Living with Polycythemia Vera (PV): Dana Turner Shares How Self-Advocacy Has Improved Her Care

Dana Turner was a young, healthy adult who had not had routine bloodwork for a decade. While she now reflects on having high platelets and migraines since childhood, there was no red flag on her medical charts that signaled cause for concern. So, like most young people busy with school, career, and family, when her annual medical appointments didn’t include a complete blood count (CBC), it didn’t occur to her to ask about it.

Blood tests led to a PV diagnosis

In fact, Dana’s platelets were high as a young child. When she began having migraines with visual disturbances (aura), it was thought to be “something that happens,” particularly in young girls. She also had symptoms of Raynaud’s disease, a condition in which blood vessels narrow in response to cold, most often in the fingers and toes. It can cause numbness, blue coloration, and pain.

When at age 29, her primary physician ordered a CBC, her platelet count was in the 800,000 to 900,000 range. Normal for her age would be 150,000-450,000. She was sent to a hematologist and diagnosed with essential thrombocythemia (ET). Not long after, a bone marrow biopsy confirmed she had a JAK2 mutation and also a rising hematocrit. Hematrocrit is the percentage of red blood cells. This led to a revised diagnosis of polycythemia vera (PV) in October 2019.

Dana was prescribed a “watch and wait” treatment protocol, along with a daily baby aspirin and phlebotomy treatments as needed to maintain her hematocrit under 45.

Exploring interferon therapy as a younger patient

“I became iron deficient with repeated therapeutic phlebotomy treatments and felt exhausted constantly,” Dana recently told HealthTree. “The brain fog was the worst. I was concerned about my hematocrit and hemoglobin barely improving, my platelets continuing to rise, and the risk of disease progression was always on my mind.”

So, she decided to research other treatment options. She didn’t want to try an oral chemotherapy pill such as hydroxyurea, and her spleen was not enlarged, so she wasn’t a good candidate for ruxolitinib (Jakafi, Incyte).

“I knew that interferons were successfully prescribed in Europe for younger patients as a first line of treatment,” she explains. “Besremi was on the horizon for FDA approval in late 2021. I decided that this may be the best course of treatment as a younger patient who felt that therapeutic phlebotomies weren’t accomplishing any of my goals.” 

Dana approached her hematologist without knowing how the idea of starting this long-lasting interferon would be received.  

“I remember this conversation vividly! My husband attends all my hematologist appointments with me, and during this one visit, we determined together that we would discuss Besremi now that it was finally FDA approved in January of 2022. I was grateful that she was so open to the idea of me starting treatment.” Dana started the interferon therapy within a few weeks, in February of 2022, and she has been on it since.

Interferon is an injectable treatment which is administered once a month.

Progress toward stability

“The one thing I learned early in my experience is to be patient. I didn’t notice any results in my blood work for several months, with my platelets being the slowest to respond,” recalls Dana. “Besremi also requires very frequent blood work.” This frequent blood work is to establish a therapeutic dose and determine therapeutic response.

“I was getting blood work completed biweekly for about a year, until I reached my maintenance dose and all blood levels began stabilizing and responding, around four months into my treatment,” she says.

Most exciting, she noticed that the frequency of her debilitating migraines greatly reduced. After just a few months, they only occurred about every six months, from her usual once a month or so. “I also slowly began regaining my energy as my iron levels improved over time. I’ve now reached hematological stability. I haven’t needed a phlebotomy since starting Besremi. And my blood work has been stable since the summer of 2022.” 

Dana says she hasn’t had a bone marrow biopsy since starting interferon, so she is unaware of her allele burden. This is a measurement of the proportion of mutated cells in the bone marrow compared to normal cells. While she says she isn’t very concerned right now about whether or not the interferon treatment has had any effect on the disease itself, she would certainly like to know.

“Besremi is working so well for me. I have had few, if any, side effects and tolerate it so well,” she says. “However, if there was an easier way to measure disease-modifying results, [other than another bone marrow biopsy], I’d be curious to see the molecular impacts of Besremi on my PV.”

Advocating for different treatment options can improve outcomes

“Since my diagnosis, I learned that self-advocacy is key. I was initially placed on a watch-and-wait approach due to my young age and general good health. But I didn’t want to wait for something to happen, like a cardiovascular event or a blood clot. Instead, I researched treatment options, shared my ideas with my hematologist, and started on a long-term treatment that felt right for me, within two years of my diagnosis. I can’t imagine what my daily life would look like if I didn’t self-advocate.”

Dana adds: “Sometimes hematologists and other doctors who are not MPN specialists and don’t follow all the data or current research, so it’s up to us to bring our education to them in order to take the next action step in our treatment. Don’t be satisfied with a ‘watch-and-wait’ protocol. Research medication options, clinical trials, or other opportunities with your doctor to get ahead of your PV diagnosis to avoid disease progression.” 

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