What’s Next for Improving Quality of Life in Polycythemia Vera?
What are key challenges in myeloproliferative neoplasm (MPN) care, and how do symptoms evolve over time? Expert Dr. Andrew Kuykendall from Moffitt Cancer Center discusses constitutional MPN symptoms, strategies to manage fatigue, neurovascular symptoms, and symptom management.
[ACT]IVATION TIP
“…we have a variety of symptoms that can be associated with kind of myeloproliferative neoplasms as a whole, as well as each one of these distinct disease entities. And the therapy for each of these differs based on the particular symptom.”
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Transcript:
Lisa Hatfield:
Dr. Kuykendall. I’d like you to speak to some of the key challenges in managing MPN symptoms. So based on the available treatment options, what are the most challenging symptoms associated with polycythemia vera, PV, or myelofibrosis and essential thrombocythemia, ET? And what are we learning about how these symptoms evolve as the disease progresses?
Dr. Andrew Kuykendall:
When we think about myelofibrosis, that’s probably the disease we associate with the most clear, distinct symptoms. And patients often have what we call “constitutional symptoms.” And this could be fevers, chills, night sweats, bone pain, weight loss. These are classic symptoms of a disease that is really causing a lot of inflammation, right? And driving a lot of these inflammatory pathways, and these types of symptoms are quite well-addressed with JAK inhibitors, these disease specific anti-inflammatories, of which we now have four that are approved for myelofibrosis in different capacities. But there are more symptoms beyond those. I think when we think about polycythemia vera, we get less constitutional symptoms, although that certainly can be seen in a subset of patients.
But we see more itching is probably the classic polycythemia vera symptom. This itching that is quite challenging, doesn’t necessarily respond to antihistamines, and can be something that’s exacerbated by like taking hot showers or being in hot water. There’s a fancy name for it called aquagenic pruritus. And patients may not even be aware this is related to their disease. I’ve met many patients who’ve come in, who’ve been diagnosed with PV who complain of this challenge with showering or being in hot water, who really never put two and two together.
And sometimes the itching isn’t even described as itching. It feels like fire ants all over their body. And you have patients that are really avoiding right, showering. And so they’re doing it maybe once a week or once every two weeks. And so again, this is a symptom that responds quite well to ruxolitinib (Jakafi), which is approved in the second line here.
Beyond that, I think the biggest symptom across myeloproliferative neoplasms is fatigue. And I don’t have great magic tips for fatigue other than to say what we found out is probably non-pharmacologic interventions are better than pharmacologic interventions for fatigue. Things like just making sure you have good sleep hygiene, getting good sleep, healthy diet, exercise, yoga, mindfulness, resting, these probably are more successful in treating fatigue than any specific drug or agent that we have. And I think that speaks to really a failure on our part to develop better therapies. But certainly it’s something that we’re all very well aware of. And so it’s something we monitor in any of our clinical trials when we are developing agents is how does fatigue change over time? And lastly, I’d say for ET I think you can start to see some symptoms that are more kind of neurovascular.
So things like headaches, migraines, ringing in the ears, or tinnitus, or tinnitus. These can be unique to ET and may predate or preempt the actual diagnosis. So a lot of our young patients that are diagnosed with ET may come to attention of physicians because they’ve been dealing with migraines or headaches or fatigue plus migraines and headaches for a long time. Then lo and behold, blood work shows that they have a very high platelet count.
And so over time, I think when we look at these symptoms, certainly there can be waxing and waning of some symptoms, response to therapies, lack of response to therapies. But if the symptoms really do change rapidly, which we don’t see that often, oftentimes this can accompany a change in the disease, right? And that’s the time to go in and see if something’s changed. Sometimes reevaluate the disease status. And so my [ACT]IVATION tip for this is that we have a variety of symptoms that can be associated with kind of myeloproliferative neoplasms as a whole, as well as each one of these distinct disease entities. And the therapy for each of these differs based on the particular symptom.
