New diagnosis of ET

Hi Monners and welcome to this corner of the Community. I am Mike and I help out around our blood cancer groups. So sorry to hear about your wife’s diagnosis.

I don't have Essential thrombocythaemia (ET) but I was diagnosed way back in 1999 with another rare, incurable type of blood cancer Stage 4a so I do know this journey rather well.

In the early days it’s all about understanding the condition and once you get more information and talk with others the journey will get more understandable.

Let's look for the members with this condition to pick up on your post, you can also look through all the group threads (hit the group name and scroll down) and as always you can hit reply to any older posts.

Talking to people face to face can help a lot but during these strange times it’s not that available but do check to see if any Local Macmillan Support in your area has opened up. Do also check out for a local Maggie's Centre in your area as these folks are amazing and each centre do run monthly Heamatology Support Groups most are online at the moment….. the one i attend does have a few folks with ET in it so worth checking.

The Macmillan Support Line is open 8am-8pm (timings may differ across services) 7 days a week on 0808 808 00 00. This service provides cancer information, practical information, emotional support, benefits/financial guidance or just a listening ear. You may also find our Ask an Expert section helpful but do allow a few working days for a reply.

You may also want to check out MPN VOICE UK for some good information and have various support platforms.

Always around if you need further help or just want to talk.

Hi Monners , sorry to hear of your wife's diagnosis, it must be a shock for you both. My good friend Mike Thehighlander  has listed different blood cancers. Unfortunately I don't have an ET diagnosis, my diagnosis is of MDS and I was diagnosed in March 2015. I have a low haemoglobin count and I have treatment every week in the form of an injection that encourages my bone marrow to produce more red blood cells. Just as I have been thinking about putting down the last sentence or two I wonder if the frequency of my injections is significant, I must look into that. 

The manner of your wife's diagnosis being delivered without any explanation is similar to my own deliverance. I'll put that to the side temporarily for a while.     

Has your wife been sent a brochure or leaflet about ET? I received mine through the post and my MDS type (there are several) was underlined in said brochure. Has your wife been referred to a haemotologist? Has she been told that she is being referred to one?If the answer to both is no, and that were me, I'd be on the phone to my GP and asking should I not be referred to a haemotologist who knows a fair bit about the condition.

Ok, back to my own diagnosis. After my haemoglobin level had dropped below 100, I had a bone marrow biopsy (the result is more accurate below 100). On my next appointment with my haemotologist, he wrote something down on a piece of paper and slid it across the table to me and said that I would like to look it up later. That was how my diagnosis was delivered!

I'd guess that your wife's GP doesn't know a great lot about her diagnosis and that's why I suggest that she sees an expert asap.

Good luck with her journey Monners. I'd like it if you come back to me and inform me of her progress. 

Tvman 

• google is definitely not you friend. Like your wife my high platlet  level was picked up on a routine blood test , With several other causes including infection my GP did consider ET but wanted to watch and wait with a repeat blood test in 6 months. Platlets had raised again and she referred me onto  haematology as ?ET for a none urgent appointment as platlets still below 1000. When I finally saw the haematologist  some 8 months after my initial blood test she confirmed it was definately an MPN but may be PV as oppossed to ET -  which ever it was my treatment would be the same and taking my age into consideration I was started on drugs to lower the platlet levels . The Haematology Nurse pointed me to MPN voice as the most reliable source of information on the internet and I have found it invaluable . It was another 4 months before I got a definative Diagnosis PV . My haematologist assures me that with ongoing treatment I have a normal life expectany. Hope this helps - wishing your wife well and hope you are reassured. 

Thank you both for taking the time to reply . We were really shocked when we realised what ET was. Probably slightly less shocked that our GP hasn’t explained a thing. Just repeat bloods in 6 months. 

Hi Monners. Sorry for not replying to your post sooner, but only found out about this forum yesterday, following receipt, through the post, of a leaflet from my local hospital Macmillan Cancer Support Group.

This is about my journey and I hope it will give you useful information for you and your wife.

I was diagnosed with MPN ET November 2022. This was following several blood tests, from June 2022, ordered by my GP when she initially found an anomaly in my standard regular blood test. Apparently I have had an anomaly in my regular blood tests since 2007, but previous GPs have put this down to infections, viruses or injuries. There are very few GPs that know about MPN, let alone recognise it, therefore do not investigate further. My GP also ordered a specialist blood test?  

The blood tests showed a regular platelet count in the 590s and that I had a JAK2 Mutation (the specialist blood test), because of this I was referred to a Specialist Haematologist/Oncologist (SHO) in my local hospital and had an appointment last November with him and Rebecca, a Specialist Macmillan Haematology Nurse(SMHN), bit of a clue of what the appointment would contain.

The SHO informed me that I had a very rare blood cancer and would need chemotherapy. Both very scary terms to hear, take in and mentally deal with.

Showing my blood count history on screen, the SHO went on to explain about Myeloproliferative Neoplasm (MPN) and in particular my diagnosis, Essential Thrombocythaemia (ET). He explained that too many platelets can cause clots that can lead on to heart attacks and stroke. He also explained that it was the JAK2 Mutation in my DNA that had a damaged helix which was sending the wrong message to my bone marrow and then to my stem cells to over produce platelets. The SHO advised me to take 75mg Aspirin daily (not the 200mg+ Aspirin used to counter aches and pains), from immediate effect. This dosage of Aspirin is well documented as reducing the risks of blood clots.

The SHO went on to explain that because of the JAK2 Mutation and that medical science has not advanced enough yet to determine why the helix becomes damaged, I would have this condition for the rest of my life and as Aspirin does not reduce blood cells, it needs to be controlled for me to have a "normal" life span. He explained that a chemotherapy drug, Hydroxycarbamide, is the initial go to treatment to reduce the production of platelets (it is because the chemotherapy treatment destroys blood cells that it is termed as a cancer). Hydroxycarbamide is taken in tablet form for MPN ET and I would have to take these tablets for the rest of my life.

The SHO explained that as the treatment is a chemotherapy it was my decision whether to follow his recommendation. The alternative is a much greater risk of heart attacks and strokes. However, before the SHO can prescribe Hydroxycarbamide, I would have to "go through several hoops" to find out if I am physically and mentally able to take a chemotherapy drug, especially for the rest of my life.

After 2 hours with the SHO & SMHN I left having been given a red A5 sized booklet, from Blood Cancer UK, explaining MPNs and an A4 booklet explaining about Hydroxycarbamide and it's effects, especially it's side effects. I was also given direct contact details for the SMHN if there was anything I needed to know or just to have a chat. Rebecca has been amazing and very understanding. 

I must admit I left in a bit of a daze and went for a walk to try to take it all in before driving home to explain it all to my wife. 

These "hoops" Rebecca explained, would involve an Ultrasound Scan, especially to determine the condition of my spleen, however, the Doctor who carried out the scan investigated all my organs, a Pre-Chemotherapy Assessment appointment with a Haematologist Specialist Nurse, to explain in detail about Hydroxycarbamide and all it's side effects, a Bone Marrow Biopsy and to answer any questions. This would all lead to another appointment with the SHO to determine the way forward.

Being the type of individual I am I need to understand all the WHYs, and have spent a lot of time investigating my condition and the various treatments. In the Blood Cancer UK booklet it refers to MPN Voice, which was my first port of call for understanding. However, there are many people on there that on deeper investigation were not relevant to my situation. No doubt you will find the same, but you have to determine your own because every individual's situation is different. There are, on MPN Voice, a lot of Americans and the same treatments and drugs in America, whilst being more advanced than the UK are not available in the UK. 

There are many articles on the net that refer to MPNs and also ET. Some of these articles are now dated, but give you an insight in to research and options that you might come across in your own journey of understanding. 

The scan, early January 2023, went well, spleen normal, other scanned organs would take more investigation, but eventually showed as all normal for my age.

By the time of my appointment for the Pre-Chemotherapy Assessment, early February 2023, I had a number of questions, that could not be answered by my investigations or Rebecca, especially about my diagnosis and the proposed treatment. At this meeting my Wife joined me. She needed to know. Some of the questions could not be answered in this appointment. It was suggested I put them in an email to the SHO's Secretary ready for my follow up appointment with the SHO. This we did.

Why ET and not Polycythaemia Vera (PV)?, Will I progress to Myelofibrosis (MF) and Leukaemia, How regular will my blood tests be? Why not prescribe Besremi or Interferon? Can I still travel the world? (my wife and I love wildlife and have travelled extensively, does my treatment affect some of the inoculations and medications we have to take for some parts of the world?),  How does high altitude affect the treatment proposed? (we ski every year), How does my situation affect our travel insurance? How does the treatment affect potential Pension ? All these questions, and many more, are individual to me in my situation. No doubt you will come up with your own questions.

One of the regular comments in my investigations was the need to see an MPN Specialist as a 2nd opinion and to confirm the treatment proposed. 

At my follow up appointment, my Wife present again, the email produced and the questions were all answered. My Platelet Count is still in the high 500sThe SHO still recommending Hydroxycarbamide. He knew one of the leading MPN Specialist Consultants at Leicester Hospital and would refer my case to her (I have since heard that I have been accepted as a referral and await an appointment).

Because of the above, I signed the various needed hospital documents to start treatment with Hydroxycarbamide. This can be stopped at any time if trials or a different treatment is advised.

February 6th 2023 I came away from the hospital with the prescribed Hydroxycarbamide and other drugs that counter the bodies reaction to an attack on blood cells. All these drugs to be taken every day for 28 days which is when my next appointment is with the SHO to determine affect and possible alteration.

So far I have had no side effects apart from a little tiredness. 

That so far is my journey, which almost certainly will be different to yours.

I trust you and your Wife come to understand the condition and the affect it will have.

Good Luck

InTouch 

Hi Monners, in case it of reassurance to you and others I can say that my experience having been diagnosed 8th Jan 2023  ET, in my case CAL-R (not JAK2 mutation) with ~ 900 platelet count and having started aspirin and Hydroxycarbomide on 14th Jan has been almost exactly the same as InTouch described above, I already know from my clinical specialist nurse ahead of my next haematologist Consultation on 16th March that my platelet count is "coming down nicely". It's relatively early days I know but I've carried on with life quite normally and with so far, no side affects.

Thrombocytosis- high platelets- can be caused by an infection, inflammation or other things. These have to be ruled out, and a second full blood count show continued high platelets, for a diagnosis of ET to be made. I had MPN suspected when a pre op blood test again showed high platelets (>750) following a high platelet count (580) in a pre op blood test 8 months before. My surgical consultant referred me to Haematology. This was just over a month ago. I have now had two appointments at Haematology, numerous blood tests, had the MPN diagnosis, (ET), made and will have a bone marrow test in a week and an ultrasound of the spleen in two weeks. I have just been told to take low dose aspirin daily, but will need to attend my first MPN clinic appointment, in a month’s time, to see if I need to start on another chemotherapy medication. If your wife is asymptomatic, as I am, it is likely that they will want to wait to get a continued high platelet count and some more blood tests before diagnosis. If she has already been diagnosed with ET I think they would want to give her more tests and start any necessary treatment sooner than 6 months. It might be worth her speaking to her doctor again and asking for another blood test and for the doctor to rule out any infections or inflammatory conditions which might give a high blood count. Not sure if this is helpful or annoying, I am still getting my head around this diagnosis and all the terminology- but it is very helpful to know as much as possible.