Patients with polycythemia vera are at increased risk of thrombotic events. Those can include stroke, heart attack, DVTs, and PEs. It's important that APPS are managing their patients closely to reduce their patient's risk of thrombotic events.

The way I educate my patients about the importance of managing hematocrit under 45% is helping them understand that by keeping their levels under 45%, we're reducing their risk of thrombotic events.

In addition, we're making sure that we're monitoring our patients closely for signs and symptoms of advancing PV. Advancing PV can include elevated white blood cell count in addition to elevated hematocrit of at, or above 45%. It also can include disease-related symptom burden. So, it's important as a clinical provider to make sure that I am aware of all the factors that can increase their risk of clots. And not only be aware of it, but also what can I do as a medical provider to reduce their risk of clotting events.

So, the APP survey was really important because it identified any kind of educational gaps that we have amongst our APPs in the community settings. The objective of the APP survey was to gain insight into the management of PV by APPs in community oncology settings. Responses were gathered from 114 APPs, each of whom managed at least three patients with PV over the past 12 months. It asked questions that were important about understanding what are the patterns, and traditions, and how APPs manage patients.

So, when we saw from the APP survey that 80% of community practice APPs are comfortable with maintaining their patient's hematocrit of 45% to 50%, that was a concerning finding to me. I think it's an important educational opportunity to provide our community APPs with additional education that shows that it's in their patient's best interest to maintain hematocrit under 45%.

Respondents were asked about their use of cytoreductive therapy and phlebotomy to control hematocrit. 58% believe hydroxyurea can control hematocrit moderately well. About a third of APP respondents strongly agreed that frequent phlebotomies are appropriate for persistently elevated hematocrit of more than or equal to 45%. When we see that a patient's need for phlebotomy is increasing and they're having poor tolerance to phlebotomy, we need to look at alternative options beyond just phlebotomy.

In some patients with PV, hydroxyurea can control hematocrit and signs and symptoms of disease quite well. However, there are other patients who have signs and symptoms of intolerance of hydroxyurea. Those can include symptoms that are worsening on hydroxyurea, they can also be signs of intolerance to the medicine itself. There could also be signs on the complete blood counts that the patient is not being managed appropriately with hydroxyurea. Those signs could include neutropenia or thrombocytopenia, even if the hematocrit is well managed.

Other signs and symptoms of resistance to hydroxyurea could be patients are at maximum tolerated dose of hydroxyurea, but hematocrit is still not well-maintained. So, when I talk to patients with polycythemia vera, we're talking to them about reducing their risk of thrombotic events. And the mainstays of that is that their hematocrits are controlled under 45%. And also, that we know the characteristics of their life that PV may impact.

So, what can APPs do in our clinical practice? So we can provide education and awareness among our physicians, APPs, and other colleagues to make sure that we are aware of the signs of loss of response to hydroxyurea, as well as signs of intolerance to it. We can also provide that same education and awareness among our patients and caregivers.

White blood cells are important to monitor because leukocytosis, especially progressive leukocytosis, is a risk factor for increased thrombotic events in PV patients.

In our clinical practice, it's important that I monitor patients with PV to look for signs of advancing white blood cell count. It's especially concerning to me if a patient's white blood cell count is over 11, as that could potentially indicate that my patient is at increased risk of thrombotic event.

So, it's pretty common for patients to come to our clinical practice for a second opinion because they know something isn't right. Typically, the patients that are coming to us for second consult are having worsening symptoms of fatigue. They may be having headaches. They're also showing signs on their CBC of loss of control from their hydroxyurea and phlebotomy treatments alone.

And so, we're talking to them about other treatment options that may be appropriate for them. In addition, we're talking to them about the underlying pathophysiology of their disease and why we think that their disease isn't well controlled anymore.

So, we know from the literature that patients with leukocytosis, especially those patients with white blood cell counts that are over 11 or patients with white blood cell counts that are getting progressively higher, are showing signs that they may be at risk for a thrombotic event.

In the APP survey, only 15% of APPs noted that a white blood cell count of over 11 was extremely concerning. So the APP survey was done by Incyte to gain perspective from community oncology APPs, and 114 PAs and nurse practitioners responded. Those APPs needed to have seen at least 3 patients with PV over the previous 12 months. So it was interesting to me, and to be honest, concerning that only 15% of my peers who are APPs found that leukocytosis is an increased risk factor for thrombotic events.

So that provides us an opportunity to help APPs better understand how they can reduce their patient's risk of thrombotic events by more closely monitoring and managing their patient's white blood cell counts to under 11. It's not just important to manage the white blood cell counts. It's important to manage the trends in the CBC.

So, for my patients, I'm making sure that I'm monitoring their CBCs closely. So, when a patient with polycythemia vera comes into our office, we're not just discussing what is their hematocrit and how that ties into their thrombotic risk. We're also talking to them about what are the other components of their CBC that we're monitoring and making sure that we're controlling their risk of clotting events.

It's not just looking at what is their white blood cell count at this point in time, but what is their overall trend over a period of time? Do we see progressive leukocytosis? Are we monitoring their white blood cell count and managing it to keep it under 11? Does the patient know that elevated white blood cell count is tied to their risk of thrombotic event? All those are important points in making sure that we're communicating properly with the patient and helping them understand where they're at in their disease status.

So, I've observed in some other practices that some PV patients are kind of put on autopilot in regards to their CBC and phlebotomy maintenance. And it's important that APPs be aware of what the trends are in the patient’s CBCs to monitor for signs and symptoms of advanced PV.

And so, I think that APPs have opportunities to better connect and communicate with our nursing colleagues, as well as let their nursing colleagues know when patients should be brought in for additional appointments if they're observing additional signs and symptoms of disease progression.

It's important to understand that symptom burden is a real thing for our PV patients. In fact, most patients with PV are symptomatic. Furthermore, those symptoms can impact the patient's quality of life.

So, in my experience, almost all of my patients with PV are symptomatic. Most of my patients suffer from fatigue, but they can also suffer from other symptoms like night sweats, itching, spleen pain, early satiety, and other symptoms that constitute flu-like symptoms.

So one of the reasons that I believe that PV patients symptoms aren't necessarily acknowledged by their medical providers is because they under report their symptoms. Some of the PV symptoms can be vague. For instance, fatigue for some patients may not be every single day. So they may not necessarily report fatigue unless they're having a day during a clinical visit where they're experiencing fatigue. Disease-related symptoms are a clinical characteristic of advanced PV, along with elevated hematocrit of more than or equal to 45%.

In the APP survey, the objective was to gain insight into the management of PV by APPs in community oncology settings. It was sponsored by Incyte. Responses were gathered from 114 APPs, each of whom managed at least 3 patients with PV over the past 12 months.

One of the key findings from the APP survey was that 44% of respondents reported that 25% or less of their PV patients were symptomatic. I'm concerned that the perception that patients with PV do not have a significant symptom burden is because those patients aren't being regularly assessed for their symptoms. This provides us an educational opportunity to let APPs know in the community that their patients are likely symptomatic, and they can better monitor and assess their patient's symptoms. So we know that patients receiving hydroxyurea can have good control of their blood counts and still be symptomatic.

So not too long ago we had a patient that came to us for a second opinion of her PV. Her blood counts were actually quite well-managed, but she was noticing that she had a significant decline in her energy level. Her ability to do her activities of daily living as a retired person was severely limited. When we looked at her bone marrow biopsy, it also showed that she had not transformed to a more aggressive form of PV.

Upon further assessment of her symptoms, we found that her symptoms were severely impacting her activities of daily living and her quality of life. That led us to re-investigate what kind of treatment options were available to this patient.

So one of the ways that I am investigating how my patients are doing is by not just monitoring their CBCs, but also on an ongoing regular basis monitoring their symptom burden.

It's helpful to not ask vague questions such as how are you doing? It's more important to ask specifically how many hours of sleep are you getting a night? Are you able to get through a whole day of work without feeling like you need to take a nap?

Other ways that I monitor my patients’ symptoms are to really get to know my patients. I ask them, who are you? What are the activities that you do for fun? Are you working? What kind of work do you do? All of those kinds of activities of daily living help me understand who the patient is and how their symptoms may be impacting their quality of life.

Some of my patients who are working say that they have substantial concentration symptoms because they have sleep disturbances or headaches or fatigue that impacts their work. And that helps me to understand, am I managing this patient well despite their hematocrit being managed well, am I taking into account that their PV symptoms may be worsening?

I also get additional insight from family members and friends that come with the patient, because oftentimes they're reporting symptoms that the PV patient may not recognize themselves.

So one of the most important things that APPs can do is make sure that they're having regular interaction with their patients to be assessing for PV-related symptoms. Additionally with those opportunities, you can educate your patients so that they're aware of their PV-related symptoms as well.

I love being a PA and I love working with patients with PV. One of my favorite things about being a PA is educating my PV patients about their condition. Oftentimes when they come to our clinic, they may not know a lot about their underlying disease state. And it's important that we use any educational tools that we have to provide PV patients with a better understanding of their diagnosis.

My patients mean a lot to me, and polycythemia vera is an important diagnosis to understand and to manage appropriately. I want to make sure that they're living their life to the fullest and that they can convey to me that they have a good quality of life. If any of those parameters are not being met, I have the opportunity to be able to impact my patients by not just acknowledging their symptoms or their disease state, but also impacting their treatments.

So, one of my patients that I had came in, and she was really struggling with fatigue. And we were able to assess her symptom burden. We were able to assess her CBC and acknowledged that she had the signs and symptoms of advanced PV.

The biggest key management goals that APPs should be implementing for their patients is making sure that we're reducing our patient's risk for thrombotic events and also managing their symptom burden.

So APPs have several key roles in implementing care plans for PV patients. The first is that we're able to identify signs of advanced PV. The second is we're able to educate patients about their symptom burden. And the third is that we are able to prescribe therapeutic agents that can help in the management of the PV patient.

So APPs have several opportunities to contribute to improved patient care in PV. The first is that we have opportunities to regularly assess our PV patients, especially looking for signs or symptoms of advanced PV. Additionally, we can evaluate patients for alternative management strategies. And last, it's important that we educate staff and patients about PV.

It's important to help patients understand what are the ways that they can reduce their thrombotic risks, as well as the ways that they can manage their disease-related symptoms.

When I'm reviewing a CBC, first of all, I am looking at the hematocrit because I do know that strict control of hematocrit less than 45% is key for reducing thromboembolic events and cardiovascular events. This is based on the CYTO-PV study.

The next thing I look at is white blood cell count. So there was a multivariate analysis within the same study that showed that white blood cells greater than 11,000 was also associated with increased risk of thrombotic events.

My goals for patients with advanced polycythemia vera are to manage their counts, to reduce risk of thromboembolic events, ensuring that their hematocrit stays less than 45% and their white blood cells stay less than 11,000.

So I have to look at what treatment strategy we're using and then, monitoring the trends is really the most important thing. And so, if I have a patient that I'm seeing weekly, for example, and their hematocrit is going up and up and up, then maybe in a month I'm saying, "Okay, we need to maybe implement some new strategies here." Also, in patients perhaps who have been very stable for a while, you know, maybe 3 months of increasing hematocrit levels to the point of needing phlebotomy or perhaps increasing white blood cells, that would really indicate to me that there needed to be perhaps a change.

When I am looking at a patient over time, it actually varies per patient. So I have to look at what treatment strategy we're using and then, monitoring the trends is really the most important thing.

When a patient walks through my door with a new, worsening symptom, my primary thing is to think of what could be the contributing etiologies to this change in symptom burden. It’s one thing to have fatigue that maybe impacts how you feel in the afternoon. It’s another thing to have fatigue so severe that you can’t get out of bed. So when there is a change in symptom, I need to know about it.

One of the things I think about is whether or not in polycythemia vera: is there a change of blood counts that could be related to this symptom change? Another thing is whether or not this could be related to a change in therapy. Specifically, phlebotomy over time can lead to iron deficiency and patients can actually be symptomatic of that iron deficiency, can manifest with fatigue, restless leg syndrome, problems with concentration, or cognitive issues.

Hydroxyurea is another drug that can lead to severe symptoms or problems in symptoms. So gastrointestinal upset, nausea, diarrhea, can have cutaneous symptoms that are worrisome. It’s very important, too, to monitor for disease progression, and the symptoms can help clue us in to that.

In general, it’s important to know the resistance and intolerance guidelines for polycythemia vera as these changing symptoms might actually lead to a change in therapy or a change in management.

Patients with polycythemia vera can experience a continuum of symptoms that impact how they live with their disease. There can be constitutional symptoms like fevers, chills, night sweats, weight loss. There can be symptoms related to blood clots, things like shortness of breath, chest pain. There can be symptoms related to the abdomen, especially related to enlarged spleen like abdominal pain or discomfort, or feeling full easily.

In general, when we look at symptoms it’s important to see that some symptoms will give us a whole picture of the patient, like fatigue, whereas other symptoms might be more specific, such as night sweats. It’s important to note, too, that although one or two symptoms may not seem bothersome, when taken in aggregate, these can severely compromise patient’s quality of life and ability to function.

Polycythemia vera is a chronic myeloid stem cell disorder characterized by excessive red blood cell production. In these patients, they can live a long time, so our primary aim is to try to mitigate the major causes of mortality. The prevalence and severity of symptoms in polycythemia vera makes managing symptoms a primary treatment priority.

In polycythemia vera, blood counts and symptoms can be independent factors. We know that regardless of what a patient’s blood counts are or whether or not their disease is well controlled, they can still have severe symptom burden. So this means that those patients that we otherwise might think look good on paper might have problems going home and seeing their kids, going to work, or even just doing activities that they enjoy.

Symptoms should be monitored early in the disease course as well as over time, as symptoms can plateau, increase, decrease, or just change with therapies.

For healthcare providers, symptoms should be of paramount concern. This is because symptoms are the clues that will help to guide our management decisions in polycythemia vera.

The important thing about symptoms is that they can clue us in to the overall picture of the patient. We know that symptoms can be related to specific features of the disease.

We also look at organ dysfunction. A large spleen is something that a lot of patients with polycythemia vera deal with. We also know that treatments can be related to symptoms, so potentially hydroxyurea and phlebotomy have been shown to have higher symptom burden than patients who are not undergoing those treatments for polycythemia vera.

And you have to take in the whole picture. We have patients who are of different comorbidities, baseline health status, nutrition.

So in general, symptoms can help clue us in to the larger picture of the patient, but also to disease-specific features that we should be paying attention to.

Current guidelines for therapy of polycythemia vera aim to normalize the red blood cell count as well as normalize the white blood cell count and platelets along the way. This is important because even on the cytoreductive therapy, we are decreasing the risk of thrombosis, but we are not eliminating it. That may be in part explained by not looking or paying attention to a white blood cell count. We know that the white blood cell count or leukocytosis, particularly progressive leukocytosis, has implications on the thrombotic risk of the patients.

Traditionally, we have been using two factors that would assess the thrombotic risk. That would be age 60 or over or a history of blood clotting. If one or the other is present, then that particular PV patient has a risk of thrombosis and requires introductional cytoreductive therapy – not only a baby aspirin and phlebotomy. The goals of that effort is to normalize the hematocrit below 45% and maintain it below 45% hopefully without any need for phlebotomy. The other factor will be to control progressive leukocytosis. White cell count increase over three or four measurements will certainly identify patients at a high risk of thrombosis. And the final factor would be increased thrombocytosis, increase in platelets where you would also like that to normalize.

A thrombotic episode in PV patient is of a major concern because that leads to increased morbidity and mortality. In other words, complications from having impaired circulation in a body leads to impairment of the organ, which can be a brain or a heart, and also leads to untimely death. Even in the cases of a cytoreductive therapy, although we are reducing the risk of thrombosis, there is still underlying risk of thrombosis.

So when you look at the symptoms that I deal with, with PV, if you take one in and of itself it's something you can deal with. But when you start adding them all together and they're on a daily occurrence, it becomes harder to deal with as time goes on.

I’m Dave, I live in South Carolina with my wife Betsy. 10 years ago, I was diagnosed with PV. I don’t believe I was having night sweats pre-diagnosis, I think that came on after I was diagnosed and probably within the last few years that I am really noticing it.

Imagine sleeping in something damp. If you have fans on that even exaggerates the coldness that you feel. It’s a pain. You get up to towel off, or you get up to cool down and walk around, and I'm trying to do all of this without waking my wife up.

I would say I have experienced about 30 night sweats since my last doctors appointment. I hope doctors can look at this and say I really need to inquire more with my patients about what the symptoms are that they are dealing with on a daily basis, instead of just looking at the numbers. If my doctor came in and asked me about my symptoms, I’d be very open to sharing it with them.

The greatest thing that you can give your family and your kids is your time. And if I’m not able to give them my time in any way, because I have PV and I’m experiencing fatigue, that to me is the most frustrating.

My name is Nick Napalitano. I am a PV patient. I was diagnosed about 4 years ago. Before I was diagnosed, I was experiencing fatigue, but you know, being a young father you sort of dismiss it as just normal fatigue.

Experiencing fatigue means that I can't be with friends, that I can't go on vacation, that I can't help my wife with the kids and contribute around the house, and it makes me very angry and very disappointed and very deflated. It makes me feel like I'm a burden.

So I recently experienced extreme fatigue when my son had a baseball game, and I coach his baseball team, that I was unable to make the game, which was completely devastating to me. I would say, on any given week, there’s about 4 activities that are impacted by my fatigue.

I have experienced fatigue about 40 times since my last PV appointment.

I would hope the doctors would understand what the patient is going through even if that patient doesn’t offer it. It should be part of the process where that is where we start. We start with, how are you doing from a mental health standpoint? How are you doing from a symptom standpoint? And if that patient doesn’t offer that, pull it out of them, because it’s in there.

There are a lot of activities that I used to do that I just don't do anymore because of the itching effects that I have with PV.

My name is Julie and I was diagnosed with PV 13 years ago. The list of things that I got in my life that had to change was overwhelming because it was such a 180 from what my life was at the time. One of the symptoms that I deal with on a regular basis is itching. It's all the time, basically.

When I get in the shower and it's a hot shower, it's instantly itching. If I jump in the tub. Love it. Get out of the tub. I start itching. It happens at the end of the day before I go to bed.

I have missed 36 activities due to itching since my last PV appointment.

I’ve seen a lot of doctors over the years and only one has ever talked about itching with me. It makes me feel disappointed. It makes me feel personally that I’m not getting as much as detailed care as I should be. I definitely feel that my doctor should be asking me about itching. The fact that it’s not happening is really sad. As a patient it makes you feel really alone.