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Treating
Polycythemia Vera

Understanding PV can help you better manage it

By Colleen Doherty, a Board-Certified Physician

How Does Polycythemia Vera Progress?

Polycythemia vera, also called “PV,” is a chronic blood disorder in which a person’s bone marrow (the spongy tissue located in the center of your bones) makes too many red blood cells. People with PV also make too many white blood cells (the cells that fight infection) and platelets (the cells that help stop bleeding by forming clots).

A mutation of the JAK2 gene causes PV. The JAK2 gene creates a protein that helps your bone marrow make blood cells, and a change in the gene (called a mutation) leads to increased blood cell counts.

Experts are not sure why some people develop this mutation. While PV is not inherited (meaning the mutated gene is not passed down from a parent to his or her child), research suggests that some people have a genetic vulnerability to developing PV. In other words, for some unknown reason, certain people are more likely than others to acquire the JAK2 mutation.

PV's Effects

Red blood cells carry oxygen to the rest of your body via the bloodstream. When there are too many of them, your blood becomes thicker than normal, as shown.

Since thicker blood flows more slowly, a person with PV is at risk of not getting sufficient oxygen to their organs, causing various symptoms. Luckily, the right treatment can manage these as well as PV progression.

Discussing Treatments

Doctor advising patient

Although PV isn’t curable, there are multiple treatment options available for stalling its progression and managing symptoms for a long time. Understanding why your doctor recommends a specific one is an empowering way to play an active role in your care.

If you're not sure where to start the conversation, this doctor discussion guide can help. Download it for a refresher on PV-related vocabulary and use the commonly asked questions to learn as much as you can about living with polycythemia vera.

There are several categories of therapies available. In addition to taking them as prescribed, healthy diet, exercise, seeing your primary care doctor for preventive health care, and managing cardiovascular factors like diabetes or obesity are also essential.

  • Aspirin

    Aspirin

    The vast majority of people with PV take a low dose of aspirin everyday. The purpose of daily aspirin is to prevent blood clots. It can also alleviate symptoms of headache, vision disturbances, and burning hands and feet

  • Phlebotomy

    Phlebotomy

    Phlebotomy is a procedure in which blood is removed from your body, similar to what happens when you donate blood. The purpose of phlebotomy is to lower your hematocrit. It will ultimately thin out your blood, which will help prevent the formation of blood clots.

  • Droxia, Hydrea (Hydroxyurea)

    Droxia, Hydrea
    (Hydroxyurea)

    Droxia and Hydrea (hydroxyureas) are chemotherapy drugs taken by mouth. They reduce red blood cells and platelets in your bloodstream to help blood flow more easily.

  • Jakafi (ruxolitinib)

    Jakafi
    (ruxolitinib)

    Jakafi, a JAK1 and 2 enzyme inhibitor, is FDA-approved for those who do not respond well to or cannot tolerate hydroxyurea. It reduces hematocrit and may reduce spleen size and symptoms.

  • Pegasys (pegylated interferon alpha-2a)

    Pegasys (pegylated
    interferon alpha-2a)

    Pegasys triggers your own immune system to attack overactive bone marrow cells. It’s used “off-label” most often in younger people, especially women of childbearing age.

  • Busulfex, Myleran (busulfan)

    Busulfex, Myleran
    (busulfan)

    Because long-term use is associated with higher risk of leukemia transformation, busulfan is typically reserved for older individuals or people who cannot tolerate hydroxyurea or interferon-alpha.

Each therapy is prescribed under a unique situation, so not all options are right for everyone. Take this quiz to learn more about the circumstances each may be prescribed under.

Managing Signs and Symptoms

PV symptoms result primarily from an elevated red blood cell count. That said, a high number of platelets and white blood cells also play a role in certain symptoms and complications.

It’s important to note that the course of PV is quite variable, meaning some people have a mild disease with few symptoms whereas others have a more severe, debilitating disease. Those with few symptoms of PV are often only diagnosed incidentally after a routine medical visit.

And while nonspecific symptoms like headache, dizziness, facial flushing, weakness, excessive sweating, and unusual tiredness are common, there are also specific symptoms to note, along with helpful strategies for managing them.

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