it didn't agree with me.

So much to say about the transplant process and more than happy for you to give a call, pls just ask Steve to email you my contact details...

Best wishes

Warwick

[AnnBee]

Hello Craig
Anita here. Sorry you have to join us. I was diagnosed with ET in around 2001. CALR2 TET2 I am now 67.
Used Hydrea on and off ( mostly off) with platelets upto over 1 million with nil events. Transformed into Post ET MF though that is not clear and became Hydrea resistant ( too many side effects) I’m presently on Pegasys with rising White Cells and Blasts upto 6% and developing under active thyroid from Pegasys. I am not interested in a SCT as would not cope with any side effects. This is all such a personal road we tread but we have the facility here to share and support each other in a way others who do not have these conditions can. Please ask me anything at all and I look forward to hearing how your journey unfolds Welcome.

[Hatchie]

Hi Warwick, nice to meet you. As tough as it is, I'm relieved to hear that your experience with bone pain mirrors mine. I used to get it in my legs at night, so severe that I had to crawl to the toilet, only to find it gone by morning and back at the gym. Often, when I share this with others, even doctors, they look at me in disbelief.

I'd love to hear about your experience with the BMT.

Thanks a lot for reaching out.

Craig

[Kinsale]

Hi Craig
I’m Mark I’m 66 and want to welcome you to our wonderful group. I was diagnosed with ET Jak 2+ in 2014 and in 2016 was put on Hydrea after a brain scan revealed some ischemic changes in my brain. Hydrea wasn’t a good fit for me and in October of 2018, was able to swap to Pegasys when it was put on the PBS. In short Craig, Pegasys has been brilliant for me. Almost 6 years on and I’m injecting every 6 weeks and have recently been dropped from 90 micrograms to 67 micrograms. All my blood test results are in range with my platelets stable at 306 my Allele Burdon is 5.9%. So I’m very lucky that Pegasys has worked so well for me.

Likewise this wonderful supportive group supports me and allows me to tap into a group of people who understand the daily challenges that living with an MPN.

Cheers

Mark

[Merry]

Hi Craig. I’m so glad you found your way to us. Lots of lovely people with helpful advice here to assist you. I was diagnosed with ET JAK2 in 2015 and at present am coping quite well on Apixaban (blood thinner) and CBD oil. I can see that you have had some great advice from ppl who identify more closely with your MPN journey so I will just say that you are more than welcome to join our 6 weekly online Cafe catch-up Teams meetings where we get together and have a chat. I find these meetings really helpful as I am in WA so quite isolated from MPN specialists etc. All the best, Merry

[Hatchie]

Hi Merry, Mark, and Anita,

Thank you all for the warm welcome and sharing your experiences with me. It's reassuring to connect with others who understand the challenges of living with MPNs.

Merry, your suggestion about the online Cafe catch-up Teams meetings sounds wonderful—I'll definitely look into joining. Mark, I'm glad to hear that Pegasys has been such a positive treatment for you over the years. Anita, your journey and insights are also valuable, especially navigating through different medications and their effects.

I appreciate the support and willingness to share knowledge. I'm still early in my MF journey, but having this community means a lot. Looking forward to learning more from all of you.

Best regards, Craig

[sydunipete]

Welcome Craig,

I was diagnosed with ET over 11 years ago at age 50. Later that was classified as CALR+ and I was on hydroxy for about 10 years gradually increasing to 4 tablets per day. I recently changed haematologists and received my first Bone Marrow Biopsy. It appears I may never have had ET, but I'm an unclassifiable MF with CALR and single-HIT TP53.

I'm being referred to a SCT specialist (I'm now approaching 62 years of age) and am grappling with all the decision making processes that go with that.

It's a difficult journey and it affects all of us in different ways. Part of the challenge is finding information about our rare conditions while avoiding much of the noise on social media. It's all quite triggering and anxiety is a natural part of what we're going through. I hope MPN Mates can help you with you journey and I wish you nothing but successful outcomes.

[MPN-MATE Admin]

G'day Pete,

Just thought that you should possibly have a chat w/ Warwick at some stage, (especially so if considering going down the ASCT path). Warwick is now 2+Post ASCT, and I am sure that he would be happy to discuss in greater detail some of that which his experiences have taught him.

Personally, I implore other members to have those discussions here on MATES, so that all of us MPNers, might gain some future knowledge that might aid each of us in different ways. Anyways, Warwick should be at our next Cafe Catchup meeting this coming Sunday evening between 6-8PM. Here are those details again:

MATES MEETING DETAILS:

WHERE: ONLINE – MICROSOFT TEAMS

WHEN: SUNDAY JUNE 16

TIME: FROM 6 – 8PM AEST

MEETING LINK:

https://teams.live.com/meet/95392473204 ... RUtKRH3x2V


Naturally, all of us are just a tad different from one another, and yet their are also always commonalities too...

For example, I am also CALR(2), and now Post ET / MF being treated by Ruxolitinib, and other complications include a High Risk Mutation (ASXL1 n– designated as indicating a poorer prognosis), and Von Willebrands Syndrome (VWS), which makes my blood extremely sticky at times, which could also lead to further TIAs, (minor brain strokes – had X3 thus far).

Anyways, I seem to be able to manage my condition reasonably well through my anti-inflammatory diet & exercise (cycling) regime... (On hold for the moment while I recover etc)
Nevertheless, my preference at 65yo, is to continue on this path, as for me the ASCT path might prove more problematic and deduct from my current 'Quality of Life'. It might not be perfect, however, there are many more people out there far worse off than myself...

The Human body is such a fascinating thing, and I believe I must keeping pushing myself across those invisible boundaries to live and continue to experience the best life possible for me...

Anyways, hope to catchup again soon, perhaps this Sunday if you're available (?)

Best wishes

Steve

[sydunipete]

Thanks Steve, I’m currently in Sweden so due to timezones etc I’m unable to join the online catch up but I wish you all the best for the event.

The suggestion for me to consider the ASCT came out of the blue following my BMB. I’m 62 and am yet to come to terms with exactly the implications of it all. And I’m still feeling well. I’ve reduced my hydroxy dosage (to 3 tabs / day) during this current trip and that seems to have relieved my itching. The plan is to commence Rux when I return in September. At that stage I’ll be referred to a transplant specialist and we’ll see what happens from there.

I battled a bit of anxiety following those last results but things are on a more even keel at the moment. Let’s see what September brings.

[MPN-MATE Admin]

Hey Pete,

Great to hear from you again... Hope you're enjoying Sweden too...

Pete, everyone's MPN journeys are similar yet quite different for each of us of course...

Not sure that I ever mentioned any of what I am about to share with... Prior to my diagnosis (Dx) in 2016. I had recently completed a BA in the Humanities, and then commenced another in Communications w/ majors in PR & Journalism. In my past of attempted a couple of documentaries of my own, and always felt draw to writing really...

In any event, prior to my DX in 2015, I had my first TIA (minor brain stroke). Caused some paralysis in both my right side and my ability to speak and see clearly. It was all over within less than a 5 minute period. However, I shall never forget the sensation of being locked inside this vehicle that is "Me" and yet seemingly have lost almost entire control of my ability to motion and or communicate effectively. It was quite an epiphany of sorts...

Shortly after that event, my father was to pass away, and I wrongly attributed all the ramifications of those associated events as to the validity of why I somehow had lost my ability to study effectively. Eventually, I decided to take a break from my studies... I simply found it impossible to keep my eyes open while trying to read, it continued to become progressively more intense. However, I erroneously thought it was just all that intense study catching up with me etc... Wrong!

During those days I was still applying for a Position in SE Asia working as a Volunteer in International Aid / Development. When I finally landed a role, I could barely believe it... All my Xmas presents seem to have finally arrived. It would've been an understatement to suggest that I was elated, I was over the moon with joy!

On that fateful Friday morning, I was involved taking my aged / immobile mother to another one of her many medical consultations, as I was required to lodge a final formamedical check prio to my departure on the following Tuesday. When I would've been flown to Adelaide for orientation before departing for my first assignment...

... On the following Sunday evening circa 20:30ish... I received a call from the cheif medical officer advising me of some inconsistencies in my blood work. Then my MPN journey first commenced with me trying every which way to propagate some other valid logic for my blood work... Denial would soon set in.

After more extensive testing, my mood became slowly more despondent as I gradually learned to accept my illness... I believe it's fair to say that my senses were greatly disturbed for the next 12 mths... However, shortly after all of that, I did the "Taylor Swift" thing and found a way to "Shake it all off!"

Shortly thereafter, I strated building our website FORUM, which later became our registered Australian Health Promotion charity...

Pete, like you, I am also in my 60s (65), however, and in my case, I have shown myself that my anti-inflammatory diet, and my intense cycling exercise regime most successfully aided me in managing my MPN well... Along with the assistance of Ruxolitinib...

As you might recall, I am also POST ET / MF, w/ (CALR (2) & HRM ASXL1+, along w/ acquired Type 2 Von Willebrands Syndrome. In my case, I am still happy with the "Quality of Life" that I am still able to maintain today, nevertheless, I was recently asked to consider another BMB to see if my condition might be progressing(?)

After some further thought on the matter, I decided against going down that path knowing what's involved in doing so... However, some people like our own Warwick, most likely would no longer be with us today had he not undergone the ASCT procedure.

Another few of members, (Anita, Wendy & now Craig) are also contemplating the ASCT path...

Just remember, if you are truly considering that course, (ASCT), throw a few questions up to Warwick as he is most knowledgeable in that regard...

BTW, not sure if you've seen my recent Post concerning my accident, still on crutches atm. But I am recovering well...

Stay in touch Pete...

Best wishes

Steve

PS. Link to Accident POST. – viewtopic.php?t=2856