Anyone living with Essential Thrombocythemia with JAK2?

I live in Nellysford Virginia. My doctor is with the University of Virginia Cancer Center. I am extremely pleased with the care.

Hi all,
I'm following several of these ET threads and find them all very helpful and reassuring! A big thank-you to all who contribute. Janemc, your advice seems to always be especially calming and comforting as well as very helpful.

As I've posted in a few other threads, I was diagnosed with JAK2 ET in April at age 77 with a platelet count of 581k, which had been steadily increasing over the past couple of years. My PCP had done tests to rule out other causes such as iron, D, inflammation, thyroid, etc. Referred to O/H specialist who told me to take a low-dose coated 81 mg aspirin a day to make the platelets more slippery and less likely to cause clots which could cause a stroke, heart attack, or pulmonary embolism. This seems to be the standard initial advice for many of us who don't have any contraindications for aspirin if we are at an age (over 65?) that puts us at high risk for clots. He did not mention a BMB and I didn't ask for one. Both he and my PCP agree on the diagnosis of JAK2 ET and I'm trusting them.

He said my age and family history put me at high risk and if my count gets above 600k I would need to go on a chemo pill (presumably HU although he didn't specify). I'll share some other things he told me in hopes it might be of use to someone else:

He said I was not born with ET nor the JAK2 gene mutation and they don't know what causes them nor how to prevent them. He said it was nothing I had done and it is not hereditary (a relief, as I have three adult children). It is something some people develop over time and is rare. He said there is no cure for ET at present but it can be managed well. (Through my reading about it life spans don't seem to be affected if it is treated.)

He said although it is classified as a blood cancer, he prefers to call it a blood disorder, so that's what I'm calling it too in my mind. (I had learned from my own reading that this is not a cancer that spreads to other parts of our bodies, thankfully.) He said it can sometimes turn into a different blood marrow disorder or a form of leukemia over time, but that is not common and isn't likely to happen. Should it ever happen, we'll address that at the time.

He said to not worry that the treatment is a "chemo" pill, that for most people it is tolerated well and doesn't cause you to throw up or your hair to all come out. That was reassuring.

Btw, the first time I was told the word "cancer" was when I received a welcoming email from the medical practice of the specialist, from a "cancer institute." That was a shock but I did a lot of reading about ET. I think that's how I found these discussions. I agree no one should learn they have any form of cancer from an email or an online discussion forum, but I haven't let that bother me.

My platelet count has since actually gone down to the 540s at O/H visit, then up to 560s last PCP checkup, but still under 600k. That's the first time it's ever gone down. I have to go back to my O/H in January for a checkup. In the meantime I will have cataract surgery. I did not want to be on a new chemo drug and then have eye surgery while my body was adjusting to the chemo pill, so I'm so very thankful I won't be. I also have to get my second shingles vaccine.

Sorry this is so wordy!

One thing I really wanted to say is that is is obviously difficult to know WHAT exactly is causing symptoms for those of us with ET. I don't have any severe symptoms, but after reading these posts, I do have some mild ones, but from what?? Maybe not ET and certainly not HU, as I've never been on that yet. Maybe they aren't really symptoms of anything but age?

For example (and I hope this might be comforting to some), I tire more easily than I once did although I have far less to do, being retired. But I'm 78, so I blame that on being age-related. Also, my hair has thinned just a little on top and I have to clean out my hairbrush much more often, but again I figure that's age. My fingernails have some small vertical ridges and have had for some time. I've had problems sleeping through the night for several years although nothing is on my mind. Other than some constipation I don't have GI issues, though, and I've never had a headache or any bone pain.

I said all that to say, even before I was diagnosed with ET and my count got above normal, I was having these mild "symptoms." I've not yet seen much less taken a HU capsule, so they can't be from that either. All I can surmise is that they are just age related or "just life."

My point is that I really, really don't want to have to go on HU, but these "symptoms" I already have aren't from the drug. I don't even take any over the counter meds. However, I've convinced myself I will have to try HU if my platelets get too high, because the alternative would be very high risk for a stroke or heart attack. I'm going to have to remember that I had some mild "symptoms" before any of these new things happened. Therefore, I would urge those who are (like me) very hesitant to take their HU to not jump to conclusions about side effects unless they are severe. I'm going to have to re-read my own post if/when my doctor has to prescribe it for me. I don't want to have to take it. But I also don't want a stroke!

Sorry for my rambling, but reading about all these symptoms made me realize I have some of them in a mild form, and had them before ET and still without any HU. Everyone has to make up his or her own mind, but ET doesn't need to be left untreated because of the risk of fatal blood clots.

God bless all of you and prayers for good health.

Again, sorry so wordy and rambling! I'm a fast typist on a laptop and that can be a bad thing on forums, LOL.

@cec2
thank you for your thorough reply. I was diagnosed with jak2/ Et and had a bone marrow biopsy to rule out other cancer ,( which was a breeze ) . I started on Hydrea 500mg daily (after my platelets climbed to 600.000 ) and a baby aspirin in January of this year . I had very few side effects ( severe migraines were the worst ) ; then in August I began to experience constipation , and bloating( my belly was distended like I was 5 mths pregnant ) . I cried when I couldn’t even button my pants , so I attributed this to the Hydrea . I restricted my diet to one meal a day, exercising more , and eating very healthy to no avail . I discussed this with my H/O ,and she wasn’t willing to decrease my dosage or number of days that I took H. She almost dismissed my symptoms saying I needed to see a GI Dr, and colonoscopy because “most people “ tolerate Hydrea?? . I was tested for thyroid TSH and all normal . After several other medication were suggested ; Analagride ($200/mth after Medicare and supplement ) ) and Interferon ( a shot in my belly once a week and very expensive) , I decided to just monitor my platelets with my PA and take 2 baby aspirin . My platelets have risen from 350 to 460 , but my side effects have all subsided , but another TSH test recently indicates extremely low levels levels , which I will address with PA tomorrow . Might still see a GI also , as in the past I have had IBS from stress .
This diagnosis is forever and everyone is different . I do believe your H/O should address side effects ;,not just focusing on lowering your platelets.

We each experience ET and HU differently.

But we all have one thing in common: When we tell our doctors about issues we're experiencing, we're dismissed.

I for one am going to acknowledge my symptoms (fatigue, headaches, congestion, constipation), try my best to address them, and not expect myself to be brave and jolly every minute of every day.

I am glad to take HU, because I don't want to die of a stroke, or to deplete my bone marrow. But I refuse to say that ET is a walk in the park.

HU comes from China, India and Europe, so every capsule is subject to punishing tariffs. Dramatic price increases and reduced availability loom.

We each experience ET and HU differently.

But we all have one thing in common: When we tell our doctors about issues we're experiencing, we're dismissed.

I for one am going to acknowledge my symptoms (fatigue, headaches, congestion, constipation), try my best to address them, and not expect myself to be brave and jolly every minute of every day.

I am glad to take HU, because I don't want to die of a stroke, or to deplete my bone marrow. But I refuse to say that ET is a walk in the park.

HU comes from China, India and Europe, so every capsule is subject to punishing tariffs. Dramatic price increases and reduced availability loom.

@janemc Droxia, the brand name drug, is subject to US tariffs. My pharmacy uses generic hydroxyurea, and generics are not subject to tariffs right now. However, the political situation with countries that supply generic prescription meds is fluid. They could be tariffed or the countries that manufacture them could reduce supplies to the US as a retaliatory move. Your pharmacist can give you info about what med supplies look like in your location.

@janemc I had what I think was an extreme autoimmune reaction (joint / tendon pain and stiffness, muscle weakness, including difficulty swallowing and severe swelling with red sores on ankles) after taking HU for 1 month. My hematologist said he didn’t think it was possible that my symptoms were caused by HU, but by another medication. I stopped both for a few weeks, but restarted HU after my platelet count doubled. I then had a similar reaction just a few hours after taking the second daily dose. I saw him a few days ago for a follow up, and he finally said he was going to believe me! So big of him! But at least I don’t have to continue looking a second opinion. Now he is hopefully going to work with my rheumatologist to come up with a treatment plan. They are considering Jakafi, but will probably have to try anagrelide first.