Progression in MPNs: Allaying Fears and Taking Action

Published on

Topics include: Treatments , Understanding and Patient Stories

Many myeloproliferative neoplasm (MPN) patients worry about progression, life expectancy and the uncertainties that come with a rare cancer. In part two of our Partners Series, Patient Power founder and myelofibrosis (MF) patient Andrew Schorr, is joined by leading MPN expert Dr. Mark Heaney and two people living with polycythemia vera (PV), Ann and Jonna, to discuss diagnosis, risk stratification, progression, and actionable steps to take to access the best care available. Ann and Jonna walk through their treatment journey, obstacles, fears and drive to continue the life they enjoy while Dr. Heaney provides research-based insight on progression rate, potential complications, disease management and risk-reduction techniques for MPNs. Watch now to learn more.

Sponsored by Incyte Corporation.

View more programs featuring , , and

Transcript

Please remember the opinions expressed on Patient Power are not necessarily the views of our sponsors, contributors, partners or Patient Power. Our discussions are not a substitute for seeking medical advice or care from your own doctor. That’s how you’ll get care that’s most appropriate for you.

Andrew Schorr:

Hello, and welcome to Patient Power. I’m Andrew Schorr in San Diego, living with myelofibrosis now, about six years. Welcome to this Partners—program. We have some wonderful guests. These Partners Programs are with patients discussing with a leading expert and we’re delighted that we have two PV patients—polycythemia vera—joining us today, along with a leading MPN expert and researcher from New York City. So let’s meet our guests. First of all, let’s go to Atlanta. Jonna Hartman is with us and she’s been living with PV from—since 2005. Jonna, thanks so much for being with us.

Jonna Hartman:  

Thank you.

Andrew Schorr:  

Okay. And then…

Jonna Hartman:  

…glad to be here.

Andrew Schorr:  

Yeah. Thank you so much. And now, let’s go to Champaign, Illinois. Ann Zelinski joins us and you’ve been living with PV since 2001, right?

Ann Zielinski:       

That’s correct. Thank you for having me.

Andrew Schorr:  

No, thank you. And then, what about our expert? Back on Patient Power is Dr. Mark Heaney from Columbia University, an MPN specialist there. Dr. Heaney, welcome back to Patient Power.

Dr. Heaney:         

Thank you so much, Andrew, for—for having me back.

Andrew Schorr:  

Okay. And I want to thank the Insight Corporation through sponsoring this program. They have no editorial control, but we appreciate their support as we produce these progress programs. Okay, so let’s get started. We worry about progression. I’m at one end of the scale: myelofibrosis. Some people are at another end of the scale: essential thrombocythemia, and we have two ladies with us with polycythemia Vera. So, I know that, Ann, when you were diagnosed, you were pretty despondent, worrying what the future was. Maybe you could just describe that for a second.

Ann Zielinski:       

Yes, I went in, like Jonna, on a normal yearly check-up and the doctor called me at work and said “Take this down. You have polycythemia vera.” And he scheduled me with a hematologist, and that hematologist told me “You have five to 10 years to live. Get over it. There are people worse off than you are.” I—because I’m connected with the University of Illinois and part of my faculty were in the College of Medicine, I found people to get a new hematologist at the same clinic who directed me for the next 12 years and the first thing he said was “You and I are going to deal with this for decades to come.” And I think that’s the first time I took a breath.

Andrew Schorr:  

Okay. And the, eventually, you consulted out-of-state with a—one of our leading MPN specialists, Dr. Moliterno. And then, closer to home now, Dr. Brady Stein, up the road in Chicago. And—and then you’ve had your journey along the way. How’s your mood, now, about progression?

Ann Zielinski:       

It’s still on my mind. I don’t think anyone can dismiss it. But now that I see—every time I get a CBC, I get a happy surprise. I don’t have any more immature cells of either line. And my spleen is down significantly. So I’m feeling much, much better.

Andrew Schorr:  

Okay. Good for you. Now, Jonna, you’re more recently diagnosed and you had sort of an adventure to get to the right doctor and now you go to the Winship Cancer at Emory and, like Ann, you’re now on Pegasys, but along the way, you’ve had some real fears about this, haven’t you?

Jonna Hartman:  

Yes, I have. You see things on Facebook support groups where somebody has progressed after 12 years or 17 years or 10 years and it just gives you some concern, so I think about it some. But then I also try to just live my life every day and enjoy every day and not really stress all the time about the conversion.

I’m now on peinteferon alfa-2a (Pegasys), and I just went for blood work after four-and-a-half weeks, and I had really good results. It took the higher dose, but I was so happy that my counts are starting to go down, so I kind of feel like I’m doing something about it, now. I’m on a drug that could possibly prevent me from progressing—at least, that’s what I think. So I’m excited about my results yesterday and—and hopefully what Pegasys is going to do for me.

Andrew Schorr:  

Well, good for you. One—one thing I wanted to just recall for you. You mentioned that you heard a presentation at one point and saw a slide that kind of scared you. What was that?

Jonna Hartman:  

I was in an MPN event in my community and my doctor, actually—Dr. Elliott Winton—was speaking at this little, informal event and he had slide and the slide was showing medium life expectancies and PV was 14 years, so—you had seen numbers like that before in your research and so, when I went in for a doctor’s appointment, I said “What about the slide you used? It says 14 years.” And he was like “I probably shouldn’t have used that slide.” And I’m like “No, you shouldn’t use that slide because it’s very scary for us that have the disease when we see things that say 15 years, 20 years—I’m young. I want to see my daughter get married and have grandkids.”

Andrew Schorr:  

Okay, well, the title of this program is “Allaying Fears.” Each of you have had it. I’ve had it, to, as a myelofibrosis patient. And taking action. So, both of you have taken action by connecting with specialists. So we have one with us, Dr. Heaney. Dr. Heaney, tell us, what do we know about progression, because people know, if it goes—blows through myelofibrosis, you could even have acute leukemia, and maybe there are some medicines changing for that now, but it’s very scary. So what do you tell patients today on what we know about progression and how variable it is?

Dr. Heaney:         

So, I think the truth is that, most patients with polycythemia vera and essential thrombocythemia don’t progress. In fact, the progression rate to myelofibrosis is less than ten percent. So it’s—it’s certainly a concern and, as a physician, that’s my job to watch for that, but most patients don’t go on to progress. And so, there are other complications that can come with the disease and having a blood clot is probably the most common.

So that’s—that’s something that we can sometimes try to reduce the risk of and all those things are part of managing the disease in total. It’s—progression is scary and, you’re right, if you go to myelofibrosis, then there is that fear that there’s another layer of progression beyond that, but—but fortunately that’s not the case for most people.

Andrew Schorr:  

Okay. So, we’re not necessarily on a freight train from ET through PV to MF to acute leukemia, so that’s good to know. So then it sounds like monitoring and the proper treatment for the condition you have. So with these ladies with PV then, what would be the monitoring you would be doing to lower their risk of complications from PV, since we have two ladies with it.

Dr. Heaney:         

So the—the most common risk of PV is thrombosis or blood clots and we think that, over a patient’s lifetime, somewhere between a quarter and a third of patients with diseases like polycythemia Vera and essential thrombocythemia may have blood clots. I—I think one of the things that’s really critical to remember and—and I know that you mentioned the—the life expectancy slide that was scary, but all of the data that we have right now is already 15 to 20 years old, and this is a disease that extends over such a long period of time that the really knowing what’s modern and now is extremely challenging for—for us as physicians.

And so we do know that there is an increased risk of thrombosis and we think that we can reduce that risk by giving medicines like aspirin in low dose, by controlling the hematocrit to keep it 45 and less, and in some cases, using what we call cytoreductive medicines, like hydroxyurea and pegylated interferon and possibly other medicines like ruxolitinib or Jakafi.

Andrew Schorr:  

So one of the things we have talked about before, Dr. Heaney, particularly related to myelofibrosis and some polycythemia vera, is genetic mutations that may be fueling your condition. Does that apply that in PV where you would be treating it differently based on that?

Related Programs

Preventing Progression in MPNs: Researcher Perspectives From ASH 2017

MPN experts Dr. Angela Fleischman and Dr. John Crispino share their insights on clinical data released at ASH 2017 related to disease-modifying agents that may impact progression. Watch now to find out the latest.

Published:

Tuning In to Your MPN Symptoms: Monitoring Disease Progression

How do you know if your MPN is progressing? Watch as Physician Assistant Lindsey Kalhagen discusses progression and what might indicate a need for an adjustment in treatment.

Published:

Patient-Doctor Perspective: Regaining Control of Health and Happiness After an MPN Diagnosis

An MPN takes a toll on a person’s body, mind and spirit. How can patients reclaim and enjoy their lives? Watch now for tips on regaining the control many patients feel they’ve lost during or after cancer treatment.

Published:

Advertisement
Join Our Community Register for Events Read Our Latest Blog
Advertisement

Page last updated on May 20, 2019