ET and approaching 60

[Freethorpe]

I am new to this forum. I was diagnosed with ET with CALR genetic mutation 5 years ago and have been on 75g aspirin per day since then. NHS guidelines say that I must start taking Hydrea or similar at 60. My platelet level is around 900. I feel well and am very healthy and fit plus reading on the internet that CALR is less risk for strokes etc. I never see a consultant at the hospital and no one will discuss taking into account risk levels and maybe delaying the start of taking the stronger medication. I am 'on my own' if I continue to only take the aspirin but I don't want to move to a stronger drug until I have to.

[Mwalimu]

I understand how you feel but think you should be more pro-active about seeing someone to discuss this. I would not be happy if my platelets were as high as yours - of course, I am older and almost certainly fatter!! Sallie

[Freethorpe in reply to Mwalimu]

Thanks you Sallie ! I agree - I am trying to find a new consultant who knows more about ET - it is difficult in the UK NHS - they don't like referring you out of your area. Am trying though.

[lindyloulou in reply to Freethorpe]

Hi

I too am in the uk, I live in Leicestershire but see an haematologist in Nottingham, this was not a problem at the time as I was working in Nottinghamshire. I have been more than pleased with my treatment. I would not be happy with those readings either. Pegasys is both my and my consultants treatment of choice, I do recognise that we are all different though and decisions should be based on that.

I am 63 and have been on Pegasys for 3 years, started it after the age of 60.

Good luck, ask lots of questions and remember you have the right to ask your GP for a second opinion.

Regards Lynn

[lindyloulou in reply to lindyloulou]

I was on Anagrelide prior to Pegasys, was 53 at the time of diagnosis, I too have Calr ET.

[Dovme]

Hi and welcome

I am 59 Jak 2 with a count of 840 on 100mg Aspirin. I live in Germany the rule here follows WHO guidance is age 60 unless your count is approaching 1500. I think much depends on your level of fitness, medical history blood count secondary factors etc. l probably will start the meds at 60 as l feel emotionally prepared and hope l will tolerate as most seem to do. I personally would also prefer to reduce risk of a clot. Discuss with your doctor it’s important to self advocate in these situations and do what’s best based on good medical advice

[IrishSarah]

Great to hear you’re feeling fit and well!

ET is such a rare condition and varies so much from person to person that I think it’s definitely worth going through the hassle of finding an expert to get their advice on your individual situation/treatment. You don’t have to take their advice if you don’t want to, but it’s good insurance to have that relationship started in case you need more help at some point in the very distant future.

Take care,

S x

[Mazcd Partner MPNVoice]

hi Freethorpe, welcome to our forum. We understand how you feel about having to start taking medication and it really would be best to discuss this with your healthcare team. When you go to the hospital for your appointments do you see a haematology nurse specialist at all, or do you just see a different doctor each time, as you said you never see a consultant.

This is a big decision and one that you do need to discuss so that you can make a decision, and of course your consultant will make the decision as to whether or not to start you on medication based on your blood counts, your overall health and any risk factors, like any previous blood clots or heart problems etc, so it is a lot of different things to be taken into account and discussed.

Best wishes, Maz

[Freethorpe in reply to Mazcd]

Thank you Maz. I commented in my reply below re the haematology team at my local hospital - I don't think they have an ET specialist and I have not seen the consultant once. Just a different haematology registrar (I think) each time. I agree there are lots of things to take into account - but I can't get them to discuss these with me. It has really helped posting on this forum because I have realised that I need to insist on something different !

[mhos61]

I understand your very genuine concerns.

You are CalR+ (lower thrombotic risk), very fit and healthy with platelets below 1500. This is definitely worthy of a discussion with your haematologist/team. You say NHS Guidelines say that you ‘have’ to begin treatment at 60. Has your Consultant or CNS actually had this discussion with you?

I have ET and was convinced by my Haematologist to begin treatment with hydrea at age 60, even though I was also fit and healthy. This was the right choice for me, as I have the Jak2 mutation, which is an additional risk factor for thrombotic events.

You can always be referred for a second opinion, preferably with an MPN Specialist, it is your right.

[Freethorpe]

Thank you. I attend my local NHS hospital in Norfolk every 4 months for a blood test and review. I have never in 5 years seen a consultant haematologist despite my GP sending a letter and requesting that specifically. I see a different registrar each time and I suspect many of them are trainees. All they do is repeat the NHS guidelines on hydrea at 60 to me and will not engage in a discussion about this being different depending on individual circumstances. I will find out how to ask for a second opinion as I am currently talking to my GP about it.

[mhos61 in reply to Freethorpe]

I sympathise, I always see the CNS too.

I was diagnosed in 2016 by a lovely Haematologist. I made a point of asking her would she be my regular Consultant, she confirmed that she would. That was the first and last time I’ve seen her. I understand this is because my condition is stable, but if it changed or, I wish to see her I can ask for a consultation. I do think I should be reviewed once a year though.

In your case, frequently seeing different Consultants paints a picture of an inconsistency of care, made much worse by a lack of engagement in your consultations, how frustrating that must be. Your thoughts with regards to treatment are relevant and deserve adequate discussion, so you can make an informed choice.

I hope you get that referral soon. Keep us updated.

Mary

[socrates_8]

Hey there Freethorpe...

My name is Steve... (61yo), I am Post ET / MF 'CALR+' Type2... i also have Von Willebrands Syndrome (acquired Type 1 or 2?)

Earlier on, (when diagnosed 4 years ago), I had two (2) TIAs, (minor brain strokes), and on both of those occasions I could feel that I had extremely high Platelets.

There are a few clues, (symptoms), like: impaired vision issues, severe headaches, and Extreme Tinnitus...

Today, I am taking Ruxolitinib, (Jakafi), and Low Dose 100mg Aspirin (Enteric-coated), ...

... however, my platelets are still a problem. My last Bloods they were 947...

I do not recommend allowing yourself to have a TIA, and therefore you might well need some type of help to reduce your Platelets back down to less dangerous levels, in my view...

There are a few options, but maybe Pegasys' Interferon might be the way to go... Just a thought, and at least worth chatting to your medical team about all the options that you might have, few though they are... unfortunately...

Stay safe & well...

Best wishes

Steve

[lizzziep]

I hope your GP can push for you to see an MPN specialist, it sounds like the people you are seeing don't know anything about ET except what they have read in books. My niece is a specialist nurse in a cancer department (not haematology), she had never heard of ET and has asked colleagues including doctors and no-one else has either. I usually see the specialist nurse, I very rarely see the consultant.

[Sksarj]

I was diagnosed aged 66 with ET I'm not Jak2 though. At the time of diagnosis my platelet count was 800 and rising. I was put on hydroxy and aspirin straight away. I saw the consultant at diagnosis and a few times afterwards then I once my platelets settled into the 300s and stable I saw a specialist nurse. That was over 7years ago. But I feel sure if I wanted to see my consultant she would see me. Hope this helps.

[mhos61]

nature.com/articles/s41408-...

The above link is an interesting article written by MPN experts on ‘Risk Adapted Therapy’ in ET.

Take a look at ‘Intermediate Risk’ and the reference to ET patients who are not Jak2 or MPL positive and have no prior history of thrombotic events and or cardiovascular issues??

[hunter5582]

Not having a consultant who is a MPN specialist is unacceptable, sub-standard care. If you do not have a MPN-expert doc locally, then travelling out-of-area to find one is what you need to do. The approach I use is to have a local hematologist (who I really Like - but is not a MPN expert) handle my on-going care. I travel 2 hours to periodically consult with a doc at the MPN Clinic at Johns Hopkins Hospital. This doc consults on my case and provided expert advice on my care. While I am doing this here in the USA, I know there are others who do something similar in the UK NHS. Here is a list of MPN-expert docs mpnforum.com/list-hem./ .

There are different treatment protocols for ET. I believe the WHO and British Hematology Society now have different protocols. There are different definitions of "high-risk" based on age. Some have moved it to age 65 . Regardless, just know the protocols for assessing risk vary as do treatment recommendations. Here is an alternative view and a great presentation regarding ET by the doc who used to be my consultant, Dr. Spivak. youtube.com/watch?v=hbVr9u3...(Not working as expected?) .

You will note that Dr. Spivak does not favor the use of hydroxyurea, though others do. My own view is colored by the fact that I am in fact HU-intolerant. Others have a different experience with HU. The good news is that if you need to take a medication to reduce platelet levels, there are other options. Some docs prefer the PEGylated interferons and some patients tolerate them better (but not all). There are other options as well.

The key question is whether you "need" to take more medication at all. Most of the standard protocols call for something at age 60 or 65. This is based on broad statistical projection of risks that may or may not apply to you individually. Standard protocols apply to many, but not all people. There is also an issue of individual choice and a decision about your own risk-tolerance. Which risks/benefits do you wish to take? Understanding what your individual risks are and what risks/benefits may come from different treatment options is what will need to drive your decisions.

Ultimately, you will need to decide what your course of treatment will be. You need to consult with a true MPN-expert doc to be sure you have the information you need to make the best decision for yourself. You need a doc who has the KSAs to evaluate your individual case and make a recommendation based on your unique MPN profile. One size does not fit all with MPN treatment. Do bear in mind that you are the one who makes decisions about your treatment. Doctors provide expert advice and recommendations. The patient makes all of the decisions.

Please do not take "no" for an answer regarding seeking expert care. You will need expert consultation to make good decisions for yourself. Assertive patients receive higher quality care. Passive patients do not. Do be sure to ensure yo get the care you deserve,

[JaK2ET]

Belated welcome to the forum! Have you managed to arrange a consultation with a real MPN expert? Good luck!