Essential Thrombocythemia: Making treatment decisions

I have a strange correction. ET was getting under control and I kept saying I felt worse. My cardiologist said I have POTS and each med made me feel worse - turns out it was Me and not the meds.
I don’t have POTS - I have NPH, Normal Pressure Hydrocephalus. The neurologist sent for an MRI and the fluid buildup in the brain was even visible to me. I have every symptom - you can read them in a hydrocephalus info page.
Next is a spinal tap, then if I show improvement, a shunt.

I was afraid they’d call me a hypochondriac because nothing helped…
I love having a diagnosis.

@exon9 Statistically speaking, patients with the CALR mutation tend to have fewer blood clots than patients with the JAK2 mutation. Practically speaking, that means that hematologists may start hydroxyurea later for CALR patients under 60 with no clot history than for JAK2 patients.

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I've been taking Hydroxyurea for several months now for ET and Polycythemia both, and it appears to be working well. I was afraid too, but I've found that it's not too bad. It just makes me get very tired (exhausted) if I do too much, and I just have to go sit down. Don't be afraid.

@exon9 I don't claim to be a huge expert on this, but here goes. CALR is a protein that is involved in signaling the production of blood components. With MPN's in general, the DNA that codes for the CALR protein has been changed in someway to cause the increase in platelet production. Think of the DNA and proteins as letters and words. If the letters are in the correct order it makes the word we know, but in the wrong order its meaning is changed - think of meat vs meet or plane vs plain or rain vs rane [one is correct spelling, the other not]; they sound the same but the meanings are not. There a number of great resources to explain this in much greater detail (MPN Research Foundation - https://mpnresearchfoundation.org/ , Blood Cancer United - https://llsorg.widen.net/view/pdf/juiq8xkd2b/en-booklet-mpn-detail-ps81.pdf , Voices of MPN - https://www.voicesofmpn.com/essential-thrombocythemia) as well as your doctor.

Meds to help control this are constantly evolving and the scientific community is making some really interesting strides in this area, from some of the things I have seen lately there is reason for optimism in the not to distant future.

My platelets got over one million. I had been on HU but after 6 months I had a reaction. Hematologist started me on Besrimi injections. I found out I had a bruit in my carotid artery and my hematologist said my platelets had to be in the 400 range before I could have surgery. I continued with the injections with those being in increased every month. When I started having symptoms from the bruit I called my vascular surgeon and they told me to go to the ER. When I saw the dr he said he was admitting me and was going to go ahead and clean out the carotid. I was concerned about my platelets so when I was admitted they immediately started me on heparin. Had the surgery the next day and my platelets have been in the normal range since then. I was still on the Besrsimi injections but I started cutting it back each month until I got down to 50. I was up to 475. My last injection was in October and they are still in the normal range. I don't know if the surgery had anything to do with it or not but I am thanking the Lord for helping me.

I told the hematologist that I had started backing down on the Besrimi and she asked me why? My answer was I have a lot of people praying for me and how will I know the Lord has healed me if I continue to take this. She was in agreement with me. However, I have not seen the hematologist since I quit taking it. I have an appointment next month.

@tree7737 I have been on Hydroxy for a year. My platelets were very high at over 1 million. Medication has brought them down to the 500,000 level. My side affect's dry eyes, I have lost a lot of hair in the last year. I have been using systane of cream at night. My problem with that is that my eyes stay cloudy most of the day. I also use warm compresses, that seems to help.I am 84 I’m sure my fatigue, has something to do with my age. Good luck to you.