Hi everyone I'm new here, from NZ with ET JAK2+ plus low Von Willebrand Factor VIII

[Wentry]

I live near Wellington, New Zealand (originally from the UK) and I was diagnosed with ET JAK2+ on 24 Nov 2017 after 3 months of waiting to find out, my GP only referred me in August when my platelets hit 780 and 620 (still 650 2 months ago and don't have latest result yet). Looking back through my blood results (regular for high blood pressure) that I have now managed to get, I have had high platelets (over 500 and sometimes over 600) for over 5 years, but the GP hasn't ever told me or explained the relevance or done anything till now. I've been on the site reading posts and learning as much as I can for over a month now, so I feel like I know some of you already, but I wanted to wait for my diagnosis before posting for the first time

I have had bleeding complications from surgery at least 3 times even though my platelets aren't high enough to be associated with it, so have been screened for Von Willebrand which came back quite low, re-screening in January, if it's low again they will do more tests to see what type it is and if I've always had it or acquired it. Does anyone else have ET and Von Willebrand disease or Acquired Von Willebrand?

Wendy

[Melaniem59]

Hi Wendy

I’m also Et, jak2 & have low vw, I see Claire Harrison at Guys & was referred to the haemophilia clinic at st Thomas’s in London

I was told that it is common in ET but is not usually tested for unless there’s a reason, ie. bruising or bleeding so people are not aware

As the platelets rise, the vw goes down, at first I was told not to take aspirin while they looked into it but I’m now back on aspirin

Hope this helps

Melanie

[Melaniem59 in reply to Melaniem59]

I forgot to say if you’re blood group o then vw will naturally be low (I’m not) it’s more likely ET but that’s a possibility 😊

[Wentry in reply to Melaniem59]

Oh I didn't know that, I'm actually O negative, I'll ask my Haematologist about that next time I see him I've actually had the surgery bleeding problems for at least 5 years and also had other signs of low Von Willebrand even before the platelets rose so I'll just have to wait and see if anything comes of it. My Haematologist won't let me take aspirin until the Von Willebrand is investigated further, the second screening results won't be until mid Feb as I'm not doing the test again till I get back from the US and Europe mid Jan. It is slightly worrying not being able to take the aspirin as I'm flying four long haul flights over 10 hours over the next month, two of them with less than 24 hours between, I have my flight socks and will drink lots and move around as much as I can and hope for the best!

[Melaniem59 in reply to Wentry]

Hi Wendy

You are probably inherited vw & not acquired then, as you already had the bleeding

Does your haematologist know about your flights, maybe ask if you should take the aspirin before you go back as it does put your mind at rest a bit, I fly a lot & was more worried not being on the aspirin

In the end the haemophilia clinic dismissed me & implied it was all a waste of time as the two go so closely together!

It was just more worrying about something they then said was not important 😕

I think they are doing more studies into the link & one dr wanted to investigate mine more but the other didn’t so it wasn’t dropped

Good luck anyway with your trip & nice to talk to you

Melanie

[Wentry in reply to Melaniem59]

Hi Melanie

Yes he does know about the flights, but he said unless my platelets get higher then he doesn't want me to take it yet until the Von Willebrand is investigated further, they were 650 at the beginning of October. My next appointment with him won't be until May unless it gets much worse, but I'm on monthly blood tests (had one Friday last week) so he is keeping an eye on them and I've asked the lab to copy me with the results too, plus I'm seeing my GP tomorrow so I'm hoping he will also have the results. If my platelets are still in the 650 or over range then I will email the Haematologist and ask him if I can take the aspirin, hopefully it's not over a million as he said he'd put me on Hydroxyurea if that happened, it could complicate going on holiday next week so fingers crossed! I do get symptoms including fatigue, headaches, occasional dizziness and the crescent aura that quite a few other people seem to have, with flashing coloured triangles.

I take multiple medications to control my blood pressure (inherited from my Dad) and I'm already at a higher risk of stroke as it hasn't always been very well controlled in the past, though thankfully it seems to have settled down on the newest tablet I'm on, and I also have arrhythmia since three years ago (I wonder if this was related to the ET), controlled with medication, so to have this on top of those was a bit depressing at first to say the least but there are lots of people worse off than me so I'm staying happy and positive now I've accepted it

So the haemophilia clinic didn't think your low Von Willebrand would actually cause bleeding with surgery etc. or that you would need to bump it up somehow in advance of surgery, or they didn;t even mention it? I have read a study looking a ET and Von Willebrand and they estimated I think that up to 20% of the people with ET had low Von Willebrand factor so I guess they could be right about the two going hand in hand.

Yes lovely to talk to you too, I don't know anyone else in the Wellington region with an MPN and I think there only two possibly three others in New Zealand on MPN Voice, but we're scattered around the country so not easy to meet up! This forum is my only way to talk to people who understand what it's like. Thank you for the good luck, I feel like I need it ha ha

Wendy

[stillkicking in reply to Wentry]

Hi Wendy,

I am living near Dunedin NZ, and also originally from the UK:-). After deteriorating health last year, I was finally diagnosed earlier this year as having ET JAK2 negative. Like you, I waited months for test results to come through. Dealing with haematologists and GPs has not been straight forward, but I do feel supported by my local GP, even if she knows very little about MPNs, she at least does treat me as a human being and has battled on my behalf at times when I have needed it! The fatigue, headaches, visual migraines, were a worry, but most things, other than the fatigue, has been helped greatly by the daily aspirin that I take. My platelets are probably around 1100 or so (I'm waiting for my latest results). I'm 59 now, so may start HU when I turn 60, although my current haematologist says that there might be some flexibility with that, depending on how all the risk factors are looking.

Anyway, kind thoughts to you, I know that we MPN people are rather scattered around NZ, but if you are ever down this way, you are most welcome to drop in for a cup of tea!

Peter

[Wentry in reply to stillkicking]

Hi Peter

I have noticed I have more fatigue than usual and have had the visual aura occasionally for years but it has become much more frequent in the last year, I don't get frequent headaches at the moment but have had in the past. I can't take aspirin yet until they sort out if my low Von Willebrand is a problem. With the risk factors I have I think my haematologist will put me on HU if my platelets go over 1000 even though I'm only 55. I kind of feel my GP should have referred me sooner item my platelets have been well above normal for quite a long time and with my other risk factors but he didn't so it is what it is

I'm just off to the US, UK and Germany tomorrow, but in definitely come for a cuppa if I'm down your way. Fiona isn't far away from me in Palmy

Have a great Xmas and good health for the New Year!

Wendy

[stillkicking in reply to Wentry]

Lovely to hear back from you. Wishing you all the very best for your travels and a happy Christmas. P

[Mazcd Partner MPNVoice]

Hello Wendy, welcome to our forum, if you haven't already, I would urge you to read as much as you can on our website mpnvoice.org.uk I am sure this will help you, there are also lots of very useful videos and real stories that may help you. Best wishes, Maz

[Wentry in reply to Mazcd]

Thanks Maz, yes I've been having a good look around for a month or so now and it's been so helpful, my Haematologist was quite impressed about how much I knew when I finally got to see him, I think it has encouraged him to discuss everything in detail with me because I know what he's talking about I'll have to try and time one of my UK visits in the future so that I can come to one of the Forums

[Mazcd Partner MPNVoice in reply to Wentry]

Hi Wendy, it's nice to hear that you were armed with information. Hope you can make it to one of our forums in the future, it would be lovely to hear about how MPNs are managed in NZ, and also to meet you of course. Best wishes, Maz

[linds]

Hello Wendy

How nice to welcome you on the site all the way from New Zealand. Have you ever thought of having a buddy? When you are so isolated from other MPN travellers it could be a helpful thing for you. Hope you have a lovely holiday.

Linda x

[Wentry in reply to linds]

Hi Linds,

Thank you for such a warm welcome I have thought about getting a buddy but haven't looked at it to work out how I go about it yet, I will do that very soon though as I'm sure it's a great idea. And thank you, I'm really looking forward to my holiday although I will have to do some work as I work for myself, I feel like I need it after all this happening

Wendy x

[linds in reply to Wentry]

Hello Wendy again. Just a quick message - Maz would sort the buddy out no problem xx