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Patients with myeloproliferative neoplasms (MPNs) may experience a number of symptoms, with fatigue being one of them.

MPNs are types of blood cancer and occur when there is an abnormal change in stem cells within the bone marrow, according to Leukemia and Lymphoma Society. This abnormal change can lead to increased amounts of white blood cells, red blood cells and platelets. The main three types of MPNs are essential thrombocythemia, myelofibrosis and polycythemia vera.

MPN-related fatigue can take a toll on a patient’s quality of life, notably because of the change in the blood’s consistency.

“Fatigue has a profound effect on patients’ quality of life. A lot of people don’t realize that if you have chronic fatigue, especially from MPNs, because your condition is unchecked, then a lot of times it can actually be debilitating,” Patrick Buxton, a clinical nurse coordinator in the hematology department at Fred Hutchinson Cancer Center in Seattle, Washington, said during an interview with CURE®.

“(Patients are) not having the energy to go out and work, participate in family events and just (engage with) life in general. And they just don't really feel well overall,” Buxton said. “And so, it’s important to monitor these patients’ labs to make sure that they stay within the range for their condition to ensure that they don’t end up developing those symptoms again, because when (a patient’s) blood counts become so high that (their) blood basically becomes a sludge, (they) definitely feel it. And it can cause a whole lot of problems centered around fatigue, but not just fatigue. It can cause a lot of inflammation issues.”

To work through the weight of fatigue, Buxton urged patients to hydrate, eat nutritious food and exercise.

“Especially with MPN, patients maintaining good hydration and adequate nutrition is very important. Because once (patients) kind of get stuck in a downward spiral, it can be very hard to get out,” he noted. “So, making sure that … patients are trying to get a good amount of exercise daily, even if it’s just getting out of bed, pushing (themselves) a little bit each day to try to get that energy going again and making sure that (they) eat and drink enough to keep going.”

Staying hydrated is essential for patients with MPNs, Buxton emphasized.

“Hydration cannot be stressed enough. A lot of people don’t hydrate with water, as I have come to realize, and so there’s been a lot of education patients have like, ‘Oh, I drink all the soda.’

And I'm like, ‘That’s not really helping. You need to drink water, especially when you have this condition, you need to keep on top of things.’”

For patients with MPNs who experience fatigue, Buxton advised them to have open communication with their care teams.

“That line of communication is very important. And even if the patients don’t think it’s important, they know their bodies very well. So, if something is not working right, communicate that to the team,” he said. “And know that it really is a lot of teamwork with the nurses, the patients, the doctors, that the patient is an integral part of the team; without the patient, we wouldn’t be here.

“So, know that (patients are) the center of all of this, and we are here to help, but if (they) don’t report these issues (to us) and just let them be then we won’t know. So, with that information, be a good self-advocate, because that’s how (they’re) going to get better care is being an active participant. Don’t let small things go because sometimes small stuff adds up to something big. And especially with fatigue — patients know their bodies well. So, follow up. Make sure that you are getting the care that you need.”

For more news on cancer updates, research and education, don’t forget to 

It’s key for patients with MPN-related fatigue to communicate changes they feel, especially as they “know their bodies very well,” a nurse told CURE®.

Patients With MPN-Related Fatigue ‘Know Their Bodies Very Well’

Understanding which of the three types of myeloproliferative neoplasms (MPNs) a patient has can help guide them to receive the appropriate treatment and management of the disease.

Of note, MPNs are a group of rare blood disorders where the bone marrow creates too many blood cells. This can lead a patient to have an imbalance of red blood cells, white blood cells and platelets, all of which can lead to several symptoms. These can include feeling tired, issues with clotting or bleeding and potentially an enlarged spleen.

Unfortunately, the number of patients diagnosed with MDS in the United State each year is not exactly known, according to the American Cancer Society. The organization notes that estimates start at 10,000 and can increase from there. The risk for MDS increases as a patient ages and diagnosis of the disease is uncommon before age 50, according to the American Cancer Society, with most patients diagnosed in their 70s.

Each of the three types of MPNs have their own set of symptoms and management strategies, highlighting the importance of knowing which disease a patient has.

In patients with polycythemia vera, the bone marrow produces too many red blood cells, which typically carry oxygen throughout the body. This overproduction of red blood cells can thicken a patient’s blood, leading to issues like blood clots and an increased risk for stroke.

The exact cause of polycythemia vera is often not clear, but it is typically related to genetic mutations in the bone marrow cells, particularly in a gene called JAK2. Diagnosis is typically made through blood tests that reveal elevated red blood cell counts, hematocrit levels and the presence of the JAK2 mutation.

Patients with polycythemia vera may experience symptoms like fatigue, headaches and redness or itching of the skin. Treatment for this type of MPN often involves medications and occasionally procedures to reduce the number of red blood cells a patient’s body makes. In particular, treatment for polycythemia vera may include phlebotomy (removing excess blood), medications to reduce blood cell production and management of symptoms and complications.

With essential thrombocythemia, the bone marrow creates too many platelets, which are the cells that help blood clot. Too many platelets can lead patients to experience clotting and bleeding issues. In particular, blood may clot too easily, which can block blood vessels and cause issues like strokes or heart attacks. Paradoxically, patients may also experience bleeding problems because the blood doesn’t clot properly in some cases.

The exact cause of essential thrombocythemia is not well understood, but it is often associated with genetic mutations, particularly in genes like CALR, JAK2 or MPL.

Some patients with essential thrombocythemia may not have any symptoms or they may experience things like headache, dizziness or red spots on their skin.

Patients are recommended to work with their care team to manage the disease and potentially reduce the risk for complications, both of which may be achieved with medications or procedures like plateletpheresis (when platelets are filtered from a patient’s blood through a machine) to control the number of platelets in the blood.

In patients with myelofibrosis, the bone marrow becomes scarred and experiences difficulty in creating enough healthy blood cells, which may lead to fatigue, anemia, an enlarged spleen and an increased risk for infections. Symptoms may also include pain and early satiety, or feeling full even when the patient has not eaten much.

Similar to essential thrombocythemia, myelofibrosis may also be linked to specific genetic mutations like CALR, JAK2 or MPL.

A patient’s care team may manage myelofibrosis with blood transfusions to manage anemia, supportive care to address symptoms, medications to reduce the size of the spleen or, if necessary, a bone marrow transplant.

There are three types of myeloproliferative neoplasms, all of which come with their own set of symptoms and management strategies. 

Understanding the Three Types of MPNs

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Patients with myeloproliferative neoplasms (MPNs) — a group of blood cancers that causes the bone marrow to overproducer red or white blood cells or platelets and which includes polycythemia vera, essential thrombocythemia and myelofibrosis) — are at particular risk for thrombotic and cardiovascular events.

“It's really a problem for all patients with cancer because it does increase your chances of developing a clot, for example,” said Kim Noonan, nursing and patient care services chief nurse practitioner at the Dana-Farber Cancer Institute in Boston, during a conversation about the risks faced by patients, and the ways in which nurses can help patients prevent cardiovascular and thrombotic events.

However, such risks are of particular concern for patients with MPNs, as literature has shown.

“Clinicians have long recognized an association between MPNs and increased risk for both thrombotic and hemorrhagic complications,” stated the authors of an article published in 

 “A recent meta-analysis of 13,436 patients with MPNs showed a pooled prevalence of arterial thrombosis of 16.2% and of hemorrhagic complications of 6.2%. Indeed, cardiovascular events often accompany this cluster of hematologic conditions and contribute significantly to morbidity and mortality.”

Symptoms of a clot or pulmonary embolus include shortness of breath and chest pain, especially following exertion, as well as an elevated or irregular heart rate and an unexplained fever noted Noonan, the co-author of a 

study on the assessment and prevention of venous thromboembolism and cardiovascular disease among patients with another form of blood cancer, multiple myeloma.

“I'm always thinking about thrombosis first, and then I can relax if I have maybe another explanation for their shortness of breath,” Noonan said. “But we're always working it up, we really do due diligence to not miss some kind of thrombotic event that's going on.”


I think when people see me, they just feel like they have to start jogging, because I'm always saying, “You (have) got to move, you have to move, you have to move.” And it's so, so important. That person that is sedentary is really at a huge risk to the point of where if they have a sedentary lifestyle, we're thinking maybe that person needs to be on needs to be on anticoagulant therapy right from the beginning, as opposed to just using an aspirin.

We really want people to stay hydrated. We want them to get up and walk around. We want them to be aware of what the symptoms are (and be aware that) they can be doing everything right and still develop a clot because of the medication that they're on. But I think also, education is essential, (letting a patient know) that you are on a medication that can increase your chances of developing a clot or thrombosis, and (you should) just be aware of what the symptoms are.

Transcript has been edited for length and clarity.

“When people see me, they just feel like they have to start jogging,” as one chief nurse practitioner tells CURE when discussing how patients with MPNs can reduce their risk of cardiovascular and thrombotic events.

Hydration and Movement Are Important for Patients With MPNs

Can you explain how MPNs have become more of a chronic disease in the cancer space?

So MPNs have always been a chronic disease, and sometimes distinguished as a more aggressive type of cancer. So often, patients with MPNs will have either a normal life expectancy with very little symptoms. And of course, there's people who will have a much more aggressive disease. (It’s important) to recognize that investment of time patients will have the disease for decades. It has an impact on how are we going to manage these diseases, we have to have a broader perspective, then just say, well, we're going to manage disease, we're going to cure the patient, because most of the patient we don't cure. But we certainly can prevent lots of the long-term complications. And we certainly can improve in a very significant and meaningful fashion quality of life. So I think that's where we have to have a broader and more very holistic approach to the management of a patient with MPNs.

Why is it key to ensure that patients are taking care of themselves outside of their treatment?

So in the case of MPNs, in particular, for the essential thrombocytopenia or polycythemia vera, or even with myofibrosis, one of the biggest complications we want to prevent, in the mid to long term, vascular complications or strokes, heart attack, or blood clots. So, if you have one of these complication, they really can affect not only your life, your quality of life, but potentially your life expectancy, but it can leave patients with some disability. So it is crucial to do what we can to minimize the risk. So of course, we have some treatments but there's lots of things other than that, that patients can do and physicians can advocate and help managing lifestyles, diet and managing the other comorbidities. So oftentimes liaising with the other specialists or family doctor to make sure that the cholesterol is well controlled, that diabetes is well managed is absolutely key. And it is important whenever the patients are being seen by their health care team at the MPN clinic that they these different topics are being addressed.

How can we implement integrative care into the treatment approach then with this?

I think it's at least my approach to have that as a checklist. So when we see people with MPNs, depending on the state of their disease, or the expected symptoms will be different. So, for instance, somebody who's got polycythemia vera will typically ask symptoms about the providers or the itchiness. Symptoms of erythromelalgia pain in their hands or fingers, but also ask about symptoms related to the screen size. So, I think to systematically ask about those is helpful. But also ask questions about and measure the blood pressure, but also ask questions about the management of the diabetes and blood pressure and make sure there's no other new medical concerns, again, because you've got a perspective of this patient being alive in five years, 10 years from now, so you want to prevent or in a way you don't play the role of the doctor, but you have to kind of ensure that all the preventable disease are in as much as possible taken care of.

How does diet and nutrition play a role to in kind of that long term care?

I'm glad you asked. Because, you know, one thing that I find is that patients, one of the first things they ask when they get a diagnosis, is why me and often the what of the answer is it's bad luck. But there are things patients can do, some cannot cure the disease, for the most part. But certainly, by modifying risk factors, and through diet and exercise, you actually go a long way and in multiple ways, not only if you have a healthy diet. So you know, if you eat some unprocessed food, on saturated fat, no trans fat, for instance, or a well-balanced diet that comprises fruits and vegetables, you're probably going to be healthier, feel better, you're less likely to be overweight, your energy is better. And that will basically give you a better quality of life.

The same way, exercises will go a long way in making you feel better, give you a better quality of life, reduce anxiety, help with your sleep. Other aspects that are important are to reduce alcohol consumption. You don't have to be abstinent. But certainly, to have a moderate alcohol consumption is probably better for everyone, including for patients with MPN. And reducing or stopping smoking is also better. All of these aspects will empower patients and will have an impact on their quality of life will have an impact on how they feel about themselves, and will decrease the chance of them having vascular complications, which is a big deal. It's also if ever they need more aggressive treatment, they will be stronger and more able to tolerate treatment, including a bone marrow transplantation, if they're a better fit.

What's your biggest piece of advice for patients to make sure that they are having good well-being throughout their cancer journey?

I think it's important that they are informed about that, right. So that's part of the role of the health care team to naturally open discussion and not just focus on the diagnosis and specific MPN symptoms. But really to ask questions, when patients see that the physician asking about, you know, the diet, then they have a sense, that's important. So if you have that discussion, about diet, exercise, alcohol smoking, then you're basically telling your patient this is important. And this is something you can do something about and it's up to the patient to some extent, to decide what to do about that.

I think it is important for patients to realize that it is important to ask questions, often people will ask, “Okay, what about this health supplement? What about this type of exercise?” And I think it's helpful for them to have the discussion with their physician and nurse so that they can have a sense of what's realistic, what's possible. So it's not the expectations, not that people will train for the Olympics, the expectation is that they can have a decent level of exercise that will help with their overall well-being and improve their health. Likewise, for the diet, you're not expecting people to become pure vegetarian and have a holy diet. We're talking about people who have a healthy diet, which is realistic, but sometimes people don't know what that means. And that's fair enough. Why does she have a health care provider? They can, they can be helpful. They'll be happy to do that.

On behalf of the Canadian MPN Research Foundation, CURE spoke with Dr. Pierre Villeneuve, from the University of Ottawa, about the long-term care of MPNs.

Expert Emphasizes Importance of Integrative Care, Well-Being in Long-Term Care of MPNs

Why is it important for patients and their loved ones to seek support after an MPN diagnosis?

Well, I think it is important to recognize for patients be told that an MPN diagnosis can take a significant toll on them both psychologically, it can sometimes be very overwhelming. And I think that not only psychologically, that's very stressful, but also so it can lead to emotional trauma, but also has depending on the stage of the disease, some people will have very mild symptoms, some people will be much more symptomatic, but it can affect other aspect of their life. So such as their work-life balance, it can affect their towns for future kids and work ambitions, for instance.

Can you give examples of the type of support that patients and their loved ones can seek?

I think, if you appreciate all the different aspects of their life, and then you can address the different needs of support. So we talk about the emotional challenge that goes along with that, I think it's true. And I think depending on the stage of the disease, from the time of diagnosis, people will evolve. But some people will benefit, sometimes not only from support from their peers, support from their family, but also sometimes professional support. And typically, lots of the cancer centers will have professionals that are really dedicated to that. And I think that's key to ask for that help when needed to. I've seen patients really benefiting, that reduces the burden on the shoulders of caregivers, and also patients, they really feel they've got somebody who can give them the tools to deal with all the financial stress and logistical issues.

Sometimes social workers can be very, very helpful. So to deal with disability, worker compensation issues, for instance, to help get additional financial help social workers are really trained to help and find the right resources. So I think that's important. The cancer care societies are helpful to figure out, making sure that you're managed by or identified the right place for you. So if you have an MPN issue, and that is more complex, maybe you want to be in a place that has expertise or dedicated expertise in MPN to ensure that you figure out where your treatment, where you are in the treatment, and what are the treatment options, and also what type of clinical trials, if at all, may be relevant to you. So these are all things that you can get through information through your cancer societies.

What's the best way for patients and their loved ones to start to seek that help?

Well, I think that's where having an open dialogue with the healthcare team, so the nurses and physicians is very, very important, I think one thing is important to appreciate. And that's something you learned in med school. And sometimes we don't do as well as we should, but it's really to think of the patient as not just a disease and not say, I'm just going to treat your myelofibrosis I'm actually going to manage your care. So that means dealing with the fact that you've got anxiety that you've got financial issues, that you need additional help at home. So, to have a more holistic approach. So when you meet, I think it is, in part, when you meet with the clinicians, that is part of the responsibility of the physician and the nurse to ask about other things not just purely the medical aspect of your care, but to talk about how are things at home, as work as a financial aspect, as your exercise and diet and so on.

So it is important for physicians to do that, the nurses, but also for patients to say, Well, you know, that's actually important for me, you know, I'm not eating well, or, I have lots of stress at home, and is there anything? And can you have any condition can make and, and I think, you know, if you don't talk about it, then you can't address it. And Failure to do so will lead to suboptimal care. And at the end of the day, the patient will suffer. And that's very unfortunate, because these are chronic disease. Right? So it's very important to have a very long term perspective and and think of the quality of life and in really a broader sense than just focusing on the disease.

How can advocacy groups like the Canadian MPN Research Foundation also help?

So they're extremely helpful with a number of things in a number of ways. One is that they've got resources in terms of information that are true, are the different treating MPN physicians or groups. They will also have information typically about different trials, they may have information about what type of drugs may or may not be available in whatever jurisdiction so in the states or different cities, or in Canada different provinces. They also will be able to begin to hook you up with patients with MPN support group. And I think I certainly want to emphasize how helpful it can be for some patients at least, to have that opportunity. The other thing is that support research groups have an ability to identify needs that patient may have and say, Okay, well, you know, what this aspect is, you know, quality of life is not studied enough. And maybe we think it's important and maybe we want some money devoted for research. And so there were more and more I think in medicine, we recognize that patients, it is important to have research that is devised to answer the patient's needs not just focusing on life, your gain or some symptoms, but we need to have again, a broader perspective and say, Okay, we're treating you what is it that you think how, how we could improve your care and patient group can steer research questions.

On behalf of the Canadian MPN Research Foundation, CURE spoke with Dr. Pierre Villeneuve, from the University of Ottawa, about MPN resources and support groups available to patients and loved ones.

Improving Overall Care of MPNs with Support Groups and Advocacy Research

We talk a lot to our patients about being their own best advocate, but can you explain what that means?

For me, that means that patients should learn as much as possible about their disease and, now, about any potential new treatments that may be available to them and keep an open mind even if those are at a different center.

What's the best way for patients to learn more about their disease so that they can have an active voice in their treatment decisions?

There is a lot of information out there and it is important to look for credible resources. People can find all sorts of information on the internet and not all of that comes from resources that are useful for the patients, I believe they should look at websites like the MPN Research Foundation, Canadian MPN Group, the patient support groups across Canada. In addition, we also have patient education days that are hosted by MPN physicians, where we try to provide our patients with the most up to date information and answer all of their questions.

With that, why is patient-physician communication so key when it comes to a patient being their own best advocate?

It's absolutely key. The physician-patient relationship is a partnership, each one holds a piece of the puzzle. If the patient does not convey their symptoms or the side effects of their treatment, for example, effectively to their physician, then the physician may not be able to find the most effective therapeutic pathway for that patient.

How does this communication play a role, not just in in deciding on treatment, but voicing on what's important to a patient, like quality of life, for example?

Again, it's a partnership. It's the patient's journey, and for the physician to effectively help them along this journey, they need to know what each patient's goals are. A physician, for example, might focus on blood counts or delaying progression, but the patient might have an important event in their life that they're working towards attending. So definitely the communication is key at this point in time.

We're very lucky. We're at an unprecedented time in MPN treatments, we have several options, including newer options that will be coming up that are being studied in clinical trials. This gives our patients hope, but it also adds to the complexity of the decision-making process. And communication cannot be overemphasized when it comes to that.

To bring it all together, what is your biggest piece of advice for patients to help them find their voice in their care?

Education. I think that, as we say, knowledge is power. I personally love that my patients are educated about their disease. They often know when a major hematology conference is going to take place. They come to their appointments, and they ask me, “So doc, what new information did you learn in this conference that can help me?” We, as physicians, are privileged to learn from our patients about their disease every single day. And at the end of the day, it is always the patients who will drive the science forward and the patient advocacy groups that will help us move forward with things like drug reimbursement, especially in a place or health care system like we have in Canada.

On behalf of the Canadian MPN Research Foundation, CURE spoke with Dr. Shireen Sirhan, from the Jewish General Hospital-McGIll University, about patients being their own best advocate in their MPN care.

Taking an Active Voice in MPN Treatment to Improve Communication About Care

To start, why is important, in particular for MPN patients, to seek a specialist in their disease?

 So MPNs are rare diseases, and although many hematologists in the community treat MPNs, there are certain centers that are more specialized. They have special clinics for patients with MPNs, with access to molecular testing clinical trials, multidisciplinary teams that can assess their needs. MPNs are complex, both to diagnose and to treat. So MPN specialists are the most up to date with current therapies, and they can help navigate this complexity of which drugs are best suited to each patient.

With that, can you also discuss why it may be important to seek a second opinion?

 It's important for patients to be comfortable with their MPN physician and a second opinion can sometimes offer them that reassurance. Also, patients who seek a second opinion with an MPN specialist can still be followed by their hematologist closer to home, and then what we call a shared care model, where they continue their regular follow up and have an occasional consultation with their MPN specialists in cases of, for example, disease status changes or new treatment recommendations.

Do you have recommendations on how patients can find a specialist or a cancer center that specializes in MPNs?

On behalf of the Canadian MPN Research Foundation, CURE spoke with Dr. Shireen Sirhan, from the Jewish General Hospital-McGIll University, about finding a care team that specializes in MPNs.

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