Graphic: At what point should a patient express concern about their symptoms staying the same or worsening to their doctor?
Dr. Yacoub: I think this is a continuous process. Patients with polycythemia vera will live with this disease for the rest of their natural lives. Patients with polycythemia vera have normal life expectancies, so we expect them to live with this disease for the rest of their lives. And we absolutely would like to encourage them to be very open about their symptom burden and symptom pattern and always seek improvements in what we're offering them. There are common polycythemia vera symptoms such as itching, fatigue, fogginess, muscle cramps, and bone pain. Some of those symptoms are chronic in patients, and we try, with our therapeutics, to improve the symptom burden to the maximum we can achieve.
Now, there are certain higher-risk symptoms that we warn patients about that they should always bring to their doctor earlier, such as high-grade fevers, especially in patients who have not had that as part of their baseline symptoms.
So a new-onset fever, new-onset drenching night sweats. That is basically sweating at night, in which the sleepwear or the sheets are wet, and patients have to change, especially if it's happening multiple nights a week. These are definitely abnormal and high-risk and should be discussed with the physician early. And then unexplained weight loss, early satiety, and inability to eat a large meal, and this is resulting in weight loss. These are definitely symptoms that are more high-risk. So for patients with any of those three concerning symptoms, I think this requires a more expedited evaluation by their physicians.
Another concerning trend of symptoms is for patients who have a stable regimen of phlebotomy or cytoreductive agents that has been stable for a while – years – and suddenly, they don't need those agents anymore. Suddenly, the hemoglobin drops or the other white blood cell elements change in an erratic way. That is definitely a concern, and that's when patients should get a disease reassessment and reevaluation.
Graphic: How can patients manage extreme fatigue?
Dr. Yacoub: Unfortunately, fatigue is a symptom of polycythemia vera. It's also a common side effect of every single agent we use for polycythemia vera, as well as a symptom of life. If everything you do in life might cause fatigue, successful therapy for PV can improve fatigue. On the other hand, excessive phlebotomies and iron deficiency can make that worse. So there's a sweet spot between reducing the hematocrit to the right level versus excessively that can result in worsening fatigue. And then, that might be one indication to change therapy in some patients, in which we can find that "sweet spot" where we're reducing the hematocrit and not inducing fatigue, and that could be an indication to change therapy.
There are non-pharmacological interventions. So, aerobic exercise can improve one's energy level and should be recommended for most patients. Better sleep hygiene and better dietary habits can also improve that. There are no specific agents that would actually increase energy, and those are not necessarily options we use in our patients as a pharmacological recommendation. But really, it's all the non-pharmacological interventions that we can apply for PV or other diseases, or even in healthy individuals.
Graphic: Should a patient communicate with you when they have a big life event coming up?
Dr. Yacoub: Absolutely. So again, patients with polycythemia vera can live with these diseases for a long time, and life happens. Pregnancies, traveling, medical complications, elective surgeries, and emergencies. All of these things are part of living with this disease as a chronic disease and a cancer, and we definitely recommend keeping your doctor involved with those decisions and those events as they come – and they will come – and open communication is always helpful for all parties involved.
Graphic: What do you want your PV patients to know?
Dr. Yacoub: I am very excited about the future of polycythemia vera. We have had significant advances recently. There has been an effort at using early intervention in treating patients with low-risk polycythemia vera with interferon, which seems to be very fruitful and effective. There are many clinical trials that are going on, including first-line agents, agents beyond first-line, and very novel agents, such as rusfertide (PTG-300), in which phlebotomy can be eliminated.
So we're very excited about all the efforts and all the patients enrolling in those clinical trials to complete these efforts. So I would like to commend all the investigators, the patients, and their families who have supported these studies. And I would encourage everybody who can hear this to find a clinical trial that would be meeting some of the needs they have and find if there's a good match for them. Talk to your doctors. Go to ClinicalTrials.gov and see if there are any of those trials that you could contribute to, or you could benefit from.
