Update to Specialist 1st visit

[Cmg5935]

Hi everyone,

Thank you for the support and information shared to my original post.

As promised, I’m sharing what was explained to me by my referring hematologist regarding the letter sent her by the specialist.

This is my experience and I want to be careful to not alarm any other forum members. This is one dr opinion and I would of course seek others to verify

The portion of the letter that was a surprise is quoted here. “ Triple-negative patients also displayed inferior LFS (P¼ 0.003). The current study identifies ‘CALR- ASXL1þ ’ and ‘triple-negative’ as high-risk molecular signatures in PMF. Leukemia (2014) 28, 1472-1477; doi:10.1038/leu.2014.

We sent blood for more detailed genetic profiling today and I will see her back in four weeks. Depending upon the results of the genetic sequencing, we will decide when to repeat the bone marrow.

At this appointment, I did not bring up the question of a transplant. I would rather wait and see what other mutations we find before addressing this issue.

She will be back in four weeks and she is to continue with the same care as before until then. Thank you for allowing me to consult on this delightful patient. Needless to say, I will do everything possible to help.

Sincerely,”

Granted she did call me delightful 😅, but our appt never touched on any possible need for a transplant. So caught off guard I needed some assurances.

My hematologist said that as a triple negative and should there be no other obscure genetic mutations to explain my ET & MF I would be a candidate for a bone marrow transplant, because my odds of developing PMF Leukemia are much higher. (like a woman with a positive BRACA and family history can opt for a double mastectomy to prevent getting breast ca. I could have a marrow transplant.)

I’m still digesting this information, but am hopeful an explanation will be found for my ET so transplant would not be needed.

Sorry for long post. ( to be continued after next appt October 16)

Christina

PS: THIS IS 1 DR. OPINION SO I WOULD GET ANOTHER BEFORE EVER CONSIDERING A TRANSPLANT.

[Dianne-Guisborough]

Thank you for letting us know Christina. As you say, alot to take in and also you will not, nor have to rush to make any decisions just yet . On the positive she seems like a very pro-active haematologist. My friend's Aunty had PV which after 10 years progressed to myelofibrosis . Last Xmas she underwent a bone marrow transplant and has done fantastically .

I hope you are keeping and feeling well and sending you e - hugs across the pond .

Love Dianne

[Cmg5935 in reply to Dianne-Guisborough]

Thanks Dianne,

I’m trying to remind myself that nothing has to be decided until all my results are in. Can’t believe I’m praying they show a rare mutation 😅.

My other very palpable fear is our current government. They are trying very hard to take away our health coverage. If successful I now have a pre existing condition that would make my insurance so $$$$ that I wouldn’t have any. And that thought is terrifying considering the constant monitoring and potential treatment my ET/MF will require.

Are you folks accepting American refugees??? Lol.

Thanks again for reaching out with your support !

Christina

[Dianne-Guisborough in reply to Cmg5935]

Oh Christina

That is awful - it is hard enough having to come to terms with and cope with your diagnosis and a complicated one at that without the extra stress of worrying about financing your monitoring , treatment and managent ! I know that here in the UK some people moan about our NHS but we definitely never should.

We pay our taxes and a small national insurance contribution and then receive free care , yes we pay for prescriptions but even then, if you have a cancer diagnosis and some other long term conditions you are exempt from payment.

Is there any chance if you need certain drugs that your Haematologist could apply to the drug company direct to see if they would sponsor the drugs for you in a compassionate grounds basis. I dont want to talk about me, this is about you Christina, but I have PV and my haematologist tried me on anagrelide as I cannot have hydtoxyurea due yo history of melanoma , but it didn't work then I had interferon injections which started to reduce my blood counts and haematacrit but I felt as though I had flu all the time I was on it. I was still needing weekly venesections to kerp my Haematacrit within target range . Unbeknownst to me smy Haem wtote to Novartis who produce Jakavi ( ruxolitinib) and they have agreed to provide me this drug indefinitely on what is defined as compassionate grounds . It is when you have tried the standard drugs but they dont work or you cannot tolerate them or they are contraindicated and you canmot get them funded via the NHS . In this case Rux is licenced in the UK for myelofibrosis but not for PV Recent trials are showing promising results with Rux for PV hence they have agrred to provide it indefinitely for as long as it is helping me or it gets NHS funding approval in the UK.

I was just wondering can you discuss your concerns re the future funding of your treatment witj yoir Haem and see if there is a route similar to mine through the drug companies direct albeit for different reasons they are equally valid .

As we say here in Yorkshire "Shy bairns get nowt!" So it is always worth asking the questions .

Sorry I have been so long winded but I really feel for you and want to wish you all best for you and your health. In the meantime be viligent with your health , drink plenty of water , kerp as active as you can and rest when you need to.

Keep Strong xx

With love

Dianne xx

[Cmg5935 in reply to Dianne-Guisborough]

Dianne, I do appreciate the suggestion. Should it come to that I absolutely will have my haem directly contact the drug companies. Fortunately, for now they still have me on aspirin therapy. Though labs every 6 wks and bmb a year would be incredibly costly without proper insurance.

Our government is voting this week. It seems they are more concerned with a campaign promise to repeal than properly fix our health system. 85% of our population doesn’t want the plan they are voting on.

Believe it or not, preexisiting would include things such as asthma, post partum depression etc... A breast ca pt would pay estimate of $141,000 a year for coverage. We don’t have those types of income here for average Americans.

Now I’m going long. It’s just so scary to think that a bunch of racist old men are deciding our fate.

I don’t believe this law will pass but the fact it could is disturbing and unnecessary stress as you said.

Christina xo

[Dianne-Guisborough in reply to Cmg5935]

Fingers and toes crossed that your new President doesn't get his way . He doesn't seem to live in the real world xxx

[mhos61]

Thanks Christina for the promised update. Feel like I'm on this journey with you!

Wow! I can see why you needed the assurances. It's unbelievable - but not surprising that you were left to make sense of the 'said quote' without any former discussion of its content at the consultation. You appear quite a level headed person; I dread to think how someone with a nervous disposition would have reacted?

On the other hand, it does sound like you have an excellent medical team at the cutting edge in their field; so you should take some comfort from this. It's a real shame on top of all this you have the additional worry of health coverage being threatened too.

I really hope your October appointment is as favourable as can be.

Mary xx

[Cmg5935 in reply to mhos61]

Mary it is an honor to have you involved and advising me along the way. I am so relieved & happy to have found this forum. We are all on our personal journeys, but united.

I was surprised when the specialist felt that my at times extreme exhaustion shouldn’t be due to the condition. Every post I’ve read makes mention of it as do some websites about ET/MF.

I’m drinking plenty of water and trying to up my exercise as well as eating better, but still have days where I can only sleep. I’m most definitely not depressed. I know what that feels like and this Isn’t that.

I’ll post when the vote is in on our healthcare. Disgusting what they are trying to do in order to save $ and offer the wealthy donors tax breaks. I love ❤️ my country but am embarrassed and horrified by what is happening here.

Thanks for following along with me as I transverse this new path I’m on.

Christina 💕

[mhos61]

Always here to support you and others in any way I can.

Yes, this is a great forum. I only found it by accident in perusing the internet for any information on MPNs, so glad I found it.

It's bewildering and annoying how some of the health experts don't recognise the dreaded fatigue as part of the MPNs. We can't all be wrong!

Really hope the healthcare vote goes in the favour of the 84%. 🙏

Take Care

Mary 💕

[Bridie123 in reply to mhos61]

Hi Mary, I'm coming in here on the fatigue issue. My husband and I are going to the conference in London in November and I am going to ask those specialists why we all have it and why it's denied that it's anything to do with our mpns! I don't know what is worse, having fatigue or being told it's nothing to do with the disease or medication! I think the latter because it adds another burden. Regards Carole.

[Cmg5935 in reply to Bridie123]

Carole, I cannot wait to hear their response. You are so right that it is frustrating to at times have debilitating exhaustion and be told it must be due to something else. I know it’s not. Just as Mary stated. How can it not be real when everyone of us experiences it.

Glad you’re taking up the flag in this battle for us!

Christina

[mhos61 in reply to Bridie123]

Well said Carole. Look forward to their response.

Mary x

[Cmg5935 in reply to mhos61]

Morning☀️ Mary, good news on the American healthcare front. With the 84% actively protesting the proposed bill was voted down for a 3rd time. Unfortunately each time our governing party revisits healthcare it ratchets up everyone’s anxiety.

For now at least, I and millions of Americans that would have lost coverage are safe.

Christina

[mhos61 in reply to Cmg5935]

Great news! I did hear this on the news and thought of you.

So pleased for all of you Americans out there who relied on the vote going this way.

Mary ❤️🌷☘️💐

[Bridie123]

Hi Christina, you sound calm and controlled, easy to portray on a post. I hope with all my heart that you can really be that way particularly while your waiting. In the meantime keep ☺🤗😄🙂☺xxx Carole.

[Cmg5935 in reply to Bridie123]

Thanks Carole, I suppose I am rather circumspect. Not to say I’m not anxious, but I’ve learned to compartmentalize over the past 5 yrs. I believe it was in a reply to my 1st post “ New Here” that I shared my recent family history. I’ve lost a sister to a rare heart condition, had my younger sister dx with stage IV breast cancer with a 2-5 yr prognosis and have helped my older sister with her breast ca dx over the past yr from chemo to reconstructive surgery. I also handle all matters for my bipolar 86 yr old Father. So it takes a lot to rattle me at this point. Lol

In some ways my dx has been the easiest of all because it’s just mine. I’m solely responsible for me and my decisions. In somehow less stressful than being responsible for others. I actually feel fortunate that though not curable, ET/MF is treatable. And should I be told a bone marrow transplant would prevent subsequent PMF leukemia it will rock me but I’ll tackle it head on.

Curve balls are nothing new. And the light at the end of that tunnel would be a cure.

So as you said, I will try and stay positive and with you and everyone here for support I will share the good & the tough days and my support for others on our forum

Christina xx

[cwayman11]

Big hugs xxx

[mjfin72]

Hi Christina. My goodness what a lot to take in for you in such a short space of time. Really hope these tests show up with something that explains why you have Et. I'm sure the next few weeks waiting will be tough but you know you always have this forum and a virtual ear if you need to offload.

Really hope you get the news you want and that a transplant isn't necessary.

Take care

Mandy x

[ChelseaF]

I'm curious what the biopsy showed and what things point to PMF rather than just ET? I'm very curious about this because my own biopsy showed some mild fibrosis (it did not give a number, just said mild). I still have no diagnosis other than two hematologists believing that I have ET though they can't officially diagnose me as the biopsy could not confirm it and I have no mutations.

I hope your further testing gives you answers. It's no fun waiting and feeling uncertain.

[Cmg5935 in reply to ChelseaF]

Hi Chelsea,

Check out: online.epocrates.com/diseas...

I also was told I have MF mild. It was explained to me that this was determined by the bone marrow biopsy slides. They could see changes to the marrow Structure. The stage is determined by what is noted as the marrow becomes more fibrous.

The specialist did confirm my diagnosis, but as I explained, absent the commonly seen mutations she is doing a full genetic panel in search of a more obscure mutation to explain why I have ET&MF. Once determined she’ll give me treatment recommendations.

It really is frustrating having such a rare condition as even some haems are not equally knowledgeable as the next. I suppose it a matter of finding the right haem and as patients being proactive & advocating for ourselves. That’s why I’ve found this forum so incredibly helpful.

Hope I helped a bit.

Christina

[ChelseaF in reply to Cmg5935]

Thanks. I am curious to hear what you find out. MF does concern me a bit since I do have some fibrosis and no found mutations. I don't meet any diagnostic guidelines but my hems think it's ET still.

Please let us know when you get the new test results back & best of luck to you!

Chelsea

[Cmg5935 in reply to ChelseaF]

Definitely will keep you and everyone informed as I get more information.

I was told the MF can be caused by the ET. So it’s a secondary condition, and verifiable by the bmb results.

Christina

[Lainy1]

Hi Christina, i can not say anything that has not been said by other people. Sending you great big e-hugs 💖💚💖💚💖💚💖💚💖💚💖 will be thinking of you.

Lainy xx

[Cmg5935 in reply to Lainy1]

E-hugs are much appreciated and a very welcome! Thanks Lainy ❣️