Newbie to mpd/et

[Jackybuckley]

Hi I've recently been diagnosed with mpd et and looking to learn more about it and how it could affect my life,lifestyle and longevity ,also as I'm on hydroxacarbomide was wondering if anyone on here their mpd/et had turned into leukaemia because of the medication used? Strange thing I've had bmb which confirms I have et,but so far all the genetic testing's have come back negative. The consultant is still waiting on some of the tests and he is confident due to bmb that this is what I have got ....but I'm just confused as to why nothing has turned up so far in the tests I've had done

[WileyFrench]

Welcome to “the club”. I have ET, no mutations and my blood work said not ET but like you, my BMB said it most likely is. So I’m on HU (for the past year) as well. Loads of people on this forum have had ET for decades and have been taking the meds, and are just fine. My haemo says that’s the majority of cases and the Canadian Cancer Agency concurs: very small percentage become leukaemia. Plus research is going on all the time, so new treatments come along. I think you’ll get some good info on this site. It’s certainly been useful for me.

[Jackybuckley in reply to WileyFrench]

My blood tests did reveal ET as did bmb my platlets and neutrophils are all over the place at the moment, and I'm on hydro for 5 days per week,one week then every day my haematologist is seeing me every week and at moment my bloods are still concerning them. I was hoping they were starting to stabilise,but unfortunately they're not yet ,I have great faith in the team at new cross and they have been so thorough in telling me everything that's going on,but obv I suppose until they've got the full picture of my genetics in front of them then they're as much in the dark as I am it's so good to be able to find a forum with people in the same boat .....thank you

[ELIZBEN]

Hi and welcome.

I also have triplet negative ET. I only take a daily dose of aspirin at the moment and have regular check ups every 6 months with my haematologist.

This is a great place to get advice and sometime just let of steam

J x

[katiewalsh]

Hi and welcome to our group. It can take awhile for the HU to bring your numbers down so don’t worry. Also, don’t worry about your ET developing into that particular type of Lukemia. As you learn more you’ll discover that most people with ET never progress or transform to MF or that. In fact, literature says that most people with ET will live a normal or near normal life span & die of something else. But all your concerns are normal and most of us have had them too so asking questions on here is a great way to learn. The NCCN website has excellent & detailed information on the various MPNs. It’s the best source I’ve found to learn from as a beginner or just as a refresher. (Just google “NCCN MPN”. The most important thing is to have a good MPN expert overseeing you if possible to make sure your treatment is appropriate. Either directly if you live near one, seeing her/him periodically or working in conjunction with your local doc. I was diagnosed in January, 2014. I thought my diagnosis was ET but recently learned it was actually pre-fibrotic MF. Even though this diagnosis would suggest I had no fibrosis, I actually had stage, or grade, 1 fibrosis. Im also jak2 +. I’m very stable so I just see my specialist twice a year and my hematologist oversees my labs. This is a great place to ask any question even if it seems silly to you, express anger, frustration or complain, ask for support if you’re having a hard time and anything else that’s going on. I’m sorry you had to join this group but the people on this forum are amazing. One other source for information which you might like is Patient Power. Best of luck to you! Katie

[Wyebird]

Welcome!- I can’t add much more it’s all been said- I was very active prior to diagnosis. ET Calr.

It took me three years to come to terms with my illness and even now it gets me down occasionally.

Give yourself time and ask as many ‘silly’ little questions as you want. Someone on this fab site will advise you.

[hunter5582]

You will find a whole realm of information on thrombocythemia and the other MPNs. These rare disorders we share require a specialized set of knowledge and experience that is not always easily found. Many docs, even hematologists, do not have much experience with MPNs and thus they default to the "standard protocol" for your treatment. While these protocols may be r the right choice for many patients, they are not the right choice for all. Each person can have a unique presentation of their MPN and the cooccurring medical conditions that are sometimes related and sometimes not. You deserve a treatment plan that is based on your unique presentation of the MPN and your other medical conditions. You deserve a treatment team that has the requisite knowledge skills and abilities to provide you with quality care. You will have to educate yourself about MPNs and then be a tireless advocate for your own care to receive the care you need for your MPN.

Here are a few factoids from the current and emerging research that you might find relent. Thrombocytosis can be a primary disorder (essential) or secondary to another medical condition. Thrombocytosis alone does not necessarily increase your risk of thrombosis. It can actually increase your risk of hemorrhage. Alteration of von Willebrand factors/function that drives the coagulation cascade can occur when platelet levels are in the 800Ks (this is a replicated finding, but new in the research). You are at increased risk for thrombosis if thrombocythemia occurs together with erythrocytosis and/or leukocytosis (e.g. polycythemia vera). You are also at increased risk if you are JAK2+. There is lots more to know - these were just a few snapshots of the information available to you.

Regarding hydroxyurea (HU). HU is a commonly used medication that has been around for a long time and many docs rely on it routinely. It is cheap, readily available, and many payers may require you to try it first if you need chemo. Some people do in fact benefit from it with few adverse effects; however, it is not a benign substance and some people are HU-intolerant. HU is teratogenic, mutagenic, carcinogenic (e.g. skin cancer), and is suspected to be leukemogenic (controversial finding). You must not get pregnant while on HU (men wait 1 year post d/c/ HU, women wait 6 months). Women of child-bearing years not on HU should not even handle the bottle without gloves. HU passes into both semen and vaginal secretions so sexually active couples are advise to use condoms to protect the partner from exposure. For men, HU can cause hypogonadism (oligospermia/azoospermia and may cause decreased testosterone (the latter is new in the research). HU affects your immune system and you must avoid live vaccines and be careful about exposure to infections. Mouth ulcers are a fairly common adverse effect and should be considered a first sign of HU toxicity. One serious sign of HU toxicity is skin ulcers, usually on the lower extremities. this is caused by compromise to the vascular endothelium and can lead to gangrene. This is not common, bury very serious when it occurs. I am one of those who is HU-intolerant. Even on low doses, I experienced mouth ulcers, thrush, leukoplakia, and compromised intestinal endothelium (constipation and flatulence). None of this is to say that HU is not the right choice for you. you may benefit and do well on it. However, you have a fundamental right to informed consent. You cannot meaningfully consent to your treatment if you do not fully understand the risks and benefits.

I have had an MPN for 30+ years. It is not until the last year that I really came to understand my own disorder. I chose to educate myself be diving deeply into the professional literature and emerging research. I also had the opportunity to consult with a true expert in MPNs. I have two hematologists on my care team. My ongoing treating doc is a terrific hematologist who actually spends time and listens to me and answers my questions. I am only his 5th MPN patient in 20+ years of practice. I also have a consulting hematologist who is the Director of the MPN Clinic at Johns Hopkins Hospital. He has a unique perspective on MPN treatment that is informed by experience with hundreds of MN cases. He does not rely on standard protocols, evaluates each patient on a case-by-case basis and individualizes their treatment plan. Tis is a much better approach. It is worth the time, expense and effort to travel to see this doc when I need to. I found this doctor on this list of patient-recommended MPN experts mpnforum.com/list-hem./ .

As intimidating as it is when you first find out you have an MPN, know that most of us live rich, full lives despite the MPN. Sure - it can effect your quality of life, but there are ways to manage the symptoms and there is some really promising research and treatment strategies emerging. As you enter this journey along with all of us who have MPNs, I would encourage you to take control of your treatment. Educate yourself and learn how to advocate for our own care to ensure you get your needs met in the way you want them to be met. This forum is a wonderful resource for all of us who have MPNs. I have learned a lot from other MPNers and always benefit from the support I receive here. All the best to you. I hope you get the answers to your questions soon.

[Mazcd Partner MPNVoice]

HI Jacky, welcome to our forum. I would suggest that you read as much as you can on our website mpnvoice.org.uk about ET and Hydroxycarbamide, I am sure it will help you understand more about both. And also look at some of the videos of patients talking about living with ET, I am sure you will learn a lot from them. Kind regards, Maz

[Doggy1903]

To support you, I have been on hydroxy for about a year now. My doctor stated that I was not to worry so much about leukemia . There is a very small increased risk of this. However I think its good to concentrate on the fact that the meds help reduce your risk of a clot or stroke.I have not experienced any side effects and really wish you well too.