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Starting 17th year with ET. What's ahead?

Blood Cancers & Disorders | Last Active: Jun 18 10:52am | Replies (78)

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@mikecaldwell

@nohrt4me: I was diagnosed with ET when I was 50 with JAK2. My platelet level was successfully controlled with anagrelide for 7-8 years. After it started being less effective, my hem/onc doctor switched me to Jakafi, which was not common to use for ET at the time, but it successfully controlled my platelet count for 10 years. I also took aspirin daily. At no time during my ET diagnosis did I have fatigue issues. Then 2 years ago, at age 68, my ET converted into secondary ET myelofibrosis (“MF”). My main issues with MF were severe anemia from dropping hemoglobin levels and shortness of breath. I had to start getting regular transfusions to keep my hemoglobin above 7.0. When I went to MD Anderson for a second opinion, my doctor there told me that if you have ET as long as I had, it was pretty certain that it would eventually convert to MF, which my coal hem/onc agreed. MDA recommended that I consider these options in order: (1) a clinical trial, (2) pacritinib or momelotinib oral drugs and (3) a SCT. I first tried a MDA recommended clinical trial, but closer to home at Vanderbilt. It was not successful for me so I then tried pacritinib with no success. I then switched to momelotinib with some success raising my hemoglobin levels and stretching out the periods I needed transfusions. But, a BMB determined that the fibrosis in my bone marrow was increasing and I was told by my local hem/onc that I needed to consider a SCT or risk a likely conversion to AML, which would make a SCT even riskier given my age at the time, 69. I had no comorbidities and was in good physical shape, so I had an allogeneic SCT in October 2024. My donor was an unrelated 28 YO male from Germany and a 12/12 match. After 4 weeks in the hospital (I elected to do this in my hometown of Memphis vs. MDA or Vanderbilt), I have been recovering at home with no infections, and my most recent BMB shows me to be in remission. Recent blood tests show I continue to have 100% engraftment with donor cells. I do have some Graft vs Host Disease issues, but they are manageable so far. I feel most fortunate in my journey. This is likely more information than wanted, but that has been my experience that started with ET 20 years ago. Good luck to you!

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Replies to "@nohrt4me: I was diagnosed with ET when I was 50 with JAK2. My platelet level was..."

Thanks! I appreciate your story. I do know that progression to MF is likely if you live long enough, and we CALR+ may be more likely to that than the JAK2+ folks.

I have two cousins, both in their late 50s, who had AML, both had SCT with family donors. One is alive, but very immune compromised. Often in hospital. The other died of heart failure soon after the SCT.

I have a cardiac issue and am 70+, low income, so not a good candidate for SCT. I would choose hospice if I were diagnosed with AML. But glad to hear SCT has worked for you!

Meantime I'm trying to stay as healthy and interested in life as possible.