Just a newbie but desperate for independent info... - MPN Voice

MPN Voice

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Just a newbie but desperate for independent information and experiences!

Quercus53
Quercus53

Just a newbie with ET CalR. Still a little bit in denial as my diagnosis was not handled well at all. I was having regular blood tests for RA and my practitioner noticed my platelets were slightly elevated, 500ish.

I was given an appointment for Haematology and suddenly found myself in the Cancer centre. Surely this wasn't right, I just need my blood checked?

I emerged from my appointment quite shell-shocked, I had blood cancer! I went to the appointment on my own so I probably missed a lot that was said but when I got home , I felt like it had been a bad dream. and to be honest felt completely $#it!!

A few years ago my Dad had died from PV but, oh no, it's not hereditary. Really? Two rare blood conditions?

I have constantly refused to go onto treatment other than aspirin, as after a bit of internet trawling, the numbers don't look too bad. A few years ago my platelet count would have been acceptable. I am now under pressure to start Hydroxycarbamide but I don't like the look of the side effects, plus I already take a chemo drug for RA (MTX). Also I travel a lot to the Tropics and on this drug I would have to keep out of the sun!

I feel I need to reach out and ask people about the treatment, have you had side effects, how about the sun bit, any concerns?

Is my count really that high?

Help!! Real info and experience needed please!!

36 Replies
oldestnewest

It is very frightening and shocking when you are first diagnosed. We have all been there and can sympathise.

I was on Hydroxycarbamide for 17 years and I didn't get many side effects at all, just mouth ulcers when my dose was increased from time to time. It is a very well established treatment. It doesn't agree with everyone but I was one of the lucky ones and you might be too.

These days, the decision to medicate is generally based on your age so if you are relatively young, with platelets of around 500, you might want to question your doctor about why you would need anything more than aspirin at this stage.

Please continue to ask for support on here. We all need it from time to time.

Very best wishes, Jennie

Quercus53
Quercus53 in reply to Otterfield

Hi Jenny, thanks for your reply. I should have mentioned that I am over 65 (a girl never tells the rest!) So I guess I'm in the high risk category?

However, without any symptoms, apart from a bit of itchiness, which I had put down to my new rescue cat's excess fur, I don't really feel ready to start taking a toxic substance every day.

I think I'm right when I say back in 2008 when you were diagnosed, ET was not regarded as a cancer?

I do feel like I have been put onto a central conveyor belt, the easy option, where I am fighting to get off. Still, I've always been a bit obstinate, so maybe I'll keep fighting!

Thanks again, much appreciated. x

Otterfield
Otterfield in reply to Quercus53

It was 2001 when I was diagnosed. At the time there was a trial comparing Hydroxycarbamide/Anagrelide /Aspirin only. I was randomized to Anagrelide. After two years the trial was stopped because they found that Anagrelide wasn't so good at preventing clots and Iwas given Hydroxycarbamide. I was just 39 at diagnosis so now I would presumably just have been put on Aspirin. MPNs were designated as cancers in 2008 but I never really thought of ET that way as I suffered so little with it or the medication.

I would probably get a second opinion. I was diagnosed with a ET Jak 2 positive in 2008, and my platelets were about 587 at the time. My hematologist put me on hydroxyurea right away, but looking back and after reading so much about it over the years, I think he should have waited. He didn’t even call it cancer at the time. He called it a blood disorder. So I didn’t really worry until I read about it in 2015 that it was a blood cancer. I was only 52 back then, and most sites recommended cancer drugs after age 60. Now, when my platelets are in the 500’s, I’m happy. I really feel that taking that drug made me more fatigued and light-headed and made it difficult to concentrate and that it affected my work. But, in no way am I an expert. But as Hunter has taught me on this site, you need to be more assertive in your treatment and do what’s best for you.

I can absolutely relate! I giggled some when I read your post, I felt the same way on Monday when I got my diagnoses ET CALR+ and honestly still wobbly from it all. Real ups and downs in the last 48 hrs and hoping I can gather myself up soon. What I can tell you is my last platelet count was 681, when I met with my hematologist she felt comfortable putting me on aspirin and wait and watch checking every 3 months. If I become symptomatic or platelets hit 1000-1500 we will talk then about meds. Hope you feel better soon it is very shocking indeed. I agree with a second opinion.

oops sorry, I used the wrong name!!

Hi thanks for your input. I would be happy to have a haematologist say that to me. I'm quite happy to watch and wait before taking meds..

Keep as well as you are xx

Quercus53
Quercus53 in reply to Cja1956

Hi Cja1956, thanks for replying.

My Dad had PV as I said and I was so impressed by the way he was treated by a different consultant at the same hospital. They gave him 15 years max to live when he was in his late 40s/early 50s. He died when he was 88, PV was cited as the main cause.

How do I go about getting a second opinion on the NHS without annoying my current doctor?

Originally after about six months I set myself the 1000 platelet target before any treatment. Being over 65, maybe that is too high?

Thanks again for your help

x

Cja1956
Cja1956 in reply to Quercus53

It was difficult for me as well to think about getting a second opinion. I was with my original hematologist for over 10 years and I felt I was being disloyal. . But when I developed anemia and was feeling really poorly, I felt he had reached the limits of his expertise. He couldn’t seem to figure out how to stabilize my numbers. I didn’t find this site until about 2018 and that’s when I got the courage to leave him. I even told him I thought I had progressed to MF, but he didn’t think so. When I finally got my appointment with my new doctor last year, I brought my original Diagnosis and the last 6 months of records. She ordered a new BMB, an ultrasound, and tons of bloodwork, and confirmed my suspicions. I’m actually feeling better than I have in years with her treatment. So, again, You shouldn’t feel bad about seeking out an mpn specialist. It has made all the difference in the world to me.

All the best,

Cindy

Quercus53
Quercus53 in reply to Cja1956

Hi Cja1956

I think a second opinion may be a good idea. My consultant is really not happy with my decision at the moment. The consultant who looked after my Dad described my platelet count as mildly elevated, which sounds about right to me. The count has stayed at 500 - 575 over the last couple of years, since I was diagnosed.

Luckily I am over 65 and not working. When I was, the RA was a bit difficult to work with but I was given no quarter by work and almost forced into retirement.

Not that I would go back now!

Thank you so much

x

The initial reaction to the ET-CALR dx is understandable. We have all been there and done that. The good news is that there is some good news.

I was diagnosed with ET about 30 years ago. It progressed to PV about 7 years ago. Most of the time, I have been on an aspirin-only tx protocol. I have never had a single incident of thrombosis. In fact, while on aspirin, I have been more prone to hemorrhage than thrombosis. Do be aware that with thrombocytosis, you can be at risk for either. I am age 65 and managing PV successfully with a phlebotomy-only tx protocol. It is working for now.

Unless you are symptomatic or in a high-risk group, standard treatment for ET is aspirin-only if your platelet levels are below 1 million. Some docs set the level higher than that. There is no value to sanitizing your blood cell numbers in the absence of symptoms. There is no straight-line correlation between platelet levels and risk of thrombosis below 1 million. Risk is more complex than that. It depends on how your ET manifests. That is why is is so important to consult with a MPN Specialist rather than a regular hematologist. Many docs (even hematologists) do not have the KSAs to provide optimal treatment for MPNs due to their rarity. Here is a list of MPN Specialists mpnforum.com/list-hem./ .

Cytoreduction is needed in some cases; however, hydroxycarbamide (AKA hydroxyurea aka HU) is not the only option. PEGylated interferon, Ruxolitinib, busulfan and other meds are also options when needed. HU used to be considered to go-to med for ET. While still a standard of tx, not all docs agree on this anymore. HU is a "highly toxic medication with a low therapeutic index" per the American Society of Health System Pharmacists. As you already know, it does have significant side effect risks. Many people tolerate it OK; however, not all do. FYI - I am HU-intolerant. I experienced significant adverse effects when I did take it the last time. The MPN Specialist I see took me off it and was clear I should not take it based in my profile. Do be clear. Some people need cytoreduction. Some people do tolerate HU. ALL of the medications used treat MPN have their own risk/benefit profile.

Here are a few resources re, HU if you heed to review them

drugs.com/monograph/hydroxy...

hillman.upmc.com/patients/c...

There is a known phenomenon regarding Familial MPNs. The predisposition to acquiring some of the driver mutations is believed to be genetic. I have the JAK2 v6127f mutation with ET that progressed to PV. My daughter also has the JAK2 v617f mutation currently dx with ET - may be progressing to PV. My son has idiopathic erythrocytosis with no JAK2 mutation detected. Genetics DOES play a role.

So more good news. With ET-CALR you can expect to live a normal lifespan (or at least near-normal). The issue is with managing the symptoms. What that looks like will be unique to you. More good news - there are ways to manage the symptoms. That is not to say it is all just a walk in the park. It is not. There can be significant challenges. Look at some of my posts and you will see just what a unique learning opportunity MPNs can be. More good news - you will have plenty of time to have your own unique learning opportunities. The not-so-good news - you will likely have some learning opportunities. The best way to cope is to educate yourself and become your own best advocate. Assertive patient receive higher quality care. Passive patients do not.

There is some not-so-helpful info out there when you Google ET and other MPNs. there is also very good information to be found. Here are some links you may find helpful

MPNs - Spivak

legeforeningen.no/contentas...

About PV, but relevant to ET

vjhemonc.com/video/4kie_7r9...

How I Treat ET - Spivak

youtube.com/watch?v=hbVr9u3...

More excellent presentations about MPNs. All are worth watching

mpninfo.org/conferences/201...

I hope this helps. ALl the best on this journey.

Quercus53
Quercus53 in reply to hunter5582

Hi Hunter, I was kind of hoping you would spot my dilemma and reply, having read all your informative replies to other queries.

Well, I was going to do the garden this afternoon but it looks like you have provided me with enough links to keep me studying into the small hours!

I'm so grateful.

I am a naturally assertive person but equally I don't like to upset anyone, especially where my health is concerned! So how do I go about getting a second opinion and can I specify who I would like to provide that opinion?

I'm the wrong side of 60 and an avid traveller, when allowed. Up to COVID I was always given a syringe to inject myself with a blood thinner when flying more than 4 hours, to avert DVT.

I had read up about the radioactive potassium but it doesn't appear to be on anyone's treatment list. Do you know anything about it?

Thanks for all the good news you provided me and all the links.

Can I get back to you if I have more queries?

x

hunter5582
hunter5582 in reply to Quercus53

I am glad to hear that you are naturally assertive. Assertive patients receive higher quality care. Passive patients do not. You are the one in charge of your treatment. Regarding getting a second opinion, it is quite simple. Pick a MPN Specialist and make an appointment. I do not know what your healthcare system/insurance is, but nearly all permit you to do this. Some do put barriers in the way, but it can still be done. I saw your note about not wanting to "annoy" your current doc. Do not worry about that. If that doc gets annoyed, then he/she is putting his/her ego ahead of your health. Any doc that does that should be fired. Most docs, even hematologists, do not have the KSAs to provide optimal treatment for MPNs. Assert your right to the highest possible quality of care for yourself.

I was able to find a terrific local hematologist who is not by his own definition a MPN expert. I am inly his 6th MPN/2nd PV patient in a 25+ year career. I consult with a MPN Specialist at Johns Hopkins, which is too far to go for regular care. The MPN Specialist advises me and my regular hematologist on my care. My regular doc has no problem at all with this arrangement. Ethical physicians never mind consultation with a more knowledgeable colleague. A doc that does mind should be fired.

I am also on the upwards side of 60 - 65 as of July. Despite this, I am not following the standard protocol for PV age 65. Based on reviewing my presentation of PV, which remains relatively indolent, the MPN Specialist recommended a phlebotomy-only treatment plan with no aspirin. There are very good reasons why this is the right approach for me. My situation and presentation are what drives my treatment, along with my treatment preferences. What is right for me would not be what is right for someone else. This treatment protocol will not always be the correct protocol. At some point I will almost certainly need to resume the use of medication. I will almost certainly choose a PEGylated Interferon, unless something better has been developed. I will also be looking into a hepcidin mimetic once it is approved.

I have heard of radioactive potassium being used, but do not know much about it. It is not a standard therapy. I am more familiar with PEG-IFN, ruxolitinib , anagrelide and busulfan. I would suggest arranging to see a MPN Specialist and asking specifically about all of your options, including things in clinical trials. That way you can weight the risks/benefits of each of your choices. If you are in good health and asymptomatic, you also have the choice of no meds to treat the ET. The recommendation for cytoreduction based solely on age is a statistical projection of risk based on studies of large numbers of patients. It may or may not apply specifically to you. That is something to discuss with the MPN Specialist who can do a far better job than I can of explaining your risk profile.

I hope that helps. Do feel free to reach out anytime you like. You can message me if you like. All the best to you.

Hi, I found out I was ET Jak2 6 months ago approx. Just like you thought - what's happening. Then got fed up with analysing day and night. So took a deep breath and took first hydrea tablet. Think it's the fact that it's a chemo tablet that initially spooked me. But you know - my platelet count was 990 and last time check down to 670 and nothing has really changed. Feel ok and just get on with my day. Never hardly on my mind now - just take tablet and get on with my day.

Will say though - that if my count goes down to 450 'ish going to give body a rest from meds. But that's my individual choice.

All the very best

Quercus53
Quercus53 in reply to elliebongo

Hi elliebongo, I think if I went onto meds I would also consider dropping them once in the safer area. Problem is, would this have a yoyo effect?

elliebongo
elliebongo in reply to Quercus53

It may well, but I'm also new to this so hopefully this will be a discussion with my haematologist, who has been very good so far.

Think I'm living for now and not looking to far into what it may/or may not develop in to.

Cja1956
Cja1956 in reply to Quercus53

Hello, again. When I first started my meds at diagnosis, my platelets went from around 600 to the 300’s. I remember calling my doctor several times to see if I could stop the meds. When I didn’t get a response, I stopped them on my own and shortly thereafter, I ended up being rushed to the hospital with a blood clot in my spleen. My platelets were at 800. I really thought I was having a heart attack, the pain was so sharp. I was hospitalized for 3 days. They called it a splenal infarct. I never stopped my meds again. So the yo-yo effect, in my limited experience, seems like a real thing.

Hi

I was diagnosed in 2018 started on aspirin and hoped that l would avoid the dreaded meds by staying healthy eating well,supplements lots of excercise. These all helped with my general health but unfortunately my platelets climbed from 450 to 1200 and other fears took over increased Covid vulnerability,having blood clots etc. On my haematologists advice l started on Hydroycarbamide and like most was petrified by the long list of side effects. I am now taking 17 pills a week possibly 18 soon. Many people on this forum were very reassuring about there experiences with HU. My platelets have dropped to 560 in almost three months. I have not had any side effects to date. Long term effects who knows? All Medications have side effects even aspirin. But since being on HU my fatigue has gone. I try not to obsess about my condition snd l don’t want it to define me. I recently learnt to my surprise of a MPN clinic at Charité hospital in Berlin which is my home city. So l plan to visit and inform myself about the range of services on offer and seek a second reassurance opinion on my current treatment. Think second opinions are good things to do. I am particularly keen to hear about new treatments like Besremi which has replaced Peg here and also other trials - combination therapies etc

I also travel some four months each year.l am looking forward to resuming this with my meds

hunter5582
hunter5582 in reply to Dovme

I am really glad to hear you found a MPN Clinic close by. That is so great to hear! It really does improve the quality of your MPN care. I also want to learn more about Besremi, which is under FDA review here in the USA. I hope it will be available soon. There is also a hepcidin mimetic for tx of PV that is under review. The MPN Specialist indicates it would be on option for me rather than phlebotomy-only. Like you said - ALL options have potential side effects. My phlebotomy-only no drugs approach has side effects. I am very iron deficient. Ferritin is only 6 - so low many labs can not measure it. It only causes mild side effects, but they are annoying.

Like you and Quercus53 I also love to travel. That is a big part of why I continue to work part-time post-retirement. My jobs take me all over the USA and up to Canada. I am working virtually these days, but it is not as much fun. I am will be first in line for a vaccine when it comes out. I volunteered to be in clinical trials at Johns Hopkins if they want MPN patients as test subjects. I hope they offer it.

All the best to you.

You could be me based on your description or lack of symptoms. I was diagnosed earlier this year, platelet count at 840. HU dropped me back in the normal range, low 300s. I still wonder if this true. My only symptom was itching and still is. I have no reaction to the drug and feel simply wonderful and enery like I haven't had in decades. I'm approaching 80 and feel half my age. However, I trust my doctor, a hematologist/oncologist, highly educated at me Anderson. I take great comfort in hearing from so many that you can continue a normal life for some time. I hope you read the reply from Hunter...he is very knowledgeable and advice, website recommendations, etc are the best on this site. I have an attitude that tells me my doctor is trustworthy, much wiser than me, has no motives other than making me better, and at this age i have put my faith in him. When first diagnosed the realuzation i could have had a stroke was frightening. You sound very active, alert and intelligent. I, too, want to continue being that way. Perhaps you should get a second opinion to put your mind at ease. My very best to you in your journey.

Quercus53
Quercus53 in reply to Mollymag2

Hi MollyMag, I think I was in denial for about the first year or so, convinced they had got it all wrong. As I have rheumatoid arthritis which can raise platelet levels, I'm still wondering whether that is partly to blame for the 500-550 count.

Hunter sent me a list of drugs and their effects. Hydroxyurea is described as carcinogenic, so why would I want to be on such a drug? It's like pushing a stick into a wasp nest, completely irrational!

Keep on scratching!!

xx

Mazcd
MazcdAdministrator in reply to Quercus53

hello Quercus53, welcome to our forum. Completely understand how you are feeling at the moment, very scary having to go to a cancer centre, and I am not surprised that you didn't hear most of what was said. Hopefully the replies you have had from the lovely people on this forum have helped you, I would also suggest that you read as much as you can on our website mpnvoice.org.uk, there is a lot of really helpful information about MPNs, symptoms, and also about all the different medications used to treat MPNs.

It is understandable that you are scared about having to take Hydroxycarbamide, especially when you read the list of side effects and that it is a cytotoxic medication. I have been taking it for my ET for nearly 13 years and quite honestly, it gave me my life back, as it reduced my awful symptoms that were debilitating me every day. But I was absolutely petrified when I was told to that I would have to start taking them, but I thought all I could do was try and if they didn't reduce my platelets or I couldn't tolerate them for any reason, then I could stop them and try something different.

There are many many people taking Hydroxycarbamide, it has been around for years and is used to treat other conditions, it is a tried and tested medication, however, it is not suited to everyone, there are many people who can't tolerate it because of different side effects, but this is the same for any medication, there are people who will get some side effects and some people won't, for example I am allergic to any and all anti-histamines, so good job I don't get the PV itch.

It is a very difficult decision to have to make and you do need to have a discussion with your haematologist and explore all the options available to you, and what will work for you and with to your other medication. Have a look at the information about the different medications and then write down questions that you have about them, and when next you see your haematologist, take the list of questions with you, and take someone with you if you can, it does help to have someone else listening to what you are being told.

Hope this helps, best wishes, Maz

Quercus53
Quercus53 in reply to Mazcd

Hi Maz

you're right everyone on here are fantastic, helpful souls who all know what we go through. I have looked at a lot of the website and also been helped by the very knowledgeable Hunter. Some of the people here are not from the UK so processes vary a little when handling, for example, getting a second opinion.

The doubling up of chemo drugs is something I really want to avoid, the alternatives are equally frightening, maybe heart problems, depression, nausea, aching body etc etc, all made the worse due to both chemo drugs at once.

As I have told the many lovely people who have replied, I am going to seek a second opinion. I just have to find a real MPN expert to consult. The list I saw didn't have anyone for my part of the UK but will look further.

Onwards and upwards....

Thanks again

xx

hunter5582
hunter5582 in reply to Quercus53

It is worth travelling to do the consult. I usually spend the night when I go to Johns Hopkins MPN Clinic. It is totally worth it, It is harder to do in these crazy COVID times; however, COVID craziness - tele-medicine. I do many of my appointments by tele-video platforms. It is WAY better than a phone call and almost as good as being there in person. It is so easy to set up that for the life of me I do not know why anyone would do a mere phone call. When the provider and the patient can see each other it humanizes the process. Suggest choosing a MPN consultant that offers tele-vid as a priority if no one is nearby.

Welcome Newbie, I am 78 and have had PV since my 30s. My doctor stressed to me that I could help myself by keeping a diary of how I felt so I could tell him every thing. For two

years I was on just aspirin daily and my low hemi iron diet with EXTRA fluids mainly water.

After that I was getting two blood draws per year for about four years. worked good!

Started having higher R/ W/ and platelet count. My Doctor put me on Hydroxyurea and no iron vitamins. I get my blood tested every three months, See my specialest yearly. I have had one blood draw in the past 10 years. My Doctor has had me walking 1 mile a day and I have a friend that makes sure I do. I pray you will do as well as I have. PS I still put out a garden and pressure can my vegetables,.

Quercus53
Quercus53 in reply to soisew

Hi and thanks for taking time out from the garden for me!

My real worry is to be taking two different chemo drugs which apparently worsens any side effects. I live on my own and don't have anyone to look after me if I get bad side effects.

Having 'spoken' to lots of people on this forum I am now of a mind to visit my GP and ask him to arrange a second opinion for me.

Everyone has been fantastically supportive and I am so grateful

Best wishes xx

Hi and welcome to the forum.

It certainly won’t do any harm to get a second opinion, and hopefully you will be more informed to make that decision.

Treatment with hydroxy is about lowering your thrombotic risk. The CalR mutation is less of a thrombotic risk than Jak2 or MPL mutation. However, any other health conditions will have a bearing on this too. RA increases your risk of heart disease, so you definitely need professional guidance on this.

I have ET Jak2. I have been on hydroxy for four years and have absolutely no issue with it. It works well for me, and it is tolerated well in most cases.

Just a quick note about your worries about taking methotrexate with hydroxy. There is a forum member (Judy) who has RA and PV and takes both drugs. She has had no adverse effects.

Quercus53
Quercus53 in reply to mhos61

Thanks for your email. I am going to find out from my GP if he can request a second opinion. Whilst I am pleased that Judy is getting along well with the two drugs, only one person's experience is not comfort enough. What I really want to know from this second opinion is at what level I really need to make a decision about meds. A completely realistic view other than the text book version.

mhos61
mhos61 in reply to Quercus53

I get that, and absolutely agree.

We are all different, and the more information you can gain is obviously going to be helpful; however, it is the MPN experts who will offer you the best professional guidance in making your decision.

I wish you well, it’s never an easy decision.

piggie50
piggie50 in reply to Quercus53

My platelets were in the high 900’s when I commenced Hydroxy, I refused it for 4 years.

Judy

Quercus53
Quercus53 in reply to piggie50

That was the kind of level I was thinking, around 1000 but the pressure from the doctor is to go on now.

piggie50
piggie50 in reply to Quercus53

Me too, not my Heam but one of his team really upset me once, I went back to work in tears. I made a complaint and on my next appointment she apologised.

Hi, I’m sorry I didn’t see you original post, I only log on occasionally now. Everyone’s experience and situation is different so only you can make the decision on your meds. I have had RA for over 25 years, I originally took a drug called Penicillamine, which was the first line drug at that time. Regular blood test etc as it also can affect bone marrow. When I was diagnosed with PV 15 years ago the Penicillamine was stopped as my RA was not so bad. Seven years ago at the age of 59 I started Hydroxicarbamide, one per day and two at the weekends. This has varied depending on blood test results. Early 2019 I had a huge flare up of my RA. Rheumatologist wanted to start me on methotrexate, naturally I was concerned to be taking two toxic drugs. Maz very kindly contacted Prof Harrison for me who advised that she does have patients on both drugs with careful monitoring. I commenced 20 mg per week of methotrexate September 2019. I have had monthly blood tests since until now when I am now on every 10 weeks. I keep a close eye on my bloods and contact heam and rheumatologist if I need to between telephone consults.

For me, once I got over the worry, it was a no brainier. I need the Hydroxicarbamide to keep my platelets lower and I need the MTX for my RA as that was unbearable.

When I started the MTX my Heam took me off Hydroxy for 4 weeks , in that time my platelets went from normal to 850!

Good luck with whatever you decide.

Very best wishes

Judy

Quercus53
Quercus53 in reply to piggie50

Thanks very much, hope you continue to stay well x

Hi like you l was recently diagnosed with ET and now 2 months in on Hydroxycarbamide.There are some side effects but it is early days and lm aiming to be one of the lucky ones. Good luck,stay positive and check into this forum for added support and information.

Quercus53
Quercus53 in reply to Adiewon2

Thank you, will do

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