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! It can also be difficult to understand this diagnosis, because it is not a single disease. MPN comprises several specific diseases, including essential thrombocytosis (ET), polycythemia vera (PV), and myelofibrosis (MF). In the simplest of terms, MPNs are a closely-related group of rare blood cancers.
Though MPNs were originally described in the late 1800s (See Sidebar: The History of MPN), it wasn’t until 2005 that several scientists discovered that patients with MPNs share a common gene mutation—the JAK2 mutation—within bone marrow stem cells. Gene mutations or alterations can cause many diseases, including MPN and other forms of cancer.
Our bodies function through a type of internal communications system. JAKs are specific proteins (Janus-associated kinases), which act as signaling mechanisms with cells. In other words, they “tell” blood cells in the bone marrow to divide and grow. When JAKs are working normally, they help the body make the proper amount of blood cells. However, if a mutation is present, the signal malfunctions and stays on permanently.
If you think of JAK genes as a factory, then a mutation flips the “on” switch and leaves the factory in constant production mode, causing too many cells to be manufactured. Just as a factory would eventually have difficulty handling this type of malfunction, the overproduction of cells begins to cause problems within the body, including blood clotting/bleeding, organ enlargement, bone marrow scarring and other symptoms.
Since the JAK2 mutation was discovered, additional gene mutations have been found in MPN patients, including MPL (myeloproliferative leukemia protein) and CALR (calreticulin protein), among others. Although no one knows exactly what causes MPN, most people with the disease have one of these gene mutations. The presence of these mutations helps to diagnose the disease, and with newer techniques, can help us to better understand how the disease may progress, as well as provide a prognosis.
As mentioned, MPNs are a group of linked disorders, and this grouping is referred to as disease spectrum. However, because they are so closely related, they tend to share certain features and there is variability between the individual types of MPN. This means there is the possibility of transformation