Newby to ET

[StigerP]

Recently been diagnosed with ET.

Consultant suggesting Chemo Pill, but only after "going through several hoops"

Did not explain what the process will be.

Any information of what others had to go through before medication?

[Cat1001954]

hi I’m Cathy I’m 68. I was diagnosed in august I have triple negative ET. I’m on oral chemo drugs and clopidogrel blood thinner as I’m asthmatic I go once a month for blood works. And then once a month to get results I had bmb to confirm my ET

[Cat1001954 in reply to Cat1001954]

hope this helps a bit and good luck on your journey

[StigerP in reply to Cat1001954]

Thanks again Cathy.

[StigerP in reply to Cat1001954]

Hi Cathy. Thanks for reply.

Don't know what triple negative ET means, not heard it.

Have appointment with Consultant 16 January and will ask him.

Also, what does bmb stand for?

Sorry for being nieve. I'm totally knew to the terminology. No doubt I will learn a lot over the next few weeks and months.

Stuart

[Cat1001954 in reply to StigerP]

hi bone marrow biopsy. Is bmb. And iv got a rare form of ET that’s why it’s triple negative.

[StigerP in reply to Cat1001954]

OK. Your the 1st to add to my new dictionary.

Good luck with your medication.

[EPguy in reply to StigerP]

One of the hoops might be your genetic test results. This can help confirm it's ET type MPN rather than another non MPN condition. They will look for certain mutations that have these symbols: Jak2, CALR, and MPL. With ET you are likely to have one of these mutations in your bone marrow. But ET can also have none of these three, hence "triple negative". This is not as common but possible.

You won't necessarily get the BMB (bone biospy as noted by Cat1001954) right away. But this is usually worth having for future reference if you are found to have a likely MPN condition since it shows details not possible any other way. BMB takes a sample of your marrow.

Chemo pill is likely Hydrea (Hydroxyurea) It is not expensive so the cost should not limit your access.

[Cat1001954]

your very welcome we can do this journey together if you don’t understand something write it down and ask heamatologist x

[Cat1001954 in reply to Cat1001954]

that’s what I do if I don’t know something

[StigerP]

Yes. Thank you. I have started a list ready for my next appointment.Thanks for your help.

[Zeppelin11]

Hi There! I'm on Hydroxyurea 1500mg per day but I'm about to transition to Pegasys. I don't have very many side effects with the medication I'm taking now but I'm on a high dose and want to avoid any long term side effects, hence the new medication. Best of luck!

[Mirror368 in reply to Zeppelin11]

That seems like a very high dose. What is your current platelet count?

[ciye]

Welcome. You have come to the right place and you will always find lots of help and wisdom.Your consultant should have talked you through your treatment options, mine did and have me a booklet .

Google mpn VOICE it explains et, and it's treatment options.

[nightshadow]

The hoops you need to go through are probably going to be based on your location. When bringing insurance and medical access into the equations, the problems in the US are different from the ones in England.

This is my experience as a well insured American living in a large metropolitan area with close access to the specialists in the field.

I was diagnosed with ET in 2020. I had tests to make sure that I had ET as opposed to a thrombocytosis caused by something else and then tests to see if I could tolerate hydroxyurea and what the dosage should be.

The tests for verifying that I had ET took a month, I had low iron levels, which can cause an elevation in platelets, so the anemia needed to be corrected before they decided that I had ET. Then it was several months, nine I think, before the correct dosage was determined.

This site is a good resource for finding out about ET in general and I would recommend doing some research about the disease so everything you hear from the consultant won't be brand new information. You'll be in a better position to understand what you are being told and also what options are out there for you.

Some of what you read may be really rather scary, but ET is one of the slow cancers, I believe the term in indolent. As the saying goes, people are likely to die with ET, not die from ET.

It also seems to be highly variable in it's effects, as is the tolerance for the most commonly used chemodrug, hydroxyurea. So this becomes a very personal journey that, though we've been through it before you, your experience is going to be unique. So get information and be prepared to be your own advocate. You'll do fine and if you need help, or just to vent, we'll be here.

[Smudger0122]

Hi Stiger

Im 46 year old Male, diagnosed with Essential Thrombocythemia Calr June 2021.

Platelet count was high, reached 1665 in February last year.

I to was confirmed with Bone Marrow Biopsy last year.

I currently take hydroxycarbamide 3 on one day, 2 on next. Then 3 again and so on.

Also on clopidogrel blood thinner and omezaprole.

I have blood tests every 2 months, my decision. 4 monthly was suggested by blood consultant. But i have become so anxious regarding my health conditions lately.

Don't Google too much.

All the best

Smudger

[Scaredy_cat]

There is a lot to take in and your appt with consultant should answer them. Depending on your mutation and blood counts you may find the doctor adopts a watch and wait approach.

[dancingfiend]

I too am 72 and live in South West UK. Diagnosis of ET JAK 2 in June 2021. Huge shock as had no idea what platelets were, let alone that mine were sky high! My GP clearly did not understand significance either, as escalating levels had been overlooked for almost 4 years!

Local haematologist prescribed Hydroxycarbamide and daily dose 75 mg dispersible aspirin immediately. Took Hydrea 1000 mg 5 days a week and 500 mg 2 days for about a month.

Bloods were taken, and reviewed by haematologist, every two weeks for first couple of months until platelet level reduced to acceptable level; followed by ‘tapering’ over about 6 months.

Initial period of Hydrea resulted in noticeable fatigue and some headaches.

Now on regime of 1000 mg one day and 500 mg six days. Seems OK. Just need to manage daily activity to control fatigue.

It’s a scary time at first, I know, but gradually it becomes part of life.

[Notmyusername]

Hi Stiger,

I’m pretty new to this too having been diagnosed with Prefibrotic myelofibrosis in dec 2022 - a somewhat annoying cousin of ET.

I’m also in the UK and getting treated out of UCLH in london. I’m happy to provide you with my consultants details if you want a 2nd opinion, he is an MPN specialist. I’m due to start pegasys interferon in a couple of weeks so looking forward to that! In the mean time my platelet counts are off the scale.

As others have said, it’s really shocking to get this diagnosis out of the blue, I’m certainly still digesting it and while there’s a lot of terminology and background info to learn initially, as others have mentioned, it’s quite hard to find really clear information. MPN voice has been a good starting point and just following others thoughts and experiences on here has been helpful to me too.

I think the advice others have given on here which is to make a good list of questions for your consultant and work through them so that you get answers you are comfortable with.

[Mazcd Partner MPNVoice]

hello Stuart, welcome to our forum. It is very daunting when you are newly diagnosed, I would suggest that you have a look at the information on our website, mpnvoice.org.uk, there is lots on there to help you understand more about ET and the different medications used to treat it. And the lovely people on this forum will also help you with advice and support. Not everyone starts medication straight away, though most will likely have aspirin, it will depend on many factors, what your blood counts are, your overall general health, your past medical history and any symptoms you might be experiencing with your ET. Many people take Hydroxycarbamide, which is very likely what your haematologist is suggesting, it is a very well tolerated medication and has been used for many years to treat MPNs, though some people don't tolerate it too well, but generally most do.

Read the information on our website and start writing down questions and things that you want more information about from your haematologist and take these with you at your next appointment, it does help you then to keep focussed during the consultation so that your questions are answered, ask how often will you have a consultation; what are the treatment options. mpnvoice.org.uk/living-with...

Also when you next go for an appointment, find out the details for your clinical nurse specialists (CNSs), who they are and contact details so that you can contact them during appointments if you have any queries or concerns, they are very knowledgeable and can liaise with your haematologist.

This list of terminology will also help you mpnvoice.org.uk/about-mpns/...

We are here to help and support you, we understand how you are feeling, best wishes, Maz

[Threelions]

A Huge Hello & Welcome Hug 🤗

So glad you have found your way to this forum. A place where fellow MPNers help and assist one another with all things MPN.

Feel free to ask any questions, any time & the good friends here will endeavour to help.

I was diagnosed with ET over 3 years ago & found the people here so valuable. When others in our life may not understand things we go through, you’ll find plenty of understanding here.

I’ve been taking PEG Interferon since diagnosis & it’s kept my blood nicely in order.

It’s very important, at the stage you’re at, to insist that you are referred to an expert in the field of MPNs . As many here have said before, MPNs are not widely covered or understood fully by medical staff and you need to make sure you get the right course of medication & care. Once that’s in place you can get back to living your life👍

[Hopetohelp]

I was put on 75mg aspirin when first diagnosed with a watch and wait approach with blood tests every 3 months. As figures started to rise I started Pegasys. We are all here to help so feel free to ask as many questions as you like

[Mishie14]

hello from US where process may be different but the disease is not. Many helpful comments are posted that are great reference for you. I’ll add from my experience some things not touched on yet re drugs and side effects. I am 72, didn’t get sick not even a head cold until 2022. I have ET JAK2 positive, bone marrow biopsy negative and platelet count jumping around 600k to 1M since diagnosed last March. It was a surprise diagnosis from advance blood tests taken for a gastro endoscopy test. Within 2 weeks I was on ET drug plus blood pressure, blood thinner vitamin B12, vitamin D and 81 mg aspirin capsules. Primary ET drug was low dose hydroxy which showed good results but along with awful known side effects—extreme fatigue, loss of appetite, diminished sense of taste, headaches, and near constant pain in bones and joints. Then my liver rebelled and enzymes rose to about 10x normal causing alarm. Hematology consulting MPN specialist stopped hydroxy to let things settle down for a couple weeks. Then started ET drug anagrelide low dose for plan B. It was slower acting on platelets and with less severe side effects than hydroxy. Hematology gave me an iron infusion treatment to help with anemia left from hydroxy. It really helped me feel better. When anagrelide dose was increased in late November to get more aggressive on reducing platelets, the side effects got worse and included heart palpitations and skipped beats. This is a known possible side effect that sometimes goes away after a short while so with cardiologist approval I kept taking higher dose. It didn’t go away and then skyrocketing liver enzymes appeared and anagrelide was stopped. Currently working with liver specialist. Strangely, platelet count dropped in recent blood tests after stopping anagrelide. Side effects have mostly gone away and I’m feeling a lot better overall. This is not going to last, interferon Pegasys Plan C is likely in my future once liver is improved. Key points are there are multiple treatments which side effects vary in variety, intensity, and by person; frequent blood tests are important so you know what the drugs are doing to your body in addition to side effects you are feeling; MPN specialist is required—hematology is key but is not qualified to address MPN alone; and take advantage of knowledge base and resources available through this group. You are not alone. Good luck!

[Mirror368 in reply to Mishie14]

Your journey is very interesting, I live in the US also, I am 78 and was diagnosed in June 2022 with ET JAK2. My platelets had been rising very slowly for several years, I was referred to hematologist when my platelets hit 621. At my first appointment they had jumped to 735. I had extensive labs and bone marrow biopsy. I was put on HU 500 mg a day. Headaches started daily. She took me off it for a month and I was then put me back on 500 mg HU every other day. Platelets have been lowering nicely, My last visit they were down to 434. However, I have been experiencing headaches and legs aching. I do not think I could take Anagrelide as I have paroxysmal AFib and palpitations are a risk with that medication. Pegasys has not been discussed yet but it is expensive and seems to have many side effects also.

My current treatment plan is monthly labs and continue taking HU 500 mg every other day. Hope everything stays stable,

Good luck with your continued journey, Eileen

[hunter5582]

Hello and welcome to the forum. This is a great place to get support and information from fellow MPNers.

I was diagnosed with ET about 30 years ago. It progressed to PV about 9 years ago. Now age 67, I have lived a rich life while managing a MPN and continue to do so. ET is a condition that can be managed successfully. It is important to note that there is more to managing ET than just preventing thrombosis (clots). There is also risk of hemorrhage and microvascular symptom. Mant with MPNs, myself included, have more trouble with the secondary or constitutional symptoms than the primary risk of thrombosis. Much of this has to do with the overproduction of inflammatory cytokines associated with the JAK2 mutation.

MPNs are rare disorders. Most doctors have little experience with MPNs, including hematologists. That is why it is important to consult with a MPN Specialist. This is the only way to ensure optimal MPN care. Here is a list. mpnforum.com/list-hem./

There are multiple options to treat ET. Cytoreduction is usually recommended for patients with ET age > 60/65. The two first-line treatment options are hydroxycarbamide (chemotherapy) and Pegasys (immune modulator). There are also second line treatment options like Jakavi and anagrelide. It is important to review all of your treatment options so you can make a choice that lines up best with your goals, risk tolerance, and treatment preferences.

Mazcd already referred you to an excellent source to start learning about ET and your treatment options. The MPN Voice website is excellent. Here are a few other resources to get you started on your MPN journey.

ET/MPNs

mpnjournal.org/how-i-treat-...

legeforeningen.no/contentas...

Hydroxycarbamide (AKA hydroxyurea)

drugs.com/monograph/hydroxy...

online.epocrates.com/drugs/...

Pegasys

drugs.com/pro/pegasys.html

nssg.oxford-haematology.org...

We each respond differently to the medications used to treat MPNs. Some respond better to one but not the other. Some can tolerate one but not the other. Suggest reviewing all of your treatment options with a MPN Specialist in order to make the decision you feel is best for yourself.

Wishing you all the best as your enter this journey.

[Oscarsboy]

Hi I'm Yvonne 74 years old. I went through a similar process to that you are describing 18 months/2 years ago. At the time we were in the middle of the covid pandemic which I know did not help, but it took a lot of perseverance to get through those "hoops" and get an appointment. I had a BMB which confirmed I was ET JAK2 but trying to get an appointment to get the chemo tablets (Hydroxy) which is what I think you are referring too, was a real uphill struggle. I was given telephone appointments, but you cannot start that treatment without a f to f appointment as you have to sign papers of agreement to start it. However eventually we got there and I have been on it since. For the most part I tolerate it well. I do have blips every now and again, ie symptoms kicking in and some side effects. I find this is often sparked though by a stressful occasion or incident and it then takes a few weeks to settle down. You will need a f to f appointment to start your medication and everything is/should be explained to you clearly at that point. If you find it difficult to get an appointment with the haemo team etc to commence treatment or see a consultant to explain everything to you I would suggest you contact PALS at your local hospital who are usually very helpful with assisting to get things moving. (Pals) Patient Advice Liason Service. Hope all goes well for you.

[Annula]

Hi - I had extensive blood tests, liver & spleen scanned & bone marrow test before treatment started. Now on Hydroxy, aspirin & statins - ok so far 2 years down the line - thankfully! Lots of luck ...

[Ovidess]

Hi, Just to add another ET Jak2 profile: I'm 65, just diagnosed with ET this fall after about 30 years of high platelet counts that doctors merely checked each year. Was I at risk for all those years? It is a mystery. I was only sent to oncology/hematology when other blood counts went haywire as well. I bucked at the idea of chemo, and realized my small city oncology office probably did not have the most recent info, so I arranged a video Mayo Clinic visit in December. That doctor was helpful. He agreed with the diagnosis (done through BMB, etc). but said I may be on the road to Polycythemia Vera as well, but that the name does not matter as much as getting the blood issue under control. He said that Peg Interferon is something I could consider in place of chemo (HU). Although it has a scary black box warning and has plenty of possibly dire side effects, it has a track record of addressing the mutation itself, as well as slowing or stopping the progression of the disease to PV, MF, Leukemia, etc. I have yet to start treatment because I postponed the decision to the first week of the new year, and Bang! I now have flu and had to postpone again. I'm in the USA, new to Medicare, and am not sure what my local oncologist will say about me skipping the usual order of treatment (that is, trying HU first.)

I have other rather interesting material to add to my profile. I recently did a 23andMe genetic test out of curiosity (hereditary) and for any health info to be gleaned. I have no idea how their testing is regarded by the medical community. Here's something I learned about my genetic response to drugs: I have one copy of a variant that predicts rapid metabolizing --through increased enzyme activity-- of various drugs in the areas of Cardiology, Gastroenterology, Infectious Disease, Neurology and Psychiatry. I also have a variant copy that hints at predicted decreased function through decreased protein function. I am wondering if this has to do with the liver cleaning the blood inefficiently. Both my diagnosis and this genetic information are new to me, and what with Christmas commotion and a family falling one by one to the flu, I am still slowly learning about it all. If anyone has insight on these pharmacogenetics results, I'd be interested to hear from you.

Best to all! May 2023 treat you well.

[EPguy in reply to Ovidess]

Reply to Ovidess: On result insights from 23 and me, there is a thread discussing a "haplotype" of 46/1 that can be found from this test. Our Hemo tests don't look for it but it seems may be relevant to MPNs. Did your result show anything there?

[Mirror368 in reply to Ovidess]

I read your post with much interest. I am thinking of doing 23andMe.

I am 78 and diagnosed ET JAK2 in June 2022. Started on 500 mg HU and reduced a couple months ago to every other day because of headaches. It is working nicely as my platelets are down to 434 after two months of every other day. I was already on a blood thinner so I do not need to take low dose aspirin.

Pegasys is very expensive..HU is inexpensive. Some Medicare insurance complies in US might not approve Pegasys or want you to do step down…first HU before switching.

Have you thought of trying HU at low dose and then later trying Peg? If you go on HU remember it is recommended to drink a minimum of 64 ounces of fluids daily.

Good luck with this very confusing diagnosis. Eileen

[Ovidess]

EPguy, I looked at that thread you mentioned but didn't understand it. Even the nomenclature "46/1" does not look like any info I got from 23 and Me, unfortunately.

[EPguy in reply to Ovidess]

I don't really get Haplotype either, but I'm slowly learning. It seems of special interest for genealogy hence their interest in it. 46/1 is just one sort of a haplotype. It's possible if you don't see 46/1 that you don't have this in your genes, which would actually be fortunate. It's also known as GGCC.

It's also connected to these results which 23&me could report:

"The following SNPs are (all) associated with the JAK2 46/1 haplotype that appears to predispose to V617F-positive neoplasms; since they are all part of one haplotype (and are not independent of one another), having one usually implies having the others as well:

rs12343867

rs12340895

rs3780374

rs4495487

rs10974944"

snpedia.com/index.php/Rs123...

--

This old report from 23&me discusses this subject, so it seems they do look for 46/1. They report a 2X increase in odds of MPN with 46/1.

blog.23andme.com/articles/j...

--

Like you I'm not sure what to make of all this but with so many getting 23&me tests it seems of interest to us.

[hunter5582 in reply to Ovidess]

I also ran the more detailed report of the 23&me data through Promethease. I have the same same data that EPguy reports.

23andMe blog People with a rs12340895(G) (equivalent to rs12343867 in the study) had nearly four times higher odds of developing V617F-positive myelofibrosis (MPN) compared to people without the variant. "23andMe recently replicated this association, though we see a smaller effect — in our database, people with a G at rs12340895 have about two times the odds of developing V617F-positive MPN compared to people without the disease. People with an A at rs3780374 in the JAK2 gene (equivalent to rs4495487 reported in the study and highly correlated with rs12343867) have about three times higher odds of developing V617F-positive MPN compared to individuals without the A version."

The following SNPs are (all) associated with the JAK2 46/1 haplotype that appears to predispose to V617F-positive neoplasms; since they are all part of one haplotype (and are not independent of one another), having one usually implies having the others as well.

• rs12343867

• rs12340895

• rs3780374

• rs4495487

• rs10974944

I am positive for an alteration in the genome at the three bold locations - associated with the JAK2 46/1 haplotype. This is not the somatic JAK2 mutation that causes MPNs. It is a germline genetic variant that is thought to predispose a person to acquiring the JAK2 mutation.

[Ovidess]

Thanks, EP, I found some of this in 23andMe's "your raw data" page, and the articles you cited helped some. The JAK2 mutations seemed associated with chromosome 9 (in my case-- or always??), and I noted several CCs or GGs, associated with higher odds of developing MPNs-- cells with colonizing habits.😦

I'm sorry, Stiger P, to introduce this esoteric info here as it is likely not helpful to you right now. Perhaps this information should go under its own topic (like "genetic tendencies")--but I find conversations have a natural sprawl to them that is hard to control.