found the highly impactful MPN Research Foundation, which proactively has made a huge impact on this field by directing philanthropically-gathered resources to drive research advances in MPNs and advocate on behalf of MPN patients. Joyce Niblack, J.D. broke new ground in developing online patient communities that provide real-time moral and informational support for MPN patients every day. Joyce also helped start the efforts to better understand and impact the difficult symptoms associated with MPNs. Back in 2001, Joyce came to me and shared her view that the symptoms MPN patients face were common and problematic. However, since many MPN patients overall do not look ill, they felt their healthcare providers were not responsive to their symptomatic concerns. The engagement of MPN patients in advocating and determining their own future has only grown from these examples and include many others, such as Antje Hjerpe, David Alexander, Jon Mathias and Zhenya Senyak, to name a few, along with the continued efforts of the MPN Research Foundation.
In response to Joyce’s concerns, as well as other advocates, we conducted the first large scale assessment of MPN symptoms. Leveraging the extensive online community of MPN patients, we conducted a survey of over 1,400 MPN patients and demonstrated symptoms that were both prevalent and sometimes severe, including fatigue, night sweats, itching, headaches, bone pain, and spleen-related problems. We then realized we needed a way to capture and quantify these subjective symptoms to be able to track them (better or worse) in the context of a clinical trial, or even in practice. The Myeloproliferative Neoplasm Symptom Assessment Form (MPN-SAF – 27 questions) and shorter version (MPN 10 – 10 items), has helped quantify the symptoms patients with MPN experience. These tools have now gathered data from over 5,000 MPN patients, in over 30 countries, and asked in almost 20 different languages. The data has revealed some key observations, including:
1. MPN patients have frequent symptoms, with fatigue being almost universal.
2. MPN patients suffer a very similar pattern of symptoms regardless of geography or culture; however, culture may impact the severity at which a patient reports those symptoms.
3. Symptoms are overall worst, as a group but not by individual, for patients with MF, then PV, and then ET.
4. Severe symptoms can be present in any MPN subtype, and the severity is not only associated with risk-factor scores (i.e., DIPSS in myelofibrosis or IPSET in ET).
5. Certain symptoms are associated with disease progression, including inadvertent weight loss, fever and bone pain.
6. Monitoring MPN symptoms is important in assessing the impact of any therapy intervention, including phlebotomy or medications.
7. JAK inhibition has perhaps had the greatest impact of any pharmacological therapies on MPN symptoms.
8. Despite the availability of medical therapies, and beneficial impact of JAK inhibition on MPN symptoms, most patients have residual symptoms ranging from mild to problematic.
9. Mr. Senyak (or Zhen as we all know him), who is an MPN patient and advocate, raised the call-to-action regarding the burden of fatigue in MPNs and directly collaborated with our MPN Quality of Life Study Group to understand this burden further.
These observations leave many MPN patients searching for answers on how to help alleviate the problematic symptoms from which they suffer. This book was written explicitly to apply the lessons learned from integrative medicine to tackle unmet needs, including symptoms, in MPN patients.
Living Well is a great choice for focus, as living well or wellness does not imply the absence of disease, but rather balance and achieving the best quality of life (QOL) an individual can attain. Achieving wellness can include working on many aspects of our lives—stress, sleep, nutrition, pain, lifestyle, relationships, and disease understanding—that can improve or worsen an individual’s QOL regardless of the status of their disease. LIVING WELL with a Myeloproliferative Neoplasm (MPN) answers several key questions to help patients achieve that balance:
1. What is the MPN disease spectrum, and how is the disease monitored?
2. What is the background and the elements of integrative medicine?
3. What is the role and benefit of a healthy lifestyle while battling an MPN?
4. What is the information, role, and potential benefits of specific complementary therapies, and how might they help an individual?
I am certain readers will learn many lessons to help them on their journey with their disease. In addition, I suspect that the information presented on healthy lifestyle is relevant to both MPN and non-MPN patients alike.
Finally, I congratulate Dr. Gowin for bringing her passion for holistic patient care of MPN to this wonderful book. Indeed, I think it will be a welcome addition to the library of any MPN patient.
Ruben A. Mesa, M.D.
Director
Mays Cancer Center at UT Health San Antonio MD Anderson – An NCI Designated Cancer Center
Mays Family Foundation Distinguished University Presidential Chair
Professor of Medicine
Introduction
Over recent decades, the wealth and pace of knowledge acquisition surrounding MPNs has been astounding. From the discovery of the JAK V617F gene mutation and the eloquent description of symptom burden, to the development of targeted therapeutics and validated symptom assessment tools, the landscape has truly matured in the field of MPNs.
While these developments are both exciting and hopeful, it continues to be challenging for those afflicted with this disease. Despite recent advances, patients continue to struggle with symptom burden and the chronic nature of the diagnosis of MPN. Many patients seek advice on ways to live better when confronted with a diagnosis of MPN and pursue methods to positively impact their disease and improve quality of life. Simultaneously, an emerging culture of integrative medicine is occurring within cancer care.
Integrative medicine offers the personalized, cutting-edge approach of traditional (allopathic) medical care, while concurrently recommending evidence-based lifestyle medicine and complementary therapies. This new era of integrative medicine is not unique to cancer care and is pervasive in
all aspects of medicine. In fact, I suspect it will be the universal medicine of the future.
With that in mind, it is with great enthusiasm that I offer Living well with a Myeloproliferative Neoplasm to the MPN community. As with all integrative medicine, it begins with a strong foundation in the understanding of the disease spectrum and standard therapies. It then builds on how the MPN patient or caregiver can empower their own health through lifestyle habits and intentional care of the mind, body, and spirit. It is intended as a guide for either patients or caregivers while on their paths to wellness in the face of MPN.
Sincerely,
Krisstina Gowin, D.O.
Chapter 1:
Understanding Myeloproliferative Neoplasms (MPN)
Understanding Myeloproliferative Neoplasms (MPN)
Disease Spectrum
Simply pronouncing mye-lo-pro-lif-er-a-tive neoplasms (MPN) is challenging!
