Wednesday, June 26, 2013



I learned this week that my life saving blood stem cell donor is a 22 year old man who lives outside the United States. He has agreed to the stem cell collection schedule my doctor proposed. How cool is that?! Young, healthy stem cells ~ it doesn't get any better.  As a patient, I am grateful that this young man agreed to take time out of his summer to save my life.  As a mother, I feel a sense of pride that this young person learned about the need for bone marrow/stem cell donors, joined the registry, and agreed to help a stranger in another country.  Both my kids say they hope they can do for someone what this young man is giving me. I get teary every time I think about this.

As people share wishes, prayers, and positive thoughts for my health, I ask that you include my selfless, nameless donor in those efforts. 


We are ONE MONTH from my Re-birthday! The process begins with a thorough Medical Evaluation. Tomorrow (June 27, 2013) is my big test day.

7:00 am:   Pre-BMT labs, Vitals
   I was told they will draw the blood from my Power Port, so I take that to mean they need a lot of blood.

7:30 am:     Drink jug of Barium.  What a refreshing treat!

8:00 am:     Bone Marrow Biopsy   [need another 'tag line' for my rump!]

8:30 am:     Meet with Clinical Health Psychologist  (this one is most worrisome ~ what if they realize I'm a bit nuts?)

9:00 am:     Meet with Research

10:00 am:    Pulmonary Function Test w/DLCO

10:30 am:    Meet with Atlanta Blood Services re: my need for platelet donors

11:00 am:    Register for CT scans

11:30 am:    CT scan of Brain / Chest / Abdomen / Pelvis

1:00 pm:     Lunch (probably the highlight of my day)

1:30 pm:     Register for Echocardiogram/EKG

2:00 pm:     Echocardiogram

It is no secret that among my numerous quirks, I have 'text anxiety.' 
Whether it's my car's emissions test, the vision test to renew my driver's license, or weekly blood tests, I get a rush of the 'heebie jeebie jitters'. This may not yet be a recognized technical term, I think you know what I mean.  

To calm those jitters, I mindfully picture the test administrator in his/her underwear and focus on my breathing. 

In this situation, I recognize that all these tests will form a baseline of the health status of all my systems pre-transplant. My biggest threat to surviving the transplant is organ failure. The results of these tests enable the doctors to anticipate issues that may arise during chemo and post-transplant and plan accordingly.  

I will find out the results of all these tests on my Education Day, scheduled for July 10th.
As the song goes, the waiting is the hardest part.

Jakafi to the Rescue

Myelofibrosis Treatment Banishes Behcet's... Coincidence? I think Not

About one month after my Autumn 2009 PV (polycythemia vera) diagnosis, I started getting unexplainable lesions in most inconvenient locations (I've shared this over a year ago). After several months of excluding many possibilities, it was diagnosed as "Behcet's Disease/Syndrome" or "Probably Behcet's" in March, 2010.  

I found it odd that I would acquire an MPN (myeloproliferative neoplasm) and an auto-immune disease around the same time (mid-40's). My internist said anything is possible; my hem/onc and rheumatologist said there was no connection. 

While the medical literature didn't show any links with Myeloproliferative Neoplasms (MPNs) and auto-immune diseases, the question persisted in my mind.

Chemical Reactions or Physiological Changes?

I took Hydroxyurea (HU) to manage the polycythemia vera, along with occasional phlebotomies. When the Behcet's became unbearable, I was treated with prednisone for over a year to manage the flares. I then switched to Remicade infusions when I couldn't stand all the side effects of prednisone. The Remicade worked like a dream for six months. The last six months of using it, however, I endured debilitating inflammation and joint pain. My rheumatologist pointed to the PV and my hem/onc pointed to the Behcet's. Even getting second opinions in both fields kept me in a painful mobius loop.   

Last November, a gastroenterologist friend said that the inflammation symptoms were consistent with what some of his Crohn's patients experience after a time on Remicade.  I was in a conundrum: which pain do I prefer -- the hands, feet, and joint pain or the lesions pain in other equally important body parts. It was a false choice. 

At the same time, all my blood counts were on a slow but stead descent and we knew something new was happening. I quit taking the Remicade and decided to use prednisone when a flare was just beginning. It took about eight weeks for the Remicade to fully leave my body.

Enter Jakafi... After being diagnosed with post-PV Myelofibrosis in February 2013, I began a low-dose regimen of Jakafi to shrink my spleen, ease bone pain and night sweats.

My last Behcet's encounter was the first week I started Jakafi. I hit it with prednisone to keep it from going full flare. It subsided within 4 days. I haven't had another flare since.

Apparently, there has been research on the JAK pathways and their relationship to auto-immune diseases. Pfizer now has TV ads for Xeljanz (tofacitinib), a "JAK inhibitor for Rheumatoid Arthritis."  RA is another auto-immune disease.

I realize that I am but a sample of one, but this experience gives me solace that my hunch that these two rare/oddball diseases share something in common. The JAK pathway!

When Focus Needs Perspective  

We are living in a most remarkable time of scientific discovery. 
Researchers are able to focus on changes at the molecular level and tie to proteins, genes, kinases and other terms I can barely pronounce.  

Many times I've been frustrated by the very narrow scope of this research because it doesn't help me NOW when I'm hurting. 

I must remember the adage: Follow the money. 
Research funding is tied to very specific research questions and tight timeframes, influenced by the funder(s). Funding is extremely competitive. And those research dollars rarely fully-fund a project, much less encourage researchers to follow new paths that emerge as they do their work. 

So blood cancer researchers continue their diligent work and auto-immune disease researchers continue their work. All the work is vital to understanding the disease processes which will ultimately inform us on effective prevention strategies. 

At the same time, a funding mechanism that supports tying all this knowledge together must occur to make the whole larger than the sum of its parts.
Stand Up to Cancer is a funder of innovative and collaborative research and information sharing among experts of different types of cancers.  

Perhaps it's up to patients/trial subjects (whole beings) to share our experiences, observations and questions. We can help the subject matter experts "connect the dots" in ways that may be outside a study protocol, but useful nonetheless.

After all, we are not only the patients, we are the consumers of the eventual treatment or cure.

Thursday, June 13, 2013

I Blame the Hemoglobin

There seems to be an inverse relationship between my Hemoglobin level and Anxiety level. Hemoglobin is a protein in the red blood cell that carries oxygen through the body. As the hemoglobin decreases, I become more easily annoyed -- with myself and everyone around me. I get the anemia double-whammy from the alpha thalassemia trait (red cells are smaller and oddly shaped) AND low hemoglobin count.  

The difference between me with hemoglobin at 12.0 and 9.5 is 'Marina the Upbeat, Can-do Gal' and 'Marina the Grouch.'  12.0 is fun to be around; 9.5 and below is best to avoid.

The filter between my thoughts and my voice has thinned over the course of prolonged illness. I used to be a pretty tactful person, able to smooth ruffled feathers and still get a point across. Active listening, patience, body language and good humor are reliable communication techniques. I've studied non-violent communication and practiced appreciative inquiry as a part of my spiritual growth and leadership development. 

Yet when the hemoglobin dips below 9.5, all bets are off. 
Active listening morphs into "what the heck were you thinking??"  
Patience evaporates in the midst of household procrastination (isn't it a good idea to save big clean-up projects for the hour before company is to arrive?). 
Humor is replaced with sarcasm: "of COURSE I know where your [insert any random item] is. Let me turn on my magical internal GPS device that tracks items I've never seen or used and find it for you."   

While I'm not quite ready to appear on the Jerry Springer show, it seems like I'm trying out the role.  My gripes, rants, and sarcasm are not how I'd like to be remembered, especially by those who get the brunt of my mis-placed frustration ~ my loved ones. 
If life is my Advanced Placement exam, I'm clearly not ready for graduation.

I offered several "reasons" for my crankiness: 
*  teenagers are self-aborbed                     *  it's the anemia talking  
*  'no one' shares my sense of urgency         *  not enough oxygen to the brain   
*  we don't live in a barn                           *   fatigue causes frustration

The correct answer may be "all of the above PLUS fear"

When my blood levels are closer to normal, I feel good enough to keep busy with the kids, help with the marrow registry drives, and spend time with friends. I don't make time to contemplate the upcoming stem cell transplant.

When the need for a blood transfusion approaches, I am so fatigued (this is different from feeling 'tired') that I physically cannot do as much. Climbing the stairs sets my heart pounding and I rely on the handrail to rest. I spend time in bed or on the sofa with hopes of 'recharging' enough to complete a basic household task.

When I'm forced to be still, questions and thoughts about what lies ahead bubble up in my head. Will I get a matched unrelated donor?  Will it come through in time? 
What happens if the myelofibrosis progresses too fast to acute leukemia before a donor is found? 
What kinds of complications could happen during transplant process?  
My organs have endured a lot of stress the last 6 years with the blood clots and medications. Will I be strong enough to over come the challenges? 
How will my marriage change? What about Alex's senior year in high school ~ how will I keep track of him? What about Katrina's first year in college?  I've seen enough movies on the Hallmark channel to know that mom's illness can reek havoc on kids' academic performance.

These thoughts remind me that control is truly an illusion, albeit one of my favorites. The feelings of vulnerability and powerlessness over my future stir an inner rage. It is not my happy place. I've read enough pop-psychology books to know that anger is a mask for fear. No amount of scenario planning can sooth the ever-present frustration that lies beneath a seemingly smooth, confident veneer.

It seems that I can accept the uncertainties when I'm busy. I comfort myself by doing what I can to help others, be present with my family and friends. I embrace my positive outlook and an inexplicable but real sense of peace that everything will work out for the best. 

Wednesday, June 12, 2013

The Call and The Twitter


Yesterday afternoon I received "The Call" we've been hoping for. I had to pull the car off the road so I could concentrate. It was my stem cell transplant coordinator. She said that there are 2 perfect matching donors (10 for 10 match of HLA tissue sequence) for me. One of them is bound to come through for me so we can transplant next month (July). We should have confirmation and a scheduled date sometime next week.  
Of course, nothing is final until it's final.  But we are over the moon happy with anticipation.


On my way home, I managed to reach Robert, my mom, my dad, and my siblings by phone to share the news. When I returned home, I gathered Katrina and Alexander to let them know. I then told some dear friends. 
I was too excited yet too tired to cook, so the kids and I went out to dinner. 

Before we place our food order, their phones began vibrating and beeping like they were about to explode.
The kids were trying to ignore their phones and focus on me. Between their furtive glances and the sounds and vibrations from the phones, my curiosity got the best of me.  "What is going on with your phones? Go ahead and check them," I said.  

"It's Twitter."
"And Instagram."
"Your news is getting re-tweeted all over the place, mom!"

Just then, my phone started dinging with text messages.. Congratulations from friends who learned from their kids that I've got a match.

My head started spinning. How could my news be tweet-worthy?

I confess to be in a bit of shock and quite tired. Here is how I can answer the questions most of you have.


Q:     What do you now about your donor?
A:     I only know that this person has a big heart for others and our HLA sequence is a great match.  We will be anonymous to one another for a year. I don't know gender, age, ethnic background, or where this person lives.

Q:     When will the transplant occur? Does the donor come to Atlanta?
A:     I should know the transplant date and prep schedule next week.  The MUD (Matched Unrelated Donor) Coordinator at Northside Hospital will coordinate with the marrow donor registry with whom the donor registered (e.g., Be the Match or Delete Blood Cancer). The registry staff will communicate with the donor to determine timing and logistics and report back to the MUD Coordinator. The MUD Coordinator shares the info with my Transplant Coordinator who gives me the transplant schedule and plan.

My hero (aka donor) doesn't leave his/her community. The donor will get a daily injection of Neupogen (filgrastim) to stimulate stem cell production for several days before the stem cells are collected from the bloodstream. 
When the blood stem cells are collected, they are shipped to Atlanta immediately so I can receive them via transfusion the next day.

Q:    What happens between now and the transplant?
A:    I'm told my primary job is to remain healthy ~ no colds, viruses, infections. I need to learn to count backwards, too. 

- 3 Weeks:  I will undergo a series of tests to check the condition and health of all my major systems. 
- 2 Weeks:  Meet with all medical personnel to go over test results, my care plan, what to expect, etc. 
- 1 Week:  Prepare for Transplant Day with daily chemotherapy.
                My donor receives Neupogen shots to stimulate stem cell production
                Day -1:  Donor's stem cells are collected by apheresis; stem cells shipped to  Northside Hospital.

Day Zero:  Transplant Day (also known as my "Re-birth day") 
                 I receive my donor's stem cells by transfusion.

When I get the official transplant date, you can be sure I will be filling my calendar with lots of "to do's" ~ mostly around getting Katrina ready for college, Alex and his college applications, scheduling bills, etc.

Also, I'm setting up a Caring Bridge page so we can keep anyone interested up on the latest in my big adventure.

I need a nap and a transfusion, so that's it for now.

Sunday, June 02, 2013

Beating the Odds Starts with a Swab

When I began this journey 3 months ago, I was pretty confident that one of my four siblings would be my match (a 10 for 10 match of HLA-types; it's not about matching blood type).  Each had a 25% chance, so wouldn't 1 of the 4 have to match?   That's not how probabilities work, I learned. 

I figured the Northern European ancestry on my mom's side would ensure several potential donors. Apparently our Greek ancestry is a bit more diverse than we thought.  

My family and friends immediately jumped on my challenge, determined to find me a donor. They have already run nine (9) Bone Marrow Donor registry drives ~ in Houston, Tempe, Grand Rapids, and four here in Georgia.

My mom even brought donor registry kits to her retirement party yesterday in Dallas ~ I believe she swabbed 32 new willing donors! 

556 new healthy people have joined the 

National Marrow Donor Registry 

because of these efforts!

Need for Marrow Donor Goes 'Viral'

My plight has hit social media!  While I've been public about my journey, I struggle with asking for help for myself ~ especially from perfect strangers.  

Friends convinced me that a Facebook page would help promote awareness and organize volunteers. I've seen similar pages (Genny's Hope Foundation and Help Nalini Now) that share a common goal:  to increase the number and diversity of healthy people registered to be bone marrow/blood stem cell donors. 

So now we have "Marina Needs Us" to promote the marrow donor drives and blood drives that are organized in my honor across the country. We'll see how helpful it is and how it changes organically as conditions change.

Donor drives have been done in partnership with AHEPA, Be the Match, and DKMS Delete Blood Cancer.  All the donor information feeds into the National Marrow Donor Program. 

Some use SignUpGenius, a free on-line volunteer organization site for community organizations to post volunteer opportunities. We learn as we go.

Goal #1:   Register 1,080 New Potential Donors 

Since I always work best with a goal, I set my sights on adding 1,080 donors to the registry. Why 1,080?  Because 1 in 540 people on the registry end up donating their blood stem cells to a patient like me, I'd like to pay it forward to cover a matching donor for me and one more patient. 

I continue to be humbled when I see neighbors, friends, friends of friends, and parents & siblings of my kids' friends volunteering to work these donor drives, get swabbed, and talk it up in support of our efforts to increase the donor pool.  

Helpful hint:  students can earn volunteer / community service hours for their assistance with these efforts.

Goal #2:    Dispel the Myths

I readily admit that I didn't know anything about bone marrow transplants until I met people with various MPNs (myeloproliferative neoplasms) that progressed to burn-out the bone marrow. 
What's worse that no information?  BAD information!

A Friend In Deed for a Friend In Need
None of this happens in a vacuum, and I certainly have not done much to make these drives happen.
Lynn Agnes, fearless friend
I must take a moment to openly thank Lynn Agnes for taking on this challenge of finding my match. We haven't seen each other in quite some time, yet Lynn showed up to help with our big drive at Peachtree Ridge High School on May 10th. Lynn researched my illness, the transplant process, and how marrow donor matching is done. During the drive at my kids' high school, she began brainstorming and calling people to schedule more drives.

Lynn secured several drive locations in a matter of days:  Gwinnett Chamber of Commerce, TPC Sugarloaf Country Club, Gwinnett Medical Center, Gwinnett Technical College, downtown Duluth (Georgia), and the Annunciation Greek Orthodox Church in Atlanta.  And she has more activities in the works. 

Lynn inspires me and my family with her bold tenacity. She is teaching my kids how to take action when there is an urgent need.  We know that her efforts will save lives.
Thank you, Lynn!