A Patient’s Story: Advocating for your Care

By Sherri J.

My platelets were reported to be above 450 in 2015 at my first doctor’s appointment after returning to the United States from an 11-year assignment in Italy. My doctor told me it was nothing to worry about, to take a low-dose aspirin, and he would watch it. I inquired a time or two at follow-up appointments but was assured not to worry, without any explanation. Every time I got tested it was above normal, but I knew nothing about the seriousness of a potential MPN issue.

About a year ago I started having what I know now to be increasing symptoms of ET. I felt as if I was significantly aging with fatigue, lack of appetite, headaches, dizziness. Over the next 3 to 4 months, the symptoms became unbearable. I blamed all of my symptoms on other things: stress and anxiety, menopause, allergies, work life balance issues, my diabetes, my pursuit for my doctorate while working a fast pace full time job with 5 hours or less of sleep. You name it, I was able to tie almost every issue that I was experiencing to something else, so I never brought things up to the same doctor. Soon my platelet count jumped almost 100K and that was after a steady incline over the previous year. My PCP finally put some things together and decided it was time to see an oncologist.

The oncologist I saw was not an MPN specialist. My first sign that this oncologist was not a good match for me was at the initial appointment, I shared in writing and verbally my complete history, chief symptom complaints, and as he was leaving the room, he told me my platelet count wasn’t high enough to cause symptoms for what he has seen with other patients. He totally wrote it off. I didn’t say anything at the time because getting into specialists in my area is quite difficult. I told my husband on the way home, this was not the doctor for me.

The doctor did run extensive bloodwork, and one significant finding was that I am JAK2 positive. Knowing I was JAK2 positive, I decided to call UVA who has MPN experts and see if they were accepting new patients. She was surprised the first doctor did not order a BMB and said I would have that procedure the day of my first appointment if I agreed to it. An hour before my scheduled appointment, the doctor called and advised me I was JAK2 positive and to come back in 3 months. I asked him “what about my symptoms?” He said take a low dose aspirin and he would see me in 3 months, gave me a 1-minute explanation that I likely have an MPN. No talk about a BMB, any treatment, risks, explanation of what an MPN is, nothing.

From the moment I walked up to the desk at the UVA Cancer Center, I felt I made the right decision. I handed the intake nurse the same organized document and she passed it to the doctor before he came in. He was so happy to have that information and said he wishes more patients did that. He spent over an hour with my husband and me explaining what an MPN is, what the test results I had meant, what he would learn from the BMB, what each outcome could be (PV, ET, MF). He explained progression. He made sure every question was asked and fully answered. I advised him that my relationship with my medical team would be just as important as my treatment and that I wanted to feel as if I had a seat at the table for treatment planning. I knew I was in the right place.

Once my BMB confirmed my diagnosis, my doctor felt Besremi was the best course of treatment, but insurance approval could be an obstacle until it is FDA approved. We agreed I would start on 500 mg of Hydroxyurea twice a day and continue the low dose aspirin. At my next follow-up my platelets came down, the doctor said I wasn’t resistant to HU but I wasn’t tolerating it well, so he wanted to approach my insurance company about the Besremi and got approved a few days later.

After some tweaks of my dosing and some side effects, my numbers were perfect and what my doctor was hoping to see. Although my blood counts were all in line, I still have pretty significant symptoms, so he increased my dose of Besremi which really helped. I decreased HU to one 500 mg dose on Monday only. He said I’m super reactive and I may not have to go up to 500 mcg of the Besremi. The trial pushes folks as high as they can stand but he doesn’t think it is always necessary.

So as of early September, clinically, I am in good shape and any raises in the dosage will alleviate my symptoms, the worst are fatigue and headaches. He will continue biweekly dosage increases up to 300 mcg as long as I can tolerate it. He will then leave it up to me whether to titrate or not. There is a balancing act between toxicity and alleviating symptoms, so my feedback is important to him. My doctor is hopeful because HU brought my numbers down and Besremi made them perfect after only two doses. He has seen that that HU success coincides with success with Besremi.