Newly diagnosed Et Edinburgh

[Green1988]

Hi is anyone from Edinburgh that has been diagnosed with Essential Thrombocythaemia Calr mutation?

[Batman66]

Not from Edinburgh not too far though, I'm in Stirling.

[Green1988 in reply to Batman66]

Thank you for your reply hope you are well?

[dabs121]

Hi, I'm just south of Edinburgh, in the Borders. ET with Jak2. Welcome to the club. I have found this site excellent and the information from others helps answer those many, many questions we may have that you don't have time or the inclination to ask your Dr's.

[Green1988 in reply to dabs121]

Thanks for our reply. That’s good to hear

[Kizzy03]

Hi yes! I’m in Edinburgh and have had ET with Calr mutation for several years. Sorry I can never remember how many years!!? Anyway! I was only on 75mg of Asprin but because I’m now 60 I’ve desired to take the Hydroxycarbamide which was offered. I attend the Western General and did have Dr Teh but he’s since moved on, but I do feel I’ve still been given good advice from the other Doctors I’ve seen and both Specialist Nurses I’ve had. Ive also had very good information being on this forum. To be honest I’ve never really had any problems with anything and still lead a normal life, but if you have any questions I’m happy to help if I can. 😊

[Green1988 in reply to Kizzy03]

Thank you for your reply. We are really struggling just now my husband got diagnosed 3 weeks ago and we were not expecting this. It’s just so difficult to get my round this.I feel my everything has just flipped upside down. We have an appointment at Spire next week. So hopefully get to ask more questions. I can see it upsets my husband he got a bruise on his arm yesterday and he was really upset cause these symptoms are all new. He’s 35 .

[Smudger0122 in reply to Green1988]

I can completely relate to you, i too am still struggling with the diagnosis of this. But time is making it easier, plenty of support on here.I just take my medication and get on with daily life, yes blood tests will become frequent. But just think of them as like popping to the dentist for a check up.

I would asvise not to google to much, like i did!

Ask any questions on here, so much support and knowledge on this forum.

With regards to his bruise, he may of just banged his arm at some point.

Keep positive

Smudger

[Green1988 in reply to Smudger0122]

Yes I definitely think time will make it easier. I couldn’t eat for the first week when we found out I literally sat on my phone all day and waking up through the night sitting on Google joining forums. I don’t obviously show it in front of my husband I try to be positive but deep down it’s hard.

[Scottishterrier in reply to Kizzy03]

Hi kizzyI have had et jak2+ since 1994 I am on Hu once a week I have so many consultants Dr Farquharson was brilliant I to was under Jean who was my nurse specialist and also to was under Dr Teh I have met my new consultant Dr konstavalous he was really nice my only blip in the road was three clots to liver so under a great gi consultant for many years and the WGH dental unit so they have results on file

Stay safe

Scottish terrier xxx

[Smudger0122]

Hi Green1988

I am male 45 and was diagnosed with Essential Thrombocythemia Calr last year, and confirmed diagnosis march this year with Bone Marrow biopsy.

I am on hydroxycarbamide and clopidogrel.

I don't live in Edinburgh, but happy to answer any questions that you may have.

If I am unable to, plenty of experts on here that will.

Keep safe

Smudger

[Green1988 in reply to Smudger0122]

Thank you for your reply. If you don’t mind me asking why did they start you on medication I thought it was when you turn 60?

[Smudger0122 in reply to Green1988]

My platelet count reached 1665 march this year.Hydroxycarbamide was used to bring it down quickly, but looking at going onto interferon in the future advised by Guy's hospital.

[Green1988 in reply to Smudger0122]

I see. We are seeing a private Haematologist next week. I hear Claire Harrison is good and that we should see an MPN specialist as to a haematologist?

[Smudger0122 in reply to Green1988]

Yes, i am now under Claire Harrison at Guys. They will be keeping an eye on me as we go forward.They oversee my care, and keep in contact with my local haematologist team.

[Green1988 in reply to Smudger0122]

That’s good to hear. Stay safe and thank you for answering my questions 🙏🏾

[Green1988 in reply to Smudger0122]

Also we have not been offered a Bone Marrow Biopsy as the doctor said nowadays blood testing is advanced?

[Smudger0122 in reply to Green1988]

I was given bmb as confirmation of diagnosis.My blood swipe was taken back in June last year, when i was in hospital for brain surgery and platelets were to high for safe surgery. This was back then confirmed as ET Calr, but bmb was down for a 100% confirmation.

[Smudger0122]

Remain positive, chin up all will be ok.Keep in touch.

[Green1988 in reply to Smudger0122]

💯💯 thank you will do.

[Scottishterrier]

Hi I am from Edinburgh I have had et jak2+since 1994 I am on Hu once a week due to blood clot to the liver I attend the wgh also to be extra careful they also referred me to dental unit so they have my details on file I have had many consultants due to them retiring and sadly one she died of cancer I can safely say in all these years never had a bad one they have been fantastic I have only met my new one once he is Greek also the secretary is great you have a problem it gets passed on and a phone call straight back you are under safe hands if you are attending the WGHStay safe

Scottish terrier

[Green1988 in reply to Scottishterrier]

Thanks for your reply. Yes it’s the Western we go too we have only seen consultant once and she is moving next month so not sure who we will get. That’s reassuring to hear they are efficient. I’m just really struggling to take all this in its really difficult.

[hunter5582]

Hello and welcome to the forum. You have definitely found the right place to be. this is a great place to get support sand information from people with lived experience dealing with a MPN.

I was diagnosed with ET about 30 years ago (about the same age as your husband). It progressed to PV about 8 years ago. I have lived a rich life while managing the MPN and at age 66 continue to do so. The first thing to know is that ET can be successfully managed and most people with ET can expect to live a normal lifespan.

It is is a shock to find out that you have a "blood cancer." Many of us look at this as cancer with a little "c" rather than cancer with a big "C". It is similar to a condition like diabetes in that it is a chronic condition that can be successfully managed. One of the core truths is that you are more likely to die with ET than from it.

It is quite common for us to have more issues with the secondary or constitutional symptoms than we do with the primary risk of thrombosis. There is also risk of microvascular symptoms (e.g. erythromelalgia, headaches)and hemorrhage (bleeding/bruising). These issues can be successfully managed.

It is very important to consult with a MPN Specialist. Most hematologists lack the KSAs to provide optimal MPN care due to how rare they are. There is an excellent MPN Specialist on this list who others on the forum speak highly of (Dr. Mark Drummond).

mpnforum.com/list-hem./

Symptoms like unexplained bruising are annoying and worrisome but they can be managed. As you both learn more you can expect to find ways to deal with the ET successfully.

All the best to both of you.

[Green1988 in reply to hunter5582]

Hello and thank you so much for your reply. This was all really unexpected as he was referred cause of his high platelets never would we have thought it is a blood cancer. The first 2 weeks I couldn’t eat couldn’t sleep just literally sitting on Google and joining forums to find out as much as possible. I just feel all everything has turned upside down. I don’t show this in front of my husband but when I’m on my own I literally just cry all bad thoughts cross my head we have 2 young children. We have an appointment next week with Mark and have questions ready to ask. It’s just so difficult most of our immediate family know but now I just don’t feel like seeing or talking to anyone else.

[hunter5582 in reply to Green1988]

I am glad you have an appointment with Dr. Drummond. Best thing to do at this point. He can answer your questions and help work out an optimal treatment plan. In the absence of a thrombotic incident or co-occurring medical conditions, your husband will most likely fall into the low-risk group. This means that he will be just monitoring and managing any of the secondary symptoms that may be present. Some docs no longer recommend event he daily aspirin for someone in his situation. Dr. Drummond will help him sort that out.

The main thing the docs worry about with thrombocytosis is thrombosis (clotting). Please do review this with Dr. Drummond, but he is likely considered low risk for thrombosis with his profile. Note that I have had thrombocytosis for 30 years and have never had a single incident of thrombosis, even after I progressed to PV.

The easy bruising that he may be experiencing is something we experience with thrombocytosis. That is the weird thing that we can experience both thrombosis and hemorrhage. The more minor increase in hemorrhage we experience is mostly just an inconvenience. Shaving nicks take longer to stop and we get bruises too easily. Do be sure to let the doc know about it. He may want to do a von Willebrand Panel. He will also most likely check his prothrombin times (ptt/aptt - INR). This is all just a standard part of evaluating a MPN.

Please be assured that he will be able to manage the ET. Look forward to a long rich life together. There can be some issues to manage, but they can be managed. Particularly when he obviously has such a supportive partner.

All the best to you both.

[Green1988 in reply to hunter5582]

You are amazing and this is great to hear I really appreciate the support from others in the same position. Thank you so much 🙏🏾

[Green1988 in reply to hunter5582]

Also what’s the difference between calr type 1 and 2?

[Smudger0122 in reply to Green1988]

Not sure, i haven't had a number mentioned to me.

[EPguy in reply to Green1988]

It is a complex genetic variation as seen in the report below. One type has something missing, the other has something added. There have been studies of the prognosis for each, the reports are not conclusive or are conflicting from what I have seen. This study found type 2 had higher PLT.

The terms type 1 and 2 are starting to fall out of favor lately as they are finding more CALR variants.

---

<<exon 9 deletions or insertions; type-1, 52-bp deletion (p.L367fs*46), and type-2, 5-bp TTGTC insertion>>

researchgate.net/publicatio...

[hunter5582 in reply to Green1988]

Since I am JAK2 positive, I have focused most of my learning on this mutation. There are others on the forum who are more knowledgeable than me regarding CALR. Here are few tidbits I do know.

CALR1 - more common in MF - lower platelets

CALR2 - more common in ET - higher platelets

ashpublications.org/blood/a...

CALR is generally associated with a lower risk of thrombosis.

pubmed.ncbi.nlm.nih.gov/332...

As you delve more deeply into learning about MPNs and CALR Specifically, it is important to build a base of knowledge. There are some very good webinars here.

mpninfo.org/conferences/202...

This presentation on MPN Molecular Biology is particularly helpful It will help to have a basic understanding of the underlying biology of MPNs.

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

As you learn more about CALR please be sure to pass it along. We can all benefit from what you learn.

[Scottishterrier]

Hi please don't Google best site if you want information is mpn voice that gives you accurate information and everybody on here are members of health unlocked and also mpn voice and know exactly what you are going through yes we different MPN's and on different medication and as someone said it is a small c not a big c it can't be cured but can be managed by the right medication many of us are on different medication your husband will have good days and bad days don't look on the bad side look on the good side because if you do it keeps you positive don't worry what might never happen I have had three blood clots to the liver and I count myself lucky it could be alot worse been through brain surgery didn't go to plan but still getting on with life hence Maz giving me nickname Scottish terrierStay safe

Scottish terrier xxxx

[Green1988 in reply to Scottishterrier]

Aww thanks so much for your support. Yep I’m trying be keep positive ❤️