Save a Life. Why Would You Not?

Saturday September 14^th, I attended a fundraiser hosted by Julian’s Dinosaur Guild (http://www.juliansguild.org/julian/home.html).  The guild was started in honor of Julian Blackwell, who was diagnosed with Aplastic Anemia at the age of 3, in 2010, and underwent a bone marrow transplant in 2011.

The event was emceed by Keith Eldridge of Seattle’s KOMO 4 News, and donations raised help fund Dr. Akiko Shimamura’s research of bone marrow failure diseases at the Fred Hutchinson Cancer Research Center and Seattle Children’s Hospital.

Dr. Shimamura is a leading expert in Aplastic Anemia and spoke at the event about some exciting work being done in her lab right now.  One particular study has identified gene markers that can help predict up to 10% of patients who will likely not respond to ATG treatment.  I imagine that, in the future, this may help develop a routine screening protocol that can spare “non-responders” from a harsh round of ATG treatment, and perhaps direct earlier efforts toward bone marrow transplantation or participation in clinical trials.

At the event, I had the opportunity to introduce myself to Dr. Shimamura and chat with her briefly about Ryan.  She reassured me that there is still time for the ATG to work, but that she would be happy to see him at her clinic for a consultation.

Last week, Ryan’s doctor determined that we are at the point where he recommends a consultation with Dr. Shimamura.  Since we are not quite to the “3-month post-ATG” benchmark when initial evaluation normally occurs, I simply asked the doctor if he wished that he were seeing more improvement in Ryan’s counts at 2 ½ months in.  He said that he has had some patients who were “late responders”, so he is still hopeful, but that he would like to have seen Ryan not so dependent on transfusions by now.  I asked him if another bone marrow biopsy would be scheduled soon to see how things are looking. Unfortunately, he said that it didn't make sense to do one right now, because without much cell growth (i.e. increases in counts), he expects that Ryan’s biopsy would look the same as it did in June.  Enough said.  With that, our conversation shifted back to setting up the referral to Dr. Shimamura, transferring bone marrow biopsy slides to her, etc.

Back to the fundraiser for Julian’s Dinosaur Guild…   The event also paid tribute to Julian’s two year bone marrow “transplantversary”!  It was encouraging to see Julian running around full of energy and hear that there were other bone marrow failure survivor’s in the room, including another child who has successfully been treated to remission with ATG.  However, the most touching moment in the evening came when the audience was introduced to Andrew, Julian’s bone marrow donor.

Two years earlier, Andrew had been in the middle of planning his wedding when he received the call asking him if he would be willing to donate.  Just days prior to the fundraiser, Julian and Andrew met in person for the first time.  Onstage, Julian’s family presented Andrew with a framed photo of them both together, with messages of thanks filling the matting around the image.  In that moment, Keith Eldridge asked Andrew why he had made the decision to donate.  An emotional Andrew put his hand on Julian’s shoulder, then looked up at the audience and simply asked “Why would you not?”

Would you be willing to save a life? Did you know that you can easily register online as a potential bone marrow donor?  A kit will be sent to you and you simply swab your cheek and return it – no initial blood draw is needed.  Chances are, you will never be contacted and have to decide.  However, even with over 9.5 million people already in the registry, some people requiring a bone marrow transplant will only have one match (or no match at all).  What if you are the one in 9.5 million match needed to save a life?  Would you donate? Why would you not?

To register, visit www.bethematch.org  Please, don’t just think about it as something nice you might do someday.  Take a few minutes right now to sign up online.  There might already be a patient out there that may not have a “someday” without the few minutes it takes you to register today.

Two Months Post ATG: Do we have a Partial Response to Treatment? Maybe… or Maybe Not…

Years ago, when I was a 20-something bachelorette, I was microwaving a frozen burrito when I received a phone call that would take my breath away and leave me feeling dazed and sick to my stomach.  It was the feeling of getting really bad news, and it was the first time I had ever received this type of bad news.  A friend had been killed in a car accident overnight.

I remember dropping the burrito, plate and all, into the garbage and then wandering aimlessly around the house in the hours that followed, until I finally crawled into bed and spent a restless night trying to sleep, but then continually waking to thoughts of this person and the tragic events.

Throughout the years, I have experienced this same “bad news” feeling at times.  I have felt it when learning of the death of my grandparents, the death of my uncle, the cancer diagnoses’ of friends, layoffs at work, the death of a coworker last year, and of course Ryan's diagnosis of Aplastic Anemia.  I think this feeling might be related to the temporary “fight or flight” response our bodies have to danger (or a bad situation).  However, I have been having this feeling on a regular basis lately.

For the first two months after Ryan’s diagnosis, an adrenaline rush of sorts kicked in.  The first month, I was on a mission to understand as much as I could about his condition and prognosis.  I read everything I could find on the internet about Aplastic Anemia.  I read websites, blogs, medical journals, information on clinical trials, researched hospitals throughout the country, researched doctors, and watched pre-recorded webinars.  The list goes on…

I also conducted many fruitless searches on anything that I could think may have been a potential cause of his condition.  My theories ranged from it being something in his sandbox, off-gassing from the building material in the new home we moved into when he was 9 months old, the Zantac his doctor prescribed him for acid reflux when he was an infant, something from a plant outside that he may have put in his mouth, something he was exposed to at daycare, or something I was exposed to when I was pregnant.  I was looking for something to help me make sense of it.  I now realize it will never make sense to me.

The second month, I read every book that was a patient’s story on Amazon:

Anatomy of a Miracle: Drew’s Story by Jennifer M. D’auteuil

Drew had an unsuccessful round of ATG and then received a Bone Marrow Transplant from a non-related donor.  He has had complications along the way, including a near-fatal lung infection, but is doing well today.

Danielle’s Story: A Daughter’s Battle with Aplastic Anemia by Shawn Williams

A heartbreaking story about 11-year old Danielle’s battle with Aplastic Anemia and her death due to complications of the disease.

Out of Nowhere by Esther M.R. Hougham

Esther recovered from Aplastic Anemia as a result of a sibling matched bone marrow transplant.

Blood Counts: My Triumphant Battle over Aplastic Anemia by Asa J. McMahon

Asa recovered from Aplastic Anemia as a result of a sibling matched bone marrow transplant.

I have since been in contact with Jennifer M. D’auteuil , the author of Anatomy of a Miracle: Drew’s Story, and have joined her Facebook page “BAND OF MOTHERS in the fight against Aplastic Anemia”.  I have enjoyed Jennifer’s positive attitude and find that she is a daily cheerleader of sorts for AA patients and their families.  I encourage you to buy her book and check out her Facebook page.

I have also continued to follow the blogs of several AA patients, including a blog about Nora Getchell, a young girl in New York.  I found the blog, written by her mother, the night Ryan started his ATG treatment in the hospital.

Nora is in her third year battling AA.  She initially received, ATG.  It looked like she was responding for a while, but then her counts fell off again.  She then received a bone marrow transplant last year, again things were looking good for a while, but in recent months the transplant failed.  The donor provided additional stem cells and Nora was given a “boost” last month.  She is still in the hospital, has developed several near fatal infections, and the graft of additional stem cells has officially failed.  They are now trying to manage infections and coordinate a second bone marrow transplant, from a new donor, to occur within the next 2-6 weeks.  My thoughts and prayers go out to Nora and her family.

While it is hard for me to read at times, Nora’s blog has given me a realistic idea of what a rollercoaster rider this disease can be.  Here is a link to Nora’s blog if you would like to follow her journey: http://norasstars.wordpress.com/

During Ryan’s second month post-ATG, I also analyzed every aspect of his lab results from every blood draw on record.  I created spreadsheets to track every result and corresponding graphs to look for trends and potential improvements in his counts.  I combed through stories of other patients who had ATG and, where numbers were mentioned, I compared them to Ryan’s for any potential clues regarding his progress.

My theory so far is that he is having a slight “transfusion dependent partial response”. That isn't great.  His neutrophil count has made some progress and seems to be hovering around 700 (still about half of normal).  He has also had a few spikes in his platelet count, which seem to be followed by his T cells aggressively attacking them at a rate of around 11,000 for the first few days, and then leveling off at a “burn rate” of 3500 a day.  When he was first diagnosed, he seemed to be burning through about 3500 a day, so this is where I get concerned that we haven’t made much progress.  While it is encouraging to see the spikes in his counts at times, it is just as discouraging that his T cells seem to kick into overdrive once they realize his counts have gone up (rogue T Cells are our enemy and are the attackers of Ryan’s healthy cells).  I have included updated charts of Ryan’s counts below.

Now we are entering the third month post-ATG.  Upon completion of this month, we will have a good idea of whether Ryan is responding to treatment.  I have completed my research, I have read the books, I have analyzed the data. We have an appointment Monday and Ryan’s blood will be drawn to HLA tissue type match him for a potential bone marrow transplant.  Unless his counts drastically improve this month, it is likely the doctors will ask us to start considering a transplant, and they will start looking for a potential donor.

In the meantime, I am to the point where I feel like there isn't much I can do to help.  I can pray that there is a perfect match donor out there if Ryan needs one.  I can make sure Ryan has his medication twice a day.  I can monitor his temperature for potential infections.  I can keep him away from large public gatherings. I can try to help him deal with the anger his medication seems to be giving him.  I can pray for a miracle. I can also love him more than he will ever understand.  Other than that, I am left to deal with my constant “bad feeling” and look for positive ways to channel my nervous energy and deal with all of this.