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.
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. This way, they 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.
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.
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.”
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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