ET or RT, that is the real question?

[socrates_8]

Hey everyone out there...

My name is Steve from Sydney (down under) Australia etc...

Thought it might be useful to have a chat and share some experiences here with the rest of the MPN community. I was diagnosed w/ ET only in the last 2 weeks (May 2016). They seemed to assume this finding rather quickly and I am still wondering if I might actually have Reactive Thrombosis (RT) as opposed to ET? There are apparently a great many similar symptoms, and after some rather exhausting (peer-reviewed academic research)... Well I am still wondering (& quietly hoping) that it is RT and not ET...

Diagnosis:

Bone Marrow Conclusion:

Mildly hypercellular marrow with trilineage hyperplasia which is marked in the megakaryocyte lineage.

There is associated megakaryocyte nuclear atypia and increased fibrosis suggestive of a myeloproliferative neoplasm.

Consistent with but not diagnostic of essential thrombocytosis.

Suggest BCR-ABL and clinical correlation to exclude reactive thrombocytosis.

(JAK2 & BCR ABL - negative) Not yet tested for CAL Reticulin.

My experience thus far emerged as an unexpected discovery. After 5 years of university studying (while also feigning at being my Octogenarian mother's default carer - she refuses any assistance of course :), during a routine medical for a o/s posting working in community aid), I was to learn of a 1700 Blood Platelet (BP) count, and that a suspicious episode I suffered last August, was a likely transient ischemic attack (TIA), and not a pinched nerve.

Naturally, at first... I just went into a passive state of sensory delayed shock. Probably only just resurfacing since that time now...

My BP, from May 6 (1700), May 20 (1515) and then May 27 (1384), hence, my BP levels stayed consistently high. However, they did drop significantly without assistance too...??? Hydroxyurea (HU) commenced May 27 2016.

June 17 will determine if the prescribed medications of low dose aspirin and HU has been a successful remedy in returning my BP levels to around 450 etc.

Of course, I do not wish to suffer a stroke either...

Side-effects:

During these past two (2) weeks of HU medication I have noted the following:

Extreme fatigue, some bleeding of the gums on flossing and cleaning my teeth, some headaches, some heat flushing, some dizziness and vertigo, mild bouts of constipation and diarrhoea, and finally a rapid gain in weight which I have no doubt is now contributing to intense back pain (I am really having trouble walking about, trying to sit or even to lie down etc).

Hopefully, the final diagnosis might change to Reactive Thrombosis RT and I will be able to escape this poisonous treatment.

I am yet to ascertain what (if any) natural remedies might work for reducing BP production, if it does turn out that I do have ET after all...?

But very little to report as yet.

Retrospectively, I now realise that I have had a host of other symptoms like:

Seriously affected blurred & monocular vision loss that might last for seconds or minutes (I thought at that time I needed reading glasses so acquired them etc), erythema, which can be a superficial reddening of the skin,which causes irritation & dilatation of the blood capillaries and scarring to almost all of my finger nails and a couple of toes too, Infrequent - but serious throbbing burning pains mainly in my fingers that might last for longer periods of time, some loss of balance that has never really become what it once was (however, I am 57 years old)...

All of these MPDs are largely age related (in most instances), so much the crueler when it affects those in their youth. In age, it seems to correspond to degenerative causes.

In any event, I would be most happy to hear from anyone here concerning my post or even those just wishing to say hello of course...

Best wishes to all...

Steve

[mhos61]

Hi, Steve and welcome. I have recently been diagnosed with essential thrombocythemia, and like you I was hoping for a diagnosis of reactive thrombocytosis. However, for whatever reason I somehow knew that wasn't going to be the case. I also have been started on aspirin and hydroxy and am awaiting appointment to see if it has lowered my platelet count. It sounds as though you have armed yourself with plenty of knowledge on ET, which I believe is essential. You will find as I have that the MPN community here are extremely supportive.

Good luck Mary

[socrates_8 in reply to mhos61]

Hey Mary...

Thank you for your response. It seems we share a few similarities. And, like you, for whatever reason... I also believe strongly that my case is RT and not ET.

In my dysfunctional adolescence, I played with intravenous drugs, and contracted hepatitis at an early age. Although it has never really bothered me since, I have always suspected that someday it might... Hence, my leaning toward the RT diagnosis over what the medical profession seems more content with. However, they have not ascertained as yet that my liver has suffered a previous hepatitis event, and I have not as yet volunteered that key piece of information as I had always believed it to be detectable considering the range of examinations of thus far been exposed to etc.

Either way, the underlying cause would still need to be eliminated (if possible) to have a complete cure... So I am not out of the woods by any means if the RT diagnosis becomes a more concrete factor.

In retrospect, perhaps it would have been best for me to have volunteered that information, however, its stigma is an assault of another classification entirely...?

In scanning through the Blog Feed here on MPN, I did not see anything of those that might also suspect RT over ET...? Until you have just mentioned the same now Mary...

I am beginning to wonder if there might not be a latent form of attached Narcissism, to that which is the more fashionable form of disease classification...

Nonetheless, it is an interesting disorder, ET I mean. Of the six basic myeloproliferative blood disorders, it seems that ET is not really a cancer as such but a Blood Platelet (BP) disorder proliferates an over-production of BP cells, whereas all the others MPNs appear that they might be various stages on the way to Chronic myelogenous leukaemia (CML), wherein the White Blood Count (WBC) falls dramatically affording little or no natural immunity to disease and infection etc..

Interesting too that there is quite a large volume of peer-reviewed literature that suggest that HU might also be a contributing cause to transgenic formation from ET to CML (although always accompanied by the adverb - RARELY).

One of the effects of HU suppressing the BP count is that it can also lower the WBC, and over a longer period of treatment our bodies system start to resemble someone that is suffering from CML etc... And that for me is quite a concern in and of itself... Don't you agree...?

I fully understand that the issue of stroke MUST be managed but I cannot help but wonder why, in the case of ET, that there is not another way of achieving a better result than living with such a loaded gun and a diminishing quality of life for the rest of what must be, a sadder existence...?

If Stem Cell Transplant (STC) is an option for those whose lives are being more seriously threatened, then why wouldn't ET sufferers attempt this operation before our lives deteriorate further...?

Yes, there are always prevalent risks. Most drive automobiles everyday too...

In other words, why wait until our quality of life has been in chronic training to lessen our bodies ability to successfully undergo STC before we attempt to reverse the course of our disease?

I suppose one answer might be found in the economics of doing so... However, if I can no longer find or manage to maintain work... what economics do I have to speak of?

In such a 'Catch-22' I believe that I would naturally opt top risk the STC surgery.

What about you Mary, how do you rationalise this wicked problem?

I look forward to hearing back from you or any of the MPN community again soon.

Thank you for your support and for listening to my controlled rant.

Steve

[mhos61 in reply to socrates_8]

Hi Steve,

Admittedly, I do find it hard at times to rationalise this ET diagnosis - but then I think to myself that things could have been a lot worse. For example, I could have got it when I was a lot younger and I suppose the thought that ET is the lesser of the MPN evils is somewhat 'comforting,' albeit that is a strange choice of adjective. Also, I count myself lucky that it was picked up on a routine blood test, rather than the first symptom being a dvt, heart attack or stroke - so that is how I am dealing with it at the moment. I am not looking too far into the future either, just taking each day as it comes.

Are you going to be checked out soon for the CAL Reticuline mutation? I would definitely tell the Consultant about your past liver problem as this could be a contributing factor to your present health problems.

Good Luck

Mary

[socrates_8 in reply to mhos61]

Hey again Mary...

Yes, I too could so easily have been missed, and gone on to have experienced a much more severe stroke episode. Although, as it turns out, I did have a TIA previously it only lasted seconds (in August 2015) it was a minor event in the greater scheme of things, and was allowed to have been considered a 'Pinched nerve'?

Unfortunately, in these latter years of my existence, I am not as financial as I once was... In Australia, there is a great contrast between how a patient might be treated depending upon whether they are Private vs. Public etc.

The CAL Reticulin test in Australia is a tad expensive to undertake at the present moment. However, if I can find some work where they do not insist upon my undergoing a 'medical screening' as a part of the process. I would then arrange for that test to be completed. Mainly so I could eliminate it as a cause of ET etc. Leading back to my assumption of an RT diagnosis, and a liver complaint.

I have been contemplating seeing another GP and asking to have a Liver Function analysis completed although all my previous bloods did not suggest that my liver was a problem, nor did the Ultrasound that I have recently undertaken etc...?

The medical screening that would ultimately reveal my condition was being conducted so that I might be declared fit for an overseas volunteer posting working in International Development/Aid. Hence, my reason for keeping the Hepatitis undisclosed due to the stigma that I have noticed that accompanies any frank admission of the same etc.

I am still endeavouring to make that o/s posting, if I can, but if I was to now acknowledge my omission of a previously known of health condition (c. 40 y ago), well that too might jeopardise my chances...

After 5 years of studying the theory, this o/s posting would have gone a long way to providing me with the much needed exposure toward establishing my new career in International Aid work etc.

My study support allowance ends next week... Interesting times ahead ...

But as you quite rightly surmise... Things could be much worse... :))

Death is starting to look like more of a release from this ceaseless tide of struggle...

Sorry for sounding so morbid, I am fine really.

Thanks Mary.

Steve

[mhos61 in reply to socrates_8]

I see now Steve that you're in of a bit of a 'catch 22 position.' You sound so passionate about the overseas posting, and I truly hope that it works out for you. Equally, I hope that you manage to work out your health issues, and above all, get that most important accurate diagnosis.

Good Luck (keep us updated)

Mary

[socrates_8 in reply to mhos61]

Thanks Mary...

Me too...

S.

[jillydabrat]

Hi down under, all of your symptoms look like an MPN, if you read back on other people's symptoms you will see your a perfect match. Everything you are experiencing whilst taking the hydroxy is normal as well. Sorry but I cannot understand all the terminology. Hope you find your answers from this wonderful site and we are always here for support. Good luck next week, the brat

[socrates_8 in reply to jillydabrat]

Hey Jillydabrat...

Nice handle.

Thanks, I guess I will be taking things a step at a time for the moment.

Cheers.

Steve

[Phelpsy]

Hi Steve , you've found the righ forum to put your mind at ease & get all you Questions answered ,

There's plenty Knowledgeable people on here to help you Maz for one ,

your symptoms sound my alarm bells ringing ,

I was diagnosed with ET 3 years ago now after a Tia , ( horror shock for me & my family ) I was 50 yr old ,

And MPN voice & the lovely people on here kept my focused & Possitive ,

I'm on Hu , blood thinners & simvistatin

Just keep my blood health

It's working platelets were 900 now down to 390 , still room for improvement

But I'm so greatful for HU it's so not the Greeneyed monster it's made out to be ,

For me any way

I hope you get the diagnosis your hoping for , stay fit healthy & drink 8 glasses water a day keep me ticking & feeling Tip top ,

Good luck & keep Possitive

I always say I have ET

It doesn't have ME X

Pam Newcastle England X

[socrates_8 in reply to Phelpsy]

Hey Pam...

Thank you for your supportive message. To be honest, I have been a tad afraid to exercise as I usually do in fear of aggravating my condition, so to speak. However, my weight gain has been a swift event over this period 5-6kg, and this can only be accentuating my risks even more. I guess finding the balance is somewhere in between these tow extremes...?

I shall endeavour to drink more water, but I really don't eat very much as it is and that is why I am concerned about my weight gains. I am a vegan/pescetarian, which just means a vegetarian that eats seafood too... Yet my weight is now at 120kg from 112 only a month ago.

I am not diabetic and I do not have any added sugar at all, in any of my foods, drink mainly green & herbal teas, and try to avoid coffee and alcohol because they tend to dehydrate me (and caffeine I believe might also be stimulating my infrequent bleeding gums that transpires because of the aspirin). To assist that not happening I try to clean & floss before taking the aspirin in the AM.

While I appreciate your assurances on HU, I remain unconvinced at this juncture that its effects long term are benign. But naturally, I am moat grateful for your feedback & will take what you have said onboard.

Thank you Pam.

Best wishes.

Steve

[tessa46]

Hi Steve, like you I was recently diagnosed with ET, but JAK2+. I had a stroke last October (luckily mild) which eventually led to my diagnosis in March this year. I am classed as high risk because of the stroke and I'm 68. I have always been careful with my diet and take a lot of exercise. I have a history of poor tolerance of drugs, so very unkeen to take hydroxycarbamide, espcially as a life long medication which addresses symptoms but not cause. My platelets have varied between 576 - 695, 625 when last measured and I have no night sweats or spleen enlargement. Although seen as high risk, my haematologist has been unable to quantify how great the risk is for someone like me who is taking Clopidogrel (platelet aggregation inhibitor) and Atorvastatin 10mg and blood pressure tabs Ramipril 2.5mg. My blood pressure was fine before all this happened, so I feel alot of that is caused by stress. My own doctor is less interventionist and feels that, while its my choice, the drugs I am already on will cover all the bases as regards a recurrence of stroke. I have decided not to take the hydroxycarbamide for the next 3 months and to enlist the help of a nutritionist and see if diet and supplement changes can help. I am due to go back to my consultant in September. I will let you know whether I find out anything of interest that is helpful. I hope you get your diagnosis sorted out, I had every test and scan going before mine was finalised, and another look at your liver might be helpful. I hope you make some progress. Give my love to Sydney, one of my sons lives there and I love it!

[socrates_8]

Hey Tessa...

Thank you for your response.

Yes, I agree on being unfavourable towards any types of chemical intervention... All my life I have barely taken a panadol for a headache.

I also agree that I would prefer not to have any further TIAs minor or otherwise.

Your medication regime does sound interesting: Clopidogrel (for BPlatelet suppression), Atorvastatin, (which is mainly for lowering of cholestrol), & Ramipril (which is mainly used to relax heart muscles and lower Blood Pressure issues) apparently.

That is interesting as I do have periodic Blood Pressure issues that concern me but noone has thus far suggested I require help w/ my Blood Pressure in keeping it low. It is frequently in the high 150s but rarely above that mark, as yet...

I shall ask a few question regarding the Clopidogrel, but I am a tad concerned as it may or may not work and my gums do bleed occasionally and I don't want to exacerbate those events if I can help it. But I would like to know what the main differences are between Clopidogrel & HU, they do sound constituently very similar...

I have always believed in regular exercise and a good diet, but here I am anyways...?

However, there is little doubt that a person always feels much better within themselves when maintaining fitness at a certain level etc.

Are you in the States Tessa or the UK, or somewheres else...? I have a sister who lives in LA, and another who will possibly be moving there sooner as she recently married a cool jazz/blues guitarist San Francisco, I think?

Sydney may be lovely but it is FREEZING right now...

Best wishes and I shall look forward to hearing more about your results too...

Thanks Tessa.

Steve

PS. It is comforting to have so many people to discuss these matters w/ even if briefly...Thank you everyone...

S.

[Mazcd Partner MPNVoice]

Hi Steve, welcome to our forum, nice to hear from you. We can all understand your feelings about your diagnosis, it is quite a shock, and also having to take Hydroxycarbamide, we all struggle with that one, and it is something that you have to decide for yourself, whether or not to take it, the side effects you are suffering aren't very nice, and hopefully they will settle down soon, they are side effects suffered by a lot of people who take Hydroxycarbamide and they do tend to settle down after a couple of weeks, but if they persist then speak to your haematologist about an alternative, or changing the dose, this can help with the symptoms. Keep us updated with how you are getting on. If you would like any booklets on ET and Hydroxycarbamide and other medications then let me know, I can post them to you in Australia. Best wishes, Maz

[socrates_8 in reply to Mazcd]

Hi Maz...

Thanks for dropping by and offering some support. It is greatly appreciated of course.

Maz, as I have stated in the subject line, I am not completely certain or resiled to my current diagnosis of ET ahead of RT at this juncture...

I am planning on obtaining a 3rd opinion (as the 2nd Op seemed a tad afraid of my feminist inclined haematologist), I am certain that she is a learned individual although she is a tad abrupt all the same...

That is not really at issue here, what is in my view, is being rushed through the Public medical system without even a word about RT being a possibility when a CAL Reticulin test is yet to discounted.

Apparently, in my bloods, there is a noted Calcium deficiency, which of itself alone could indicate the possibility of the clonal marker CAL R etc...

Obviously, if it did present as positive, I could no longer argue the possibility of RT instead of ET. However, while ever there are unanswered questions I will pursue them with the gusto, even if that gusto must be tempered by the speed of a tortoise (given my financial constraints etc).

As to side-effects:

At the present I am on the minimum does of 500mg per day. Although it has been strongly suggested that this will most likely be elevated shortly. Possibly after my next blood tests this week.

It seems that many here are in both camps when discussing this issue. Some who have been around for quite some time say that they hardly notice a thing, and others speak of leg ulcers and a range of other unwanted phenomena...

There is also quite a range of peer-reviewed literature that suggests HU might also promote ET to other extremes of MPNs, although many also suggest that it is a rare occurrence...

If indeed I do have ET, as has been suggested, then I will continue to seek out alternate solutions because I do not wish to be ingesting poisons for the remained of my existence with a ever increasing/decreasing 'quality of life'. (Life as we already know is far too short as it is...)

Age, obviously, has such an effect upon us all (quality of life) but I have no wish to summon those symptoms any sooner than they might have normally emerged etc...

Maz, are you also suggesting that the weight gains I am currently experiencing will also dissipate? Now that would be lovely...

In all honesty, I do feel that I have always struggled to keep my weight down to what I would categorise as athletic, since my early 40s. I was still playing cricket until circa a year ago, when the aches, superficial injuries and pains of doing so were becoming a little too much to bear, and I decided that I could no longer afford to carry any injuries, especially if I was ever to eventually return to work.

The ET diagnosis would make working even more difficult than it already is, whereas, if it is RT and the underlying cause can be treated successfully then at least I might be able to support myself into my most uncertain future...

As an ET patient, I believe that insurers may not accept me for a range of certain positions... My age is already impediment enough. Until recently I have never needed to have a degree in order to be employed. Now I have a large debt to the state/fed, and no way to ever repay that debt. IF? I am unable to resume some well paid form of employment...

There is little point to one's existence if we cannot contribute in some meaningful manner, while supporting our own livelihoods...

Thanks Maz.

Steve