Random question

[Assoonas]

Hey everyone. I was just wondering if you need a biopsy to determine if you have Essential or Reactive thrombocythemia. My consultant is very dismissive of ET as she does not consider it a cancer.

[Paul_1971]

Hi

Many are moving away from using a BMB to diagnose as other pointers should tell if something like ET or PV can be diagnosed, such as blood test for mutations, blood counts etc.

If you have high platelet counts and test positive for JAK2/CALR etc then you shouldnt need a BMB to diagnose

All the best

Paul

[Assoonas in reply to Paul_1971]

Thanks. I am Jak2 positive and my platelets were at about 1000 before I was diagnosed in April 2018. The level had been rising since early 2012 but only after a mild heart attack was I referred to haematologist. Sadly no mpn specialist in this lovely wee country and sadly my GP isofix the same view as consultant with regards to mpn not being g a cancer. Biopsy never even been mentioned !!!

[Paul_1971 in reply to Assoonas]

Its not rare for GP/Consultants to say they dont feel its cancer. It was classified as a cancer in 2008.

I had the same with my GP when trying to get free prescriptions. Had to go elsewhere to get it signed.

The problem is they dont want to be told either, proof is readily available but i guess they dont like to be proven wrong.

I do feel for you if you are struggling to find a specialist to help you. Not sure what country you are in maybe someone on here might know of one.

[Assoonas in reply to Paul_1971]

Thanks Paul. Quite sad for all of us that the medical profession have their heads in the sand over mpns. Hard enough fighting this disease without having to fight them as well !!!

[Otterfield in reply to Assoonas]

I note from your profile that you are in the UK and there are indeed MPN specialists here. Perhaps you could print out some information from MPN Voice which will clearly state that these are cancers. I'm surprised and shocked that a haematologist would not know this unless he/she is assuming it is reactive. Good luck!

[Assoonas in reply to Otterfield]

I'm in Northern Ireland so sadly no specialist. My consultant has said it is ET and the first time she told me she just said it was nothing to be worried about and handed me a piece of paper with the side effects of hydroxycarbramide on it. The fact it was a Macmillan headed piece of paper got quite a reaction from me with a nice swear word sneaking out lol.

[Otterfield in reply to Assoonas]

Being in NI is no bar to having a telephone appointment with an MPN specialist if you feel that you need one. However, for many people ET really is something that you don't need to worry about. Make a list of questions to ask your consultant and hopefully you will come away from your next appointment much better informed. For example you could ask why she considers HU to be the best option for you and what the alternatives are. You could also ask her to clarify her statement about it not being cancer, as this means that you should get free prescriptions.

[Assoonas in reply to Otterfield]

Thankfully prescriptions are free here anyway . I am going to ask my Renal consultant about talking to an mpn specialist and if she can help. She was the one who referred me to haemotology to begin with. She is always very helpful.

[socrates_8]

Hey there Assoonas...

My name is Steve, & it sounds to me like you need to find yourself an MPN Specialist... "As Soon As Possible!" Pardon the pun, but truly...

Doctors being ignorant of MPNs, is almost a crime, in my view...

One of the biggest issues most of us face becoming diagnosed, is the simple fact that there remains a great deal of ignorance surrounding the diagnosis & treatments of MPN.

Even just the basic questions, (possibly most asked question), like: Are MPNs a form of cancer?

In 2008, the World Health Organisation (WHO, determined to change the former name of Myeloproliferative Diseases (MPDs), to Myeloproliferative Neoplasms (MPNs), because the condition more often than not meets the criteria of a 'Blood cancer' because of the cloning and over abundant production of mutated cells...

Hence, ALL doctors should at least be aware of that simple event, in my view... Providing ambivalent responses is not acceptable, & they should simple say, "I don't know..." When they really don't know, in my view...

However, because MPNs are often long term chronic illnesses, that can be indolent & even asymptomatic, they are often discounted as being non-life threatening by many within the medical fraternity, & that too is very dangerous...

However, all it takes to arrange an app't w/ an MPN doctor, is a simple referral letter from your GP, (or any GP), and then just make the appointment etc.

Here in Australia, the level of MPN ignorance is far worse, & we have so very few specialist options to select from... Fortunately, for you over there in the UK, you have better opportunities than do we... One of the most informed is Prof. Claire Harrison, at St Thomas & guys, & the MPN Voice coordinator Maz probably has a list of candidate MPN Specialists to find one closer to where you reside etc.

Remember, MPNs are generally considered as 'Chronic' illnesses. Hence, whoever our doctors are, we might be having to manage & nurture that relationship for quite some time, & therefore it is imperative we are happy that the doctor selected is both knowledgeable & practices an empathetic 'bedside manner', in my view...

Reactive v. Essential Thrombocythaemia?

I recall upon my own diagnosis, I spent months trying to prove to everyone that they had made a mistake, & that this was going to be a 'Reactive' illness I was suffering from... When really, that was me going through those first initial symptoms of being overwhelmed w/ my ET diagnosis, which shortly thereafter became MF... (Denial is part of the process I am afraid, or can be?)

Knowing what one's 'Driver Mutation' (I.e. - JAK2, MPL or CALR) , and then what one's bloods reveal, for example: extremely high Platelets, high Red Blood Count (RBC), & of course White Blood Count (WBC), are usually enough to strongly indicate which MPN one might have...

Bone Marrow Biopsies (BMBs), usually tend to show the level of bone marrow fibrosis occurring, (scarring of the bone marrow tissue), which might in turn indicate that a person might be 'Pre-fibrotic' & in the early stages of MF, as it was in my case...

Other tests to help establish a diagnosis of ET, might be a test to derive the 'Stickiness' of one's Platelets,because this might also reveal other conditions like Von Willebrands Syndrome (VWS), which can lead to having a stroke, (Transient Ischemic Attack - TIA), if not closely monitored etc. I have had a few of those too...

I am not trying to alarm or frighten you at all, just really endeavouring to help you comprehend why it's paramount for all people w/ an MPN to be their own very BEST advocate, when it comes to their health. We must be responsible, & direct & manage our illness as best we can, & in my view... That starts w/ finding the right medical team to assist us to do just that...

Hope that this might help a little... Never be afraid to ask as many questions as you need to have answered, & if the doctor is not responding adequately, then find one who will...

Very best wishes, stay safe & well...

Steve

[Assoonas in reply to socrates_8]

Thank you Steve. I have only seen my consultant twice since diagnosis and not really a very approachable person. One of the registrars was a lot easier to talk to but sadly only there short term and he admitted he didn't know much about mpn. If I had to fly to England for a consultation with a specialist I wouldn't think twice about it although I still havent been released from shielding as I am also a transplant recepient. Sadly I'm the only patient with mpn at my current hospital so they are really not interested. I'm really surprised to hear Australia are not more up to date. Thought they would have been at the forefront of most medical treatments.

[socrates_8 in reply to Assoonas]

Hey again...

There may be another solution available to you, even if only as a 'short-term' bandaid...

ZOOM consultations... Because of CV-19, Tele-consult's are very much in vogue right now. I would suggest that you first obtain a Referral from your GP, to see a dedicated MPN Specialist, and then call them & ask for a 'Tele-consult', under the circumstances I am fairly certain that it should be achievable etc...

Of course you will need to provide some of your medical history for backgrounding ...

Ever since I was diagnosed, I have kept all of my own results of all tests, whatever they were etc...

Unfortunately, the problem in Australia is quite possibility exacerbated more by what is sometimes referred to as the 'Economy of Scale'.

Australia's population is quite small by global standards, c. 26M, and the exceptionally rare incidence/prevalence of these types of blood cancers, simply means there are simply not enough people who are sick w/ an MPN to warrant the case for more MPN Specialists to help improve their case for greater longevity... Sadly money is the prime motivation in our 'market-driven' ethically challenged 21st century world...

In my view of course...

Anyways, do let me know how you get on w/ it all...

Best wishes

Steve

[Assoonas in reply to socrates_8]

Brilliant idea Steve. Thanks very much. I will contact my Renal consultant and see if she can even refer me. She is a lot more approachable than my GP.. totally agree about this being a money oriented world. Must admit I'm not rich but I would give away everything that I own to have no health issues. I will let you know how I get on but in the meantime stay safe and well in these weird and wonderful times 👍

[socrates_8 in reply to Assoonas]

Pleasure...

Just remember too... You can go to ANY GP, & ask for a Specialist referral...

And please don't allow yourself to become somehow caught up in some misplaced feelings of 'disloyalty' ... This is about you & your health, & the ignorance of others should not become your stumbling block on the way to you gaining a "better quality of life", (during your MPN journey).

Best wishes...

Steve

[piggie50]

Hi,

Prof Mary Frances McMullin is based in Belfast. She has talked at many of the MPN Voice conferences I have attended (before Covid). I don’t know if she sees patients but you may be able to get some information from her regarding who you can see. This is on her bio page....

My main research interests are in myeloproliferative disorders, acute myeloid leukaemia and chronic myeloid leukaemia. I have an active clinical practice and collaborate with scientists looking at the pathogenesis of the myeloproliferative disorders and in particular investigations of patients with idiopathic erythrocytosis. I also work on the pathogenesis and treatment of Acute Myeloid Leukaemia and Chronic Myeloid Leukaemia.

Good luck

Judy

[Ebot in reply to piggie50]

Hi. I was thinking exactly that. Prof is a well recognised leader in the MPN field and a thoroughly lovely person.

[Assoonas in reply to piggie50]

Thanks so much for the info. My friend is actually a patient of the Profs and sad news the lady in question is retiring this year. She will still be involved with her transplant patients on a part time basis only. Sounds like a huge loss to our particular group. Still going to try and get a referral before she departs though 🤞. Thanks everyone for all your information. Even if the Prof can only give me leaflets etc it is still more than I have right now lol. I do look online for info but not always sure I'm in a reputable link when foraging about online. I'm definitely not very Tech savvy 🙄🙄

[piggie50 in reply to Assoonas]

Oh what a shame. mpn voice.org.uk is a reliable source of info, up to date and correct. It was founded by Prof Claire Harrison at Guys. Maz our site coordinator can also send you leaflets as Ebot says.

I hope you can get some better care than you are getting at the moment.

[hunter5582]

i will add to the chorus - you need to consult with a MPN Specialist. Do whatever it takes to see one. Here is a list mpnforum.com/list-hem./ .

Regarding ET being a cancer - it has been considered one by the WHO since 2008. Some of us look at it a cancer with a little "c" as opposed to Cancer with a big "C" - just one view of it.

Note - if you do need cytoreduction, there are certainly options other then hydroxyurea. Your doc should have told you that. here are a couple of articles.

mpnjournal.org/how-i-treat-...

legeforeningen.no/contentas...

Hope you get better answers soon.

[Assoonas in reply to hunter5582]

Thank you. I will get everything printed and take it you my GP.

[Ebot]

Hi. See my endorsement of Piggie50’s post. Also get hold of the leaflets published by MPN Voice - check the website. You might like to wave those around in front of your GP / consultant as in ‘I was interested to read these and thought I would share ...’. :)) Good luck.

[Assoonas in reply to Ebot]

Thank you. All information is greatly received. Learning more from this lovely forum than from any doctor.

[Wyebird]

Hi take solace in the fact it took 5 years to find the correct dose for me. It was heart breaking when at nearly every consultancy I found yet again I had to increase my meds. I have an amazing team.

Now this is my RANT!!

AS a retired teacher, every time the curriculum changed I had to deliver syllabuses on topics I was unfamiliar with. So plenty of reasearch on my behalf was needed.

Why don’t ALL GP’s research when faced with a patient with an unfamiliar or rare illness???

If this was done then your GP would know that you have a rare BLOOD CANCER!

😡

[Assoonas in reply to Wyebird]

Thank you. All about learning how to stand up to the "professionals" .