MPN Voice
2,933 members4,459 posts

Confused

Hi

I got diagnosed with E.T Jak 2 + in 2007/2008 after a Portal Vein Thrombosis.Also got pancreatitis and oesophagal varises (but never had them banded since then). I have been on Hydrea, Clopidogrel, Propranolol and Omeprazol till now. Anyway, I now asked for my spleen to be measured and to have a BMB. Today I had the ultra sound and I am not sure it will do me any good because....I have gall stones, a little fatty liver and an enlarged spleen......

I feel more sad then ever now! As I know my self , I have tried to avoid to read too much about E.T as it depresses me, but now I really feel my life is going down the drain. The reason I asked for these tests was that, in a FB group, I was told that it was the only was I could know I had the right diagnosis and medication.

What will the BMB show then???? I don't know if I can take this, really. I thought I was doing ok........we had plans for the future, my partner and me, but now I don't know if anything will happen.

Just wanted to check with you folks, if I am the only one feeling like this?

20 Replies
oldestnewest

Sorry to hear your symptoms are an increasing burden. I think you are right to get all the information you can and please don’t worry unduly. ET is the lowest risk of all the MPNs so the best one to have if you can see it that way.

Are are your blood counts still stable under HU?

It may be that you need a change of drug. I’ve read plenty of other posts where people have changed drugs with very beneficial results to quality of life and spleen reduction.

I’ve had two BMBs, if in doubt it’s the best way to make sure your treatment plan is optimal.

3 likes
Reply

Hi, thanks for the answer.

Yes, all my blood tests seems ok and I never had any trouble with pain from the spleen or so. Really, I have not had any real symptoms from anything except the medication (as far as I know)! I had a little gall bladder pain when I was pregnant but I was told that is normal and that went as soon as the baby was born.

Problems with medication is feeling ill a while after taking it and also stomach reactions...Since a good while, I would say about 8 months, I am taking Kefir and my stomach loves that!

As you say, E.T is the least bad of the bad but still...I spoke to the doctor and asked why I was on just Hydrea. She did not think anything else was for me so, that was that.

One thing is...I cannot stand cold! I came home last night from Spain, where I spend as much time I can, and now I am blue from the cold weather!

I need to go back as soon as I can, but first the BMB...not looking forward to the result at all. Sigh...

Reply

Since your bloods are stable and no progression re symptoms it sounds like you should try not to worry too much. It’s a scary time I know though, the moment of truth!

Wishing you the best of luck, I’m afraid these hurdles are now a part of our lives. Hopefully you are still drinking red wine!

Best Paul

2 likes
Reply

Hahahaha...I have some now and then, for sure!

Thank you for your support!

1 like
Reply

Despair not! You have just come from lovely warmth (?) to cold cold cold! It’s enough for any of us to hit rock bottom faced with that. I’m afraid I can’t offer you advice re the way to go as I’m still in the early stages of learning about all of this but try not to worry about the BMB results - whatever the outcome, at least you are forewarned and can plan ahead, if indeed you need to do so. I had one done in November so I would know there were no more surprises and seeing as the doc was pretty relaxed about the results, I took it to be a good sign. It’s only my opinion but with a BMB at least the doctors get a better picture of what is going on.

Just dream about Spain and before you know it, you’ll be back!

Sending e-hugs and all best wishes

Gill 😚

2 likes
Reply

Hello Swede,

I’m really sorry to hear that you’re feeling sad. It appears that you have got yourself into a bit of a state. Firstly, I would give the Facebook group a break for awhile. I could be wrong - but it comes across as if you were pressurised a bit.

The positive in all this however is - you are now being investigated thoroughly. If there is a need for a change in treatment etc., it will be in response to this, which can only be a good thing and in your best interest. You are in good hands.

It’s easy for me to say, but try and remain calm until you can discuss the results of your scan and bmb with your Consultant. I think we all have anxious moments with our MPNs, so can truly empathise.

Those future plans you have with your partner will materialise. 💕

Keep us updated

Mary x

3 likes
Reply

Hi I’m 62 year old male started with enlarged liver and spleen originally

I had a first bone marrow test shown nothing they tried predisolone steroids high dose but to no avail

I was referred to a gastroenterologist he asked if I worked with chemicals which I did and said there’s your answer

I was then referred to Manchester Royal from Wigan hospital they did lots more blood tests all negative and second bone marrow test also negative but my spleen was getting bigger killing off my red blood cells

I had a splenectomy a normal spleen is 105 grams mine was 580 !

I was ok with splenectomy out in 5 days now on V K penicillin daily

But after my splenectomy my platelets rose from 250 normal to 945

I got blurred vision my liver was fatty I had an angina attack and Haematoma

I’d been dismissed by the Haematolygist for 12 months appointment

But my GP sent me to another Haematolygist who done a Jak2 test two years after my original symptoms and it came back positive!

9 Haematolygist said they’ve never seen this clinical presentation before

Chemical cause severe and unusual symptoms

Obviously yours I hope are not through chemicals but I’m on hydroxycarbamide 1500 daily my platelets 420 now I’m on Clopidogrel asprin allopurinol and other medication but apart from fatigue and still getting blurred vision joint pain it’s managable

Don’t get down keep positive they will sort you out I’m sure

1 like
Reply

What chemicals do you work with?

Reply

Skydrol contains Benzene Mastinox now banned substitutes used now organic solvents contained Xylene

Trimethylbenzene petroleum barium chromate and strontium chromate

Reply

I too was asked by dr if I worked with chemicals and the answer is yes. Also organic solvents etc. I am sorry to hear you have a complicated condition.

Reply

Hello! I was struck when I read your post here about how many similarities there were with my own medical history. From being a relatively healthy 42 year old, I ended up in A&E with really bad stomach and back pain a couple of years ago. I was diagnosed with a portal vein thrombosis, and then with PV as the underlying cause.

They put me on blood thinners (warfarin), omeprazol, anti nausea and strong pain relief to deal with the thrombosis and used venesection to deal with the PV, though since then I’ve moved initially to Aspirin, then Aspirin and Pegasys as my blood counts weren’t that stable (they venesected me so often to control my red blood cells that I became acutely anaemic and my platelets and white blood cell counts were both rising above safe levels).

The symptom burden was immense for me - even today I am still on really strong pain relief (tramadol and morphine) daily just so I can function - I think the source of pain for me is a combination of the damage done by the thrombosis and the PV. I get significant fatigue - to the point where I need to sleep for an hour or two in the late afternoon most days and I still live with near constant nausea (from the clot and the damage it did to my digestion system).

Like you, I had osophegeal varicies banded, initially every three weeks, but now I’m out to every six months now (I hate that procedure!).

Anyway, back to your questions. The BMB will likely show that you have ET, but you already know that anyway. It will also tell you if there is damage to your bone marrow. Eventually, with all MPNs there is a risk that your bone marrow will over-work creating so many new blood cells that it will fail and turn into scar tissue, leaving your body unable to create new blood cells at all. This risk is small but increases over time, so the comparative risk of progression is relatively higher for a younger person diagnosed with an MPN than an older person.

The BMB will tell if you have scarring in your bone marrow - it is likely that you won’t, so hopefully that will give you some peace of mind, or if there is you’ll know how bad it is, and whether proactive treatment is necessary.

The spleen can become enlarged with any MPN, as it’s dealing with more blood cells than it should need to, but particularly if you have a portal vein thrombosis then the flow of blood in that area can be restricted and the portal vein back pressure can also make the spleen enlarge too as it’s not as easy as it should be for the blood to leave the spleen. My own spleen was over double the normal size when I was diagnosed, and it has caused me some problems because it displaces your stomach somewhat, making you feel very full when you eat, but you didn’t mention anything like that so it’s possible that your spleen is not too enlarged - again it’s better to know if there is a problem there to be managed or not.

All of this is a massive thing to deal with, and it’s no surprise that you end up feeling down. For me, dealing with the uncertainty of where my PV is going over time has been hard, and the constant pain and nausea has made “normal” life really difficult since diagnosis. The thrombosis adds to what your body has to deal with, and the combination of PV or ET and a PVT is pretty uncommon, so my consultants (and I have many - 9 at last count) rarely agree on the cause of a particular symptom, of how it should best be treated. I have found a need to do a fair amount of medical research myself so I can try and arbitrate between them to be necessary, which again adds to the toll your illness has on you.

I have young children too - our twins are seven now, and all this uncertainty really upsets the planning of a normal family life and the impact of this inability to plan for the future takes a real toll on a family. I can’t predict how I’m going to feel at any given point in the future - am I going to be having a good day or a bad day, such makes even planning family days out or holidays really hard. The impact of my symptoms and drug side effects have made maintaining a full-time job really difficult, but I’m the sole income for my family, so I can’t afford not to work.

When combined, all these things make life really quite difficult, and as much as I’d like to say that I’m confident that none of these medical problems will impact my life expectancy, I can’t. For me, before I was ill, I guess I thought I was about halfway through my life; we’d have decades to see the kids grow up, retire and reap the rewards of a lifetime of career working - suddenly all of those plans seem in jeopardy. The impact of this - not the purely medical symptoms, but the realisation that decades may now be 5 years, 10 years even 20 years - I won’t even have hit retirement age by then - it’s a lot to take in, and it causes a lot of pressure on both the sufferer and their family. Many doctors don’t understand these psychological impacts - they want to treat the physical symptoms but dont address to mental stress that accompanies these rare and complex illnesses.

So, you’re not alone in feeling the way you do - I’m incredibly lucky to have a hugely supportive wife and family, and we tackle all these challenges as a team, but don’t be afraid to ask for help if you’re struggling to cope. Your doctor should be able to refer you to a psychologist or a psychotherapist if you’re feeling depression, and groups like MPN Voice, Bloodwise and Macmillan can help with everything from buddy programs to complimentary medicine, advice on medical issues, employment and financial advice - there is a huge amount of help if you need it, but you’ll need to be proactive and ask for help (and that can be hard, I know).

The diagnosis you received is a life changing event - no matter what path your illness actually takes. No-one can tell you what it means to your life expectancy (though everyone wants to reassure you), as every MPN sufferer is different and that uncertainty is another burden you have to live with. The good news is that you don’t have to deal with it alone - there is lots of help available to deal with the non-medical as well as the medical impact.

Sorry for such a long reply - I hope your BMB and spleen ultrasound don’t show anything unexpected, and your time without symptoms continues for many years!

Paul.

PS. Before my diagnosis, I never felt the cold - I’d happily wander around in the dead of winter in shorts and tee shirt but now I feel the cold terribly. One of my consultants told me that this is entirely down to the blood thinners I take to combat the thrombosis - I hadn’t even thought of that being related but apparently having “thin” blood really does make a huge difference to how your body copes with cold temperatures! My INR has gone from 0.6 when diagnosed to about 3 now so I guess this makes sense.

7 likes
Reply

Paul

Good post. Just to clarify, my understanding is that the progression risk per every 10 years is significantly lower the younger you are. I think you are saying that the longer you live the greater the cumulative risk.

Also I was told progression risk is average 10-15% every 10-15 yrs, same for ET and PV but it seems clear from research on the Web that ET about half that.

I assume you have had a BMB? Was this clear of fibrosis? And do you have a JAK2 Allele Burden %?

I’m WHO Grade 1 fibrosis, Allele Burden 60%, Spleen 14 cm and still on venesections/Aspirin but hoping to now start Pegasys. Low symptom burden.

My thoughts on life expectancy are:

We are at greatest risk of a stroke whilst on aspirin/venesections, HU or Peg significantly reduces this risk.

If/when we progress to MF, Rux and Peg (sometimes in combination) can slow down progression for the majority of patients, perhaps buying another 5 years. Ideally at this time would get chromosome testing to identify whether one has those mutations that fast track MF progression. If +ve, this would clearly swing decision towards early BMT.

The final decision is if/when to have a BMT. I note that recent breakthrough re GVHD (the principle risk) so I’d hope that within 5 years, this will transform prognosis.

Plus there are some encouraging trials ongoing such as Ropeg and especially RG7388 which is targeting other mutations than JAK2. Since JAK2 was identified, the bulk of the research has been focused on reducing Allele Burden but there is clearly something else as well that is causing progression. Now that this is getting more attention, I feel that there is good chance of a major breakthrough within next 5 years. Perhaps RG7388.

Bottom line is we are so lucky compared to those dx 10 years before us. Our experts are learning more every day. We just have to stay as well as possible (ie eat good diet and exercise, don’t stress) and give them five years to crack the progression nut. The ‘cure’ nut may take longer but it’s progression that is our priority.

How are you getting on with Peg?

Best

Paul

2 likes
Reply

I was so upset to read your post and the anguish you are suffering. I was also upset to read one reply about only having 5, 10 orc 20 years left. That is poppycock. Once your blood is stable and remains so you can live a normal length of time. The bone marrow biopsy is the big one.

All of us with an MPN have enlarged spleens, it's all part and parcel of the disease

I have to correct this statement as I don't want people to be alarmed

I have checked this with Prof Harrison and she has said that not everyone with a MPN will have an enlarged spleen. Maz

but it's all down to the BMB. Your bone marrow will have been working overtime producing all these red blood cells and like all things that work too hard it can become damaged, in this case scared. If it becomes to scared it will then struggle to produce the red cells.

Please wait for your biopsy results and then tell your consultant all of your fears. The worst thing you can do is hop from site to site reading horror stories and things that are just not true which will only scare you to death. This site is the only one you need because everyone of us has been through the same thing. This is not a death sentence but it will change your life because of the symptoms but we all pretty well live a normal life around the fatigue.

Please go to the MacMillan Cancer site and read up about ET then you will officially read that people with this disease live a full life.

Sending hugs to you now get out there and enjoy yourself

Jill xxxxxx

6 likes
Reply

Tomorrow is the BMBday! Just a pitty, my partner needs to go to his mothers funeral so he cant come...so, that is that.

Reply

Hi Swede. I am so sorry you are feeling depressed and bleak about the future . From what you say this came about re Facebook advice. To have a bmb is just another check on how our bodies are dealing with our MPM. You have been feeling ok with your meds and as it was you that requested it not your Heamo, suggests that he/she is quite happy with how you are doing. So I don’t think you need to be worrying so much , ( we all wobble at times re our futures and longevity ) but reading all the posts here from these good knowledgeable fellow sufferers really is uplifting and positive. I totally agree with Jill when she writes not go onto other sites and be drawn into depressive reading. I think this has caused you unnecessary worry. Once your BMB results are back ...(I know we all are jumpy till we get them....) .you will be reassured.

I do hope that you have been uplifted somewhat by the posts you have received.

Love Sandy x

3 likes
Reply

I too have ET, I am now on Anagrelide, (plus aspirin) after I developed side effects to hydroxy after being on it for 3 years. Carry on with your plans for the future, ET shouldn't affect them! ET sufferers usually have the same life span as others who don't have it! You could have something a heck of a lot worse. I get loads more trouble from my arthritis than the ET. Also don't believe everything you read on the internet, guess what it's not all true!! I sometimes look at facebook groups for ET and there are some very strange remarks about what ET does and doesn't do to your body, I wonder whether the people who put them on actually have ET. You'll get some good info from this forum. I have had a lot of reassurance from other members on here.

Best wishes for the future

Lizzie x

2 likes
Reply

Thank you all for taking your time to answer! You see, I have always said that I have really been well, all my life.BUT when I recently had to tell a friend that asked...I seem to have been ill, in various ways and things ALL MY LIFE! E.T is the one that really got the best of me. I have bronchiectasisis, asthma, had malaria, measels, mononucleosis, alopecia, cyst on one ovary et cetera... knee operations, hernia operation, ankle operation...the list goes on BUT I was thinking I was well, compared to others! When E.T struck, I then became ill for real and now I might get total depression and a new diagnosis I can't handle.

As I now know that there are changes (after the ultra sound yesterday), I am thinking there will be sad results from the BMB as well... ...

I do understand that I can't just sit here and be worried and sad but it is easy as the weather is horrible and I (we)can't plan for going back to Spain again, as I haven't got the appointment date for BMB yet.

Just need to mention that my partner is the best! He just don't won't me to see him worry so he hides that. Also, we sold the practice a year ago and the new owners are just making our life a hell, so he is dealing with that, but the stress is ....well, stressfull!!

Anyway, I am glad you are here and I will be back,somehow. As the song goes -" thank you for being my friend"!

Reply

Hi Swede

You mentioned you have had to get over (and still have) so many other issues. The main point is that you always considered yourself well. ET is no different. You just need time to accept you have it, maybe make a few changes to your everyday life, and feel sure that the strength you had to overcome your other issues will still be there to get you through this worrying time.

I felt totally adrift when I was first diagnosed in November but the kind comments and support from people here made me realise that if they can live with it, so can I. I feel much more at peace with my ‘lot’ now and realise that I can be involved in my treatment and make the changes to my life that’ll give me a good life - whatever the fates have in store for me!

Try not to worry. Perhaps if you share your worries with your partner (if you haven’t already done so) - he sounds very supportive from what you have said and may just need to hear from you how you are feeling about things. A big hug works wonders!

All the best to you and keep posting to your MPN family.

Gill xx

1 like
Reply

Thank you, that is very kind of you. I have today had such a strong pain in my lower back so now I have a million other diseases aswell!!! ;-)

Yes, Mr Swede is very supportive and he is medically trained so he knows what's what. BUT right now he has so many other problems to deal with, so for example tonight he probably need to work all night (just because we been away so long). I feel he must stay well and strong for my sake. He does hug very often or just stroke the shoulder or so.

Noone in my family is ill, my grandparants and their parents died at 90+ age...I am the only one with an illness or 7....

I must as you all say, get a grip!

I will tell you the result from Wednesdays meeting with the Haematology doc.............so watch this space!

Reply

Hi there,

Well, one BMB later.....the result shows no fibrosis or blasts so that is good. I still have the same diagnosis and I was asked by the doctor to take less medication. So, I guess that is good somehow. My mental state is a bit better I think, and hopefully can keep that up a bit better than before. Just worried about...I never used to worry about things! Stupid me!

Anyway, thank you for your support! I will be back...

1 like
Reply

You may also like...