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!

The Outs and Ins of MPNs

My last phlebotomy was on June 29, 2012. It took about 15 minutes to withdraw 500 ml of that RBC-rich blood from my body. Now, almost eight months later, I'm receiving my first transfusion of packed red blood cells.  What a ride this is!

When my hemoglobin (hgb) and hematocrit (hct) was not stabilized with Hydrea last summer, I needed a couple of phlebotomies to get me back in the zone. Since last fall, these counts have slowly but steadily fallen. 

Now, with Hgb at 8.2 and Hct at 27.4, I can't walk up the stairs without my heart pounding. I get light-headed and dizzy when I walk or stand for much time around the house. (You know something is not right when you find yourself looking for things to lean against as you move around. Similar to when I was pregnant and had to scope out the nearest restrooms)

One of the great take-aways from the Joyce Niblack MPN Patient Conference held in Arizona this month was encouragement from experts like Susan LeClair, PhD and Ruben Mesa, MD that blood counts and ranges are not absolutes for every patient. We are encouraged to pay attention to our individual symptoms and let our doctors know.

This gave me the confidence to let my local hematologist know that I need a blood transfusion now (with at Hgb of 8.2)  -- I don't want to wait for it to reach 8.0 (the standard accepted level to begin transfusions). I've been very fatigued with it in the 9's for the last few weeks; when it fell lower this week, I had to cry "uncle."


Additionally, I plan to start taking Jakafi when it's approved by my insurance. Since blood counts often drop during the first few weeks, I want to start from a position of strength.


So yesterday I went to the hospital's outpatient infusion center to get my blood typed and cross-matched. Here is a good explanation of the blood transfusion process.

My "energy pack"

Today I'm getting 2 units of B negative packed red blood cells from a donor in Riverdale, Georgia. A nurse came in with a cooler that contained what I'm now calling my "energy pack." After verifying the info on the blood bag with the info on my wristband, the transfusion commenced.

As I sit here and write this while someone's generous gift is flowing into my veins, I feel quite humble. Someone took time (and energy) from his/her daily routine to help a stranger. It is an unconditional gift. No questions or judgments about my race, religion, age, gender, political views, sexual orientation, family situation, employment status, or the reason I need the blood. A pure gift of care and concern for others. 

One of the nurses explained that this donor helped me with the red cells, and another with the white cells, and a third person with the platelets. How is that for leveraging a donation? 

My mom was an ICU nurse while I was growing up and we learned how important blood donation is to saving lives. I started donating blood when I was 18 and in college. Because "B negative" is not a common blood type, the blood bank would sometimes call me in. I continued donating until my early 30's and quit due to chronic anemia. Later, in my 40's, I became symptomatic and diagnosed with Polycythemia Vera. I've been assured that my blood was good and safe for donation in my early years; the JAK2 mutation that triggered the PV occurred later. 

I have no idea how much blood I donated in my healthy years. But I'm sure it not enough to cover the blood I will be receiving over the coming months. Some have told me that they would like to donate to someone they know. I remind them that should they ever need blood (due to illness or accident), the donor who saves them will likely be someone unknown to them. 

Blood donation is the ultimate random act of kindness. I receive this gift of kindness and life with love and gratitude. It is yet another reminder of the interdependent web of life. 

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

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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 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)