Confused and concerned

[annem22]

My story is a bit of a long one. It all started out at the beginning of the year when I went snowboarding in temperatures close to 0 degrees. Before we went on this trip, I was trying to recover from a cold that I was having difficulty shaking. I went to the doctor and the only thing he could prescribe me was Advair for my cough. I used it once during my trip and then decided I didnt want to use it bc I hate putting any type of drugs in my body.

So I went snowboarding and I had one hard fall on my tail bone that left it bruised and very sore. Later that day, I had a major dizzy spell where I would have fallen if someone wasn’t there to catch me. I didn’t feel right all weekend - i would get a little dizzy here and there. After I returned home from our ski trip, I went to the doctor and saw a physicians assistant and told him what happened. I asked him if I would die of a brain aneurysm and he said no, prescribed me with meclazine for the dizziness. 3 days later, I had a stroke. I had a vertebral artery dissection (VAD) that the doctors said was caused from the impact of the fall even if I didn’t hit my head. As a result of the stroke, I couldn’t walk normally, I suffered from double vision, I lost sensitivity to the left side of my body and the right side of my face. My face is always numb and though my vision is no longer double, I still have some vision challenges. Throughout my stint in my rehab, my blood was drawn from time to time and my platelet levels were elevated. The doctors said that they’ve seen that with this type of stroke/trauma, platelets tend to increase before they decrease. The highest it was in rehab was in the 700s. It eventually dropped to 600s. A hematologist even reviewed my labs and wasn’t very concerned. So I felt reassured. I only had to take aspirin daily 325mg.

A few months after this whole debacle, I was able to get to my PCP (primary care doctor) for my annual check up. I got my CBC And was told that my platelet levels was high 720s and he wanted to do some additional testing, get some blood smears. Luckily I was tested negative for jak2 and CALR. The MPL came back with something but to be certain, I was told I needed a bone marrow biopsy. From that, my pcp diagnosed me with early onset of essential

Thrombocythemia. He said that there is nothing to be done and that he wouldn’t be putting me on hydrea. He said maybe at some point later in life and that I should just enjoy life as I would if none of this had happened. I also had an ultrasound done of the abdomen to make sure my spleen was not enlarged and it wasn’t.

It’s not that I didn’t trust my pcp but I wanted to get a second opinion from doctors that dealt primarily with this type of MPN. SO I asked for my records and went to memorial Sloan Kettering. Upon having my blood drawn, for my first visit with MSKCC, my platelet levels have shot up to 960s. They tested me for Von Willenbrands disease which luckily came back negative. They are going to redo the genetic testing to be absolutely certain, but they are leaning towards ET as well.

I have to go in for blood work next week 2x to see how my platelet levels are. I really do not want to go on hydroxyurea but understand that it’s extremely important especially for someone who has suffered from a stroke. The doctors are going to connect with my neurologist as well to get their opinion to make a final decision on how to proceed. I have read that some people don’t experience side effects from the drug, but I am most concerned about the long - term effects of it. Will it make me more susceptible to cancer. Actually the fatigue that people experience, how will it effect my job? My employer has been extremely understanding since my stroke, but now this?

I am hoping to not have to get on hydroxyurea, but from the sound of it, the doctor is leaning towards putting me on. I am now 39, have two kids, 9 and 4 and a full time job.

I just don’t understand, or can’t accept the fact that I have this disease. I went from being healthy to now having all these problems since having the stroke. I know it could have turned out tragically worse but that thought doesn’t always get me by.

My husband keeps trying to comfort me in saying that right now nothing is for certain bc The doctors haven’t decided. But Ive been through so much since 1/5/18. I am struggling with staying strong and positive. I hate telling my kids that “mommy can’t play” bc she has a headache or is tired. I don’t know if it’s from the stroke or now the ET. I’m still on the mend from the stroke and now to layer on ET.....I want to be there when my kids graduate college and get married and see some grandkids. I want to have a mother/son dance at his wedding and see my husband walk my daughter down the aisle. I know ET is not a death sentence. It’s the future of what ET can become and how my job will be affected (I am the bread winner) that worries me and stresses me out.

I’m sorry for the long winded post. Thanks for hearing me out.

[Aime]

Hi, you’ve really been in the wars! But now you know what you have with the correct monitoring and treatment the expected lifespan with ET or Polycythaemia is normal. As far as I understand, there is an outside chance of transmission to leukaemia but this is very unusual.

If you go to the MPN Voice website which is closely monitored like this forum, so you know the information is trustworthy, you will find out a lot about your condition. This forum is full of lovely people who truly understand what you are going through and will support you - a lot of them have been around with their condition for quite a number of years and are still alive and kicking.

Keep in touch, kindest regards Aime xx😺😺

[annem22 in reply to Aime]

Thank you so much Aime. I am just trying to figure out what to do about taking hydroxyurea. I’m very torn. I will check out the MPN Voice website.

[mhos61]

Hi Annem22,

Welcome to the forum.

I’m sorry you have had such a rough time leading up to what is deemed to be a potential diagnosis of ET.

Although you are young because of your recent stroke your haematologist will consider you ‘high risk,’ hence the hydrea discussion.

I don’t know if you’ve seen an MPN Specialist, if not, it’s something really worth considering. Many young ‘high risk’ ET patients are offered pegylated interferon now rather than decades of treatment with hydrea. An MPN Specialist will be best placed to advise you. If that isn’t possible, ask the haematologist that you’re seeing about peg interferon as an option due to your age.

Don’t get me wrong, I’m a great advocate of hydrea, I have been taking it for two years with absolutely no problem; however, I am 62. It works well for me but I totally understand your concern being only 39.

Keep us updated

Mary x

[revfee]

Hello, and sorry you have had such a difficult year.

Do discuss your fears and hopes with your specialist who should be able to explain the pros and cons of different treatments, or none. You are right all drugs have unwanted effects as well as beneficial ones but sometimes its a balance. I have PV not ET but I have been on Hydroxycarbamide for most of the last 30 years without any major side effects. I'm always trying to take the minimum necessary, but the possible risk of leukaemia in the future is less than the risk of stoke of other clotting events so I take it. I was only 29 when I started taking it, though there were less options in those days, so do discuss the other medical options available now so you understand which is best for you.

Good luck

Fee

[annem22 in reply to revfee]

Hi Fee. I am fortunate enough to not have ever had any complications with medications like some people do. I sure hope that I am the same way when it comes to taking hydrea or interferon.

I am sorry to hear that you’ve been dealing with this disease at such a young age but am also glad to hear that haven’t had any major side effects. It must have been very difficult because I can’t imagine 30 years ago, there being much information out there for you like there is now. Thank you for showing me that I can get through this.

[Rachelthepotter]

Hi. So glad you found this forum: having a rare blood cancer can be lonely. If you want more info. the MPN Research Foundation .mpnresearchfoundation.org/Understanding-MPNs is based in Chicago and is a great resource for information and updates on treatment options. Which are developing fast, as you'll see.

Getting medical advice from a haematologist who is part of the specialist MPN network of reseach and treatment is very worthwhile for expertise and access to clinical trials If you have a look at some of the research papers you’ll see the same names cropping up, and if you look at some of the online video presentations you can get a feel for the personality of the doctor and whether you’d get on.

As to drugs, its a question of balancing the risks of drug side effects against the risks of a stroke or other thrombotic problem. It does look as tho there will be new, and better, drugs on stream soon, so hydrea for a while to get your platelets controlled and reduce the present risk of clots , untill better options come along, might be worth trying, as your haem has suggested.

Do keep posting.

All the best

Rachel

PS I guess I ought to say that I have MF, and tried hydrea, but am now on ruxolitinib ( to keep my platelets down) and erythropoetin injections once a week to help counteract the anaemia the ruxolitinib causes. As I write that, I can hear Burl Ives voice in my head singing “I know an old lady who swallowed a fly....”. But the drugs are developing fast, and the newer ones won’t have the same side effects.

[annem22 in reply to Rachelthepotter]

Hi Rachel,

Thank you for the information and support. I’ve been on the website when I was initially diagnosed and sometimes it was just so overwhelming that I had to stop googling.

When I had the most recent blood test done, I was just about starting my menstrual cycle and on oral antibiotics for a staph infection (I feel like my medical problems are never over). This staph infection is another thing I have to deal with and it’s been almost 2 months! The doctors are all aware, but I am hoping the next few blood tests will show decrease in platelets somehow. A girl can hope, right?

Thank you again. I appreciate all the support.

[Susana7]

Hi and welcome to the forum. That was a scary way to find out you have ET... it increases the likelihood of having a stroke, which might explain why you had one in such unusual circumstances. Your pcp did well in taking the high platelets seriously and ordering genetic testing. If I were you, at your age, I would ask your MPN haemo to consider Pegasys interferon as the first line treatment. It is often prescribed to younger patients because it is not chemo (that increases slightly the chances of leukemic transformation in the long term) but instead it is a form of immunotherapy. It is thought to reduce chances of disease progression over time and in some cases it can lead to remission. There are many good articles about it. I was diagnosed initially with ET four years ago, further testing revealed it was PV, and i have been on Pegasys for almost 3 years, with great results. Pegasys is self-administered by a subcutaneous injection every week. It is a slow acting drug. My bloods got progressively better and normalised after a year and I now inject only every 3 weeks, a tiny maintenance dose. I lead a normal life, have two teenage boys and a full time highly demanding job. I am still more tired than before all this, that does not go away, but it is manageable. So please do not worry, there are effective meds out there to control the disease (Hydroxyurea also works well for many people) and you can lead a normal life. All the best to you and let us know how you get on. Susana x

[annem22 in reply to Susana7]

Hi Susana,

Thank you for your note. I am definitely going to discuss interferon with my hematologist. I am glad to hear that you are doing better and live a normal life. I’m a worrier so with the newer drugs, I’m sure I’ll start worrying about how they haven’t been tested long enough and how this could be a problem as well.

I am curious as to how you were initially diagnosed with ET and then to PV.

[Susana7 in reply to annem22]

Please try to focus on the positives: there are people in this and other forums that have had the disease for decades and are doing very well 😊 These days, I forget I have it, except when I go to the haematology clinic for my check up and when I am on the MPN forums!

My platelets were very high (over 1 million) and I have the Jak2 mutation, all else was normal, so was diagnosed with ET. I did not have a bone marrow biopsy. In the next few months my periods became irregular and the hematocrit (percentage of red cells) started to increase (this was before starting interferon). Further tests (a red cell mass test) revealed I had PV, that had been masked by the periods, which act as a natural phlebotomy! The doctor says masked PV is not uncommon in women because of the menstruation. It did not change much for me: interferon was still the recommended treatment as it controls everything, platelets, reds and whites! X

[annem22 in reply to Susana7]

I just read that a lot of insurance companies will deny interferon because it’s an off-label drug. Let’s hope that’s not the case.

[Susana7 in reply to annem22]

I am in the UK which thankfully has a different non-insurance based health system. But many US-based MPN sufferers manage to get interferon approved on their insurances, sometimes on appeal. There is an interferon Facebook group for MPN sufferers with many US patients that exchange their stories on these matters. You need support from your haemo in the initial request or in an appeal.

[GolferCL in reply to annem22]

You should suggest to your Haematologist that HU may not be the best option for someone of your young age and inevitable long term use of medication. Hopefully your insurance company will listen to the doctors!

[GolferCL]

Hi. I’m 43 and diagnosed ET JAK2+ in June. I’m on Interferon rather than Hydrox as I’m “young”, spend a lot of time outside doing sport, etc.

Ask about the options, don’t just take the HU as the first option suggested. I had standard flu-like symptoms at first, but manageable with paracetamol, and 2-months in my platelets are heading down and I have no side-effects from interferon.

[annem22 in reply to GolferCL]

Thank you GolferCL. Did you have trouble with your insurance approving to pay for this drug. I am glad to hear your platelet levels are heading down.

Also did your doctor prescribe you with interferon from the beginning? Were you ever on Hydroxyurea?

[GolferCL in reply to annem22]

I’m in the UK, so luckily none of that to worry about, hopefully you’ll find it’s covered by your insurance.

I was only diagnosed in June, with platelets about 1000 at that time (blood clot in portal vein was the trigger to diagnosis) . I was offered either HU or interferon, with information on both. I chose interferon with support from my Haematologist. My platelets are still coming down, around 510 now, so interferon is working, so not contemplating HU for now. Check out mpnvoice for some great information

mpnvoice.org.uk/about-mpns/...

[BonnieJ]

Hi, glad to hear you are going to Memorial Sloan Kettering here in the USA. As you may know, it is the #2 leading cancer center in the nation. If you can, you should try to continue your care there for the ET as they are also a research facility and would be the most knowledgeable regarding the most promising treatment for this.

Best wishes,

BonnieJ

[annem22 in reply to BonnieJ]

Thanks Bonnie. I do plan on continuing my care at MSKCC. Wish you all the best too.

[Bippers]

Hi Annem22! Sorry you've had such a rough time since January, 2018. I know the feeling. I was diagnosed with ET in 2004. Went on 500mg Hydroxuria daily until 2010. Then, my oncologist started reducing the frequency to 5 days a week, then 4, etc., and finally off completely in 2012. Platelets have been in the normal range since. I take 81mg aspirin 2 - 3 times per week. My hemoglobin count bounces around 10 - 11 and should be 14+. That indicates anemia but I don't notice it. I've had two bone marrow biopsies that show platelets are released earlier than they should be.

I take 1000mg of Niacin (non flushing - important), two 65mg Iron, and at least 1000mg B12 each day.

The only thing I noticed about Hydroxuria was my thinking seemed to be dulled. It improved after I stopped taking the drug.

Others complain about issues with their feet and hands. I did not experience physical issues.

Hope some of this information helps you.

Good luck to you....Bippers

[Mazcd Partner MPNVoice]

Hi Anne, welcome to our forum, as you can see you are in the right place for help and support. We can all understand how worried you are about the whole thing, especially as you have young children, but the outlook for people with MPNs has improved immensely over the last 10 years or so, the research being undetaken around the world is also finding out so much which is all very encouraging, so try not to worry so much, I know that's easy to say. If there is anything we can do to help let us know. Best wishes, Maz