Monday, February 18, 2013

These Tired Bones Need a Makeover

After several days of tests and consultations with experts at Mayo Clinic Scottsdale, I returned home with a worse-than-expected diagnosis. Here's the quickie Bad News/Good News version:

My bone marrow has gone from over-achievement to under-achievement. Instead of producing too many red blood cells, it is now in the "spent phase" and is not producing enough blood. This transition called my spleen and liver into action to produce blood. They are working hard, but will not be able to sustain their efforts forever. I'll soon need blood transfusions to keep my hemoglobin at a livable level. What irony! From phlebotomy to transfusion in less than one year.

A blood stem cell transplant is in my near future;  a successful transplant will cure me of the myelofibrosis AND the auto-immune conditions that rage inside.
Without a transplant, I'm at great risk for acute leukemia (no cures) and could expect less than three years on the sunny side of the ground.

Now that the test results and reports from Mayo are in my local doctors' hands, I'm preparing for the insurance approval / denial / appeal process. Quite frankly, I'm more anxious about getting the transplant approved (with the best transplant center for my particular leukemia) than the transplant process itself.

The kids and Robert are taking the news like champs. So are my mom, dad, and siblings. We're going to get through this together as we do all challenges ~ with love, laughter, the occasional cuss word, and toasts to the present and a bright future. 

Some have asked what they can do to help.  If you are so inclined, please consider:

  1. Become a blood donor. You can be sure that me, or someone like me, is grateful for your gift of life.
  2. Join the National Bone Marrow Donor Program. With a simple swab of your cheek, you will be "typed" (not blood type, by the way) and entered into the database. When a patient needs a donor, our info is compared. Donors are contacted and must agree to be considered for donation before the patient is contacted. Donors can say no at any time. Also, I won't need your bone marrow ~ just your blood stem cells (it's like donating platelets).
  3. Keep us in your happy thoughts, prayers, meditations. My mom alerted her international, multi-faith 'God Squad' and I am here to tell you that I do feel the love. It manifests in hope, energy, and strength.  

NOTE:  I especially encourage my friends of Asian, African, Jewish, and Native American descent to sign up. Matches for are very limited for children and adults of color compared to white European descendants. 

Here are some specialty donor programs (they tie into the large database):  

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If you like more technical terms, read on:

I. My Polycythemia Vera (PV) has transformed to post-polycythemic Myelofibrosis (MF) in recent months.

  • I have completed the “spent phase” of Polycythemia Vera and my bone marrow now has lots of reticulin, which crowds out the blood-making stem cells. 
  • Extra-medullary hematopoiesis is now at work – the spleen and liver are beginning to produce blood due to the bone marrow fibrosis.  Over time, this puts extreme stress on both organs.
  • The overall goal is to prevent the MF from progressing to an acute leukemia from MPN;  it is very difficult to treat and very high mortality.
  • The auto-immune conditions and portal vein blockage add complexities to my circumstances. My “youth” is an advantage.

II. I will start a newly-approved medicine called Jakafi to help improve the symptoms of the disease.  
While it won’t slow the progression of the disease, it should reduce the spleen size, night sweats, itching.  May cause more anemia.  I’ll likely need blood transfusions (when hemoglobin falls below 8.0

III. Need to plan for a blood stem cell transplant in the next year or so.
Because of the rapid rate of disease progression, I'm not a candidate for a clinical trial.
We need to prepare for a transplant:

  • “Type” me
  • Look for a match – my siblings, then
  • Get insurance & local docs on board with this plan
  • Stay as healthy as possible

I was hoping that a transplant could occur after Alexander graduates high school and heads out to college (mid-2014).  The doctors said that is highly unlikely.  There is a window of opportunity for successful transplants and weekly monitoring of my blood counts will inform us of the timing. That bums me out because I want to be fully present for his last year at home. He said, "Don't worry about me, mom. The sooner you get the transplant, the sooner you feel good enough to do all the things you want to do." 
As long as I make sure he has gas money... (wink, wink)


Living Life and Loving It said...

I am sending you as much love as I can via fingers and the worldwide web. I will be praying for you and your family. I pray God's goodness, grace and mercy cover you with healing and a donor match found quickly. I am on the Be the Match registry.
Much love, Ossie

Bonnie Evans said...

At least with a BMT you would tackle both your diseases. Mayo should have a case worker who will help up I get approval.i hope your HMO will allow you to go wherever you want for it to be done. There are only a handful of places that have good results thus it is imperative. First we need to get you a match!

Supposedly only 10% OF MF patients mute into AML which we had been told. The old statement of the life expectancy of an MF patient is 3-5 years which is widely believed to be outdated. Joe muted into MF from PV in 2008 but then again his blood counts did not get as low as yours except during chemo for MCL and is the only time he needed blood transfusions. You are YOUNG for MF thus your chances of a successful BMT is high..

A blood transfusion should give you some energy back but it will take a few times to get it up where you will feel the difference.

Love ya, BONNIE

OrangeMom said...

Marina - Sending you much hope! Genny had pMF and MDS and went through an matched unrelated donor (MUD) stem cell transplant a year ago. All in all she is doing very well, even while dealing with GVHD. If you go to January 2012 on her facebook timeline you can read about her isolation period, day by day. Just go to

MF sucks. Cancer sucks. But you will get through this and on to a better life. And you are so right -- laughter helps. Hugs to you.