I am new to the forum and my wife (age 46) recently had a liver bypas (TIPS procedure) due to a clot in a vein (she also had a splenectomy 10 years ago due to a cyst on her spleen) and has just been diagnosed with:
1. ET
2. JAK2 positive
3. Budd-Chiara syndrome
It is very scary and I was hoping anyone might know the answers to any of these:
1. She is on Hydrea and Warfarin and has been vomiting and constipated quite a lot - is this something that changes over time or does she need to look into some other medication? She recently went 5 days with passing a stool even after taking many laxatives.
2. The haematologist (Simon He from Australia - he is one of the best so we are comfortable with keeping him) said she would transform to secondary Myleofibrosis (MF) within 5, 10 or 15 years - I was of the understanding this occurred in only 10% to 15% of cases (that's what I've found reading anyway) but he already says it will happen?
3. Is there anyone else out there who contracted ET in their mid forties (apparently this is young) who has had the illness for a while and could advise me of what their doctors are suggesting about the long term outlook?
Thanks
Seamus
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sobrien1234
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Hi Seamus, welcome to the forum. This is a great place for support and getting some input from knowledgeable people. I have ET and I am also JAK2+. I was diagnosed at 50 (53 now). I was put on Apixaban and Hydrea after developing a blood clot in the sinus vein in my head (3 months ago). I have felt a little nausea from the Hydrea and it has certainly taken me this last few months to become accustomed to taking it. I have found it makes me very tired but no vomiting. I also suffer from mild constipation but that pre-dates the Hydrea. I know some people cannot tolerate it so talk to the Doc and see what he says. I also have MGUS so I have long term risks from that too but my haemo just said I may get cancer in 10-15 years and to just have a blood test once a year and don't worry about it. Since my blood clot (and a different haemo, the last one having retired) the focus is on preventing further clots (and getting rid of the one I have). I have not had a BMB and was told that would only happen if there was a change in my Hg.
I have no idea why he would tell you it will transform to MF - you will have to ask him as that is not my understanding either. Again, ask the Doc. Next time you see him, take a list of questions you want the answers to so everything is covered while you are there.
I'm sure others will have some good advice for you. Sending best wishes and hugs for you and your wife. She is lucky to have you to support her.
Yes Gerard, but I only realised that much later after investigations. Originally, after diagnosis I did what the Doc said and didn't "worry" about it. I wish I had learnt more at the time as I may have been more diligent in taking my aspirin and avoided a blood clot however I had a lot of work on helping my kids and looking after a grandchild at the time so my health took a back seat. I also made several long haul flights that year which wouldn't have helped the situation.
I’m so sorry to hear of your wife’s ET diagnosis. This forum will be a great source of support for you both.
I really can’t understand how your haematologist can be so confident that your wife’s ET will transform into MF? I would ask at your next appointment what it is that indicates this.
If your wife is having ongoing issues with hydrea I would at least enquire about pegylated interferon as a treatment option.
Hi Seamus, I have PV and was diagnosed just before turning 50. Has your wife had a bone marrow biopsy? If so, maybe the haemo saw some signs of fibrosis there already - do check with him. But if she has not had a BMB then he cannot know if she will progress to MF or not. The risk of progression is cumulative, 1% a year. And it is a risk, not a certainty, the majority never progress in their lifetime. Given her age, she should ask to try interferon rather than HU. Pegasys interferon can halt and even revert progression in early MF, and can lead to remission in a small number of cases of ET and PV. It is advised for younger patients. I have been on Peg for just over 2 yrs and my bloods are now normal again. All the best to you both. Susana x
Hi Susana 7 can you tell me why Pegasy is not good for someone older? or where you heard that? I'm 66 and my local is recommending Pegasy. The expert I see does not recommend HU for older patients. So I'm very curious to know where that information is "it is advised for younger patients" I understand the pregnancy reason but most experts now agree that if your HX is no clots under 60 and asymptomactic should only be aspirin. Thanks for any info.
Hi Mickey, Pegasys can be used for all ages! But the reality is that, currently, in most countries, HU is typically the first line treatment for MPNs, with interferon being offered only if people fail HU, or if they are younger. This is because interferon is compatible with pregnancy and less likely than HU to cause long term issues for those having to take it for many years/decades (i.e. younger patients). Of course, which treatment is best suited to each person is a very individual choice: interferon works well for some people, but has nasty side effects for some others. For me, Peg has been a life saver so I am a great advocate of it. If you are being offered Peg by your local, my advice is to give it a try! All the best, Susana x
Hi Seamus and welcome to the forum. I was diagnosed with ET caused by the JAK2 mutation earlier this year and was prescribed Hydroxycarbamide and low dose aspirin. I also suffered with constipation for many days at a stretch, with little effect from the usual remedies. However, once my system had adjusted to the drugs I got back into a regular routine and am no longer affected by constipation. Make sure your wife is well hydrated and eats plenty of fresh fruit and veg. I found that helped.
I hope she feels better soon and be assured that she will always find support here.
Thanks John - I will adjust diet and see how we go. Lot of vomiting and constipation early days so comforting to hear there may be light at the end of the tunnel
just to comment on the constipation bit, i ended up in hospital with this plus gall stones from processing too many rbcs, got quite complicated, so don't take it lightly. My solution: eat the right things obviously, drink lots of water and 4/5 dried figs every morning.
I agree with everything Susana wrote before, I was diagnosed with ET 18 years ago at age 19, following a clot in my left leg. I was on hydrea in the beginning because we had to take down the platelet number as quickly as possible, but for the least 13 years I am on Interferon (regular, since pegylated is not available here in Croatia for MPN patients).
Your wife should try asking for interferon and maybe some other haematologist. As Susana said interferon is able to revert even existing fibrosis so even if BMB showed some degree of fibrosis I would try interferon first and then see what happens.
I can't help regarding Budd-Chiari but if you need more info on ET please feel free to ask.
Hi Zvinkovic I know someone in Croatia who is getting Pegasy so I think it is available you just have to keep pushing for it. She did and finally got it. Good luck.
Thank you for the info, is that person here on MPN voice so I could contact her? My haematologist say it is given for hepatitis only, and even regular interferon is used off label for ET, so she says it is better not to stir things up because I would maybe end up loosing interferon at all.
no she is on a face book site called essential thrombocyemia, LOL I know I spelled that wrong. I'm going to ask her how she got it but she could not take regular interferon, HU made her sick and AG caused too many side effects. So she kept insisting and got it but she is also younger and wanted to get pregnant.
Hi Seamus, we can all understand how your wife is feeling about her diagnosis and indeed you. Have a look at the information on our website mpnvoice.org.uk, there is lots of information on there which may help you both. I would ask her consultant to explain to you why he has said that she will transform into MF within 5 - 15 years and also speak to him about different medication if your wife cannot tolerate Hydrea. Best wishes, Maz
Hello! I was just diagnosis with ET, after a very long time of trying to figure out why I was having so many symptoms and no resolution on what was going on. My CBC's have been abnormal for over a year. None of my doctors said anything! Myself, as a pre-med students I have done my own research and advocated on my own healthcare and pursued and pushed through to top doctors that would manage and treat my cancer! Which I have dobe and now have resolution! Finally!
Hi. I just saw your post from a year ago & wanted to write in case you hadn’t yet learned some better information. I was diagnosed with ET, BMB showed some fibrosis but very little. An “expert said I’d probably live only 8 years. Turns out she was 100% wrong. The real experts have said it’s unlikely it’ll ever turn into actual MF. And many on here have had ET for 20-30 years without it turning into MF. Plus, those on here who keep up with the newest research say there are new & better drugs now for MF patients. Hope you& your wife are well. Take care. Katie
Hi. Katie here again. Only an expert in MPN really knows this cancer well. So although that doctor may be an excellent hematologist, he may not be up on the latest research & medical knowledge about MPNS. Things have changed dramatically over recent years. Is there an MPN specialist you two could see? Katie
Thanks for the feedback Katie. In fairness the hematologist said in 10 years time they'll probably have a cure so don't worry too much about it. He said progress being made is just escalating every year. Thanks again!
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