MPN Voices Out Loud
MPN Voices Out Loud
Diagnosis, Symptoms, and Living with an MPN
Hear from an MPN expert—plus a group of real MPN patients! Learn about polycythemia vera, myelofibrosis, and essential thrombocythemia—the rare, chronic blood cancers known as myeloproliferative neoplasms, or MPNs.
Listen to Andrea Larson from Incyte Corporation talk with Incyte oncology clinical nurse educator Paul Larson about important topics for people living with MPNs, like tracking symptoms to watch for signs of disease progression. Also hear from Wilma, Marilyn, Dave, and other members of the Voices of MPN community who share their own personal MPN experiences.
- Learn more about tracking your MPN over time
- Receive resources to help you better understand and promote awareness of MPNs
The content presented in this podcast is not medical advice and should not replace a conversation with your Healthcare Professional, who is the best source of medical information about your individual diagnosis and management plan.
VOICEOVER/ANNOUNCER 00:00
This podcast is sponsored by Incyte Corporation and was previously recorded as part of a live Q&A.
Paul Larson, MSN, NP, Oncology Nurse Educator is an employee of Incyte, and this podcast may include his individual opinions.
The information presented in this podcast is intended to be general in nature and is not medical advice. This podcast should not replace or substitute speaking with your Healthcare Professional. Your Healthcare Professional is the best source for medical information. Always consult your care team with any questions or concerns regarding your individual medical condition.
MODERATOR 00:50
Hello, my name is Gina, and welcome to MPN Voices Out Loud—a podcast series brought to you by the Voices of MPN.
For those of you who are new to the Voices of MPN community, we’re part of an education and awareness initiative developed by Incyte Corporation that is designed to inform, empower, and inspire those individuals impacted by rare, chronic blood cancers like myeloproliferative neoplasms, or MPNs.
Today, we’re joined by Andrea Larson from Incyte, as well as Incyte oncology nurse educator Paul Larson, who will be providing an informative overview of MPNs, specifically focusing on polycythemia vera (or PV), essential thrombocythemia (or ET), and myelofibrosis (or MF). We’ll also be discussing the importance of tracking your MPN over time—and why this can be so critical to advocating for your own health and informing truly meaningful discussions with your care team.
Before we get started, I want to remind our listeners that this podcast is not medical advice and should not replace a conversation with your Healthcare Professional, who is the best source of medical information about your individual diagnosis and management plan.
So now…let’s hear from Andrea and Paul…
ANDREA 02:09
... My name is Andrea Larson from Incyte, and I am joined by my colleague Paul Larson, an oncology nurse educator…
PAUL 02:16
Thank you.
ANDREA 02:17
…Whether you’re a patient or a caregiver, we would like to talk to you about the importance of tracking your disease, identifying trends within your disease, and assisting you in talking with your Healthcare Professional about those trends and how your disease may be changing over time.
ANDREA 02:32
…Paul, in case we have people… who aren’t yet familiar with MPNs, or who may be newly diagnosed, can you please provide us with a brief overview?
PAUL 02:39
Absolutely… MPNs cover a broad range of these…hematologic malignancies. These are chronic blood cancers. The ones that we focus on…are Philadelphia chromosome-negative MPNs. So specifically polycythemia vera, essential thrombocythemia, and myelofibrosis…
ANDREA 02:58
…Great! Can you go into a little bit more detail about polycythemia vera?
PAUL 03:02
Absolutely, polycythemia vera is the most common Philadelphia chromosome-negative MPN. There’s about 100,000 patients in the US with P vera…and it’s characterized by an overproliferation of red cells. So this erythrocytosis is really what it’s predominantly known for: extra red blood cells in the bloodstream causing the blood to thicken and setting up the potential for blood clots. It is potentially going to have an impact on other cell lines like the white count and the platelet count, but traditionally most people with polycythemia vera focus on that erythrocytosis.
ANDREA 03:40
Great. And can you talk a little bit more about myelofibrosis please?
PAUL 03:44
Myelofibrosis…shares some similarities with P vera in that it is a Philadelphia chromosome-negative MPN, but it has some very distinct differences, where even though there’s this myeloproliferation—the bone marrow is overproducing cells…
P vera and myelofibrosis certainly have a lot of overlap in terms of symptomatology: itching, fatigue, achiness, and sometimes sweats, and some of these other MPN symptoms that we’re going to talk about…that are so important to track.
ANDREA 04:12
Sure. And then can you just talk a little bit about essential thrombocythemia?
PAUL 04:17
ET, or essential thrombocythemia, is the other Philadelphia chromosome-negative MPN that we’re talking about... about 80,000 cases approximately in the US. A little bit more common in women than men; P vera, a little bit more common in men than women. And it is also a myeloproliferation. The bone marrow is overproducing cells, and the blood becomes too thick with these extra cells. In ET, there’s a focus and emphasis on platelets or megakaryocytes, or what they see in the bone marrow…
…And so in ET, the patients will have a pronounced platelet count. The goal is to reduce their risk, because by having this extra high platelet count, they are at a higher risk for blood clots…
PAUL 05:02
…About a third of MPN patients are diagnosed through symptomatology. Another third are diagnosed through just routine bloodwork. So that kind of points to how some are more symptomatic than others. And then the final third are diagnosed through an event. Obviously, the biggest thing that we want to prevent in MPN is these blood clotting events, and so some patients are diagnosed because they had a heart attack or a stroke or a deep vein thrombosis, one of these blood clotting events.
ANDREA 05:31
So…can we talk a little bit about symptoms and why it’s important for patients to know them?
PAUL 05:36
So when you’re a healthcare provider you go in and talk with the patient. You get what we call a “review of symptoms”—how are you feeling? You know, activity level. Are you more active, less active? Are you doing the activities—activities of daily living what we refer to them? How is that itching, or have you developed itching? Or if it’s a new patient, do you have itching? If so, how miserable is it or is it mild? Is it just with the shower? Have you found ways to limit it?
PAUL 06:06
Tiredness is the most common MPN symptom, or fatigue, and in terms of its evolution, certainly we’re all prone to having good days and bad days, but you want to have a sense for “is my fatigue getting worse, and why is that?” Are you doing less? Are you missing more events, more social and family events? Are you less active?
PAUL 06:30
Achiness, bone pain is a symptom…And then symptoms related to the spleen are a real hallmark of some MPNs. Very much so myelofibrosis…
…the spleen is an important organ in our left upper quadrant very much involved in our immunology and our blood, and in this myeloproliferative state, people can have an engorged spleen. The spleen can get large, and the technical term for that is splenomegaly. And that can cause abdominal discomfort, aches, and pains. As it’s getting bigger, it can cause early satiety or you fill up really easily when you eat, and that can lead to some weight loss. So spleen-related symptoms are a key part of the MPN10 assessment…
ANDREA 07:13
…So, Paul,…Do patients always experience an enlarged spleen?
PAUL 07:17
They don’t always. And it may be to a varying degree. So some people have very significant splenomegaly; some may have mild to moderate splenomegaly. And probably just as importantly, or maybe even surprisingly, some people aren’t aware that they have splenomegaly. Some people have symptoms of fullness, achiness. Other people are completely unaware that their spleen is big. It should not go below your rib cage, and by definition any spleen that is below the rib cage is splenomegaly…
Your healthcare provider should be percussing and palpating your spleen. If they are not successful at doing that—perhaps you have a larger abdomen, ultrasonography can evaluate splenomegaly…but really a physical exam will usually suffice to have a sense for what’s my baseline spleen? What’s my baseline splenomegaly? And is it getting better or worse?
ANDREA 08:12
…I know some of the feedback that we hear are patients tend to not necessarily recognize some of their symptoms. They almost feel like maybe it’s a sign of them getting older. Can you…can you touch on that a little bit?
PAUL 08:23
It is unfortunately common for people to attribute their symptoms to something else. “Oh, I’m aging or I have this other—I’m too stressed out at work, or my home life.” And a lot of times in hindsight, people realize that these symptoms are directly related to this malignancy.
ANDREA 08:43
So it sounds like their quality of life is really affected.
PAUL 08:46
It can be very much so.
MODERATOR 08:53
Thank you, Andrea and Paul. It sure seems like there’s always more to learn about these rare, chronic blood cancers. If you are interested in receiving educational resources specific to one of the three types of MPNs—myelofibrosis (MF), polycythemia vera (PV), or essential thrombocythemia (ET)—please visit VoicesOfMPN.com/Register.
So, I think we’ve established that there is a range of symptoms associated with MPNs. But I think it’s also important to point out that every MPN patient is unique—and while some patients may be severely impacted by symptoms…others may have no symptoms at all. It varies.
If you or someone you care about is experiencing new or worsening symptoms, it’s important to talk to your healthcare professional. That’s because, as we’ve mentioned, MPNs are progressive conditions—which means that they can change or even worsen over time. This is why keeping track of those changes can play such a vital role in advocating for your own health.
But before we get into a discussion of MPN tracking, let’s hear from a few folks from our Voices of MPN community who can share with us what it’s really like to live with MPN symptoms…
Wilma 10:11
My name is Wilma…well, I just thought I was overworked when I was diagnosed with polycythemia vera…I kept having…dizzy spells…I couldn’t come up with complete sentences…
…as the disease progressed, I started feeling more pain...
…I had an enlarged spleen…from time to time I did have the itching. I had the headaches…
…then I started experiencing the bone pain…
Marilyn 10:39
I’m Marilyn…
…Most people don’t understand my disease…that I’m really sick…
Because, I had the ET for 30 years…
About three years ago…
I was getting really tired...I couldn’t eat very well...
…we were on vacation…my daughter looked at me and said, What's wrong with the back of your legs?
...I was bruised from my knees to my ankles…
…added to the fact, how tired and how sweaty I was…there was just a lot of circumstances that made me stop and think something's changing.
I made an appointment with my blood doctor…to get my blood rechecked…
…and they told me my spleen was enlarged.
nobody really put it all together that it was…really transforming into myelofibrosis...
MODERATOR 11:21
We’ve just heard from two real MPN patients, Wilma and Marilyn, about what it can sometimes feel like to live with an MPN. A little later on we’ll also hear from Karen and Dave about the role that tracking plays in helping them manage their condition and how that has empowered them to advocate for their own health. To hear even more personal stories from our MPN Community, you can visit VoicesOfMPN.com, an educational website sponsored by Incyte.
…So as we already discussed, disease progression is possible with MPNs, which is why it’s so important to keep a close eye on how your MPN may be changing over time. That’s not just specific to symptoms, but also other aspects of your MPN as well, like changes in your blood counts as well as an increase or decrease in the need for certain medical procedures.
Tracking your condition—whether it’s through an online tracking tool or a diary or journal that you make notes in—is a really important way to self-monitor, over time, how your MPN might be changing. Are your symptoms getting worse? Are you needing more phlebotomies than before? Have your blood counts been consistent? These are some of the things that you should be looking at and talking about with your Healthcare Professional.
As you listen to this podcast, please remember that your experience with an MPN may be different. Your Healthcare Professional is the best source of information about your disease.
Now, let’s go back to Andrea and Paul, who are going to discuss the key benefits of tracking your MPN.
ANDREA 13:23
…So you had mentioned that these are…progressive diseases, we know that they can change or worsen over time. This is why tracking changes over time can reveal very important insights about your disease…
PAUL 13:35
…They’re not going to go away, unfortunately. So they have the potential to evolve and transform even.
PAUL 13:41
You can have ET and transform into PV, and you can have PV and ET that will
transform into myelofibrosis, and sometimes a sense that when that’s occurring
symptomatology may be evolving or changing, and that’s one, you know, reason
why we’re emphasizing the importance of tracking your symptoms and these trends
because it could be a representation of some change in your disease status.
And so we definitely want to encourage patients to have a—not an obsessive watch on their numbers, but a healthy understanding of what they are, a good sense of what their baseline is—and to note any significant deviations in that. Because that may prompt further investigation and it would be something that should elicit discussion with their healthcare provider.
ANDREA 14:31
So…what should MPN patients be tracking related to their condition and why?
PAUL 14:36
…the healthcare provider’s going to be very keen on your counts. So, you want to have a healthy understanding of those, but…and they’re going to get those from the lab, and then they’re going to review them and discuss them…if numbers have changed dramatically, that warrants discussion and potential further investigation.
But the symptoms are really going to be key for the patients to report on their own, because your healthcare provider really cannot…send to the lab a symptom panel, right? So that’s your job to track these. On a scale of one to ten, how has your fatigue changed over the last three months? If you have pruritus, or bad itching, has it gotten better or worse? And a scale of one to ten is an ideal tool to use. It used to be a three or four. Now it’s a six or seven. That’s a clearly a worsening symptomatology, and then that’s really your job to report as a patient. And then it’s the healthcare provider’s job to assess why that may be and potentially investigate and make interventions.
ANDREA 15:38
…So earlier you had mentioned the importance of tracking blood counts…can you talk a little bit about what some of the health trends patients should be looking for when they’re tracking their disease?
PAUL 15:38
So I mean the key thing with your MPN is what did it look like at diagnosis? …So if you know what you were presenting with, what was your hemoglobin, your hematocrit? What was your platelet count? What was your white blood cell count? And then tracking that.
…And so your job is just to have a healthy sense of where they are and be able to bring up a discussion if they’re changing dramatically.
ANDREA 16:13
Sure. So how about tracking different procedures that are frequent for each of the three MPNs. For example, should patients be tracking the frequency of phlebotomies that they may be having if they have polycythemia vera?
PAUL 16:27
…So having a sense for how many phlebotomies you're having. If you used to only have two or three a year and now you’re having four or five, why is that?…And sometimes a healthcare provider’s not aware. They’re doing their visit with you and having a review of systems…but not completely aware of your flow of these procedures like phlebotomy or transfusions in myelofibrosis…and in essential thrombocythemia it’s very rare and atypical to have a apheresis procedure, but…they would want to have a sense for how often they were doing that.
ANDREA 17:00
…Can you talk maybe a little bit about the importance of using online trackers or how they may be effective when tracking your disease?
PAUL 17:07
I think it’s just something that keeps it all organized in one place. You know, in your pocket with your device…
If you’re old-fashioned, pen and paper is fine, but sometimes that’s harder to remember or to transition if you’re going from different sites of clinic. Certainly some people will enter things rather methodically and have charts and graphs…but just having a sense for what’s your baseline and then being able to track these trends. And so there are some wonderful tools online that one can use now. It’s just convenient and easy, so why not use it?
MODERATOR 17:48
Thank you, Paul. For anyone who might be interested, you can access an online tracker tool that’s specific to your MPN and allows for tracking key blood cell counts, logging appointments, and keeping track of medical procedures. Visit VoicesOfMPN.com/tracking to get started.
So it sounds like tracking offers a variety of benefits for MPN patients. For one, it can help you more accurately remember changes in your condition without having to rely on memory. And two, when you do it regularly, tracking allows you to see patterns—or trends—that you can discuss with your Healthcare Professional. Finally, having an ongoing record that documents your condition over time can help ensure that your health concerns are taken seriously.
So, what exactly does that tracking experience look like on a day-to-day basis? Let’s hear from a few folks from our Voices of MPN community…
KAREN 18:50
My name is Karen…and I have polycythemia vera…
When I visit the doctor's office, I make sure that I have a copy of my labs in my hands…I have hard copies of everything. I have a notebook, a very large notebook that I keep track of everything. The most current goes on the top, and that way, I can look back and see if things have changed…
The benefit of keeping track of all of this information is to really document your journey, to see where you've been and where you're going. It's almost like a journal, a diary. Keep track of every piece of information that you can get from your doctor.
I think that it's helpful for my doctor for me to keep that information because when I go visit him, I'm more educated and more prepared for his visit.
DAVID D. 19:59
I'm David…I was diagnosed with primary myelofibrosis.
I keep track of basically three things. One is my monthly blood counts including my hemoglobin, hematocrit, red blood cell count, white blood cell count, and platelets. And I also look to see whether or not I have any blasts from month to month.
Besides that, I keep track of different medical procedures, especially bone marrow biopsies. And I just keep files of all of the reports on these things. And then finally, I keep logs of certain symptoms like my pruritus.
The advice I would give to patients, new patients…who are setting up their tracking is to keep it as simple as possible. If you make it too complicated and try to track too many things, you won't do it for very long, I'm afraid. Besides that, you need to make sure that you track when things happen, so as soon as I get the numbers back from my blood test, I sit down and enter them into my tracking.
MODERATOR 21:18
In the end, no matter what kind of tracking tool you do end up using, the message here is to use one—and use it regularly. And then always be sure to share your results with your care team.
And considering that MPN patients may see a variety of Healthcare Professionals, you really do benefit from being an informed patient.
Let’s turn back to Paul and Andrea now and talk a little bit more about how tracking can play a role in making you a better communicator across care teams…
PAUL 21:51
…A common phenomenon in this day and age with chronic conditions…they’re transitioning from one healthcare team to another.
…So a well-informed patient is ultimately gonna lead to better care, because that patient is going to really be able to advocate for themselves to their healthcare team…
PAUL 22:09
…To your point about having multiple healthcare providers, but then they’ve got a whole other boat full of providers for other specialties. They obviously have other health potentials and may have other specialists. Accidents can occur and you end up in the emergency department and they might not know anything about your MPN. So really a well-informed patient can really save themselves a lot of trouble and potentially prevent, you know, more serious outcomes that would have occurred otherwise. So making sure that they communicate to an emergency room specialist for instance, “This is my hematologist contact information. If you have any questions about my blood counts, please call them so that you can talk with them directly.”
If they’re going to the dentist and they need routine care or perhaps something not routine, an intervention of some form. “Please talk to my hematologist first.” Bleeding and blood clotting is a risk with these diseases. Going in and doing procedures raises that risk. So making sure all these providers and specialists communicate together—sometimes they don’t do it on their own, but if a patient helps facilitate that, it’s more likely to happen.
ANDREA 23:18
Right. Again, advocate for your own health and make sure that you track and let everyone who’s a part of your healthcare team know.
PAUL 23:24
Absolutely. As long as everyone’s communicating, patients are going to get the best care…
ANDREA 23:30
So that was a great discussion…don’t you think?
PAUL 23:32
Thank you. Yes.
ANDREA 23:33
Thank you to everyone who joined in…We really enjoyed this…
MODERATOR 23:38
Thank you for listening to MPN VOICES OUT LOUD.
Remember, regularly tracking your MPN may help you discover important insights that can help you and your Healthcare Professionals better understand the state of your condition—and ensure that you are receiving appropriate care. However, the only way those insights will prove valuable is if you share them. Be sure to schedule regular visits and conversations with your healthcare team. And remember, the more open and honest you are about how your MPN is affecting you, the better you and your Healthcare Professionals can find the management approach that’s right for you.
It looks like that’s all we have time for today, but be sure to check out our other podcasts at www.MPNVoicesOutLoud.com. Thanks for joining us today—and allowing us to share our voice with yours in support of the MPN Community.