SK, Lambert JR, et al. The JAK2 V617F mutation and thrombosis. Br J Haematol. 2008; 143(3):307; or to estimate risk of evolution to post ET/PV myelofibrosis. Alvarex-Larran, et al. JAK2 V617F; monitoring in polycythemia vera and essential thrombocythemia: clinical usefulness for predicting myelofibrotic transformation and thrombotic events. Am J Hematol. 2014;89(5):517.
www.mpn-qol.org
Chapter 2:
Monitoring Your Mpn Disease
Monitoring Your MPN Disease
Being diagnosed with a rare blood cancer is life changing for both you and your loved ones. It can be a confusing and emotional time as you begin to deal with your “new reality.” In fact, newly diagnosed patients often compare the emotional experience to the five stages of grief – denial, anger, bargaining, depression and acceptance. As difficult as it may seem, working through these stages can help you deal with your diagnosis.
Coping with illness is an ongoing process, filled with twists and turns and unknown paths. Learning as much as possible about your disease, working with a specialist, and getting support are the best ways to navigate this journey.
It’s also very important to accurately monitor your disease, which will help you advocate for yourself, stay on track with treatments, and improve outcomes. Although there are no known cures for MPN, many people who have ET, PV or MF can effectively manage symptoms and enjoy longevity with proper monitoring and treatment.
Finding a Specialist
The first step in getting quality care is to find a hematologist specializing in MPNs. Because MPNs are rare diseases with unique symptoms and treatment options, it’s essential to find a specialist who understands the needs of MPN patients. However, the rarity of MPNs can also make it more challenging to find a specialist. Your primary healthcare provider may be able to refer you to an MPN specialist, but be prepared to do some of your own research.
Some additional places to find a specialist include:
• The American Society of Hematology, which offers a convenient search tool to help you find treatment facilities by region and specialty. Visit: http://www.hematology.org/Patients/FAH.aspx, and go to “Find a Specialist.”
• The MPN Forum, which provides a list of patient-recommended hematologists who specialize in MPNs. Visit: https://mpnforum.com/list-hem/.
Questions to Ask Your Hematologist
• How many MPN patients do you treat now?
• What is my exact diagnosis?
• Did I test positive for any mutations? What kind of genetic testing can you offer me?
• What is my plan for managing this in the short and long term?
• What treatments am I a good candidate for, and why?
• What are the side effects of my treatment options?
• What kind of lifestyle changes should I make to deal with symptoms?
• If you are of childbearing age, how should I manage my family planning?
• How should I tell my immediate family about how this will affect my life and theirs?
• What kinds of clinical trials, if any, am I qualified for?
Source: MPN Research Foundation
Understanding The Complete Blood Count
One of the two major components used to monitor MPN is the complete blood count. The body is primarily made up of water and cells. While some cells stay in place to form tissues and organs, such as muscles or the heart, others may circulate around within the blood. The major blood cells include red blood cells, white blood cells and platelets. Since blood cells play a critical role in MPN progression and treatment, these cells are routinely monitored in MPN patients.
Specifically, changes in the blood cells can help determine if treatments are working and what doses are correct for you. Blood cell counts can also indicate if your disease is progressing to a more advanced form of MPN.
Blood counts are evaluated by a blood test called the complete blood count, or CBC. Let’s take a look at the components of the CBC:
White Blood Cell (WBC) Count
There are several types of white blood cells, which help your body fight infections. The most important infection fighters are white blood cells called “neutrophils.” With a CBC, your neutrophils will be counted and reported as the “absolute neutrophil count” or ANC. If your neutrophils are low, then you have what is called “neutropenia.”
Neutropenia (low white blood cell count) leaves you more susceptible to infections. The lower your ANC is, the more likely you are to have health problems:
• If your ANC is lower than 1,000, then you have a higher risk of getting an infection.
• If your ANC is lower than 500, then you have a higher risk of getting a more serious infection.
If you are diagnosed with neutropenia, it’s important to take steps to avoid germs and prevent opportunities for infection. (See Sidebar: Avoiding Infections)
Avoiding Infections
The following tips will help you steer clear of germs and stay safe while your white blood cell count is low:
• Wash your hands often, including before and after you eat, when using the bathroom, after coughing, sneezing and blowing your nose, after shaking someone’s hand or touching anything handled by others.
• Take a shower or bath every day.
• If your skin gets dry, use unscented lotion or oils. This will help prevent your skin from cracking, which can let in germs.
• If you cut or scrape yourself, clean the area with soap and warm water right away. Then cover it with a bandage.
• Always wear shoes in the hospital and around the house, and especially outdoors.
• Rinse your mouth out with water after you eat and before you go to bed.
• Use an ultra-soft
