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My Daughter – Biological Creature

riely2My 5-year-old daughter has a genetic disease that acts similar to a slow moving, incurable cancer.  It is called Hurler’s Syndrome.  There are only about 250 kids known to have the disease in the US, and roughly 1 in every 500,000 births.  She had a bone marrow transplant in November 2010 to try to slow the effects of the disease.  Honestly though, we aren’t totally sure what the benefits of the transplant will be.

Through the transplant process, Riley had approximately a 15% mortality risk.  Significant to say the least.  Now that we are over 1 year post-transplant, we are “out of the weeds” in terms of chances of transplant related death.  This fact has not changed our lack of comfort with her having the disease.
Late last week, we found out that she has permanently lost a significant amount of her hearing over the last couple of months.  This news was hard to hear. Then, she came home from school earlier this week to tell my wife that her hips hurt because they sat “Indian-style” more than a typical day.  Both bone deformation and hearing loss are two of the numerous ill-effects that she will suffer throughout her life.  Ultimately, the disease will attack every major system in her body (except brain which they think the transplant has halted progression in).  Degradation of vision, heart issues, digestive, bone and joint, liver, and so on.   The disease will continue to progress and attack her body – and there is nothing we can do but watch and treat symptoms as they come.

As an aside, we are constantly reminded of how lucky we actually are, as we continue to receive news of the deaths of some of the other children who battled alongside Riley during our time at Duke.
I am not looking for your sympathy and neither is my daughter.  She is a ridiculously amazing little girl.  In fact, I can tell you that not only has the disease not touched her spirit negatively – it has actually allowed my daughter to flourish in her outlook on life.
What was interesting about last week’s news is that it took us back to the time when she was first diagnosed.  A genetic disease is by nature present at the time of conception.  We figured things out at the age of 3.  We lived with a “perfectly healthy” child for 3 years without knowing it.  In one instant, we suddenly had a child that was probably in the top 1% in terms of the level of illness she would face in her lifetime.  It was that day that we looked at our daughter as though she was a biological creature rather than the sweet, little girl who had the ability to run, laugh, and love.   It was that instant and that diagnosis that caused us to analyze every inch of the physical being that is our daughter.
“Oh my gosh, she can’t lift her arms above her head – no wonder she has been so difficult to dress!”
“So that is the link between her minor heart problem, sensitivity to light, digestive issues, …”
And then there were things said by doctors like, “She’s beautiful…but she doesn’t look like you or her mother.”
We began to inch by inch, symptom by symptom, feature by feature, trait by trait look for her disease in everything that she was.  She was now a creature of science and this would escalate to unthinkable heights before the day of her transplant.  She underwent just about every test imaginable in order to establish baselines and make sure we knew about everything prior to starting chemotherapy and the subsequent transplant.
Have you ever wanted to look inside your child or yourself for preventative or knowledge reasons?  We were basically given a snapshot that allowed us to see inside of our daughter – physically speaking.  It would take us a few more months before we were able to really see what was inside of her, though, and we see more amazing things everyday – emotionally speaking.
Prior to transplant, doctors found fluid on her brain and spine that required immediate brain surgery and the placement of a permanent shunt in the back of her head.  Had she had headaches her whole life?  We would also find a hernia that needed to be operated on immediately.  Finally, she had 2 tubes placed in her chest that would basically act like external veins, allowing the doctors to draw blood or give medication instantaneously and without pain.
For the year or so since her transplant, we have watched and waited for further symptoms, signs, and evidence of her disease to show up, every day wondering whether and how the transplant had altered the biological specimen that is Riley.  However, what I’ve realized this week is that it doesn’t matter.  Sure I still care.  I absolutely don’t want her to be in pain or to not fit in or to have imperfections that cause her not to learn, hear, see, or be anything but what we all expect in our children.  We all desire some sort of “normal” as long as they are to extraordinary levels.  And that is just it.  She has so much “normal” – to extraordinary levels.
At the age of 5, she doesn’t know that she is anything but normal.  She recently started back to school after nearly a 2 year absence.  On her first day back, they called her up to celebrate the birthday that had happened while she was away, “I was very sick, but I am betternow.”  And then she turned and did something that the head of her preschool said she had never seen happen in her years leading the school.  Riley turned to head of the preschool and said “thank you.”
Riley is thankful for school, for her friends, for her family and for biological body.  She gets frustrated with her limitations from time to time.  Of course she has her moments of misbehaving too.  But, she sees her life and her body as a gift.  I commonly say, “My daughter has a special purpose for her life, and I’m excited to not only watch but also to be a small part of it.”
My daughter’s body has been marked by a disease that has done nothing but empower her spirit.  It is so easy to see my daughter – the biological creature.  I am learning to choose to see my daughter – the sweet little girl whose body is far from perfect but whose spirit is very near to perfect.  Of course there will be days that we need to focus on medical related things, but I refuse to let this obsession act like a disease within me that doesn’t allow me to see my sweet little girl.  This amazing child has so much to offer me and the rest of the world, and as the head of her preschool said, “so much to teach us all.”
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