JAK2+ Questions

[P-O-T-S]

Hi my name is Barry,

I am new to MPNVoice, I'm 46 and after a rough couple of months with 9 days in hospital after a referral from my GP due to abnormal bloods that were taken as a precaution for routine check up when i rocked up with symptoms for a severe and rapid onset of pain in my right shoulder, which after around a week left me with numbness, weakness and lack of mobility which s still not corrected itself two months later. I have been diagnosed with Splenomegaly, enlarged varices of the duodenum. and have had my bloods checked for JAK2V617F which has returned positive. I have just this week been in for a BMB and am awaiting the results.

I was very ill for a time with various symptoms, hot flushes, itching, feeling like i have flu like symptoms, quite severe fatigue. when in hospital i had what seemed like little clots of blood in my nose when blown and i generally felt unwell. but two months on after much rest and recovery with only pain killers for the shoulder i am feeling much better! shoulder is improving, and although i can still feel the enlarged spleen which is uncomfortable at times its not painful, and my appetite is actually quite ravenous most of the time which is shouldn't be with SM. My question is dos the JAK2+ mean that i definitely have one of the three main MPN's? Due to the varied and not text book symptoms they have found it very hard to diagnose me and although i am aware that the BMB will provide them with all the info they need for the end game of diagnoses. until then i am kind of left a little in the dark, and curiosity is getting the better of me! Could the JAK2+ also be apparent in other similar illnesses?

Thanks you in advance for your time in reading and answering if you do

[PhilReade]

Hi Barry yes Jak2 positive means you’ll have one MPN

I to started with an enlarged liver and spleen two bone marrow tests was negative in 2013/14 but needed a splenectomy in 2014

Then in August 2015 was diagnosed Jak2 positive with essential thrombocythemia

I’m on hydroxycarbamide 1500mg daily

My clinical presentation has never been seen before but I worked with chemicals at Airbus they originally diagnosed me with haemolytic anaemia

But a professor at royal free London stayed the chemicals caused severe and unusual symptoms

I’d seen ten Haematolygist in total

My spleen was 580 grams when taken out it was pushing down into my lower abdomen when cycling I couldn’t walk was like an old man crouched forward

Hydroxycarbamide or similar can sort your spleen out normally

And your liver repairs itself after treatment don’t worry you’ll be ok I’m sure now they have you diagnosed Jak2 you’ll get the treatment needed

All the best

Phil

[P-O-T-S in reply to PhilReade]

PhilReade,

Thanks for your reply, I'm sorry to hear of what you have been through! it sounds like you have had a rough time. I believe my spleen was last measured to be about twice the size it should be which will make it around 20cm. its really more uncomfortable that painful. The main thing that frustrates me its the worry of a rupture which has stopped me cycling and doing too much sport related things at the minute. although i'm not too sure how well i would do with this dodgey shoulder which is still mostly undiagnosed. they are looking to see if it could some how be related. I guess for me its just a waiting game at the minute. I guess no point in making presumptions and causing unnecessary worry.

Kind Regards

Barry

[Wyebird in reply to P-O-T-S]

Hi Barry I’m no expert but I agree with Phil. I suspect the sooner you start meds , usually hydroxicarbomide the better. Your age, symptoms, blood counts and then of course bmb will determine treatment. I didn’t need a bone marrow biopsy as themutated gene they found confirmed the diagnosis of Et Calr.

I do wish you a speedy ‘recovery’

[Sarahjane100]

Hello. I am 53, and have had 2 appointments with heamotoligists at the hospital recently. I am jak2 negative, and also negative for all other gene tests. Meaning I'm triple negative. My platelets have been high since 2014, currently 590. They advised me to take aspirin every day, and I have another appointment in July. They think it's probably essential thrombocythemia, but won't diagnose until after a BMB. It's so frustrating! Can I ask, did you find the BMB painful? Sarahjane.

[P-O-T-S in reply to Sarahjane100]

Hi Sarahjane100,

The BMB was not painful, I was well informed of the procedure before hand. Definitely didn't watch any youtube videos or like before hand. the procedure doesn't last long. and an anaesthetic is used in the area what they enter numbing that area and the outside of the bone. the aspiration was very quick and painless, but slightly unusual feeling in the leg when they draw the blood from the centre which is apparently due to the change in pressure, levelling itself out. the second Bone marrow withdrawal was also very quick, there was a little tugging and pulling but its all numb so its not really painful just a little uncomfortable.

Its all over very quickly, it needs to be in order to keep the samples good. after you lie on your back for five minutes or so while you body adjusts to the change in pressure then your up and good to go, Its still all numb so there is no pain.

the next day its just a little sore but warrants nothing more that paracetamol if required. Generally these nurses are very well trained. i was lying on my side facing the wall before any needles or such was taken out from the cart and set ready for use. I intentionally didn't look as i didn't need to know any more than it was being done as its necessary.

I'm sure you will be fine if you need to have one done, and wish you the best of luck. Just remember these things are never as bad as we imagine in our minds!

Regards Barry

[Dianne-Guisborough in reply to P-O-T-S]

Hi Barry

What a detailed and reassurring description of your Bone marrow biopsy proecdure. I am Jak2 + P Vera and so far haven't needed a BMB but it's something that I know i may need at some point . I have always viewed it as a bit "oooh not sure whether I will like that too muvh !"

Your descriotion has really put mine, and i'm sure alot of other peoe's minds at rest.

Thank you for taking the time to post your experience

E hugs

Dianne x

[P-O-T-S in reply to Dianne-Guisborough]

Hi Dianne-Guisborough,

I'm glad it has helped put your mind at rest, for the future if you find you need to have one done. it certainly helps to share experiences, good or bad i guess.

Regards

Barry

[Sarahjane100 in reply to P-O-T-S]

Thank you Barry! I know I will need one in the next few months, so thank you for setting my mind at rest! I wish you good health and good luck for the future. Sarahjane.

[hall2 in reply to P-O-T-S]

Hi Barry were you able to drive afterwards? Staff at the hospital where I'm due to have a BMB in a couple of weeks have said yes I'll be fine to drive home.

[P-O-T-S in reply to hall2]

Hi Hall2,

I didn't drive, the Misses took me but that's mostly because i'm not driving at the minute due to the lack of movement in the shoulder! I felt a little light on my feet for say the first five minutes of walking afterwords and wouldn't have worried about driving personally.

I hope that helps

[hall2 in reply to P-O-T-S]

That's great thanks!

[Sarahjane100 in reply to P-O-T-S]

Sarahjane again! I just got home to a letter from the hospital telling me my BMB wI'll be done in 2 weeks time! So nervous, but glad to be nearer a diagnosis hopefully. Have a lovely weekend, hope the sun shines for you!

[P-O-T-S in reply to Sarahjane100]

Hi SarahJane,

Don't be nervous. I know its easier said than done. As i understand it will help to diagnose weather you have the condition and what stage it is at. I fully understand as it is the unknown that plays on your mind. Try not to worry, you'll be fine and have a good weekend yourself. It looks like its shining for most in the UK or at least that's what the weather man is saying

[Sarahjane100 in reply to P-O-T-S]

That's true, it's supposed to be lovely! Thanks so much for your support, will post and let you know how it goes!

[Mujer in reply to P-O-T-S]

Dear P-O-T-S

Thank you for such a reassuring reply to sarahjane100 and I can confirm what you say having just had my second BMB just last week. The first was carried out in southern Spain some 15 months ago and the second here in Scotland. I took paracetamol the same night and again the following day but was absolutely fine after that. The procedure is quick and painless with just the sensation you describe.

Best wishes to all in the MPN family as we journey together.

Mujer

[P-O-T-S in reply to Mujer]

Thanks for your reply Mujer,

I just tried to explain it as best I could, I think we all fear the unknown and we often don't need to. although everyone is different. I am glad you second time wasn't too bad and it supplies the results you are after. an improvement and no progression

We are indeed so few and far in-between. That said I am still awaiting my full diagnoses.

All the best

B

[Mazcd Partner MPNVoice]

HI Barry, sorry to hear that you have been having a bad time, hope you continue to improve. It would be best to wait for the result of the BMB so that you know definitely what you have and then you can move forward. Kind regards. Maz

[P-O-T-S in reply to Mazcd]

Hi Mazcd,

Thank you for your reply. I kind of expected that response and its an appropriate one, its the not knowing that gets to you! I'll update after my consult.

Thanks

[Dianne-Guisborough]

Hi Barry

Sorry I meant to saythat I i hope all goes well with your results

Kind regards

Dianne

[Paul123456]

Barry

My understanding is that JAK2+ve doesn’t mean you definitely have an MPN. Please see below.

health.pa.gov/My%20Health/E...

[P-O-T-S in reply to Paul123456]

Thanks for the link Paul123456, its very helpful. Interesting read.

[Lilly1958 in reply to Paul123456]

Thanks Paul for this site , you are very educate person , and i learn samting new . My son is 34 year old and I push him to doing Jak 2 , becouse I have Et and Jak 2 pozitiv , and my ET start at same ages 34 . He is too Jak 2 pozitiv , but platets 375, 2014 was 285, 2016 was 332, and 2018 2 times 375. I didn't now person with Jak 2 pozitiv can be normal helthy and no MPN,but I push him to have BMB . Thanks 100 times!!!

[Paul123456 in reply to Lilly1958]

Lilly

Thanks for your kind words but I’m not able to advise. Do you know his % of JAK2 mutant gene?

If he does have ET, it appears very early and pretty stable. If it was me, I’d not be worrying about a BMB unless my Hem advised it.

There can be a family link but my understanding is under 10%. One Hem I see is very interested in this aspect.

Best wishes Paul

[Lilly1958 in reply to Paul123456]

Not, I don't know his % of Jak2 jet, But yes hem advised BMB. My family history not really good , mum had bone marow cancer, my daughter (I advised her to hem) have MUGS condition and I really worrying . My daughter had BMB and confirm MUGS, but protein is only 2 And stable for 2-3 years , shy is have second baby on this condition was ok. I houp my son be ok, but only BMB can give as answer . Thanks Paul . Best wishes to you to Liv long, long and give as lot God information !!!

[Symba7]

MPNs

[Meatloaf9]

Hi Barry,

My understanding is that being Jak2+ in combination with certain blood levels may mean you have a MPN, I think it is possible to be Jak+ and not have a MPN. You did not say what your blood levels were - platelets, hgb, hct, wbc, etc. Your BMB will probably give you the answers you are seeking. Good luck to you.

[P-O-T-S in reply to Meatloaf9]

Hi Meatloaf9,

Thanks for your reply, when i was in hospital my Hct was 58 i beleive but after leaving hospital several weeks later when i returned for my Haematologists apt my levels had apparently come back down. what they were i wasn't sure and to be honest i probably wouldn't have understood what they meant. I'll have to make an effort to ask in future for the info and record it. My understanding is that My Haem thinks that it may be possible that i had an MPN and didn't know and it's progressed to another hence the unusual numbers in bloods.

All the best