Donna, a retired speech-language pathologist and public-speaking teacher who has been living with polycythemia vera (PV), a rare, chronic blood cancer known as a myeloproliferative neoplasm (MPN), joins Peg Squier, M.D., Ph.D., Group Vice President, U.S. Medical Affairs at Incyte, to discuss Donna’s journey with PV since diagnosis. Tune in as they share advice for patients who are looking for new options to help manage their MPN and the importance of being an advocate.
This episode of The Mighty Podcast is sponsored by Incyte.
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Episode Transcript:
Mighty Host
Welcome to The Mighty Podcast, where we infuse the health space with positivity, humor, and vulnerability. The Mighty is a safe and supportive community here to help you find the people and information you need to navigate your health journey. We’re so excited to spend some time together today. This podcast episode is sponsored by Incyte.
Now, let’s get into what the health we are talking about today. And today, we are talking about polycythemia vera, or PV, which is part of a group of rare, chronic blood cancers called myeloproliferative neoplasms, or MPNs, and how one patient manages her disease.
So, without further ado, I would love to welcome our two guests today. First, we have Donna, who is living with polycythemia vera, a type of MPN, and Peg Squier, M.D., Ph.D., Group Vice President, U.S. Medical Affairs, at Incyte. Thank you both for being here today.
Donna
What an honor it is.
Peg
Thanks so much for having us, Ashley.
Mighty Host
Thank you. So, I gave your technical backgrounds, but I would love to have you share a little bit more about yourself. So Donna, let’s start with you.
Donna
I’m excited to be here and share my medical journey. But a little bit about me as a person instead of a patient is that I am a retired speech-language pathologist and retired public-speaking teacher. I have devoted my life to education. I am also a mother of two incredible sons. I’ve been married to my husband for 45 years. We live in the swamp in Mississippi. And I have a dog named Bentley.
Mighty Host
Love that. What about you, Peg?
Peg
Yes, so I work in Medical Affairs at Incyte. Our team has two main jobs. We provide information on our medications to everyone who needs that — so anyone from physicians and care team to patients and their loved ones. And we also do research and support research done by the community using our medications and also better understanding the diseases that they’re used to treat.
Mighty Host
I love just the varied perspectives we get between the two of you. So, thank you for sharing a little bit about yourself. And thank you in advance for what you’re going to share about yourselves and your knowledge moving forward.
And before we really get into the nitty gritty of our topic, I just like to warm things up a little bit. I have a quick question for both of you to answer. So my question for you is, who would be your dream dinner guest and why? I’m gonna go backwards this time. So, Peg, let’s start with you.
Peg
OK, dream dinner guest. You know, there are so many people in the world that would be amazing to have dinner with so many people from history, so many amazing people in science. So many people in government or people helping humanity. But you know, when you first asked the question, the person who came up in my mind was Jim Henson, the creator of The Muppets. The reason why is he always struck me as being a really creative, and kind, and gentle, and funny person. And he always, throughout his life, helped people — you know kids, but adults learn and always help people laugh. And I just think right now, maybe more than ever, both of those things are really important.
Mighty Host
Yeah, absolutely. That’s a great answer. Think of Mr. Rogers and those kinds of folks, too, who just have left that lasting impression on so many people growing up.
Donna, who would be your dream dinner guest?
Donna
I think that Dr. Martin Luther King would be a very special guest for me. I’ve always admired him; he lived his life with such purpose. And he used the power of his words to leave a lasting legacy on mankind. And I think that is what I like to do. Also, being raised in the deep South during the time of the 60s. And so I just admire him so much for taking this stand for equity. And I would have so many questions for him. I mean, ultimately, he sacrificed his life for his cause.
Mighty Host
I love that both of your answers just kind of speak to the larger impact that those individuals have kind of had on people and history as a whole. So I think those are wonderful answers; those would be wonderful dinners.
But with that being said, let us jump in to our diagnosis journey. So, the first thing I want to ask just for anyone who does not know about the condition, Peg, can you tell us just a little bit more about polycythemia vera?
Peg
Of course, yeah, so polycythemia vera, or PV. It’s a rare, chronic blood cancer, and it’s where a person’s body makes too many red blood cells, white blood cells, and platelets. PV is part of a group of diseases called myeloproliferative neoplasms, or MPNs. Although PV can occur in people of any age, it’s more common later in life. The average age when someone’s diagnosed with PV is 60 years. About 100,000 people in the U.S. live with PV, and it affects slightly more men than women. Now, PV can be managed. There are treatments available that may help people keep their blood counts under control. But it’s important to know that PV is a chronic, progressive disease, meaning it can worsen over time. In a very small number of people, PV can lead to other blood diseases, such as myelofibrosis, or MF, a disease in which scar tissue develops in the bone marrow, or it can lead to leukemia. That’s why it’s important for people living with PV to track and monitor their condition and engage in regular conversations with their healthcare team.
Mighty Host
Thank you so much for that background. It’s really helpful to hear that kind of you know what’s happening on the body’s end. And so now I’d love to know kind of the other side of it, which is, you know, that real-life experience. So Donna, what did your journey look like from when you first started noticing symptoms to receiving that PV diagnosis?
Donna
What a journey it’s been — a very long one. I’m unsure how long I had symptoms, but I was sick for years. And I was misdiagnosed through the beginning of my disease journey after medications with a cardiologist failed to improve those symptoms that I was experiencing — which included fatigue and dizziness. I just decided to become my own advocate.
I went to a local nurse practitioner, and I requested bloodwork, believing that something pervasive was impacting me. And I was hoping that there really would be something in my results that would give guidance on what my true diagnosis was. Well, I remember that day when the blood work came back, and the nurse practitioner requested that I come back in her office. And when I arrived, she asked, “Do you have a hematologist or an oncologist?” and I took a deep breath. When I heard those two words, I thought, “Wow, there is something, and it’s in this bloodwork, and maybe it has to do with the blood itself since she asked the question about hematology.”
My nurse practitioner shared that although she didn’t know what was wrong with me, she saw that my hemoglobin, my hematocrit, and my platelets were extremely high. And then she referred me to a hematologist/oncologist, and that’s when my journey to find true diagnosis with polycythemia vera began.
Mighty Host
Great, that makes sense, and I think we, that’s something we see so much is people becoming you know their own advocate for their health because they’re not seeing the results from the doctors the way they want to, and you know you want to be able to get the support you need.
I’m curious, Donna, how was your day-to-day affected by those symptoms?
Donna
I can’t even describe the impact it had on my day-to-day life. I was so dizzy, so fatigued that I could hardly walk. I could not drive for fear that I might get in a wreck. And my career has transformed into a national motivational speaker. And because of so many symptoms with PV, I had to put my career on hold as well. So, it affected me personally and professionally.
Mighty Host
That makes a lot of sense. Peg, let’s go to you. So, based on your knowledge of PV, are these symptoms that Donna experience pretty typical for people who live with the disease?
Peg
Um, yeah, absolutely. I mean common symptoms of PV are fatigue, day or night sweats, itching, bone pain, brain fog, and abdominal pain or discomfort. We know from studies where we systematically collect information about the experiences of people with PV, what we can typically see. One such study was REVEAL, which enrolled about 2,500 patients, and in that study, the most common physician-reported symptoms included fatigue, itching, insomnia, and muscle aches or bone pain.
Mighty Host
Those things for sure line up. With that kind of in mind, Donna, what led you — you know you spoke a little bit to this already — but really what led you to seek that support from your doctor?
Donna
My symptoms were just so persistent and never decreased. Even though I was on five different hypertensive medications from my cardiologist, I was still so fatigued and had so much lightheadedness. I also did have the bone pain and the itching that Dr. Peg referred to. So, I just realized that there is a root to a problem that the medical community is missing.
And I had — true, I had hypertension — but I think that was not the real root; it was a symptom. And I am a problem-solver. I wanted to advocate for myself and to figure out what was truly wrong with my body. What was the root? What was the problem so I could get a solution?
Mighty Host
Absolutely, and when you were pursuing that and that support, what type of healthcare provider made that diagnosis for you?
Donna
Well, as I stated earlier, it started with a nurse practitioner just because I was desperate. And I’d already been to specialists, so I was to the point of just anyone with medical knowledge and a phlebotomist who could gain some blood so we could get some information.
So, she then referred me to a hematologist/oncologist. And it’s kind of funny that he wasn’t that familiar with PV; he just didn’t have a lot of patients with it because we do know this is a rare disease. So he referred me to another specialist, another oncologist who kind of specialized in it. She’s not in MPN, but she’s seen a great deal of those patients.
Mighty Host
I love that. And I really love when you know doctors say, “I don’t have this experience the same way. Let me give you somebody who does,” because that really only benefits you at the end of the day. So we talked about the symptoms that you experience, we talk about getting that diagnosis. So now you have those things going on, you know what’s happening — how did your life and relationships change following that diagnosis?
Donna
Well, my personal relationships really haven’t changed that much since my diagnosis. I’m a bit of a private person — so as far as telling people about my condition — because I believe that everyone has their own crosses to bear, and this is mine. So it doesn’t interfere with friendships; we just keep on keeping on, and we really avoid the elephant in the room. I try not to complain about what I’m going through, and I just have been blessed that I have a manageable condition. And that I have a family that supports me.
Now, my life has taken a tremendous turn. At first, you know, there were moments of despondency because I thought to myself, “What is my life going to look like from here on out?” and I spent a great deal of time researching the disease so I could be a great advocate for myself. And I became really confident in the research, the disease, medications that are available. So, I could be really passionate about my health and getting my life back.
Mighty Host
And at the end of the day, it’s best quality of life always is the best place to be that you can find. You talked a little bit about you have, you had that support in your mom and your husband. Who else did you turn to for support? What did that support look like?
Donna
As a communication specialist, I felt it’s very important to meet with other people that are walking the same walk. And I checked in to any kind of local support group. We didn’t have one in this area, so I joined an online support group for PV so I could just see what other patients were going through, what medications were working for them. And I am a faithful person so I turned to my faith to get me through a lot of the obstacles that I had to overcome.
Mighty Host
Absolutely, and I love that you were able to find some community. So, thank you for sharing a little bit about that background. At the end of the day, you end up with the same diagnosis, but the way that you get there can be so different for everyone. And it’s just always good to hear the way that somebody did because if you are questioning, it helps kind of inform who do you talk to next about it.
So, let’s move on a little bit into treatment options. So Donna, following that diagnosis and all of those things leading up to it, what did treatment look like for you?
Donna
The first treatment that my oncologist prescribed was hydroxyurea, and honestly, it just did not work well for me. I experienced various side effects. And actually, I felt worse than I did before the medication. These had such an impact on me. And I knew that I needed another treatment option.
Mighty Host
That makes a lot of sense. So what then led you, you know, to try Jakafi as that treatment option?
Donna
I noticed I was experiencing several side effects while taking that other treatment as I stated earlier, so I began doing my own research to see what other options were out there. And that’s when I, as an advocate, actually asked my oncologist about Jakafi to determine if it was the right treatment option for me. And after discussing it with my doctor and coordinating it with my insurance company, my doctor was excited to prescribe Jakafi.
Mighty Host
And Peg, for people who do not know about what that is, can you share a little bit more about Jakafi itself?
Peg
Sure, of course. Jakafi®, or ruxolitinib, is the first FDA-approved prescription medicine for adults with PV who have already taken a medicine called hydroxyurea, or HU, and it did not work well enough or they could not tolerate it. Jakafi is also the first FDA-approved prescription medicine for adults with intermediate or high-risk MF. Jakafi is available in tablet strengths of 5, 10, 15, 20, and 25 milligrams.
Jakafi can cause serious side effects including low blood counts and infection. Some people who take Jakafi have developed certain types of non-melanoma skin cancers. Increases in blood cholesterol levels can also occur. In patients who took another JAK inhibitor to treat rheumatoid arthritis, there was an increased risk of potentially fatal cardiovascular events like heart attack or stroke in patients with risk factors for these events who smoke now or smoked in the past, as well as an increased risk of blood clots in legs or lungs and new (secondary) cancers like lymphoma, especially in patients who smoke now or smoked in the past. The most common side effects of Jakafi for certain types of myelofibrosis and polycythemia vera include: low platelet or red blood cell counts, bruising, dizziness, headache, and diarrhea.
You should call your Healthcare Professional for medical advice about side effects.
Please see the Important Safety Information and Full Prescribing Information at Jakafi.com, which includes a more complete discussion of the risks associated with Jakafi.
So Donna, can you talk about since starting Jakafi, what differences have you seen in your blood counts?
Donna
I can only speak from my experiences, knowing that every person’s PV experience is different, but within a couple of weeks, Jakafi started to control my red blood counts, my platelets, and my hemoglobin levels.
Mighty Host
Peg, what should be considered when a doctor develops a treatment plan for a patient?
Peg
When developing a treatment plan, a doctor should consider a patient’s medical history. Their symptoms should be systematically assessed using an MPN Symptom Assessment form total symptom score. That’s a 10-item instrument designed to monitor the clinically relevant symptoms among patients with MPNs, and they should also use results from a physical and laboratory evaluation. Now, when considering any treatment, including Jakafi, an overall risk assessment should be conducted. Treatment with Jakafi can cause thrombocytopenia, anemia, and neutropenia which are each dose-related effects of Jakafi.
Mighty Host
So then when and why would doctors use Jakafi for treating PV?
Peg
A doctor would recommend Jakafi to a patient with PV who has had an inadequate response to or is intolerant of hydroxyurea. Jakafi is also approved to treat patients with intermediate- or high-risk myelofibrosis, and that includes primary myelofibrosis, as well as post-polycythemia vera myelofibrosis, and post-essential thrombocythemia myelofibrosis in adults.
Mighty Host
So, how important are proactive conversations between patients and healthcare providers when trying to find that right treatment option for the patient?
Peg
Proactive communication is really critical between patients and healthcare providers when determining the right treatment plan. Changes in how a patient is feeling is an indication that it may be time for healthcare teams to determine a different treatment plan. Changes in symptoms could indicate multiple things, including an elevated hematocrit or red blood cell volume, elevated levels of platelets or white blood cells, an inability to reduce an enlarged spleen, any of a number of major side effects, and that the current treatment plan just no longer works.
Mighty Host
Thank you for explaining that whole process and what that looks like. Really think helpful to know what patients might be going in for when they’re looking for treatment options. And hopefully it gives them a little bit of you know knowledge to bring into it.
So, you found the right treatment option for you so Donna, how do you feel now? And how has your life changed since starting Jakafi?
Donna
I’ve been able to resume all of my daily activities that I did before the diagnosis. And professionally, I’ve been able to continue with my busy schedule as a motivational, educational speaker. Although I still stand in front of large audiences of educators, I kind of use any platform time I can have to talk about the medication and the journey that I’ve been on with PV.
Mighty Host
Thank you, and I’m sure you are teaching so many people so many helpful things. And so I always admire people who can go up and do that kind of speaking like that. So I love that that has become a part of your life in this regard as well.
So let us jump down to our Self-Care Corner, where you know we like to know you know a little bit more about you to really help round out this conversation. And what makes you feel relaxed? What makes you feel good? So, what self-care practices have you done lately? Let’s start with Peg on this one.
Peg
Well, one of the things that I have been doing lately is I have been getting to the gym regularly. And I have come to the realization that I can’t do this alone. If it’s just up to me, I’d never go. So I make myself set appointments with a trainer, and I go see a trainer three times a week, and that has kept me going in. So sometimes you just have to recognize when you have to lean on other people to help you meet your goals, and so that’s how I can take care of myself the best it turns out.
Mighty Host
I love that, and sometimes you really just do need that accountability. You’re like, “I’m ready and willing,” but you can find any reason not to, but if you have a reason to. I love that I definitely lean on other people for that accountability as well.
What about you, Donna?
Donna
Well, I’m a type A personality who rarely slows down. And so I’ve made myself a goal — not as exciting as Dr. Peg — but my goal is just to sit on my deck, look at my pond, and plant a few flowers, and read just to kind of meditate and just reflect on my day and be very thankful for everything that I have been blessed with.
Mighty Host
I think they’re wonderful because they’re actually very different things because, Peg, you’re talking a little bit about like, “How do I wake my body up for the day? How do I move it?” And Donna, you’re talking about, “How do I you know let my mind relax? How do I unwind?” So, I think like you’re speaking to both sides of the coin there. And I love that.
Now, I’d love to go into our CheerMeOn section. What made you feel empowered recently? What made you feel Mighty this week? So, let’s go the other way around. Donna, you can go first.
Donna
Well, I’m excited to share how Mighty I feel. I just came back from Yosemite, and I walked two miles, which is something I would never have been able to do before the diagnosis of polycythemia vera. So I felt pretty Mighty about that.
Mighty Host
That’s incredible. I’m sure that was a beautiful thing to see; definitely on my bucket list.
What about you, Peg? What’s made you feel Mighty this week?
Peg
Mine is not nearly as cool as tackling Yosemite. Well, it was cool, but maybe not quite as active. Mine was getting to go to my niece’s wedding. I don’t know if you’ve had the experience going to weddings where you’re just — you have your fingers crossed, you’re kind of hoping for the couple. This one, there was just so much energy, so much happiness, so much joy between her and her new husband, and you just know they’re going to be great. And so to be there and support them was just this amazing feeling of joy and energy. And it gave me so much hope for the future that if there can be that much happiness in one place, maybe everything’s gonna be OK.
Mighty Host
That is beautiful. I love that. It is very exciting. I don’t think we can compare Yosemite to weddings. They both have beautiful impacts on our lives in very different ways.
All right, so let us start to kind of wind down and wrap things up here. So, I just like to you know speak a little bit wider to that PV community. So Donna, what advice can you give those in the PV community when advocating for themselves with their doctors?
Donna
One thing that I want to attest to is to be your own advocate, especially in treatment options, and just talk to your doctor about which one makes the most sense to you. And I also implore other patients to really research the treatment options on their own and just offer them to their doctor for their professional consideration.
Mighty Host
Wonderful. Well, thank you, Donna. Thank you, Peg, for being here. Thank you, Donna, for sharing your experience with PV, thank you, Peg, for sharing all of your knowledge about it as well.
Donna
Thanks!
Peg
Thanks so much for having us!
Mighty Host
And thank you, audience, for listening to this episode of The Mighty Podcast. And thank you to Incyte for sponsoring this episode. If you want to continue this conversation, head over to TheMighty.com or download The Mighty app to become part of our community. We’d love for you to follow us and give us a rating and review on Apple Podcasts or Spotify, or if you’re listening on The Mighty, give this page a heart. Join us on our next episode, and stay Mighty.
Please stay tuned for some important safety information about Jakafi.
Peg
Important Safety Information
Jakafi can cause serious side effects, including:
Low blood counts: Jakafi® (ruxolitinib) may cause low platelet, red blood cell, and white blood cell counts. If you develop bleeding, stop taking Jakafi and call your healthcare provider. Your healthcare provider will do a blood test to check your blood counts before you start Jakafi and regularly during your treatment. Your healthcare provider may change your dose of Jakafi or stop your treatment based on the results of your blood tests. Tell your healthcare provider right away if you develop or have worsening symptoms such as unusual bleeding, bruising, tiredness, shortness of breath, or a fever.
Infection: You may be at risk for developing a serious infection during treatment with Jakafi. Tell your healthcare provider if you develop any of the following symptoms of infection: chills, nausea, vomiting, aches, weakness, fever, painful skin rash or blisters.
Cancer: Some people have had certain types of non-melanoma skin cancers during treatment with Jakafi. Your healthcare provider will regularly check your skin during your treatment with Jakafi. Tell your healthcare provider if you develop any new or changing skin lesions during treatment with Jakafi.
Increases in cholesterol: You may have changes in your blood cholesterol levels during treatment with Jakafi. Your healthcare provider will do blood tests to check your cholesterol levels about every 8 to 12 weeks after you start taking Jakafi, and as needed.
Increased risk of major cardiovascular events such as heart attack, stroke or death in people who have cardiovascular risk factors and who are current or past smokers while using another JAK inhibitor to treat rheumatoid arthritis: Get emergency help right away if you have any symptoms of a heart attack or stroke while taking Jakafi, including: discomfort in the center of your chest that lasts for more than a few minutes, or that goes away and comes back, severe tightness, pain, pressure, or heaviness in your chest, throat, neck, or jaw, pain or discomfort in your arms, back, neck, jaw, or stomach, shortness of breath with or without chest discomfort, breaking out in a cold sweat, nausea or vomiting, feeling lightheaded, weakness in one part or on one side of your body, slurred speech.
Increased risk of blood clots: Blood clots in the veins of your legs (deep vein thrombosis, DVT) or lungs (pulmonary embolism, PE) have happened in people taking another JAK inhibitor for rheumatoid arthritis and may be life-threatening. Tell your healthcare provider right away if you have any signs and symptoms of blood clots during treatment with Jakafi, including: swelling, pain, or tenderness in one or both legs, sudden, unexplained chest or upper back pain, shortness of breath or difficulty breathing.
Possible increased risk of new (secondary) cancers: People who take another JAK inhibitor for rheumatoid arthritis have an increased risk of new (secondary) cancers, including lymphoma and other cancers. People who smoke or who smoked in the past have an added risk of new cancers.
The most common side effects of Jakafi include: for certain types of myelofibrosis (MF) and polycythemia vera (PV) – low platelet or red blood cell counts, bruising, dizziness, headache, and diarrhea; for acute GVHD – low platelet counts, low red or white blood cell counts, infections, and swelling; and for chronic GVHD – low red blood cell or platelet counts and infections including viral infections.
These are not all the possible side effects of Jakafi. Ask your pharmacist or healthcare provider for more information. Call your doctor for medical advice about side effects.
Before taking Jakafi, tell your healthcare provider about: all the medications, vitamins, and herbal supplements you are taking and all your medical conditions, including if you have an infection, have or had low white or red blood cell counts, have or had tuberculosis (TB) or have been in close contact with someone who has TB, had shingles (herpes zoster), have or had hepatitis B, have or had liver or kidney problems, are on dialysis, have high cholesterol or triglycerides, had cancer, are a current or past smoker, had a blood clot, heart attack, other heart problems or stroke, or have any other medical condition. Take Jakafi exactly as your healthcare provider tells you. Do not change your dose or stop taking Jakafi without first talking to your healthcare provider.
Women should not take Jakafi while pregnant or planning to become pregnant. Do not breastfeed during treatment with Jakafi and for 2 weeks after the final dose.
Please see the Full Prescribing Information at Jakafi.com, which includes a more complete discussion of the risks associated with Jakafi.
You are encouraged to report negative side effects of prescription drugs to the FDA.
Visit www.fda.gov/medwatch or call 1-800-FDA-1088.
You may also report side effects to Incyte Medical Information at 1-855-463-3463.
This is Donna’s experience with Jakafi. Individual results may vary. Only your health care professional can decide if Jakafi is right for you.
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Jakafi and the Jakafi logo are registered trademarks of Incyte.
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