A spooky lack of reality
by Zhenya Senyak
There is a standard of care for blood cancer fatigue. “Monitor and intervene.” In reality, it’s more like shrug and eye roll.
You’re tired, so’s everyone. Let’s move on to something we can actually treat.
There’s a good reason for this state of affairs. The MPN patient — the person — is missing.
Research biologists focus on infinitely small interactions in a complex molecular universe. Hematologists focus on the organic effects of our MPNs to slow progression, relieve our symptoms. Even we, patients, seem to separate ourselve from the chaos raging in our marrow. As a result intense fatigue, the most common effect of myeloproliferative neoplasm – as with other blood cancers – continues to be largely unexplored.
This is a story of one discovery that emerged when one MPN patient dropped the burden of fatigue.
Something was clearly missing from last month’s big hematology meeting in Atlanta.
At the core of this international gathering of brilliant scientists and physicians, amid all the multi-screen presentations, graphic wall posters, and scholarly talks, was a spooky lack of reality.
Imagine The New York Auto Show, with technology and design on display but no buyers in attendance. What was missing at ASH was…us.
Here is a massive international gathering of cutting edge bench scientists and hematologists, women and men with the courage and brainpower to explore new biologic territory. They work deep in the bowels of our DNA, explore complex molecular pathways and secret biochemical processes. Many simultaneously maintain a clinical practice.
Still, nowhere to be found was a paper on the object of all this work.
The MPN patient.
Within the American Society of Hematology MPN section there was literally no focus on the MPN patient, the whole human being who contains the blood that is the presumptive subject of the meeting. You could catch glimpses of us in reports of clinical trial where some of our individual responses are aggregated to create a statistical dataset. But no amount of deconstruction would uncover one of us, an actual MPN patient.
So far as I could tell, the focus of the MPN section of the meeting was on developing and testing molecules that would interrupt known pathways through which the Janus kinases signaled blood production. There was some action on other fronts – telomeres, heat shock proteins, some flashy exhibits, some humble presentations of biopsy needles and assay kits. But no hint of interest in uncovering the effects of myeloproliferative neoplasm on the whole life of an MPN patient.
If it were just this professional meeting, it may not have mattered. But this mechanistic approach to MPN research and treatment extends to hematology practice as well.
We are partners in our own disappearance
It is not a lack of compassion on the part of our physicians. Everyone I know personally seems to care deeply about our well-being. Traditional medical practice continues to largely focus on things, the physical, visible manifestations of disease. Our spleens and marrow, our symptoms, genetic anomalies and sub-atomic bio-chemical processes are the basis of testing, diagnosis, and therapy.
We willingly agree. We need to avoid thrombosis, want swollen spleens reduced, want our symptoms alleviated. Those symptoms are addressed more or less successfully based on the skill and experience of our hematologist. Symptoms, but not us. It is as if we, as human beings, have somehow agreed to disappear behind a wall of x-rays, blood film and charts that take on a life apart from our own. Our primary symptom is not photographable. Our chief complaint cannot be weighed or measured with any accuracy.
We don’t wait for a thrombotic event, 3% blasts or a +2 fibrosis level before initiating treatment for MPNs. Why are we and our doctors willing to wait until we plunge into irremediable despair and exhaustion before starting treatment?
Since blood cancer related fatigue (BCR Fatigue) is a much more likely outcome of an MPN than stroke – and perhaps the most debilitating MPN manifestation of all — why do we not from the very beginning enlist professionals on our treatment team?
What we need as patients, and what our physicians and clinics might consider providing as a clinically and fiscally effective measure, is a routine strategic collaboration among traditional medical, psychological and alternative, complementary therapies. MPN is a whole life experience, affecting our plans, relationships, work, thoughts, feelings and self image as well as our spleens. It is part of our being, not simply a breakdown of machinery that needs to be repaired. Treatment of MPN requires a whole team approach….including specialists on our team to address the severe impacts of MPN on our cognitive, emotional and economic being.
The National Comprehensive Cancer Network, a non-profit alliance of 21 major cancer centers, produces a set of recommendations for healthcare professionals. They conclude that fatigue in cancer patients has been “under-reported, under-diagnosed and under-treated.” Their guidelines for CRF management call for screening of every patient for fatigue “as a vital sign” at regular intervals and management through drugs and other means, including psychosocial interventions.”
Treatment of MPN absent this component inevitably overlooks the psychological damage inflicted by exhaustion, denial, grieving, decline of physical abilities and all the rest. Denies us, in fact, early intervention and treatment of our concomitant depression and blood cancer related fatigue. Effective management of Cancer related fatigue, says the NCCN , “requires an informed and supportive oncology care team.”
Proof that a new approach to MPN therapy is needed is the MPN section of ASH. This Super Bowl of Hematology never touched on the primary issue affecting those who suffer from blood cancer according to the NIH and the NCI, among many others.
Fatigue, for example
The single biggest complaint of patients suffering from blood cancer is fatigue. 80%, at least of patients in therapy and over 60% of all MPN patients. And yet at the major annual meeting focused on sharing discoveries leading to new blood disorder treatment options, not one paper was presented by a psychologist, a social psychologist, a psychiatrist, neurologist, a mid-brain specialist exploring management of blood cancer-related (BCR) Fatigue.
A Central Reality
It might have been outside the area of expertise on display at ASH, but Fatigue is a central reality of MPNs. According to the NCI, “Patients perceive fatigue to be the most distressing symptom associated with cancer and its treatment, more distressing even than pain….”
The MPN patient view
The problem of reducing our MPNs into discrete symptoms is not limited to our physicians or the bench scientists and drug companies looking for magic molecules. It’s a reality we ourselves buy into.
Regardless of type, our MPN is not a thing, an objective occurrence outside our life. It is not an unwelcome visitor but a life-altering cascade of events affecting our minds and bodies, our families, social relations, work, and fundamental realities. Just think back to your first diagnosis to recall how it rocked your world…and still does.
A study published in the British Journal of Cancer “Fatigue and Cancer: Causes, prevalence and treatment approaches,” confirms there is a significant disconnect between physicians and their patients as to the impact of fatigue. 91% of patients report fatigue prevented them from living a normal life including 75% who changed employment due to fatigue. 61% of cancer patients report fatigue adversely affects their lives more than pain. Their oncologists, however, disagree with a similar percent (61%) believing pain affects their patients more than fatigue.
Patient input is essential to determine the dimensions of BCR-Fatigue. Only patients know the true impacts of MPN fatigue. The rest is just second hand news. We need to trust ourselves. We have access to data only available to our physicians through us. We live the disease. It inhabits us, we feel the effects. Our doctors can only note the impacts we report or exhibit.
Our physicians, the good ones anyhow, suffer with us, suffer for us, but never actually suffer MPN fatigue. They rely on us for feedback and we do not yet have the sufficiently calibrated tools or vocabulary to provide an accurate picture. But even when we can express our BCR fatigue to our physicians we often shrug it off or hesitate to take the risk.
“Physicians often have insufficient knowledge about fatigue and its treatments or underestimate the impact of fatigue on quality of life, while patients may consider it an unavoidable and untreatable side-effect and fear that reporting it may incite a change toward less aggressive cancer treatment.”
Annals of Oncology, February 2011
A simple idea.
The official diagnosis of ET, PV or MF is an event of such magnitude that, for most of us, it’s accompanied by shock and maybe a touch of fear. In general, fatigue accompanies cancer and its therapies.
An hypothesis worth testing is that fatigue is our first line of defense, a product of the physical and emotional shock of having our world rapidly turned upside down. We shut down and as a result trigger a disorder,
There are likely many components to our fatigue deriving from our MPN. But since there is no linear relationship between our fatigue levels and our HCT and hemoglobin levels, it’s possible the source of the crushing BCR fatigue is in another disorder completely, a co-morbidity that could be responsive to treatment.
The Fatigue Project
The idea of the Fatigue Project launched two months ago is to acquire data to design a clinical study of the relative effectiveness of fatigue reduction tactics in MPNs. Anecdotal, narrative reports submitted by patients can start the conversation that might be of use to designers of such a study. MPN patients can also volunteer to be part of the study by e-mailing ourMPNforum@gmail.com and typing “Fatigue Project” in the Subject line.)
The leading fatigue reduction strategies presented by the initial group of 36 patients include Exercise (28%), Nap (17%), Diet (14%), Sleep (14%), Walking (11%), Socializing (8%) and Willpower (8%). Other tactics included laughter, drugs, supplements, hydration, running, meditation, yoga, coffee, wine. Most participants employ more than one means to reduce fatigue, but 19% report giving up entirely on finding a successful way to reduce the MPN fatigue burden.
Dr. Ruben Mesa published a list of Top Ten Strategies he has found effective in his long experience with MPN patients. As the lead author of the “Burden of Fatigue” study –undertaken with Joyce Niblack and others — and primary developer of the MF Symptom Assessment Form, Dr. Mesa is one of the most visible hematologist/clinical investigators to zero in on MPN fatigue and its effects on MPN patients.
His Top Ten List, originally published in MPN VOICE rounds up the usual suspects in an empirical, common sense catalog that should surely be tried by all of us…if possible. They are: Getting enough sleep, walking, eating well, taking time for reading, wearing comfortable shoes, swimming, fresh air and being outdoors, stretching, less time on the computer and balancing work and life. (For the full text, click the MPN VOICE link, above.)
A sudden realization…
My own contribution to the Fatigue Project came about due to a sudden realization.
In the past several weeks, to anyone who would listen, I’d complain about a particularly acute and prolonged period of MPN fatigue. I’d nap, wake up, wonder what day it was and slide back to sleep. My concentration and focus were shot. When I would finally get to work, suddenly I’d lose all energy, feel my eyes get heavy and gritty and, under a black cloud, head back to bed.
With the ASH Conference coming up, I wondered how I could handle meetings I had arranged. As a stopgap, I figured I could drive down to Atlanta, park in a lot near the Convention Center and pop into my Camry for a quick nap when I felt exhausted and had to crash.
Saturday, I left my home at 5 AM, drove over the Southern tip of the Appalachians, and registered at the Press Room five hours later. I was able to complete a few interviews before a late afternoon taping for MPNforum Magazine. After touring the arena-sized poster and exhibit halls, I drove to my hotel. (The hotel, a couple of miles away, turned out to be crummy and noisy.)
By 7AM, Sunday, I was in my car headed back to the Georgia World Congress.
I can’t pretend to have covered the ASH conference but the day was crammed full of meetings, conversations and a torrent of information and new developments.
After a working dinner, instead of returning to my crummy hotel, I headed for the I-85 and North Carolina. At 2 AM, after 500 miles of driving and dozens of miles walking, I pulled into my driveway.…all without having a single nap. Without feeling a hint of MPN fatigue.
I was tired but clear-headed. The Atlanta trip had been therapeutic. What happened?
Turning back to Professor Mesa’s Top Ten Tips to combat fatigue, I saw I could check off most of the items on his list—walking, eating well, getting outdoors, stretching, getting off the computer and balancing life and work. Most importantly, I broke my routine, an isolated round of computer-based work.
To me, the conclusion was inescapable. Despite my certainty that fatigue was a result of my MF, I now felt my fatigue could be a symptom of something else altogether, something that might be treated and ended. One disorder that would respond to all that activity, socialization, engagement and exercise is dysthymia.
DYS-THYME-IA (diss-THIGH-mee-a): Mild, chronic and depression, similar but less intense symptoms, often lasting for long periods of time. Fatigue and loss of energy daily a common symptom. Chronic illness, medication, and stress believed to increase chances of onset. ICD 300.4
The choice of a suspect disease is obvious. And problematical.
Most of the classic symptoms of clinical depression map directly to the symptoms of (BCR) Blood Cancer-Related fatigue.
The NIH list of Symptoms of Depression could easily be used to describe BCR Fatigue: Loss of interest in activities once enjoyable, feeling tired all the time, difficulty concentrating, remembering details, making decisions, difficulty falling asleep or staying asleep or sleeping all the time. Overeating or loss of appetite. The Leukemia and Lymphoma Society lists similar symptoms of BCR fatigue: Cancer and its therapies are known to be commonly associated with clinical depression.
Depression, however, continues to carry a stigma in society not borne by other diseases. Despite its widespread occurrence and the body of scientific knowledge about the molecular, biochemical, social, emotional and genetic basis for various manifestations of depression, it continues to be associated with shame, failure of will, inability to cope, weakness … with serious implications for self-image, relationships and careers. That stigma is a barrier to treatment.
There are highly effective means of treating depression, and its resulting fatigue, through talk therapy, medication, biofeedback, mindfulness training and other modalities. But before we can tap into those means, we have to focus on the whole patient, understand the pain, stress, and anxiety created by our blood cancer. After that, it should not be difficult to ease the path to therapy if multivalent treatment is proven effective through controlled clinical trial.
The first obstacle is to get past the name. It shouldn’t be difficult. For example, my daughter will be cured of Asperger’s Syndrome in May, 2013 with the stroke of a pen. That’s the date the new DSM-9 takes effect, redefining Asperger’s as part of Autism Spectrum Disorder. A change in name only, maybe, but it opens a world of therapy. Which is what happened when we traded in our dented old MPD for MPN a few years back. A reasonable alternative might be Blood Cancer Related Dysthymia. (A primary symptom of BCR-Dysthymia, as other depressive states, is pervasive fatigue.)
I don’t know if my fatigue is due to depression, dysthymia or something else entirely. But I do know fatigue is taking a severe toll on my life and work with no relief in sight. My symptoms don’t conform to full blown depression since I’m pretty jolly and optimistic but do approximate dysthymia (ICD 300.4) closely enough for me to try a therapeutic path in that direction. I’ll talk to my doctor about it.
Conclusion: By and large, MPN fatigue is considered idiopathic and untreatable. By considering fatigue as a common symptom of MPNs and other blood cancers and not a serious and independent disease, a co-morbidity that could respond to treatment, we might be missing an opportunity to deal effectively with this “most common and debilitating” effect of myeloproliferative neoplasm.
The Fatigue Project is an opportunity for you to have your voice heard in determining future treatment protocols for MPN-related fatigue. Dr. Ruben Mesa and others have offered to help us evaluate alternative means to alleviate MPN-related Fatigue. To volunteer to participate in a future study or clinical trial in this area — or simply to receive additional information on The Fatigue Project — send a blank e-mail to ourMPNforum@gmail.com and put Fatigue Project in the subject line. Your identity will be held in confidence
Take me back to the Contents
© Zhenya Senyak and MPNforum.com, 2013. Unauthorized use and/or duplication of this material without express and written permission from this blog’s author and/or owner is strictly prohibited. Excerpts and links may be used, provided that full and clear credit is given to Zhenya Senyak and MPNforum.com with appropriate and specific direction to the original content.