My Journal
June 21, 2005… This will be my first journal entry on Canon's website. I'm
finding it both tremendously helpful to have this outlet but also very difficult to
start the process of begging for prayers for my child once again. Some of you,
no doubt, know exactly what I mean because when you visited this website and
clicked on my journal, your heart was also reminded of Amberly and all the many
times you checked her progress and my sanity online in the summer of 2001.
For those of who didn't know us then, our first child was a daughter born 16
weeks premature, July 12, 2001. Amberly Drew spent 22 days on this earth and
for most of those days, we had a website a lot like this that my dad created and
maintained where I reported details, requested prayers, cried out in anger, and
just opened up to family and friends. There was also, on that website, a
guestbook where those who visited could leave a note, a prayer, a verse,
anything. If I remember correctly, by the time we shut down the website, we
ended with more than 3000 visits to the site and around 500 guestbook entries
(in three weeks). There are still days that I revisit those messages from our
friends and family offering support and encouragement. All that to say… please
leave a note when you visit this website to check on Canon!
I guess I should rewind briefly and give some background information, for
those of you who don't know how this all started. Canon is now 15 months old,
and it was about six months ago that the focus of our lives shifted to his medical
condition. At his 9 month well-check with Dr. Young, our pediatrician, my only
concerns were that he was not crawling yet and was not able to bear any weight
on his legs. That day Dr. Young also commented, after prodding his abdomen
for longer than usual, that his liver and spleen seemed to be enlarged (today it is
about three times larger than it should be). From there, he had us do some lab
work called an ALT (measures liver enzymes). We discovered that his numbers
were around 250 when they should be under 40. The first possibility mentioned
was hepatitis, and we were referred to an infectious diseases specialist with
Texas Tech. That specialist, after more lab work and careful observation and
questioning about history, ruled out any infectious disease, and he and Dr.
Young began thinking it could be a metabolic disorder. We were sent to Dr.
Higgins, a pediatric GI in Lubbock, who wanted to do a liver biopsy to get to the
bottom of the question. That biopsy was done on March 2nd. About two weeks
later, we were driving to Dallas to see another doctor, this time a
genetic/metabolic specialist, when we received a call from Dr. Higgins. He told
us that the Mayo Clinic had tested the liver and made a diagnosis of Glycogen
Storage Disease Type 4, a very rare (50 cases in the world) genetic disease that
causes cirrhosis of the liver and death in early childhood. We were told that
there has been no effective treatment of this disease as far as diet or medication;
that the only option we had was a liver transplant. At that point, we were
associated with the Texas Children's Hospital liver transplant team in Houston.
Dr. Karpen, the chief pediatric hepatologist, has agreed with the diagnosis after
himself studying the liver sample and also noting symptoms like hypotonia (low
muscle tone) and developmental delay. We have since been to Houston twice,
once to meet the transplant team, and once for his pre-transplant evaluation
(blood work, EKG, ultrasound, etc.).
We have some confusing issues that have surfaced that I guess, like
everything else, we will deal with on a day-to-day basis. After the Mayo Clinic did
their test and made the GSD4 diagnosis, they sent a follow-up sample to Duke
Medical Center for the same test. Duke reported results that were "inconclusive"
and suggested we have a skin biopsy done for a more accurate confirmation of
the diagnosis. So, on April 18th, Drew took Canon to Lubbock for the skin biopsy.
About three weeks ago, we finally received results from Duke that reported his
"branching enzymes" in the normal range (English translation – not consistent
with the GSD4 that the doctors are familiar with). At this news, we were led to
wonder if we would need the transplant after all if the diagnosis was not sticking.
Dr. Higgins (Lubbock GI) consulted Dr. Karpen (Houston hepatologist) and they
are both resolved that, based on what they have seen of his liver and blood work,
he is still in need of a transplant. What we don't know at this point is the exact
diagnosis. We are currently getting opinions (well, more accurately, ASKING for
opinions) from all over the country on his test results to determine a number of
things. Duke suggested that we repeat the skin biopsy (with a sample they
already have). Essentially, we're being told that, whatever it is, it is progressively
destroying his liver, looks just like GSD4, and he is in need of a transplant. They
have extrapolated that if it's not the textbook form of GSD4, that it could be some
even more rare genetic anomaly, a mutation that randomly affected Canon. (I
have my own opinions of "randomly".) On the plus side, if it is not genetic
(meaning Drew and I aren't carriers of this disease), then there is a better chance
of our second son (due in August) not having it. If we are carriers, then our new
baby has a 25% chance of having the same liver condition.
Okay, I need to get away from the medical terminology now. Basically,
we're looking at a liver transplant that will happen within a year and hopefully,
somewhere along the way, a more concrete diagnosis. Canon goes back to
Houston for an appointment with the transplant team on July 6th. Unfortunately,
because I'll be 32 weeks pregnant and already taking precautions to prevent
preterm labor (a terbutaline pump and weekly progesterone shots), we decided it
wouldn't be smart for me to leave town, and so Drew will be a solo parent on that
trip. He's already had to do a couple of appointments in Lubbock by himself and
did great of course. But this will be the first time he's had to be by himself and
out of town overnight. He seems very confident, but it won't be easy to keep up
with Canon, luggage, car seat, meal times, baths, naps, listening to doctors, etc.
all by himself. Add waiting rooms to that equation and that's a recipe for a
meltdown for ANY parent! So, keep Drew in your prayers as that trip looms
closer.
I don't intend to make journal entries this long, so don't let this first one
make you bored and never want to come back. I just hate to leave anybody in
the dark as far as how we got to this point.
And where are we? Well, right now our pressing goal is financing a liver
transplant. We have been associated with COTA (Children's Organ Transplant
Association), who is helping us with fundraising efforts. Our goal (based on his
insurance, the type of transplant, the transplant center, and other things) is
$100,000. They think that will get us through the actual transplant and two years
of anti-rejection medication (which he has to be on for the rest of his life). If you'd
like to help with the campaign efforts, visit the "How Can I Help?" page on this
website, and God bless you!
But beyond the actual transplant and the campaign and all of that, there is
a much deeper thing transpiring. What will God show us through this? It's a
question I don't even want to ask anymore because I'm afraid of the answer. I
can clearly remember being on the phone with my mom only a few hours before
Amberly died, asking her in fear "What if God wants and what I want aren't the
same thing?" I feel the same question rising up in me all the time lately. I
frequently feel incredulous at the idea that we are facing a life or death situation
with our baby… again. How is this possibly fair? How can our family possibly
survive this? Where are you God? Recently, our preacher, Brian Mashburn,
taught a short devotional about when the Israelites, who had been delivered from
slavery, complained to God in the desert about being thirsty. He related it to his
five-year old son repeatedly telling him on a road trip that he needed food, even
inside the McDonald's! Brian said it was offensive to think that his son didn't trust
him to provide food for him as he had been doing for five years. I thought about
the times I have complained to God, sometimes in formal prayer, and sometimes
complaining ABOUT God, just to myself. That night last week of Brian's
message brought me home wondering how I make God feel when I don't trust
him. I came home, put my son to bed, and like every night, went back in to pray
over him before I go to bed myself. I leaned over Canon and watched him
sleeping peacefully, said the prayer of blessing that I've said every night, and
listened to God telling me to look at my son and know how peaceful he is. I felt
an overwhelming feeling of gratitude to God for listening to my prayer for Canon's
happiness because his days are full of joy and play and smiling. It was like God
was telling me that in the midst of all his medical trials and our stress, he is
holding Canon in perfect peace. He is protecting him from any pain now and any
knowledge of pain to come. He is allowing us this precious time to teach him
about how much he is loved. I pray that God will forgive me the times that I
doubt him or turn away in anger and provide us with reminders that he has not
left us. I know I should pray that God be glorified no matter what happens, but
I'm too scared to pray that right now. I'm afraid of his interpretation. For now, I'm
more specific. God, in four years, I don't want to be reading Canon's guestbook
messages to myself; I want him to be sitting on my lap listening and asking
questions about all the people who love us and prayed for him and the God that
heard their prayers and saved his life.
The Lord is my light and the one who saves me. I fear no one. The Lord
protects my life; I am afraid of no one. Psalm 27:1