My mind is full! And I feel affirmed and understood -- two great gifts today.
This morning, Robert and I went to our much anticipated appointment with Dr. Elliott Winton, a hematologist at Emory's Winship Cancer Institute in Decatur, Georgia. (http://cancer.emory.edu/)
After researching as much as I could on-line, connecting with other patients living with Polycythemia Vera and other Myelo Proliferative Neoplasms, I couldn't wait to sit with someone who literally studies and thinks about these rare blood cancers on a daily basis. Yes, I finally said the "c" word; the science world has raised this blood disease group from a "disorder" to "neoplasm" which means malignancy. That's okay -- I can live with it. I call it "Polly." The personification helps me focus my frustration on this "thing" that has a lot of control over my life.
Dr. Winton studied my medical history of the last two years (sent over by Kaiser Permanente) as well as information Emory kept on file from tests I had there in 2003 (which I had long forgotten). He noted that there was evidence of an MPN in 2003 when my bloodwork showed high platelets and low hemoglobin. At that time, he said, they wouldn't have made the diagnosis, as the breakthrough scientific discovery of the JAK-2 protein gene mutation didn't occur until 2005.
In October, 2007 I experienced thrombosis (clots) of the portal, splenic, and superior mesentary veins. Those clots caused a cascade of additional health issues which I've described in posts on this site from around that time. Dr. Winton explained that these types of clots in the gut are major indicators of an MPN, but because they occur so rarely, the JAK-2 test sometimes isn't ordered and the diagnosis is missed. I have been on an anti-coagulation regimen of warfarin (coumadin) ever since. This treatment has staved off more clots; it does nothing to address the root source of the problem.
I also began taking iron pills again after the surgery to address the anemia that resulted from blood loss from surgery. Guess what? That triggered more red blood cell production -- a natural process, just aggressive for someone with PV.
I never regained my full steam after the surgery and within a year, had to quit my job at The IMPACT! Group. I simply didn't have the stamina to meet the needs of the organization. Little did I know that my bone marrow was working over time. I was tired all the time and couldn't push through it.
By the spring and summer of 2009, I noticed my toes and fingers turned purple intermittently. The doctors shrugged this off and were unconcerned. By July I was itchy and in August, my left ankle began to swell, turned red/purple, and became painful. I went to the doctor in September; a wonderful internist ran some blood work and called me the next day to let me know that there are some concerns (my hematocrit was almost 70) and I needed to see the hematologist immediately. My blood pressure also went through the roof.
We started with twice weekly phlebotomies and then added Hydroxyurea (HU) chemo pills to bring the hematocrit level to a healthy range.
Dr. Winton shared that a hematocrit that high is rare, even for someone with an MPN; that it is amazing that I didn't have a stroke or worse. I knew it was serious and I knew I was scared; I didn't know that it was my second "close call" in two years!
Dr. Winton provided a clear and interesting lesson in the types of blood cells (can you name them? T-cells, B-cells, Red Blood Cells, Granulocytes, Monocytes, and Platelets) and how they are made in the bone marrow, differentiate, and eventually die (apoptosis).
He explained that the JAK-2 mutation that has occurred in me presently affects the proliferation of the stem cells that create red blood cells (that's the polycythemia vera type of MPN).
Note to reader: If you read this and there are inaccuracies or errors, they are due to my limited memory and poor note-taking. They are not to be attributed to Dr. Winton. I did not run this by him before hitting "publish post." Mea culpa in advance.
The clinical problems created by Polycythemia Vera can include:
1. Clotting & bleeding which can trigger strokes, heart attacks, etc.
This is treated with anticoagulation medications (I'm on warfarin/coumadin blood thinner because I had the major thrombotic event in October 2007). There are supposed to be some better drugs coming on the market in the next couple of years (yeay!) Other patients who have not had blood clots take "baby" aspirin -- 81 mg/day. This occurs in about 1/3 of the patients.
2. Myelofibrosis -- the PV also increases production of collagen in the bone marrow and extra medulary hematapoeisis (when blood is created in the spleen and/or liver).
This would be treated by decreasing the JAK2 clone size.
3. Acute Leukemia -- well, this is just not a good thing; very limited time on the planet. This happens in 3-5% of the patients.
So... How do we decrease the clone size?
Thankfully, there are scientists all across the globe working on this.
Right now, the only known cure is a Stem Cell Transplant.
Emory does "mini transplants" where some of the patient's stem cells remain, but receives many more from the donor.
That requires typing my cells and those of potential donors (siblings are most likely matches). It would require a 1 month hospital stay.
The downside? For a person of my age, there is a 15% mortality rate.
So this is not a wise option at this stage of the disease. I will pursue more information on this option as a Plan C or D, as it is better to prepare early rather than wait for an acute situation and have everyone scrambling.
The second option is Interferon. This chemo treatment slows the myelofibrosis, but the most common side effect is feeling like you have the flu all the time. No thanks!
There is a pegylated version (I think they use Roferon instead of Interferon) that requires just one injection a week and the side effects are minimal for most patients with the dosage appropriate for them. There is a Phase II trial coming up for this in about 6 months.
Most insurance does not pay for this yet, so some patients try to get into trials.
The third option is Hydroxyurea (HU) or Hydrea. This 40 year old chemotherapy slows the proliferation of the red blood cells. I am currently taking 500 mg of HU daily. The dosage level at 1,000 proved to be too much for me (resulted in the most awful sores in inconvenient places). There are other side effects of the HU that are annoying but bearable.
The fourth and most exciting option is on the horizon: JAK-2 Inhibitors.
There are several drugs in the pipeline that are a few years away from being ready for prime time.
So what is next for me?
1. Pay close attention to my hemaglobin level (must stay below 42).
2. Pay close attention to my INR (coagulation ratio) -- must stay between 2.0 and 3.0
3. Get a Bone Marrow Biopsy -- to gauge the progression of the disease process and Type my bone marrow for possible stem cell transplant in the future.
4. Additional tests (like D-Dimer) to gather enough information on my situation for the experts to determine what clinical trials might be appropriate for me.
5. See if my blood pressure can be stable in these conditions without the nadolol.
If you've read this far, I hope you can see how good it feels to be affirmed and have a plan of action. I'm in it to win it! It is humbling to be in this situation at a time when scientific discoveries are happening in this rare blood disease area.
My experience with Polycythemia Vera will be added to the international data base of patients and contribute to future study and hopefully discoveries. (I'm not worried about privacy. By this point, I'm essentially uninsurable in the US with these significant pre-existing conditions. If Robert lost his employment with a large company, we'd be in a sticky wicket. But I'm not borrowing any more trouble right now, thank you!)
You know I don't like roller coaster rides. But after my session with Dr. Winton, I feel like I can handle a few more bumpy rides -- with a reasonable plan and little help from my friends.
Polly kept me from traveling with Robert and the kids to Beaver Creek, CO for a couple of days skiing this week. My family is the greatest and I'm so glad that we didn't let Polly "win" by completely spoiling the family trip. Polly also brought some good friends to me to pass the time in my family's absence. Gratitude is a gift one gives to oneself. I am grateful!