Monday, April 15, 2013


National Organ and Tissue Donation Awareness Week
April 21 - 28, 2013

In Ontario, National Organ and Tissue Donation Awareness Week (NOTDAW) runs from April 21 - 28, 2013.

However,  Trillium Gift of Life Network has designated April as: “ Month

Now is a great time to help increase awareness for organ and tissue donor registration and help save more lives.

There are hundreds of diseases that could affect an organ to the point that a transplant is required. Here is a list of organs that can be donated:

Organ Donation

How did we get here?  Why did we decide to list Alexa?

I had been looking  into liver transplant to cure a Urea Cycle Disorder for some time.  Up until Alexa was about 18 months, she was so stable.  In fact, I secretly would wonder to myself, if Alexa has been misdiagnosed--I know this is ridiculous--Alexa HAD confirmed DNA testing done that showed she had two mutated genes; Chris and I both had DNA testing done and BOTH of us had 1 copy of the mutated gene.  Not to mention that she was slipping into a hyperammonemia coma when she was diagnosed.  But, she had done so well up until that point, that I allowed myself to hope that  maybe she had a "less affected" version of Citrullinemia.  I found out the hard way how untrue this was.

When Alexa had to start transitioning from baby food/finger foods to more toddler food, we encountered many problems.  Food wise--to say she was not interested is an understatement--THIS GIRL HATED EATING--We struggled on a daily basis with getting her to eat her "daily protein and calorie" intake.  The only reason she ate, was to make mommy and daddy happy.....
Even so, her "normal" ammonia numbers were slowly increasing.....It was not uncommon for her "normal" to run in the 60 - 80's...remember now, normal is below 50; some would even say below 35.  So running in the 60, 70 or 80's on a daily basis, when she was healthy and well,  was concerning to me.
Throw in a small illness, even the common cold.and those numbers would skyrocket.  Any stomach flu was a guaranteed admission and one more risk of damage to my baby's brain.
In November 2011, Alexa had a VERY rough go of things.  She was inundated with coughs,colds, ear infection, etc.  In a six week span, we were admitted 3 times.  The first two for 4 days each.  At each of these admissions, her ammonia was between 150 - 180.  Emergency medication intravenously was started each time.  On one of these, the next morning, we were all SHOCKED, that her ammonia went even higher--IT WAS 220!  This was the first time, her ammonia went up after emergency rescue meds were administered--THIS SCARED ME!  SCARED ME SO MUCH!  We did get the ammonia under control quickly--but her body's inability to respond as well to the medication as it used to, is one of the reasons I started to get more opinions on liver transplant.
And then we had to watch Alexa "come down" after an episode of hyperammonemia.  After each crisis, it took my daughter 3 to 4 days to get back to normal.  She could not settle down at all, she could not focus, she was extremely hyper and could not sleep well at all...Clearly these episodes were doing more damage than we realized.

And then there were other factors that came up that we were never concerned with before, like her citrulline levels.  Citrulline is an amino acid, that plays a key role in the Urea Cycle.  It is the marker that alerted the newborn screening department that something was wrong when she was just 9 days old.  At this point, there is NO EVIDENCE TO CONFIRM that elevated citrulline levels cause any damage to UCD children.  However, there is also no evidence to REFUTE it either.  It is my feeling that once a bit more research and progress  in brain imaging investigations(--which is currently underway), we will find out that elevated levels of citrulline does impact the brain.
(AGAIN, this is only MY opinion after doing much research and speaking to many professionals about it, in reference to Alexa ONLY) Citrulline has an upper limit of 80(according to MY laboratory).  Alexa's citrulline runs in the 3200 - 3800 range!  YES, you read that RIGHT..... THREE THOUSAND!!!! And there is absolutely nothing that can be done to lower it!  I have asked a million times--I cringe every time I get her lab results and see her citrulline level.

This was the most excruciatingly painful decision I ever had to make.....I spoke to doctors all over north america, to parents who have had their children transplanted and had great outcomes as well as those who have had the worst imaginable outcome.  It took us 6 months to finally decide. But in April 2012 when the HSC transplant team called us and informed us that after all the testing and after meeting with all interested parties, the team was confident that transplant was the best option for Alexa, Christian and I had no hesitation--we KNEW what we HAD to do.  She was listed and the waiting game began!

It has been about a year, we have had two calls--One in August (as the back up) and one in February which we had to turn down to illness.  Every time my phone rings with a blocked caller, my heart jumps into my chest and I can barely speak.  

Here is hoping that her PERFECT liver arrives during organ donation month!

And just in case, I have not said it enough before.....

You can register to become an organ donor in Ontario here:  and help save the life of someone wonderful like Alexa!

1 comment:

  1. Praying for your perfect liver. Love you guys, xoxo