I have Polycythemia Vera, a myeloproliferative neoplasm (MPN) -- the stems cells in my bone marrow over-produce Red Blood Cells (RBCs). This thickens the blood and causes potential for clots and stroke. In addition, my blood has the Alphathalassemia Trait (the RBCs are smaller than normal and oddly shaped, so they don't transport hemoglobin efficiently). I am also iron-deficient (and can't take iron supplements, as iron stimulates RBC production) With these things working together, Anemia and the accompanying fatigue is part of my daily life.
My mom traveled with me so we could learn together (and she could keep a watchful eye on me -- to keep from over-doing it). I was so glad that she was there. Much of the cellular biology and mutation discussion was over my head, and she helped me make some sense of it. There was also a huge emotional component to being there that I hadn't anticipated.
I went to this symposium with several questions:
1. What has been learned about causation of MPNs?
Nothing more than the JAK-2 mutation, and even that does not occur with 100% of the patients.
2. What progress has been made in managing the effects of MPNs?
There is no substitute for eating healthy foods (grains, fruits and vegetables), daily exercise/movement - 30 minutes a day, and more rest than the average healthy person.
3. Is there a "cure" in the works?
No cure, but there are clinical trials of drugs to affect the JAK-2 protein.
4. Is there a correlation between the blood diseases and auto-immune diseases (because I was recently diagnosed with Behcet's Disease)?
No observed correlation between MPDs and auto-immune diseases. There are some patients with MDS who also have Behcet's Disease.
5. Is there another protocol I should be considering with my physicians?
As long as the Hydroxyurea is working, no need to go to the shots of Interferon or Pegasys.
6. What is the likelihood that I will get my "old self" back?
It's time to accept my "new normal" The more physically fit I can be, the better I will feel. But there is no cure or way to overcome the side effects of the disease process and medications to be able to resume the full-speed ahead pace of my past. AGGHHH!!!
7. If "no" to #6, is there a rate of disease progression that I might expect?
Each patient with an MPN progresses at a different rate. They are still collecting data to see if there are some trends. Because most MPN patients are diagnosed in their 60's, the life expectancy figures are not directly applicable to younger patients.
While I won't begin to do justice to the vast amount of information that was shared, I will share my "Aha" moments and some of the points I found most interesting.
Dr. Mesa brought together an impressive group of specialists who prepared their remarks in printed form so we could follow along. They took their Saturday and Sunday (which was also Mother's Day) as personal time to share their knowledge with approximately 160 participants -- mostly blood cancer patients and their spouse/caregiver.
Did you know that the body of medical knowledge doubles every 36 months?
The doctors presenting specialized in the various categories of blood cancers:
- Leukemias (acute, chronic, and myeloproliferative neoplasms -- ET, PV, MF)
- Lymphomas (Hodgkins and non Hodgkins)
- Myeloma (MDS, others)
Blood is an "organ" made up of both cells and liquid plasma.
Dr. Joseph Mikhael provided the analogy of the 3 types of wine (red/white/rose).
The Red Cells carry Oxygen (like trucks, they pick up oxygen from the lungs and deliver to the organs).
The White Cells are the immune system (like an army, they protect the body from invaders)
The Platelets help with clotting (like an ambulance -- they are very social, first responders to the scene to begin clots to stop the damage).
Blood is created in the bone marrow (the factory) from stem cells. If the factory is occupied with disease, then all the cell lines may be affected. If the factory is busy making one kind of cell (in my case with Polycythemia Vera -- red blood cells), the others will not be made in adequate amounts.
Problems with the blood occur when the bone marrow ceases to function properly -- either decrease in production or over production of cells, and when collections of cells occur in the lymph nodes, spleen, or other organs.
Why are these blood diseases considered the big "C" word - Cancer?
Cancer is defined as identical, uncontrolled cell growth.
When the body's normal balance that allows cells to grow in the right amounts for the right period of time is disrupted, cancers grow. This includes solid tissue (i.e., breast, colon, lung) and blood cells.
The "double whammy" of blood cancers is that they are the cells meant to protect you. He used the analogy of a crime committed by a citizen vs. a crime committed by a law enforcement officer.
What caused the Polycythemia Vera?
I didn't get any satisfactory answers. We know that I have tested JAK-2 positive -- meaning that the JAK-2 protein has mutated which tells the stem cells to over-produce Red Blood Cells. But we don't know why the mutation occurred.
Almost all blood cancers have no known cause. Possible contributors include the environment, diet, stress, smoking, toxins, and radiation.
Management of MPNs
The standard protocols for managing Essential Thrombocythemia, Polycythemia Vera, and Myelofibrosis (the main MPNs) remain phlebotomies, Hydroxyurea, Interferon, and Pegasys (pegylated interferon).
Given the options available, I am satisfied with my current course of Hydroxyurea. The daily interferon shots provide flu-like symptoms (not an option for me) and the Pegasys shots are done weekly and are very expensive. I am not yet in a mental place where I can give myself injections and don't want to ask that of my family at this time.
Because these cancers are so rare, the big drug companies don't see a big enough market for much R&D. That is why the treatments are adapted with drugs that have more generalized effects (and additional side effects).
There are some clinical trials with new medicines that appear to work at the molecular level on the JAK-2 protein. Not viewed as a "cure" but as a better management opportunity. These trials are being conducted with patients with Myelofibrosis (MF), as that is the next progression of the disease process. There is some promise that a couple of drugs may make it to market in the next 3-5 years for MF, and then could be demonstrated valuable for patients with PV.
I had the opportunity to speak with Dr. Claire Harrison, Consultant Haematologist with Guy's and St. Thomas' Hospital in London, England. She specializes in Polycythemia Vera and provided some great information. Among her PV patients, she has one who also has Behcet's Disease. She said that the fatigue is unquestioned given the PV, Alphathalassemia Trait, and Behcet's. She encouraged me to walk and exercise as much as I can to keep my heart and bones as strong as possible. The steroids for Behcet's make me a jittery tired person, so I need to be mindful of that.
Dr. Harrison's main presentations were on patient adherence to their medication protocols. She pointed out many myths we hold regarding which patients will adhere to their medication regime over time and why.
Specifically, those of us with chronic diseases who require long-term use of medications -- we can get tired of "taking all these meds" and if we feel okay, decide that we will stop taking them for awhile. We have our own underlying belief system about drugs and we get inputs from people all around us.
Dr. Brent Bauer of Mayo Clinic presented information on Complementary & Alternative Medicine.
He said that since most cancer patients use some CAMs, the physicians need to take that into account and discuss with their patients. While some can be dangerous, many others are helpful with Quality of Life issues such as stress management, fatigue, mental focus. Among those cited as helpful to patients:
- Art therapy
- Massage therapy
- Music therapy
- Prayer, spiritual practices
- T'ai Chi
In fact, Mayo offers acupuncture, Massage, Meditation, Music therapy, and Yoga because the science has supported their efficacy.
Other CAMs like herbal remedies (like mistletoe, cat's claw, and sangre de drago) have been shown in studies to actually cause proliferation of the blood cancers.
Isn't it amazing how many people offer their non-medical advice? Here's a few that have been shared with me:
- A friend of a friend tried this herbal remedy and is completely cured!
- It's all in your head -- read The Secret and you can call the cure to your body!
- Try this special vitamin -- it's guaranteed to work.
- Pouring all those dangerous chemicals in your body is bound to make you worse, not better.
While we may politely dismiss these well-meaning suggestions, a veil of doubt settles into the subconscious. After all, I secretly want to will this illness away and return to my former self.
One thing I hear from many other MPN patients is the issue of fatigue. Many doctors dismiss this symptom, leaving the patients to feel dis-believed and unsupported. Dr. Mesa offered that most doctors don't ask about fatigue because they don't have a solution. Doctors like questions for which they have answers!
He spoke about VO2 max and studies that demonstrate the burden of fatigue and quality of life with blood cancers. He also gently reminded us of the vicious cycle we must work out from under:
I'm tired and don't feel like exercising --> don't exercise --> lose muscle mass, lung capacity --> susceptible to other illness --> feel more tired. lather, rinse, repeat.
If we keep moving, we are less likely to lose ground with VO2 max, will heal quicker from regular occurring illnesses, and are less likely to get pneumonia. Our goals are to optimize flexibility, heart & lung function, and muscle tone. These will help us improve strength, improve sleep, and reduce fatigue. He suggested we try walking, weights or other resistance work, and yoga.
He reminded us that those with PV and ET are at risk of heart attack and stroke -- so we need to get moving!
One of my favorite quotes from the session:
"Research shows that optimism has many health benefits. It can boost your immune function, reduce stress, lower blood pressure, relieve pain, and improve sleep. Being optimistic also may help you feel more creative, make better decisions, and communicate more effectively."
-- Teresa Pipe, Director of Nursing Research at Mayo Clinic, Arizona
Coming to Terms with the Illness
C.S. Lewis wrote: "You do not have a soul. You ARE a soul. You have a body."
Dali Lama: "We cannot give what we do not have."