There are 18,248 Different Types of Myelofibrosis. And Counting.

Based on ~16,000-20,000 people living with MF in the United States.

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An MF specialist nurse practices individualized care for a very personal disease.

Kathryn Johnson, DNP, MSc, FNP-BC
Icahn School of Medicine at Mount Sinai, NYC

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Written by Kristin Bundy for Scroll Health Studio
CREDITS
I

magine a disease that never looks the same in two patients—a shapeshifter of sorts. One with a varied list of symptoms including tiredness, shortness of breath, excessive itching, bone and joint pain, easy bruising or bleeding, weight loss, fever, night sweats, and side pain due to an enlarged spleen. Some symptoms come and go. Others never show up at all. Patients with normal counts may have a high symptom burden, but patients with deficient blood counts could be symptom-free.

KATHRYN SEES
30
Cases
per year
for up to
2
HOURS
PER MONTH
PER PATIENT

Myelofibrosis (MF) affects 1 in 500,000 people globally, according to estimates.

Somewhere between 18,000 to 20,000 people in the U.S. live with MF. And each one experiences it differently. Now imagine being the physician or nurse practitioner guiding patients through their unique MF journey. Looking to you for solid answers amid the uncertainty. For a clearer picture of the future. For hope.

As an advanced practice nurse at a top MPN specialty center, Kathryn Johnson sees upwards of 35 MF patients a year—while community hematologists-oncologists and nurses may only see 1-2 cases per year. And she gets to spend up to two hours a month with each patient, which gives Kathryn a deeper understanding of the nuances and complexities of this elusive disease. This makes her and the rest of her MPN team at Mount Sinai a vital resource for patients and caregivers who walk through their doors overwhelmed by questions.

With every patient, Kathryn learns something more about the disease, how it presents and progresses, and every day, she finds new insights and more effective ways to identify symptoms and communicate with patients. And, with her focus on clinical trial navigation, she is at the forefront of pushing science forward and enabling her patients and their families to find the best chance yet of managing their particular MF.

“Understanding who they are outside of the exam room gives you a better look at how things change over time, which can impact their treatment.”

It’s this bridge-building between high science and personalized care that drew Kathryn to Dr John Mascarenhas and the team at Mount Sinai. “Before coming to New York, I was working in patient-centered outcomes research, and I loved it. But I felt like I was missing that human connection that we get working with patients.” Working with patients in the ever-changing frontier of MF means more than taking vitals, going over lab tests, and monitoring treatments. “MF is unique in that it is a chronic and ever-evolving disease compared to other malignancies that can  have a shorter course, so we really get to know our patients on a deeper level,” says Kathryn.

Establishing a trusting relationship is key to understanding and responding to their individual disease.

And it’s not a one-way conversation. Patients and caregivers are viewed as equal parts of the team, and self-reporting throughout each month is crucial to care. So, in addition to regular visits to stay on top of symptoms, Kathryn encourages her patients to call, text, and even snap photos to keep her updated on even the slightest change or burgeoning symptom. A minor change could signal a shift in disease progression or trigger an adjustment in treatment strategy.
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01 • THE VERBAL EXAM

Listening between the lines.

How to get to more productive patient conversations.

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iseases like myelofibrosis don’t exist in a vacuum or the neat predictability of a lab. They bore uninvited into lives, upsetting plans and dreams and the simple routines we take for granted. That’s why Kathryn focuses on patients as people first, knowing those life changes can reveal much about their myelofibrosis. She begins every appointment as though she’s catching up with an old friend.

The “direct question” approach of “How have you been? How are you feeling?” can have limited effectiveness in verbal exams, triggering polite, automatic responses like “Fine. Pretty good.” It is natural for many patients to report back the good and not burden or blame the healthcare provider when new issues appear. And other patients may use their built-in defense system that wants things to be “fine and pretty good” even when they're not.

Kathryn and her team then put all these verbal clues together with the physical exam and lab tests to unlock the whole story and better understand how the disease is progressing, evaluate candidacy for a clinical trial, or decide if their current treatment/medication plan needs to be adjusted.

1
IDENTIFYING
FATIGUE

Kathryn may ask the same question in different ways to uncover missed pieces of information. “It’s important to remember our patients are people, and people are all different.”

2
IDENTIFYING
Splenomegaly

And she may ask the same question in different ways to uncover missed pieces of information. “When it comes to taking care of my patients, I can be a bit of a bulldog when I need to be.”

3
SATIETY

Kathryn may ask the same question in different ways to uncover missed pieces of information. “It’s important to remember our patients are people, and people are all different.”

“Walk me through your day—was there anything you couldn’t get done that you wanted to?”
“Now that you mention it, I had to take a nap around three yesterday.”
“Are you noticing any changes in the way your clothes are fitting?”
“Yeah, I haven’t been able to wear jeans lately—the waistband is too tight.”
“How’s your appetite? Are you eating regular meals? Do you eat more or less than everyone else at the table?”
“Eh, sometimes I forget to eat. My serving of spaghetti was only the size of a baseball.”
1
IDENTIFYING FATIGUE

Kathryn may ask the same question in different ways to uncover missed pieces of information. “It’s important to remember our patients are people, and people are all different.”

Walk me through your day—was there anything you couldn’t get done that you wanted to?”
Now that you mention it, I had to take a nap around three yesterday.”
2
IDENTIFYING Splenomegaly

And she may ask the same question in different ways to uncover missed pieces of information. “When it comes to taking care of my patients, I can be a bit of a bulldog when I need to be.”

“Are you noticing any changes in the way your clothes are fitting?”
“Yeah, I haven’t been able to wear jeans lately—the waistband is too tight.”
3
SATIETY

Kathryn may ask the same question in different ways to uncover missed pieces of information. “It’s important to remember our patients are people, and people are all different.”

“How’s your appetite? Are you eating regular meals? Do you eat more or less than everyone else at the table?”
“Sometimes I forget to eat. My serving of spaghetti was only the size of a baseball.”
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02 • THE PHYSICAL EXAM

It’s sort of my x-ray vision.

Looking for signs to see what may be going on inside.

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long with information gleaned from the verbal exam, Kathryn uses the physical exam to listen to what the body can tell her about that patient’s myelofibrosis. There are the apparent symptoms, of course, but then there are the “secret messages” that can give a physician or nurse a more unambiguous indication of disease progression or other underlying issues at work. These can be signs on the skin, like coloration or bruising, or simply observing the patient as they go through the motions of the exam—walking down the hall, getting on the exam table, removing a jacket, or buttoning a shirt—to detect any joint pain or fatigue. By correlating these silent markers to routine CBC/blood tests, ultrasound/MRI findings, and the verbal exam, Kathryn and her team obtain a more complete understanding of that patient’s unique myelofibrosis.

The role of the caregiver as part of the treatment team cannot be understated.

1

“I start my exam by looking inside the mouth for any sores caused by their medication. I’m also looking for changes in palate color. If the patient’s hemoglobin is low, it can become very pale. A symptom that’s not always noticeable on the skin.”

2

“And then we look at the shins. I’m looking for bruising and petechiae which is a good way to check on platelet count. Bruises may present elsewhere, but because the shins are right in the ‘trouble zone’ where people often bump into the corner of the bed or coffee table, the shins can be good places to check for bruising. If I find a bruise bigger than a couple of inches, I’ll measure it and note it in their chart so we can keep track over time.”

3

“Another technique I use to get a sense of a patient’s overall fatigue or malaise is to pay attention to them as they do things like buttoning up their shirt, lifting their pant leg to show me their shins, or even climbing onto the exam table. I look to see if they are struggling, moving slowly, or just lacking the energy to do it at all.”

4

“Now it’s time to measure the spleen, which is tricky. Tricky to find. And to measure. But this is where the humble tape measure becomes an incredibly valuable tool to have handy. And it always helps to get a second measurement, then compare.”

1

“I start my exam by looking inside the mouth for any sores caused by their medication. I’m also looking for changes in palate color. If the patient’s hemoglobin is low, it can become very pale. A symptom that’s not always noticeable on the skin.”

2

“And then we look at the shins. I’m looking for bruising and petechiae which is a good way to check on platelet count. Bruises may present elsewhere, but because the shins are right in the ‘trouble zone’ where people often bump into the corner of the bed or coffee table, the shins can be good places to check for bruising. If I find a bruise bigger than a couple of inches, I’ll measure it and note it in their chart so we can keep track over time.”

3

“Another technique I use to get a sense of a patient’s overall fatigue or malaise is to pay attention to them as they do things like buttoning up their shirt, lifting their pant leg to show me their shins, or even climbing onto the exam table. I look to see if they are struggling, moving slowly, or just lacking the energy to do it at all.”

4

“Now it’s time to measure the spleen, which is tricky. Tricky to find. And to measure. But this is where the humble tape measure becomes an incredibly valuable tool to have handy. And it always helps to get a second measurement, then compare.”

Measuring the Spleen with a Tape Measure
A
Have patient lie flat on the table and start from the lowest point of the abdomen.
B
Use both hands, one on top of the other, and gently press until you find the firm round bottom of the spleen.
C
Then, hold that place and measure from the lowest point of the spleen to the bottom of the ribs and then take a measuring tape and measure from the ribs down to wherever the spleen hits here.
D
Document your measurement and note any changes to the spleen over time.
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03 • CREATING CONNECTIONS

We’re building a
community.

The power of teamwork in addressing the challenges of MF.

The Exam Table Drawing: Kathryn uses exam table paper to communicate to patients what is going on with their MF and their treatment.
K

athryn believes in making her patients part of the care team—involving them in every step. As one of her patients recalls, “They told me from day one that I was on the team. Because without me, there wouldn’t be a team.” Educating patients on current and potential symptoms is not only empowering, but crucial to understanding how the disease is evolving in their bodies so they, as a team, know how to move forward with confidence, as well as hope. Each patient relationship is unique because every one of them is experiencing something different. This means that the willingness to share details about symptoms and everyday life is incredibly valuable for every situation. “I tell my patients: we may be the experts on the disease, but you’re the expert on you.”

Of course, being forthcoming with the care team is just one aspect. In turn, the care team must educate the patients to help them understand what’s going on inside as their disease progresses. One of the most common questions Kathryn and the MF team get when reviewing the pathology from a bone marrow biopsy is, “What are the stages of MF, and what do they mean for me?” That’s when Kathryn taps into her artistic side, reaching for the markers and using the exam table paper as her canvas. “I like to draw examples of the four different areas we use to re-evaluate where patients are in the spectrum of the disease and how they’re responding to the treatment.” The first thing she talks about is bone marrow. Next, she walks through blood counts, spleen size, and symptoms. Each area can trigger a need to re-evaluate a patient’s therapy, but it’s better to look at them together to get the complete picture. “This is when we lean on the patients to have an equal part in the conversation about their care. Having a true sense of the symptoms can play a significant role in adjusting the treatment plan to accommodate the patient’s lifestyle better.”

“I tell my patients: we may be the experts on the disease, but you’re the expert on you.”

Creating a network of interconnected relationships—from care team members at Mount Sinai to the patients and their families, to community hematologists-oncologists and their staffs, to academic research sites, to the biotech industry, and advocacy groups—enables the healthcare community to surround this complex disease that has so many different faces. “The teamwork we value so much can extend to the general healthcare community with physicians looking to academic institutions, available public resources, and professional organizations. There’s a lot more room for collaboration, which is a dynamic that has changed tremendously and will continue to do so in a very positive way,” says Kathryn. As she shares the tips she’s learned from her experience, and as research findings, plus clinical insights, are given channels to spread, this small community grows stronger. As does the future. “I think there are a lot of exciting things happening in research. I’m hopeful that we will find more treatments that can better target the progression of the disease. There are a lot of exciting things coming down the pipeline—that we won’t even know about for the next couple of years—which could change the lives of our patients in considerable ways.”

REFERENCE 
Jyotsna Mehta, Hongwei Wang, Sheikh Usman Iqbal & Ruben Mesa (2014) Epidemiology of myeloproliferative neoplasms in the United States, Leukemia & Lymphoma, 55:3, 595-600, DOI: 10.3109/10428194.2013.813500US population data to determine number of patients using prevalence rate: https://www.worldometers.info/world-population/us-population. Accessed March, 2022.

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