Life Saving Eagle Scout Project: Building the Marrow Donor Registry

Tween Saves Lives

When the time came for my nephew Patrick to design a project for his Eagle Scout, he decided to take on something that is usually done by adults. He organized a registry drive to get healthy adults to join the National Marrow Donor Registry.  Did I mention he is 12 years old?

Creative Collaboration

Patrick worked with DKMS Delete Blood Cancer, the nonprofit registry that provided the swab kits and educational materials. 

When considering ways to get the most donors possible, he looked to see what big events were coming up that would already bring people together. When he saw that the Full Moon Four-Miler race was coming up, he contacted race officials and asked if he could run the swab drive during the race. They readily agreed. 
"I figured healthy people run, so that would be a good event." Patrick told me. 

Then Patrick approached local businesses to promote the event. One man told him he would try to stop by the event, but he had a busy schedule. Patrick said, "I've got swab kits in my mom's car. Do you want to join now?"  The man said yes and Patrick got him registered.

He designed flyers and distributed them all over Waterford. He and his family talked it up at ball games, at the park, and anywhere people with healthy stem cells would be.

Flyers like these were distributed throughout the town, at ball games, and to neighbors and friends.




Patrick recruited volunteers from his Scout troop, neighborhood, and ball team  (including his parents and 3 siblings) and they set off for a big day of public education and donor recruitment. 

"Here's a bracelet! Go to the blue tent and save a life!"

The kids walked around with signs and handed out red rubber "delete blood cancer" bracelets and guided people to the swab tent. Who can say no to adorable youth?

Runners in the race and spectators stopped by the tent to learn more about marrow/stem cell donation.  A few people shared stories of loved ones who were saved by donors.  

The winner of the race joined the Registry after the awards ceremony. He posed with the young drive organizer here.



Donors were invited to sign the "Heroes On Call" poster. 

I'm told that 87 people joined the registry at the Full Moon race and another dozen or so joined before and after the event.  

And hundreds more learned about or were reminded of their power to save lives. 

Patrick says he will join the registry when he turns 18. But he's not waiting to start saving lives! 

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. 

The Call and The Twitter

The CALL

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.

The TWITTER

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.

FAQs

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.

A Port for Weary Veins

Port of Pireaus, Greece

When I think of a Port, I first imagine a place near land for boats to find shelter from rough seas or load and off-load goods and passengers.  

The second thing I imagine is a glass filled with a dark, heavy, rich dessert wine made in Portugal.

This coming Wednesday, I will experience another kind of port - up close and personal. I'm having a port installed in my chest. But it is for loading something other than libations.

When I went in for my second transfusion of Red Blood Cells (RBCs), the nurses told me it was time to get a port installed. My good veins for IVs are shot due to phlebotomies and Remicade infusions over the last few years. There is scar tissue and some veins have flattened out. Other veins are covered by lipomas (benign fatty tumors) on my arms and legs (fat isn't limited to cellulite, my friends!).  

The port installation is an out-patient procedure with local anesthetic. I do hope they will give me something to calm my nerves, because watching people run tubing through my chest will trigger some of my natural anxiety.

IV Educational Moment:
In preparing for this, I've learned that there are three types of intra-venous (IV) catheter mechanisms: Ports, PICCS, and Lines. Each is used for different medical purposes.

Ports:  Ports are funky-looking things that are implanted just below the skin's surface on the upper chest. A tube connected to the port is fed through a central vein towards the heart. Ports can stay in ones body for years if necessary. Ports are good for chemotherapy, transfusions, and delivering medication and other necessities. A special needle is required to connect to the port. Ports have lower infection risk because there are fewer points of entry to the body than the tubes from PICCs and Lines.

How Does A Port Work?


PICCs:   Peripherally Inserted Central Catheter 
I had one of these when I had the whopper hospitalization in 2007. They can draw blood, give medication and liquid nutrition without sticking me every time. A purple tube went from my right arm into a vein that led to my heart. I was unconscious when it was installed, but I do remember that when the nurse pulled it out it was no big deal.
PICC lines are not used for chemotherapy. 

Lines:
There are 2 types of lines, tunneled and not tunneled. Tunneled catheters / lines are used for chemo, blood draws, delivery of nutrition, medication, etc. They are not for long term use.

An on-line friend shared this brief summary of these 3 types of IV catheter approaches.


I'll post a photo or two of Marina's Port (sounds nautical, doesn't it?!) on Thursday or Friday.

Blood Stem Cell Transplants in the 21st Century

When I first heard about bone marrow transplants, I envisioned the donor getting drilled in the hips for bone marrow extraction (like the bone marrow biopsy and aspiration) and then a surgeon drilling into my large bones to implant the donor marrow. I am happy to say that I was way wrong!


In most cases, blood stem cells are harvested from the donor through a process called Apheresis. It is like donating plasma (some of you may remember college students doing this for weekend money).   

Over the years, the stem cells that get transplanted have many names:  

bone marrow, stem cells, hematopoietic stem cells, peripheral blood stem cells, and pluripotent stem cells. These are the stem cells that become red blood cells, white blood cells, and platelets. 

What's Involved in the Transplant Process

For me:    Patient and her caregiver must live near the transplant center for 6 months.
The week leading up to the transplant, I get blasted with chemo that will kill all the cells in my bone marrow (among other things) and also receive immune-suppressants to prevent my body from rejecting the stem cells.  I’ll lose my hair, get mouth sores, feel like crap…

For my donor: The week leading up to the transplant, my donor will have labs updated (to ensure no new infections) and get a growth hormone shot each day for 5 days.  Then will have blood drawn out of one arm; the stem cells are separated in a bag for me, and the blood is returned to the donor in the other arm.  Generally takes 4-6 hours. S/he will likely feel fatigued for the next couple of days. 

For me: I will receive the donor’s stem cells by IV.  Then I sit and wait for those brave stem cells to engraft in my bones and begin producing blood (takes 2-3 weeks).
I’ll also continue to receive anti-rejection medications for the first 6 months post-transplant.

At day 100: a bone marrow biopsy will be done to see if the transplant is successful.

Major Risks of SCT

Transplants for secondary Myelofibrosis are very different than transplants for other leukemias and much more intense than organ transplants.

The risks are highest in the first year of transplant.
1. Risk of Graft Vs. Host Disease: 30-50% with sibling donor.  Of those 20% will not be responsive to steroids (aka steroid refactory).  MF patients have more GVHD than other leukemia patients.
2. Risk of Death: 15-20% within the first year.  Organ failure is most common cause.
3. Relapse Rate: 20% within the first 1-3 years
4. Graft vs. MF effect:  treated with donor lymphocyte transfusions

These are odds I'm confident I'll beat.

When a Match = Life

I see lots of ads for matching companies -- match.com, e-harmony, christian singles... 
They sell the dream of happiness for life by promising to find your compatible, perfect match.

Here is a way to help someone (like me) find a perfect match that saves life -- and there are no monthly membership fees:   Bone Marrow/Stem Cell Donation 

First Fact:
A bone marrow or stem cell donor is determined by HLA (Human Leucocyte Antigens), NOT blood type.

Why match the HLA?
HLAs are protein markers which sit on all our cells. The immune system recognizes them and therefore does not attack them as foreign or infection. 

When the donor and recipient have matching HLAs, the likelihood of stem cell rejection or the new stem cells attacking the host body (Graft vs. Host Disease) is decreased. 

The ultimate match is when the patient and donor share 10 out of 10 HLAs. Most transplant centers require a 10 out of 10 or 9 out of 10 match for a standard transplant.

"Haploid match" is when the patient and donor have half of the HLAs in common. Biological parents and children of the patient are haploid matches. 
These transplants can also be successful with special care to decrease risk of Graft Vs. Host Disease (GVHD).

Get more info here:  HLA Match education

HLA Match Priorities

The priorities for my HLA match are as follows:

1.  Full-match sibling  

2.  Full-match Unrelated Donor (10 out of 10)

3.  Partially-matched family member 

4.  Cord blood

All four of my siblings did the cheek swab test through bonemarrowtest.com, as I did.
It cost $175 each, yet we got the info quicker than waiting for the doctor referrals and insurance approvals.

There is a 25% chance that a biological sibling is a perfect match. With 4 siblings, we figured that we had a good chance. One brother and one sister are haploid (half) matches with me and full matches to each other; the other two are identical matches to each other.

The doctor said that my son, Alexander, would be a haploid-match with me, and his stem cells would be preferred to my siblings because of Alex's youth. 

They also prefer younger male donor to younger female donor, so Katrina is excluded if Alex is willing to help his mom. In light of his upcoming car repair bill, I think he can be persuaded (wink, wink... both kids asked if they could be donors when this first came up).

We are now in the process to search for a 10/10 Matched Unrelated Donor (aka MUD) through the National Marrow Donor Program. 

The process takes 2-3 months, and the doctor believes that we will find a donor.

If you are between 18 and 44 years of age and interested in being a Bone Marrow/Stem Cell donor, check out these options:

bethematch.org

deletebloodcancer.org

They both contribute to the national and international donor databases. 

In the circle of life, I'm reminded that we receive what we put out in the world. Donation of bone marrow/stem cells, blood, plasma, and organs are the ultimate Random Acts of Kindness. Give with love and know that your gifts are received by the patients (and their families) with love and immense gratitude.

peace & love,

Marina

How We Chose the Transplant Center for Me

After an absolutely wonderful escape to Sicily, Robert and I returned home ready to tackle the next big adventure.  Where should I go for the stem cell transplant?  Who will be my donor?

Robert, Mom, and I met with Dr. Lawrence Morris of Bone and Marrow Transplant Group of Georgia in Atlanta. This group is under contract with our health insurance provider.

Here's what we learned:

About the Blood and Marrow Transplant Group & Facilities at Northside

Dr. Lawrence Morris came into our appointment well-versed in my condition and situation. Right off the bat he noted that I must be tired of hearing doctors say that I have an unusual and complicated case. 

The transplant group did 186 transplants in 2012 and their success rate exceeds the "Confidence Interval" (the expected success rate is determined for each center after controlling for the diseases transplanted, age of patients, co-morbidities, etc.). He said only 10 centers in the US exceeded their confidence interval. 

This center is well-within the top 10% of transplant centers in terms of scale. It's not a mega-center (like MDAnderson, City of Hope, Hutchinson, or Sloan-Kettering...)

Transplant Protocol
The transplant process would be managed mostly on an out-patient basis. This is very different from the Mayo process (5-6 weeks in the hospital). The chemo and the transplant would take place in their out-patient transfusion center. About one week after the transplant, I'd be admitted to the Transplant floor at Northside for 10-20 days, depending on how the engraftment is going, my vitals, any signs of rejection, etc. After release from the hospital, I will still go to the out-patient center daily and then every other day for check-ups. He said that at any point in the process, I could be admitted immediately to the hospital's transplant unit should that be necessary. 

They have better outcomes with the out-patient process, and I'd rather barf in my own toilet anyway. 

About My Situation

Dr. Morris explained the reasons why a stem cell transplant (SCT) is appropriate for me and the additional risks that I face. The Behcet's auto-immune issue is a bit of a wild-card. The portal vein thrombosis is also an issue; since my body has created new varices to deliver blood from the liver, we need to look and see if a similar thing has occurred in the esophagus (treatment would be required).

Transplants due to Myelofibrosis

He also identified the issues specific to transplants due to myelofibrosis: there is additional stress on the body due to years of medications, fibrosis in the bone marrow, and concern for stress on spleen, liver, heart, and lungs. That was a huge piece of helpful information for me because Dr. Fauble at Mayo said that there are unique post-transplant issues for MF patients but didn't specify what they are.

Given the stress on my body, he expects that the preparation regimen will be something between a full-in ablative chemo and the reduced intensity protocol.

Decisions, Decisions

After our Mayo and Northside appointments, Mom, Robert and I talked about the big, burning question: where to have the transplant. We want to go to the center that will have the best chance for a favorable outcome. 

The primary considerations: quality of care/experience;  quality of life for me & family;  insurance/financial considerations.

In the end, we are in agreement to have the transplant here in Atlanta.

Here's our rationale: 

Quality of Care/Experience:

• We looked at outcomes data and experience at Northside, Mayo-Scottsdale, MDAnderson, and others. Since there are very few transplants for MF, the disease specific data is hard to get. None of them do more than 2-3 per year for myelofibrosis. 
• Northside does enough transplants and participates in research; it's not a side business. It also does a great job - the docs have outcomes that exceed expectations and the transplant unit is state-of-the art.  
• They know the risks and issues associated with my particular situation. 

I don't believe we are compromising the care by staying here.  This group would be my local post-transplant support regardless of where the procedure is done. 

Quality of Life: 

• I really want to be close to Alexander during his Senior year in college. Whether I'm in the hospital, an apartment near the hospital or at home, he will be able to come visit regularly.
• Robert can do his job and he won't have to choose whether to stay with Alex on weekends or fly to see me somewhere. 
• Staying local will also help me stay out of the depression zone because I won't feel as isolated from my loved ones. I do better keeping my game face on when I've got good reasons to do so. 
• The out-patient protocol appeals to me a great deal. I recall going stir-crazy the last few days of my 2 week hospital stay back in 2007. Being confined for weeks is not appealing to me at all.    
• We have many friends who are willing to form a 'care team' to help out as the journey continues (it will be a marathon, not a spring). 

Insurance/Financial:

• I was initially inclined to go to Mayo-Scottsdale (as you all know). After looking at the data and meeting with the Northside doc, we'd have an uphill battle to get insurance to pay out of network.  I don't think we can make a compelling case that I'll get superior care elsewhere based on the data. 
• I don't want the added stress of insurance and financial burdens.
• Kaiser Permanente has excellent coverage if we do the procedure here. 

I'm glad we got the diagnosis and recommendations from Mayo first. Not only did it give us time for the info to settle into our minds and hearts, it gave me time to do more research, both data and anecdotal, before meeting with the folks that our insurance will cover.

We ran through several scenarios and the biggest question: If things go poorly here at Northside, is there going to be any shoulda/woulda/coulda discussions? 

There really isn't a stand-out, slam dunk transplant center that we dismissed. So we are in agreement.

So on we go... no regrets!

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):  

Asians for Miracle Marrow Matches

BlackBoneMarrow

Gift of Life Bone Marrow Foundation

#  #  #

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 www.bethematch.org
• 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)