A Few Quick Thoughts from the MPN Patient Symposium

I just got back home from the whirlwind trip to New York City for the 6th International Patient MPN Symposium sponsored by the MPN Research Foundation and the Cancer Research & Treatment Fund.  The day was filled with fascinating speakers (several of whom were new to me) and each of them taught me something new.

I'll organize the 25 pages of notes later, but I thought I'd tempt you with these nuggets. :)

Cool Terms I Picked up at the MPN Patient Symposium

Somatic mutation:  a change in a gene that occurs after birth (you aren’t born that way)

Molecular mimicry:  some genes mimic others

Epigenome:  the new area for research

We are all entitled to our own opinions, but not our own facts.

JAK2 and Exon 12 and TET2 – a few of many mutations under exploration

Genomic Instability:  affects the micro environment and what happens with MPNs

Specialized Niche Cell:  cells that are highly specialized (example:  Endothelial cells)

Endothelial cells:   lining of blood vessels;  they also instruct other cells and broadcast directions throughout the body.  They also instruct organ regeneration (eg, liver and lungs). 

Inductive angiogenesis:   uh, i forgot already!

Proliferative angiogenesis:   forgot this one, too!

Role of vascular niche cells in self-renewal of stem and tumor cells:

The micro environment that are created by the mutations have huge impact on what happens. 

Angiocrine Factors                

Niche cell:  activate endothelial cells

Leukemic initiating cells – they expand the vasculature in the bone marrow.

Factoids and A-Ha’s! 

• Platelet-Leukocyte aggregates and micro-particles promote thrombosis.  The different kinds of cells can act together to promote thrombosis. 

• We need drugs that target the endothelial cells.   Endothelial cells expand hematopoietic stem and progenitor cells by angiocrine expression of Notch ligands.

• The first hematopoiesis occurs with the endothelium. 

• The leukemia from MPNs is completely different from the normal leukemias.  We need different treatments because the biology is different.

• The Chronic Myeloid Leukemia is triggered by the Philadelphia chromosome, so it is regarded as a different disease process from MPNs.

• JAK2 is not a “switch” that can be flipped on or off; it has at least 10 different mutations, which cause different disease results in patients.

JAK2:  The One Mutation, Three Diseases problem.

Why do patients develop ET, PV, or PMF?

Level of JAK2 allele burden determine ET, PV or PMF – more so in men than women

How much is the disease versus the host predisposition to develop an MPN?

Are there other cooperating somatic mutations?

• Despite all the advances, we still have a long way to go – do not be misled by where we are.

• For those of us taking warfarin (Coumadin), Dr. Weksler said that the newer anti-coagulants are “not there yet”.  Some patients have tried Pradaxa and gone back to warfarin.  Pradaxa must be taken twice a day consistently and this is problematic for many patients.  She believes that we’ll have a better alternative in a few years.

• Much of the knowledge gained and progress made has occurred because patients before us and among us have shared their tissue samples, DNA, and whole selves for testing and clinical trials.

There was so much more covered.  I hope this tempts you to watch the video when it is available online at the MPN Research Foundation

Stay tuned!

peace,

Marina

4th Anniversary of my Second Chance

October 14, 2007 -- a day as important as my birthday!
Four years ago today, Dr. Darryl Tookes quite literally saved my life with his expert emergency surgery.
October 14th or 15th could very well have been the date after the dash on my tombstone (if I had one).

Dr. Tookes discovered clots in the portal, mesentary, and splenic veins in my gut.  These clots caused part of my intestine to rot and triggered peritonitis which put vital organs in distress.  During my two week stay at the hospital, the doctors searched for the cause of these clots.  I detailed the experience in posts in the Winter of 2007 and early 2009, so I won't digress today.

It wasn't until October 2009 that we learned that I have Polycythemia Vera.  The hematocrit rose to over 69 and I had symptoms that put me at high risk of a stroke.  A bone marrow biopsy confirmed that I am JAK-2 positive;  the protein/gene malfunctioned.

I have not regained the energy, stamina, and mental acuity to return to a 40+ hour career.  I want the old "me" back!  I still dream of making significant contributions to our world.  I want to be back in the compensated career track.  I am continually frustrated by the unreliable and inconsistent "good days" that chronic illness offers.

Shifting focus from frustration to gratitude is part of my daily spiritual practice.  I am aware that my life is much easier than millions of people in this country and the world.  I have a loving and supportive family, excellent affordable health care (which is expensive for chronic illnesses), great friends and community -- both local and on-line, clean water & electricity on demand, plenty of food, a safe and comfortable home...
I don't take my gifts lightly.

With great gifts come great responsibility.  That belief is ingrained in my soul.  Perhaps that is where the frustration is fueled.  There is so much I want to do to act on my gratitude and help others.  I derive intense joy from helping others, easing another's burden.  When I cannot DO, I must focus on BEING... and today I am grateful.

So today, I exclaim a heartfelt THANK YOU to Dr. Tookes and all the great Kaiser Permanente docs and staff who saved my life and keep me going.

May you share gratitude and love to someone who has been there for you.  Trust me, it feels great!

Tired of the Rat Poison? Alternatives to Warfarin (Coumadin) are "Available"

Friday afternoon I received a phone call from Andrea, my Anti-coagulation Pharmacist at Kaiser.  She wanted to know how I'm feeling and what's been going on with me.  It wasn't a personal call;  I had my blood drawn earlier in the day and the results came back:  my Prothrombin Time PT/INR was 5.7.  This is WAY higher than where it's supposed to be (in the 2.0 - 3.0 range).

The INR (International Normalized Ratio) measures how "thin" the blood is.  In truth, the blood doesn't get thinner;  the platelets get slipperier and don't form clots as easily.   People take anti-coagulants (aka "blood thinners") to avoid blood clots and strokes.

Warfarin (brand name: Coumadin) is the old standby drug.  It is affectionately called "rat poison" because it is, indeed, the poison in rat bait.  The rats eat the bait and go back home and die of internal bleeding.  Those of us who have a history of blood clots (I had the clot trifecta in 2007 that almost killed me) must stay on anti-coagulants forever.  There is no amount of exercise or dietary change that will alter the deficiencies in my blood that cause it's propensity to clot.

Back to Andrea's call and questions:
Any bruises, she asked.  I did a quick scan and noticed 2 bruises on one calf and a bruise on 1 arm and a big toe.  And I have no idea from whence they came (sorry -- no wild activities lately!)
Any changes in your medicines?   No.  
Any changes in your alcohol in-take?  Hmmm... (be honest!)  Yes -- I haven't had any all week.  
Any changes in your diet?   Well, I've got 2 Behcet's ulcers in my mouth so I haven't been able to chew all week.  Soup or broth each day.
BINGO!  When you take the same dosage of warfarin but your diet changes significantly, it can throw the blood level off.
She told me to stop taking the warfarin for the weekend and come back on Monday for another PT/INR test.  She will call me with the results and advise me on the next dosage.
Until then, she said, be careful!  If you have any accidents or falls, go straight to the hospital and tell them you're on warfarin and your INR is high.  (I know that I am highly susceptible to internal bleeding when the INR is this high).

I've become accustomed to this in the last 3.5 years, but I tell you that I do not enjoy getting "stuck" every 3-6 weeks.  Since I have been trying different medications for other chronic illnesses (sounds much more dignified than "experimenting with drugs" doesn't it?!), my body is constantly re-calibrating.

There were rumors for years about more stable alternatives to warfarin.  Anti-coagulants that are more patient-friendly would not be sensitive to how much spinach salad we eat this week (vitamin K), would not require frequent blood testing, and could be administered in a consistent dosage.

Guess what!  Such a drug exists.  It's used in Europe and Canada.  It's been approved by the FDA, but is expected to be cost-prohibitive for most of us "regular" users.  And if the insurance companies and medicare won't cover it, we won't get it.   Here's a few links to information about Dabigatran (brand name:  Pradaxa).
NOTE:  I am not recommending or endorsing Dabigatran.  


I really like MedScape website.  It has lots of helpful information:
Dabigatran -- A Good New Replacement for Warfarin

Here's the pharmaceutical company's website for Pradaxa:  Pradaxa website

If you hear more info or get to try the new med, let me know.
Peace,
Marina