Ask an MPN Expert: Tracking and Living With MPNs


Andrea: Good evening everyone, and thank you
for joining us for an Ask an MPN Expert Facebook Live Chat. My name is Andrea Larson from Incyte,
and I am joined tonight by my colleague Paul Larson, an oncology nurse educator. Thanks
for joining me this evening, Paul. Paul: Thank you.
Andrea: We’re really excited tonight to talk with all of you about general information
surrounding those who are affected by myeloproliferative neoplasms. Whether you’re a patient or a
caregiver, we would like to talk to you tonight about how a community can rally together to
raise awareness and positively affect the lives of those affected by MPNs. We’re also
looking forward to talking with you this evening about the importance of tracking your disease,
identifying trends within you diseases, and assisting you in talking with your healthcare
professional about those trends and how your disease may be changing over time.
So before we jump in and get started, I’d like to go through a little list of reminders
with all of you. Please keep questions this evening related to MPN-related topics. We
cannot provide medical advice this evening. If you have any specific questions about your
condition or treatment, please talk with your healthcare professional. Unfortunately, we
won’t be able to respond to all questions this evening, but we will certainly do our
best. And finally, we reserve the right to end the chat early. So prior to jumping in
and getting started where we’ll talk about the MPNs, first I’d like to make sure to
mention that I’d like all of you to be submitting your questions to the live feed below the
chat this evening. Andrea: First we’ll talk about general information
and topics around MPNs, and then we’ll move into answering your questions. So get those
questions in. We’re looking forward to answering them. Paul, can you give us a little bit more
information about your role, how long you’ve been with Incyte, and what prompted you to
become an oncology nurse educator? Paul: Yeah, thank you, Andrea. I’ve been
a nurse since 1995. So I started out in hematology oncology inpatient nursing, and got my nurse
practitioner in 1999. So I’ve been working with Incyte for about two years as an oncology
nurse educator working specifically with the MPN community to get more information to healthcare
providers and really helping patients advocate for themselves on the MPN community.
Andrea: That’s great. For those of you that don’t know yet, you actually may recognize
Paul from some of these events. Throughout the year, Incyte Corporation and Voices of
MPN host patient education events all over the country. We connect MPN communities and
give patients and caregivers a change to speak with MPN experts similar to Paul here. And
they get to connect with other patients as well.
Paul, in case we have people watching out there who aren’t familiar with MPNs or may
be newly diagnosed, can you please provide us with a brief overview?
Paul” Absolutely, but first I definitely want to reiterate the success of those programs.
We’ve been going out to different communities across the country, myself and four other
oncology nurse educators with Incyte. And the potential to get patients together all
in one room has been fantastic. The patients really enjoy meeting other patients with their
disease. Frequently it’s the first time they’ve met other MPN patients and it’s
a nice review of disease state and really gives people an opportunity to meet others
in their community with their disease. So to your initial question, definitely MPNs
cover a broad range of these hematologic malignancies. These are chronic blood cancers. The ones
that we focus on at these MPN meetings with the patients are Philadelphia chromosome-negative
MPNs. So specifically polycythemia vera, essential thrombocythemia, and myelofibrosis, which
we’ll be reviewing tonight for the next hour.
Andrea: Great! Great! Can you go into a little bit more detail about polycythemia vera?
Paul: Absolutely, polycythemia vera is the most common Philadelphia chromosome- negative
MPN. There’s about 100,000 patients in the US with P vera, typically shortened down to
P vera. And it’s characterized by an overproliferation of red cells. So this erythocytosis is really
what it’s predominantly know for: extra red blood cells in the bloodstream causing
the blood to thicken and setting up the potential for blood clots. It is potential going to
have an impact on other cell lines like the white count and the platelet count, but traditionally
most people with polycythemia vera focus on that erythrocytosis.
Andrea: Great. And can you talk a little bit more about myelofibrosis please?
Paul: Myelofibrosis is- it shares some similarities with P vera in that it is a Philadelphia chromosome-
negative MPN, but it has some very distinct differences, where even though there’s this
myeloproliferation – the bone marrow is overproducing cells- a lot of times folks
with myelofibrosis actually have cytopenias. They can have anemia. Anemia may be a presenting
symptom, or it may be something that progresses the longer lives with the disease. Both of
these are chronic entities, and we really try to facilitate people to have the ability
to live and thrive with these disease the long – as long as they can. But with this
evolution, things do tend to progress, and sometimes this anemia can come on either at
presentation or later on down the road. The P vera and myelofibrosis certainly have a
lot overlap in terms of symptomatology; itching, fatigue , achiness, and sometimes night sweats,
and some of these other MPN symptoms that we’re going to talk about tonight that are
so important to track. Andrea: Sure. And then can you just talk a
little about essential thrombocythemia? Paul: ET, or essential thrombocythemia, is
the other Philadelphia chromosome-negative MPN that we’re talking about tonight. Second
in incidence to P vera; about 80,000 cases approximately in the US. A little bit more
common in women than men; P vera, a little bit more common in men than women. And it
is also a myeloproliferation. The bone marrow is overproducing cells, and the blood marrow
is overproducing cells, and the blood becomes too thick with these extra cells. In ET, there’s
a focus and emphasis on platelets or megakaryocytes, or what they see in the bone marrow. If you
do a bone marrow biopsy on a patient with ET, you’re going to see an abundant amount
of megakaryocytes which are the precursors to – they become platelets later on in the
bloodstream. And so in ET, the patients will have a pronounced
platelet count. The goal is to reduce their risk, because by having this extra high platelet
count, they are at a higher risk for blood clots.
And similar to P vera, the other cells can be involved . So primarily you think and worry
about the platelets, but you do need to keep a close eye on that white count, and in ET,
you also want to look at the hemoglobin and hematocrit.
Andrea: Right. So it sounds like with all three of these MPNs it’s really important
to track your disease to be able to see some of those trends, especially because this is
so rare and it would affect every patient differently.
Paul: Very much so…they’re very heterogeneous. So everybody’s disease is going to be unique
in and of their own experience. So one patient’s P vera in just in terms of its indolence or
aggressiveness in terms of its ability for – to respond to controlling measures like
phlebotomy and P vera for instance. So tracking your numbers, having the sense for what your
baseline is, what your steady state is, and remember these are chronic conditions. They’re
not going away, unfortunately. So they have the potential to evolve and transform even.
And so we definitely want to encourage patients to have a – not an obsessive watch on their
numbers, but a healthy understanding of what they are, a good sense of what their baseline
is- and to note any significant deviations in that. Because that may prompt further investigation
and it would be something that should elicit discussion with their healthcare provider.
Andrea: Right. So you had mentioned that these are chronic diseases or sometimes we call
them progressive diseases. Because MPNs such as polycythemia vera- you may hear us call
it PV this evening, essential thrombocythemia, we’ll refer to that as ET, and myelofibrosis,
which we’ll refer to as MF – are progressive conditions, we know that they can change or
worsen over time. This is why tracking changes over time can reveal very important insights
about your disease and the status of your disease, which can help ensure that they are
having the right discussions, all of you, with your healthcare professionals or your
healthcare team. I also think it’s important to track and
track and look for your trends while you’re at home with your caregivers. There may be
things that you may not necessarily remember when you’re sitting and talking with your
healthcare team that your caregiver might be able to support you in. So let’s get
right into tracking should we? What should MPN patients be tracking related to their
condition and why? (Paul): Absolutely. Just to your point, getting
the caregiver, getting family and friends involved, is a huge benefit. When you go to
the appointment with your healthcare provider, it’s hard to remember everything that has
happened to you since your last visit, your last encounter with them, and you want to
be able to have another set of ears, another set of eyes to share what’s been going on
in the interval. But mostly, you know the healthcare provider’s going to be very keen
on your counts. So, you want to have a healthy understanding of those, but – and they’re
going to get those from the lab, and then they’re going to review them and discuss
them. And so you want to, again like we were saying, significant deviations; if numbers
have changed dramatically, that warrants discussion and potential further investigation.
But the symptoms are really going to be key for the patients to report on their own, because
your healthcare provider really cannot have a sense or send to the lab a symptom panel,
right? So that’s your job to track these. On a scale of one to ten, how has your fatigue
changed over the last three months? If you have pruritus, or bad itching, has it gotten
better or worse? And a scale of one to ten is an ideal tool to use. It used to be a three
or four. Now it’s a six or seven. That’s a clearly a worsening symptomatology, and
then that’s really your job to report as a patient. And then it’s the healthcare
provider’s job to assess why that may be and potentially investigate and make interventions.
Andrea: Sure. So let’s unpack some of that a little bit for those who may not be aware.
So can you talk about pruritis a little bit more?
Paul: Sure. Pruritus is a vey miserable symptom in MPN. In my experience, a lot of polycythemia
vera patients experience this; a lot of myelofibrosis patients – others , just like we were saying
earlier, how these are so heterogeneous – other people havenevre had pruritus associated with
their condition. Sometimes it’s referred to as aquagenic pruritus, so very much exacerbated
by exposure to water. Things like hot shower and then toweling after the shower can bring
on a significant bout of pruritus. And this is a symptom clearly driven by this cytokine
phenomenon that MPN patients experience. They have an abnormality in their hematopoiesis.
Their blood formation in their bone marrow is impacted by these mutations that occur
in MPN and these blood cancers. But there’s also overstimulation in another pathway that’s
producing cytokines. These nonspecific proteins very much involved in our immunology, and
so this excess cytokine, this pro-inflammatory state can drive a lot of these symptoms.
And as the disease progresses or evolves, the symptomatology can change. And so your
job is to report those symptoms to your healthcare providers so they can get a better sense for
what may be evolving or changing, and also hopefully they’re going to help address
those symptoms, because that’s what really makes living with these conditions day to
day such a burden in a majority of patients. Andrea: Right, and I’d like to remind all
of you out there this evening that if any of you are experiencing symptoms that may
be worsening, please consult your healthcare professional as each one of these MPNs may
affect each of you very differently. So can we talk a little bit – I know that you were
reffering to six on a scale of one to ten maybe – can we talk a little bit about the
MPN10 symptoms and why it’s important for patients to know them? What symptoms are involved
in the MPN10? Let’s talk a little bit about that.
Paul: Yeah, I mean the MPN10 to me as a nurse practitioner is just a simple review of systems.
So when you’re a healthcare provider you go in and talk with the patient. You get what
we call a “review of symptoms” how are you feeling? You know, activity level. Are
you more active, less active? Are you doing the activities – activities of daily living
what we refer to them? [You-] Pruritus would be on a- on a MPN10 scale. How is that itching,
or have you developed itching? Or if it’s a new patient, do you have itching? If so,
how miserable is it or is it mild? Is it just with the shower? Have you found ways to limit
it? Fatigue is the universal symptom that we hear
about from MPN patients. Fatigue is a very common symptom with MPN and in some cases
quite profound. Achiness, bone pain is a symptom. And then symptoms related to the spleen are
a real hallmark of some MPNs. Very much so myelofibrosis. Splenomegaly is quite common
in myelofibrosis, and to some degree in the other MPNS that we’re talking about tonight,
ET and PV, some degree of splenomegaly can be present. And the spleen is an important
organ in our left upper quadrant very much involved in our immunology and our blood,
and in this myeloproliferative state, people can have an engorged spleen. The spleen can
get large, and the technical term for that is splenomegaly. And that can cause abdominal
discomfort, aches, and pains. As it’s getting bigger it can cause early satiety or you fill
up really easily when you eat, and that can lead to some weight loss. So spleen-related
symptoms are a key part of the MPN10 assessment. Andrea: Sure. So I know we’ve touched on
several different symptoms, and we’ve gone through an talked about how they could affect
PV patients versus ET of MF patients. Let’s talk about each one of those a little bit
more in depth. So can we just talk a little bit about some of the symptoms that PV patients
could be experiencing? I know some of the feedback that we hear are PV patients tend
to not necessarily recognize some of their symptoms. They also feel like maybe it’s
a sign of them getting older. Can you touch on that a little bit?
Paul: Sure, absolutely. Although I must say that in MPN in general – and that’s why
it is this umbrella term, MPN – a lot of the symtoms overlap and the diseases share
many similarities, and that’s why they are in this same classification. So in my experience,
ET patient symptoms aren’t necessarily all that different from P vera. Myelofibrosis
is a bit more profound in that usually the symptoms that are present in those patients
are the same ones but even more pronounced; for instance, the fatigue.
But classically, if you think about polycythemia vera, the pruritus is a common symptoms in
a lot of PV patients. Some achiness ton some degree. The fatigue for sure. And just less activity than one would- was doing previous to diagnosis.
It is unfortunately common for people to attribute their symptoms to something else. “I’m
aging or I have this other – I’m too stressed out at work, or my home life.” And a lot
of times in hindsight, people realize that these symptoms are directly related to this
malignancy. About a third of patients are diagnosed through
symptomatology. Another third are diagnosed through just routine bloodwork. So that kind
of points to how some are more symptomatic than others. And then the final third are
diagnosed through an event. Obviously the biggest thing that we want to prevent in MPN
is these blood clotting events and so some patients are diagnosed because they had a
heart attack or stroke or a deep vein thrombosis, one of these blood clotting events.
So, but P vera, you know really classic for the fatigue, the itching. Essential thrombocythemia
in my experience, a little bit more complaints that I’ve heard about regarding headaches,
sometimes that foggy brain cognitive symptom, definitely fatigue. And then myelofibrosis
usually a really profound fatigue. Symptoms related to spenomegaly are really a hallmark
of myelofibrosis. Some easy bruising, bleeding events can occur in all of these entities,
but ET and myelofibrosis in my experience a bit more bleeding and bruising events.
Andrea: So it sounds like their quality of life is really affected.
Paul: It can be very much so. Andrea: For those of you that are just joining
in, you are on the Ask an MPN Expert Session this evening. I am Andrea Larson. I work for
Incyte, and I am here with my colleague Paul Larson who is an oncology nurse educator.
Just as a friendly reminder, if you want us to answer any questions that you might have
this evening, please add those questions to the chat box below the live session, and we’ll
get to those shortly. So I know we talked a little bit about quality
of life being affected, and I know that we’ve talked about a lot about tracking. Can you
maybe give some information around how caregivers can support patients within their home around
the quality of life that’s affected? Is there anything that a patient can say to their
caregiver to let them know that maybe they’re not having such a great day based on some
of these symptoms you’ve mentioned? Paul: So kin fog the caregiver’s role?
Andrea: Sure Paul: Yeah, I think that it’d be ideal for
a caregiver to have a sense for one’s nutritional status. Sometimes if we’re eating less or
not eating as well, we notice that less ourselves. So you might just keep an eye on their intake.
If their calorie intake is reducing, it would be something to bring up, and , you know,
your healthcare team may have a nutritionist that they could speak with. If you’re going
to have early satiety, if you’re going to fill up quickly, let’s make sure you eat
the right foods to get the healthiest amounts of calories that you can get.
Fatigue, and jus this sense of “Oh, I’m getting older,” not knowing that you’re
doing less. You know, so a caregiver can kind of gently suggest that, you know, you seem
like you’re taking more naps and things like this. And it’s just sometimes I think
we’re the last ones to know when we’re acting differently and doing things differently.
So I think just that extra set of eyes and ears can share to the discussion and, you
know, even bring to light to the patient themselves, “Oh yeah, that does seem different.” So,
you know, activities, nutritional intake, and maybe even mood. You know, in terms of
anxiety or, you know, your outlook on life. How much is that changing living with this
chronic condition? Andrea: Sure. So earlier you had mentioned
the importance of tracking blood counts. And we’ve talked a lot about the importance
of tracking. Can you talk a little bit about what some of the health trends patients should
be looking for when they’re tracking their disease?
Paul: So I mean the key thing with your MPN is what did it look like a diagnosis? So I
know not everyone out in the community is getting bone marrow biopsises. The World Health
Organization does recommend at this day and age a bone marrow biopsy at diagnosis. But
if you’ve already – if you’ve been diagnosed and you’re under care, I’m certainly not
suggesting that anyone go get that at this point. But what my point is why I thought
about the bone marrow is having a sense of what’s your baseline. So if you know what
you were presenting with, what was you hemoglobin your hematocrit? What was your platelet count?
What was your white blood cell count? And then tracking that. Obviously, your healthcare
team’s making interventions to try to get those numbers to a better range, whether they’re
too high or too low. However, they’re going to advise you to do that.
And so your job is just to have a healthy sense of where they are and be able to bring
up a discussion if they’re changing dramatically. A common phenomenon in this day and age with
chronic conditions and, you know, people who really are potentially enjoying their golden
years, they’re retired perhaps and they’re a snow bird. So they may be only spending
half the year at quote unquote “home” and the other half of the year somewhere south,
and so they’re transitioning from one healthcare team to another.
So they really, I think, need to advocate for themselves because the healthcare teams
are only seeing them half the year. And they’re not going to have as good a sense for changes
in their hematocrit, changes in their platelet count, and so a well- informed patient is
ultimately gonna lead to better care, because that patient is going to really be able to
advocate for themselves to their healthcare team.
In myelofibrosis they may be getting blood transfusions. If they’re getting blood transfusions
at different sites, it may be hard for the healthcare team. They may be covering each
other or signing order for each other. It’s hard for them to have a sense for how many
transfusions are you getting, but it’s very easy for the patient to track this and then
report it. I used to get a transfusion once a month, now it’s twice a month. Why would
that be, would be a fair question for them to bring to their healthcare team. Why am
I needing more transfusions? Andrea: And I think something that you touched
on that’s extremely important is the fact that they may have some more than other one
member of their healthcare team. They may have several. So it’s really important for
patients is to make sure that they’re tracking and being their own advocate when it comes
to their disease. Looking at those changes and asking the appropriate questions.
Paul: Absolutely, and they – to your point about having multiple healthcare providers,
I was thinking in just their hematology practice alone and if they travel they may have multiple
hematology providers, but then they’ve got a whole other boat full of providers for other
specialties. They obviously have other health potentials and may have other specialists.
Accidents can occur and you end up in the emergency department and they might not know
anything about your MPN. So really a well-informed patient and a patient that can advocate for
themselves can really save themselves a lot of trouble and potentially prevent, you know,
more serious outcomes that would have occurred otherwise. So making sure that they communicate
to an emergency room specialist for instance, “This is my hematologist contact information.
If you have any questions about my blood counts, please call them so that you can talk with
them directly.” If they’re going to the dentist and they
need routine care or perhaps something not routine, an intervention of some form. “Please
talk to my hematologist first.” Bleeding and blood clotting is a risk with these diseases.
Going in and doing procedures raises that risk. So making sure all of these providers
and specialists communicate together – sometimes they don’t do it on their own, but if a
patient helps facilitate that, its more likely to happen.
Andrea: Right, so continue to be our own advocate. So you’ve touched on this a little bit.
Can we talk a little bit more about the importance of tracking and how that may come into play
when patients have different medical procedures? Paul: Well, you know, tracking for procedures
I can’t really think of any specific things. You know, anticoagulation if somebody’s
taking an aspirin they’re you know. There’s going to be communication with the healthcare
team. I don’t want to lay too much of that on the
patient and then the healthcare providers will make the recommendations that are appropriate
to make. But in terms of tracking symptoms, you know,
really reporting that, because unfortunately these diseases can transform. So polycythemia
vera and essential thrombocythemia can evolve into myelofibrosis, and unfortunately all
three of them have a small but real potential to evolve into acute myelogenous leukemia.
And so, it’s really key that the patients and the healthcare team are aware of changes
so they can investigate further. So if you’re seeing significant changes in your complete
blood count, your CBC, it would warrant further investigation, and the best place to do that
would be in the bone marrow itself. Taking a look in the marrow to question if there’s
been evolution or transformation of this disease, or did something else happen? If you’ve
had a dramatic change in your hemoglobin, hematocrit, was it from a transformation or
did a bleeding event occur? So there’s a lots of things that can bring about changes
in our blood counts or out blood chemistries. And then, you know, again it’s not the patient’s
job to figure out what this is. It’s their job to report how they’re feeling, and to
have a sense for what’s my normal and when that normal changes making sure the people
overseeing your care, you know, investigate it and don’t – aren’t aware or neglect
it. Andrea: Sure. So how about tracking different
procedures that are frequent for each of the three MPNs. For example should patients be
tracking the frequency of phlebotomies that they may be having if they have polycythemia
vera? Paul: Sure, I see what you meant about procedures
now. So certainly, therapeutic phlebotomy is a common therapeutic intervention for polycythemia
vera. So having a sense for how many phlebotomies you’re having. If you used to only have
two or three a year and now you’re having four or five, why is that? Is the medicine
that you’ve been prescribed not- not doing its job? Or did- are you not taking it? Could
be multiple reasons, and so, just having a sense for I’m doing more phlebotomies or
am I doing less? I used to always get two or three a year, and now I haven’t had any
in a while. Why may that be? And sometimes a healthcare provider’s not aware. They’re
doing their visit with you and having a review of systems, not completely aware of your flow
of these procedures like phlebotomy or transfusions in myelofibrosis.
“I’m getting transfused more. Now. I’m needing platelet transfusion.” Why would
that be? And in essential thrombocythemia it’s very rare and atypical to have a apheresis
procedure, but some folks do have the potential; to have this apheresis procedure that will
bring about a rapid reduction in their platelet count. And if they were utilizing that as
an intervention, they would want to have a sense for how often they were doing that.
Andrea: Sure. Can you talk maybe a little bit about the importance of using online trackers
or how they may be effective when you’re tracking your disease? I know that we’ve
talked a little bit about you don’t have to go crazy and track every single thing,
but at the same time it should be something that becomes very routine and second nature
to you. So can you maybe talk a little bit about the benefit of online trackers?
Paul: I think it’s just something that keeps it all organized in one place. You know, in
your pocket with your device, and you can utilize these. If you’re old- fashioned,
pen and paper is fine, but sometimes that’s harder to remember or to transition if you’re
going from different sites of clinic. Certainly some people will enter things rather methodically
and have charts and graphs. I think that’s not necessary, but if somebody did that I’m
certainly not going to fault them on it, but just having a sense for what’s your baseline
and- and then being able to track these trends. And so there are some wonderful tools online
that one can use now. It’s just convenient and easy, so why not use it?
Andrea: Right. And I think it’s also an easier way to share it with your healthcare
team and also with caregivers just in case they’re in different locations and they
all want to be kept up on a family member or a friend’s updates. So I think that’s
really important. I think that we’re going to open it up to
some of the questions that we’ve received online. We’re really excited for that. So
if you can guys could continue to submit questions, we’re really going to try to answer as many
of them as we can this evening. So first we had a question that came in from Betty. It
says, “ I have PV and ET. Can you tell me the importance of watching ferritin levels?”
Paul: Oh, so the ferritin is a very- I want to say non-specific, but it is a marker of
your iron stores. But it is highly sensitive to other things that can impact it. So you
would never want to assess a ferritin when you had a bad cold for instance, or pneumonia,
an acute event, because your ferritin’s going to be falsely elevated. But a ferratin
is a very helpful test at steady state to know what your iron stores are. And in polycythemia
vera, a common therapeutic intervention as we discussed, is therapeutic phlebotomy. It
will help get those red cells down and reduce the risk for these blood clotting events.
It will lower the volume of these blood cells, but by doing that, the bone marrow replaces
these cells. Anytime we bleed or have blood removed, our
bone marrow wants to replace it, and obviously in MPN, there’s a drive to produce these
cells in overdrive already, and this you have to have iron to you- to produce red blood
cells. So in essence, through treatment, a goal to reduce the blood counts, to lower
the risk for clotting, we could make someone iron deficient. A healthcare provider when
I say we could. And so iron deficiency had symptoms and can make one not feel well. So
a ferritin is a good sense for what’s a healthy range of iron storage and what is
getting too low. Andrea: Great, thank you. For those of you
who may have just joined the live portion of this session for the Ask an MPN Expert,
my name is Andrea Larson, and I work for Incyte, and I’m joined tonight by my colleague Paul
Larson, who is an oncology nurse educator. Thank you for joining us.
The next question we received is from Janice. It’s “Is polycythemia rubra vera the same
as polycythemia vera? And is there a correlation between ITP and PV?”
Paul: So yeah. I mean P vera is P vera. I think that’s just a terminology difference.
There is an important distinction between polycythemia vera and polycythemia, though.
There’s a lot of patients who have polycythemia and they do not have an MPN. They are not
here in this discussion that we’re talking about tonight. There’s a lots of things
that can cause polycythemia, and this would be what’s referred to as a secondary polycythemia.
So something that’s driving an extra, in most cases, red blood cell production. Things
like testosterone supplementation or altitude changes or COPD, emphysema. Your body is sensing
it’s not getting enough oxygen, sleep apnea, so it’s producing more red cells. So it’s
very important to distinguish between secondary polycythemia and polycythemia vera, but P
vera and P ruba vera the same. Yeah. Andrea: Okay. We have another question that
came in from EK. Thank you for your question. “Can you be diagnosed with ET and have PV
symptoms, or do you just progress to PV? Paul: So MPN symptomatology, they definitely
overlap. So a lot of the symptoms in P vera and ET are similar, but most importantly,
everyone’s case is very unique in and to of themselves in my experience. So I’ve
never seen two MPN patients that were identical. So everybody’s case is different. So if
you have, in this situation you were talking about ET and can you have PV symptoms? You
definitely can, but you know in the same train of thought, you can PV and have ET symptoms
and myelofibrosis. The symptoms are very similar and overlap in all of these entities.
You can have ET and transform into PV, and you can have PV and ET that will transform
into myelofibrosis, and sometimes a sense that when that’s occurring symptomatology
may be evolving or changing, and that’s one, you know reason why we’re emphasizing
the importance of tracking your symptoms and these trends because it could be a representation
of some change in your disease status. Andrea: Sure. And as we’ve mentioned before,
it’s always important to note that if your symptoms continue to become more severe or
worsen, you can either contact emergency services or reach out to your healthcare professional.
So thank you for joining us tonight, Carol. We have a question form you. “I want to
know if you can progress from ET to PV, then a third stage before going to MF. Is there
any way to slow the progression down?” Paul: That’s a great question, Carol. You
can transform from ET to PV. And I’m not familiar with a third stage before going to
MF. So typically if you’re, you know – the only way you’re really going to know is
to look in the bone marrow and have your hematopathologist take a look at things under a microscope to
clearly define if somebody has evidence of myelofibrosis. But so to answer your question,
absolutely ET can become PV and both can become MF, but I’m not familiar with any interim
stage. Andrea: And you’ve talked in the past about
the importance of creating that baseline. So this why it’s also important to track
so that you can continue to see if your disease continues to progress.
Paul: Sure. I mean sometimes the bone marrow is a formality if somebody has transformed
or progressed they already know. Sometimes the patients know, have a sense even before
their healthcare provider, and then the bone marrow just kind of confirms what everyone’s
feeling in their gut. Andrea: Right. So we have another questions
for you, Paul, from Phillip. “What is platelet apheresis?”
Paul: So platelet apheresis is that very rare procedure that I was referring to and thank
you for asking, because maybe I didn’t shed enough light on that. And in my experience
not utilized very much in ET at all. But it is a rapid- so if you donate platelets. Say
you go to the Red Cross and you have healthy blood. If you have an MPN, unfortunately you
cannot donate, but encourage your family and friends with healthy blood to donate, because
all blood cancer patients need healthy blood for transfusions. And sometimes mostly people
donate red cells, but they can donate platelets. And there’s a machine, I don’t know the
name of it, but it’s a fancy machine that your blood goes out of you into this machine
and it spins the blood, and it will collect the cells that we have asked it to collect.
This technology is amazing. So when stem cell transplant is utilized, these machines will
collect the stem cells, and in this situation it can collect those platelets, and then if
for donation save them for donation, or in the case for ET if this is a therapeutic apheresis,
it will get a very high platelet count down rapidly with this machine. So it will take
the platelets off. Blood goes into the machine, spins it, takes the platelets off, puts the
rest of the blood plasma, red blood cells, white cells back into the person, and will
get that platelet count down quickly. And again, in my experience, not utilized
very much for management. Sometimes utilized in an acute setting. But it’s certainly
possible that people that they’re doing this for their disease management in ET.
Andrea: Well, something that you mentioned that I would like to just capitalize on, we
have caregivers all the time who ask what they can do to help support the MPN community,
and you mentioned that blood donations are always something that can help anyone in need
of any type of blood donation. So I think that that’s something important to note
too. We hear that a lot through those patient meetings that we discussed earlier.
Paul: Absolutely. That’s kind of my little soap box thing. I donate my red cells when
I can, and I think that family and friends want to help. They say, “You know, what
can I do for you?” and you kind of just sometimes, “You know, we’re all good.
I appreciate your thoughts.” And sometimes you just- you want to know that people want
to help, but sometimes there’s not a whole lot for them to do. And so that’s something
you can tell them. “Hey go donate red- go please go donate blood. It’s going to help
potentially me and definitely other patients.” Andrea: That’s great. Just a reminder, if
you could continue to submit any of the questions that you may have, Paul’s ready and willing
to answer those questions here up with my today. So we have a question that came in
from Liz. “If I have to have any type of surgery and I have ET, are there any concerns
that need to be discussed with my healthcare professional?”
Paul: Absolutely. That’s a great question. So any surgery that you’re going to have
– no matter how minor or major- the surgeon and your hematologist, your blood cancer specialist
should be having a discussion. Unfortunately, the risk for bleeding and blood clots in MPN
patients is greater than normal, and bleeding and blood clot risk is high to start with
for all of us when we have procedures. So it doesn’t mean you can’t have procedures.
You’re a higher risk surgical candidate, but you can still do these things. There just
needs to be coordination and discussion amongst all the specialists and healthcare providers
to lower your risk for these bleeding and clotting events that can occur in that acute
setting. Andrea: Right. Again, advocate for your own
health and make sure that you track and let everyone who’s a part of your healthcare
team know. Paul: Absolutely. As long as everyone’s
communicating, patients are going to get the best care. Might be something that was an
outpatient procedure for someone else, you might spend the night in the hospital. No
big deal. Whatever it takes to get things done and lower your risk for these complications.
That’s how thing can still get done. Andrea: Great. So thank you for submitting
your question, Paula. “Is there a correlation of the effects on hepatocellular carcinoma
with ET?” Paul: So is there a correlation of the hepatocellular
carcinoma with ET? The effects – you know I must confess, I’m not too up on hepatocellular
carcinoma, but, solid tumors and other – and blood cancers certainly can occur simultaneously,
or, we talked about these MPNs are chronic conditions. You may live with them for years
and years and everything’s going swell, and then you develop another disease, and
that other disease could be another cancer. So certainly, these thing can occur and, again,
why it’s so important to track and trend. So if you’re having new symptoms, new changes
in your blood’s counts or chemistries, it could be your MPN changing, or it could be
something else happening. And you know, the first thing that I think about when I hear
about the liver, anything hepatocellular, the liver is key in platelets. And so anyone
with liver disease can have a significant change in their platelet counts. And so hepatocellular
carcinoma/ET, I don’t feel that there’s any direct correlation, but the care and management
of [I-] both of these could be compounded, but certainly, again communication – you
would be probably be a hematologist with the ET, and a solid tumor specialist with the
hepatocellular carcinoma, and it would be key to really keep a close eye on platelet
counts, obviously. But in terms of – if the question is – is there a correlation
of the effects, you know, certainly as I mentioned they both are platelet related. The bone marrow
is impacted in the MPN, driving platelet changes. The liver is a key organ in [liver-] in platelet
production. So certainly they can be intertwined in their management, but I’m not familiar
with any correlation in terms of their incidence. Andrea: Okay. Paul, Theresa has a question
for you. She wants to know, “Are headaches experienced with MPNs?”
Paul: Headaches are quite common in MPNs unfortunately, and in my experience a bit more in essential
thrombocythemia. Can be a real challenge for some folks, so tracking these and having a
sense – headaches are a a challenge in any person who has a chief complaint of a headache,
but so if that’s one of your MPN symptoms – get a sense of what your baseline is,
know what interventions make it better, know what – know what interventions make it worse,
and really work together with your healthcare provider in terms of minimizing these. We
may not be able to make them go away completely, but hopefully with thoughtful and thorough
discussion and evaluation, we can minimize them and give you strategies to help manage
them when they do occur. Andrea: We do have a question from of the
Ilene. “Is tiredness one of the symptoms?” And I know you’ve talked about some of the
symptom -symptomatology crossing over between all three of the MPNs. So maybe you could
talk a little bit about how tiredness may affect some of the different MPNs.
Paul: Tiredness is the most common MPN symptom or fatigue, and in terms of its evolution,
certainly we’re all prone to having good days, and bad days, but you want to have a
sense for “is my fatigue getting worse, and why is that?” And so you can use that
as an example in terms of that MPN10 scale activity level. Are you doing less? Are you
missing more events, more social and family events? Are you less active? And so that can
be hard. That can sneak up on you and we have a powerful sense of denial in terms of symptoms
. “No I’m fine. You know, don’t worry about me,” kind of mentality. And so helping
people track this. So the only way to do that is with a log, with a diary, with these tracking
tools and having a sense for you know, this is definitely getting worse. This is not my
imagination. And the fatigue is real. And unfortunately we hear all too often that MPN
patients are told their- their symptomatology is -is – is doesn’t – it doesn’t – it
doesn’t warrant that degree of symptomatology cannot be related to their disease. And it
clearly is. We see this across the MPN spectrum that these symptoms occur to great incidence
and great degree and so reporting them and sharing them with your healthcare team and
then coming up with these strategies. And sometimes those strategies or just changes
in lifestyle can have a big impact. And then obviously controlling the disease is your
healthcare team’s job and sometimes the better your disease is controlled, the less
your symptoms can be challenged- the challenging to you. So you know, to answer, Paula, the
question – I can’t remember whose question it was.
Andrea: llene. Paul: Ilene, just “ Is fatigue common?”
Extremely common, and very, very pronounced in myelofibrosis in my experience, but really
shared through out the MPN community. Andrea: Great. The next question we have is
from Paula. “Is a peptic ulcer an example of bleeding associated with ET?”
Paul: It is. Yeah, absolutely. So gastrointestinal bleeding and that’s something you can be
less aware of. You know, if you’re bleeding from your nose, that’s pretty obvious. If
you’re bruising easily, that’s more obvious, but if you’re having internal bleeding,
gastrointestinal bleeding, that’s harder for you to appreciate. And so hence why it’s
important to track your counts, because if you’re bleeding gastrointestinally, you’re
probably lowering your hematocrit and hemoglobin, and keeping a sense on your- you know, any
bathroom changes. If you’re having changes in your stools. If you’re, my goodness,
if you’re vomiting blood or having anything like that you want to seek medical attention
immediately, and if you’re having GI symptoms may be related to your spleen, but may be
something else. So all of these, you know, other things can occur, and so your job is
to report it and then it’s your healthcare team’s job to figure out what it could be
related – why it may be occurring. Andrea: Sure. Summers submitted a great question.
“Does age play a major part of prognosis with MPNs?” So maybe we could talk a little
bit about the age range usually related to each of the MPNs and then the second part
of her question is more around an example. “The younger you are when diagnosed, is
your prognosis better or worse?” Paul: That’s a complicated question. It’s
obviously a great question, and an important question. In terms of prognosis, age plays
a role. In polycythemia vera we stratify people low-risk or high risk, and one of the qualifications
for high-risk is age over 60. So if you’ve had a blood clot or if you’re over 60, you’re
high-risk. So if you are under 60 and have not had a blood clot, you’re going to be
lower risk, So in some degrees unfortunately age certainly does have an impact on prognosis.
In your healthcare team’s ability to be aggressive with management, sometimes age
can be a limiting factor. The older we are, the more baggage we bring in terms of other
conditions, maybe heart or lung conditions, that could potentially limit their options
for your disease management or treatment. I think she had- and then she had a question
in terms of being younger, obviously our younger patients typically we hear about these diseases
occurring over the age of 50 and 60, but everyone out there know we have lots of young MPN patients.
And certainly the younger folks are -these are chronic conditions. They’re not going
to go away unfortunately. The younger you are the longer you’re going to live with
it, and so I don’t think that that has any significant that I’m aware of impact on
your overall prognosis, but just the longer you live with it, the more that windows open
for this evolution transformation and things like that. But obviously younger patients
are – bring less other disease – diseases to – otherwise have good health. So I think
there’s pluses and minuses. Andrea: And this is why we’ve mentioned
in the past that each of these diagnoses are so unique to each patient, that they should
be really tracking and having these conversations specifically with their healthcare professional
about their disease. So the next question we have is from James. “What should I know
about blood counts and PV?” And you talked a little bit about complete blood cell counts
earlier. So maybe it would be helpful to talk a little bit about exactly what they should
be looking for when someone has polycythemia vera.
Paul: Yep with polycythemia vera you look at the hematocrit. Classically, people come
in with a very high hematocrit. Potentially you’re looking at their hemoglobin as well.
These are both reflection of their red blood cell burden. And a target hematocrit in this
day and age is under 45. So you look at the hematocrit in polycythemia vera blood counts.
You want it under 45, but it’s important to remember you want to look at all three
cell lines. So you think polycythemia. It’s very descriptive right that- right there Cythemia
is high and poly. So there’s multiple cythemia; white count could be elevated. Platelet count
could be elevated. So you don’t look at just the hemoglobin/hematocrit.
I think we need to complete CBC. We need to look at the white blood cells as well and
the platelets. Are they above normal? If they are, unfortunately the disease is not optimally
controlled. If they were previously normal and now they’re escalating, why would that
be? So that’s a something that you could pick up if you’re tracking and trending
your numbers, and then that should elicit discussion. “You know, hey Doc. I used to
always have normal white count and platelet count. My hemoglobin/ hematocrit have been
good, but I’m noticing those other cell lines are creeping up. Why could that be?”
It should generate discussion and may prompt further investigation. It could be something
related to your MPN or it could be something else. So that’s – that’s their job to
figure out. Andrea: Just as a reminder for any of you
who may have joined, my name is Andrea Larson with Incyte, and I am joined here with my
colleague Paul Larson who’s an oncology nurse educator. We have about 15 minutes left,
and we’re really enjoying answering all of your question. So please feel free to submit
and we’ll try to answer as many as we can. So, Paul, earlier you had mentioned splenomegaly,
an enlarged spleen. Jeremy has a question. “Do MF patients always experience an enlarged
spleen?” Paul: They don’t always. And it may be to
a varying degree. So some people have very significant splenomegaly; some may have mild
to moderate splenomegaly. And probably just as importantly, or maybe even surprisingly,
some people aren’t aware that they have splenomegaly. Some people have symptoms of
fullness, achiness. Other people are completely unaware that their spleen is big. It should
not go below your rib cage, and by definition any spleen that is below the rib cage, and
by definition any spleen that is below the rib cage is splenomegaly. And so was the question
does everyone have it? Certainly not, but in myelofibrosis the majority do have some
degree, but the degree of splenomegaly is highly variable. And your sense of it may
be limited/ Your healthcare provider should be percussing
and palpating your spleen. If they are not successful at doing that – perhaps you have
a larger abdomen, using ultrasonography can evaluate spleenomegaly – I don’t think
other tests are warranted. Sometimes on clinical trials, MRIs are done, but really a physical
exam will usually suffice to have a sense for what’s my baseline spleen? What’s
my baseline splenomegaly? And is it getting better or worse? If its getting better, let’s
keep doing what we’re doing. If it’s getting worse, why would that be? And again, the patients
may not fully appreciate how much they’re actually walking around and living with. Andrea: And palpating is that physical exam
that you were referring to. Paul: Yes. Pushing. They should be having
you lay flat and pushing on your abdomen and getting the sense where your spleen is. It’s
kind of why I’m doing all this nursing stuff. Andrea: So we have a question from Judith.
What’s the importance of bone marrow testing?” Paul: You know, bone marrow testing is a little,
I don’t want to say controversial, but highly variable. So the World Health Organization
did update their MPN diagnosis criteria, and in this day and age they do recommend a bone
marrow biopsy. It’s the only way you can diagnose myelofibrosis, because you need to
see that fibrosis in the marrow. But a lot of patients have essential thrombocythemia
and have polycythemia vera and they’ve never had a bone marrow and that’s okay. You know,
that happens. It doesn’t mean they’re getting poor care. It just means that their
healthcare provider didn’t feel it was warranted. Again, in this day and age they recommend
it as a baseline. My bais is that having it is a nice baseline. Again, for that track
and trend you’re going to live with disease- this disease, potentially for the rest of
your life, and you want to know what it was like at baseline. A lot of academic centers are more prone to
getting bone marrow biopsies. Sometimes out in the community they feel less inclined.
And if you have not had one and your disease is currently diagnosed and managed successfully,
you know, it could certainly be a fair question to ask your healthcare provider. How come
I didn’t have one? Should we do one? But I think
that it would be indicated is relatively low. It would be something that could be useful
if we see the – like we were talking earlier about these changes. That’s the only way
you’re going to really know what’s – what’s driving some of these changes is actually
looking in the marrow itself. Andrea: Right. We actually have a follow-up
from Summers. “Are immunizations important with MPNs?”
Paul: That’s a good question. You know, I’m going to chicken out a little and have
you defer that to your healthcare provider. I mean, the immunizations most of them we
get as kids. And then in terms of boosters and things like that I think that’s going
to be a variable to your case and your care, but great question to bring to your healthcare
team. Andrea: And now I have a question from Carol.
“Can you speak to the life expectancy for myelofibrosis
Paul: It’s highly variable. These are all chronic conditions and based on where you
are , we can do some prognostication in terms of initial diagnosis and as that’s moving,
we have some prognostic tools and models that we have some prognostic tools and models that
we use looking at your blood counts, looking at the types of cells , factoring in how low
things are, and then coming up with some computation of these numbers. But it’s highly variable
from patient to patient. Andrea: Okay. These questions are rolling
in. We have another one from both Summers and Rosella. “Is numbness and tingling a
symptom associated with MPNs?” Paul: It is. Yeah, tingling. So erythromelagia
is a fancy medical term for pain, burning in your hands and feet. As those blood cells
are going into those smaller vessels in your periphery they can cause this numbness, tingling,
and discomfort. Andrea: Great. Cathy says, “Will having
an MPN make someone more prone to acquiring other cancers?”
Paul: Well, unfortunately, anyone has a cancer has an increased risk for other cancers. I’m
not familiar with any specific “I have this MPN and now my risk for cancer X,Y, or Z is
exponentially increased.” I’m not aware of anything. But in terms of anyone that has
one cancer they’ve already kind of opened that cancer door, so to speak. And so the
risk for other cancers is slightly higher in this population.
Andrea: Sure. And again, that’s probably why it’s always so important to continue
tracking and advocating on your own behalf so that you can have those conversations with
your healthcare professional. The next question we have is from Cathy. “Are there any comorbid
conditions that seem to be common among MPNs?” Paul: Well, not that I’m familiar with,
but certainly other comorbidities with an MPN makes one’s risk for the biggest thing
that we worry about, is these thrombosis or clotting events. So if you also have cardiovascular
disease or type 2 diabetes, hypertension, obesity, your risk for these clotting events
plus an MPN is these other comorbidities do add to the risk unfortunately.
Andrea: Okay. We have a question from Shannon. “What should MPN patients be tracking related
to their condition and why?” Tracking seems to be a trend this evening.
Paul: So tracking the symptoms – so MPN10. Fatigue, itching if you have it, activity,
spleen symptoms, nutritional symptoms, aches and pains, night sweats, weight loss, your
general ADLs, activities of daily living, your blood counts. So knowing what my normal
hematocrit is or quote unquote you know “my baseline hematocrit.” I’m generally under
such and such a level. The healthcare team makes intervention X,Y,Z, and it keeps me
there. Now it’s changed. You know, why could that be? So keeping a sense of your symptoms
and your blood counts is really what you want to track, and then get a sense. Is there a
trend? Is there a deviation from that trend should prompt discussion?
Andrea: Sure. And then, anything you might have like a phlebotomy.
Paul: Definitely, you know, medicines or interventions that are done, tracking in addition. Changes
in dosages of medicines or incidence of interventions like phlebotomy.
Andrea: This is unfortunately the last question. We have been having so much fun speaking with
all of you this evening. So Diane, “Can you discuss ocular migraines in polycythemia
vera?” Paul: They unfortunately can occur, you know,
headaches, just a more specific termino – you know a more specific termino – you know
a more specific definition of your headache. I’m not familiar with the specifics in terms
of the incidence or how to best alleviate them, but headaches in MPNs – MPNs increase
the incidence of headaches and sometimes the severity and hopefully through management
of your MPN that’s going to help manage your headaches to some degree. Headaches are
a challenge. They’re – they’re a really tough symptom. So that’s the best kind of
remarks that I can make about them. But definitely don’t let anyone tell you its not related,
because it likely is, and ultimately, you know, the best thing in medicine is, you know,
treat the cause. And so if you think that your headaches are worse when your MPN is
less well controlled, you’re probably right. And so really managing your MPN to the best
of your ability usually will help result in better symptom management.
Andrea: So that was a great discussion this evening, don’t you think?
Paul: Thank you. Yes. Andrea: Thank you to everyone who joined in
and asked questions. We really enjoyed this evening. Paul thank you so much for joining
me. Paul: Thanks, Andrea. Thanks for all your
questions and we look forward to seeing you potentially at one of these events.
Andrea: And that concludes our Ask an MPN Expert discussion. Thank you again everyone,
and we hope you do have a great night.

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