Polycythemia vera (PV) is a rare but manageable blood cancer. About 2 out of every 100,000 people are diagnosed with it. It’s most common in people over the age of 60, although people of any age can be affected.

Your doctor may diagnose PV after a regular blood test. Once your doctor reaches this diagnosis, you’ll want to see a hematologist.

A hematologist is a doctor specializing in blood diseases and disorders. Any hematologist may be able to help you with your PV. But it’s a good idea to ask if they’ve treated anyone else with this particular disease.

Most hematologists who treat PV and other blood disorders practice at major medical centers. If you can’t visit one of these medical centers, a family medicine or internal medicine doctor can treat you under the guidance of a hematologist.

After your first appointment with your doctor, you should have a better understanding of what PV is and how you can manage it.

Research has shown that the expected life span with PV changes depending on certain factors. According to a recent, multi-center study, being 67 years old or older, having a high white blood cell count (along with a high red blood cell count), and having had a blood clot in the past all lowers life expectancy.

Once you have a better understanding of the disease, the next step is talking about your treatment. Your doctor will determine your treatment plan based on the other factors of your disease, your age, and your ability to tolerate treatment.

Here are some questions about your particular disease and treatment plan that you may want to ask:

  • How controllable is my disease?
  • What are the biggest risks to my health?
  • Will it get worse?
  • What is the goal of treatment?
  • What are the benefits and risks of treatment?
  • What side effects can I expect from treatment? How can these be managed?
  • If I stick with my treatment, what can I expect?
  • What is my risk of developing complications? What happens if I develop them?
  • What are the most common long-term complications?
  • What are my red blood cell count and other blood cell counts? How can I control them? What are my goals?
  • What is the response rate to various treatments?
  • Which other organ systems are affected by my disease?

You may also want to ask how often you’ll need to see your hematologist and if your insurance will cover the costs of your appointments and medication. As well, talk to your doctor about what lifestyle changes you can make at home to help with treatment. Quitting smoking is typically an important step in treatment, especially since smoking increases your risk of blood clots.

In the last decade, there have been advances in understanding PV. Understanding the relationship between the JAK2 gene mutation and PV was a breakthrough in the research. People are being diagnosed earlier and receiving treatment sooner because of this discovery. Now, researchers are conducting studies to try to understand why this mutation occurs.

Living with PV is manageable. Talk with your hematologist often about your symptoms and treatment.