Go Hawkeyes

This month's BDSRA newsletter explained that a recent study at the University of Iowa used gene therapy to transfer enzymes across the blood-brain barrier, proving effective in treating mice with lysosomal storage disorders.  News like this means there's a chance.

***To simplify a complicated concept, the blood brain barrier is really just that, a barrier that keeps bad things in the blood away from the brain. (bad things = bacteria, viruses, toxic medicine, etc.)  At the same time, the blood brain barrier keeps good things in the brain.  (good things = neurotransmitters, hormones, etc.) It's tricky to get some good things, like medicine the body needs, into the brain.  Sometimes diseases, like Batten, seem simple to remedy: there is one enzyme the body doesn't make... give the child that enzyme and all is well!  But complexity begins with whether or not a scientist can readily reproduce the missing enzyme and continues with getting that enzyme into affected neural cells. 
That's what is so amazing about this research from the Hawkeye state!  They have successfully transmitted enzymes into brain cells in mice with lysosomal storage disorders.  Can I get a OH IO-WA?!
 CEB, Iowa trip, March 2008 (borrowed paci.  Obvi.)

Last month, two challenge grants were offered to BDSRA to help fight Infantile Batten disease, the form of Batten that Celia is affected by. Garrett's Wings and Hayden's Hope each committed matched funds, up to $25,000, for contributions to infantile Batten Disease research before February 1, 2010. These grant opportunities, coupled with donations, gives BDSRA a chance to raise $100,000 for vital research projects.  And research, like what's being done in Iowa, may soon give children like Celia a chance at life.

Consider making a contribution to BDSRA as a Valentine gift for someone you love.  But please do it before the match deadline (2.1) and please be sure that you designate your donation for the infantile research grant.  
BDSRA 166 Humphries Dr. Reynoldsburg OH 43068 or BDSRA.org
The only time we won't be rooting for the Hawkeyes will be on Saturday, November 20th 2010 at Kinnick Stadium.  On that day, the Hawkeyes won't stand a chance... The other 364 days of the year, we love every thing about the Hawkeye state.

PS. The Buckeyes handed it to the Hawkeyes last night in roundball... Go Bucks! 



We grappled with problems both mild and dire. We wiped mouths and noses and behinds, we spoke to doctors and nurses and chaplains. We followed him around undoing the undoing he did.

We invented games and discussed what-ifs, we fought fatigue and baked cookies, we cursed the dishwasher and consulted the to-do list.  But many of the mundane moments were so full of beauty and grace.  And because it feels better to focus on the one or two things that go right each day as opposed to all the things that go wrong, we deposit today in our memory banks, a day that was alive in life, a day that was good enough from the first kiss to the last straw.


Don't Wish. Don't Start.

I wish she could string clover chains and spin in a twirly skirt, or pump high on a swing till her toes graze the sun. I wish she could squeeze all of my fancy body wash into the tub, and sneak Sharpie drawings on the walls. I wish she could belt out showtunes around the family piano and sing in the childrens choir at church.  I wish she could prance around in dress up clothes and race on the swim team at the local pool.  I wish she could choose a favorite subject in school and show off her report card at home. I wish she could sport more pigtails and spout more words.  I wish she could be a big sister, take her little brother by the hand and show him the ropes. I wish she could cement friendships and flirt with boys.  I wish she could aspire to be a teacher or a marine biologist. Or a Rockette.

There's no way to predict her future, except to know that it won't entail any of the those wishes coming true.  We don't need a medical degree or a crystal ball to know that her time here won't be long, that the years ahead of her are likely fewer than the years behind. That's not to say that we don't catch ourselves wishing the good Lord would reach down and do the work that none of us can do, that we don't sometimes hope for something holy to occur that could redirect our course.  We try hard not to compare Celia to the girl we hoped she'd be, try not to hold on to so many impossible dreams. 
Because it's true, wishing only wounds the heart.



Tuck and Cover

Tucker hasn't yet shown an attachment to any blanket in particular.  He has, however, shown an inclination to wrestle with whatever blanket he can reach, rolling around until he's all tangled up.  He grabs the quilt from the couch and pulls the throw from the chair and he tries to steal whatever blanket his sister may be wrapped in.  When he's especially tired he buries his face in the covers and wrestles until he's satisfactorily wrung out every last bit of comfort the blanket has to offer.

Yes, he has a scratch on his forehead.  We realize, especially considering his affinity for wrestling, it's likely injury #1 of many...


So Big

Some parents look at their babies and wish they'd never grow up.
I look at mine and wish she could.





Figure Eight

This weekend's activities required a bit of logisticizing, but we figured out how to attend a Grant Trauma dinner party, visit Poppy John in West Virginia, and celebrate the marriage of a childhood friend.  We solicited the help of eight extra adults in order to keep Celia and Tucker and Colby well-cared for over the course of three days.  And we spent at least eight hours in the car.  But, all of the figuring was worth it.  We had fun.

And Tuck had fun figuring out how to play the tambourine at Poppy's house.



Sweet Dreams

Sometimes our situation feels worse than a nightmare, and most often it falls desperately short of our dreams.

It's easier to get through the days, though, when we consider what life has given us, rather than focus on what's being taken away. Our optimism isn't denial, but we do have hope, hidden inside, that our dreams will keep us all together even when we're apart...


Snow Biz

Properly attired, Tucker was far more entertained the second time than he was when the snow debuted.


Safe to Say

Over time, affected children suffer mental impairment, worsening seizures, and progressive loss of sight and motor skills. Eventually, children with Batten Disease become blind, bedridden, and unable to communicate. It is presently always fatal.
We'd read these words before the official diagnosis, but it was about a year ago that the words went beyond our eyes and settled into our lives. The words described the disease that was killing our daughter.
Those words were soon followed by others, kinder and gentler, from friends and family. Cards with words that filled us up when we felt empty, texts that offered sympathy and support, e-mails that reminded us how much we’re loved.
There are our own words too, the ones on the blog and the ones that are never committed to paper. Andy and I speak them to each other and they float through the air between us, private words that run through our minds and cross our lips as whispers. And we know each other well enough that, between us, the words unsaid are often the ones that roar the loudest.

She started talking early, and we envisioned her becoming precociously verbal. We imagined words tumbling from her mouth and competing for precedence in typical toddler fashion. We anticipated her spraying machine-gun rounds of " Daddy, watch me" and "Mommy, up" and "Pretty please."
She's able to say some things without words, there are still a few things we're able to hear in her silence.  Her words, unspoken, hang in the space around us.  And it seems safe to say that they are, perhaps, the most indelible, that they'll be here long after she's gone.


Ten Months

In the beginning we held him tight and watched him sleep, his eyelids fluttered closed until dark spider-leg lashes rested on cherub cheeks.
At ten months his sleepy darlingness is still just so… darling.  But so is his lively personality!  When he’s awake, happiness looks out through sparkling eyes.  Laughter spills from his mouth and, like good red wine, it warms our veins.  Joy threads through his voice stringing together brightly beaded syllables.  A mini-mechanic, he ducks his head to peer under the hood of life at his level, and invariably his fast fingers find items to reach and examine, to gum and to throw.
Now we hang close at his heels and keep a careful eye.  Brimming with curiosity he lunges ahead, stretching the space between us wider every day.




In the 1999 Caldecott winner a Vermont farm boy, mesmerized by ice crystals, describes snow as being as beautiful as butterflies and apple blossoms.  

"Under the microscope, I found that snowflakes were miracles of beauty; and it seemed a shame that this beauty should not be seen and appreciated by others. Every crystal was a masterpiece of design and no one design was ever repeated. When a snowflake melted, that design was forever lost. Just that much beauty was gone, without leaving any record behind."  
- Wilson "Snowflake" Bentley, 1925

Because snowflakes were too complex to draw before they melted, Bentley attached a bellows camera to a compound microscope. After years of trial and error, in 1885 he became the first person to successfully photograph a single snow crystal; he captured over 5000 images of snowflakes over his lifetime.

Bentley described snowflakes as "miracles of beauty."  She is, too.  And we hold her gently, knowing she will disappear, knowing that much beauty will be gone.



Not Cool

Tucker is rather fond of our lint roller.  He carries it around, giving it the kind of attention that no other toy has rivaled.  We talk about how sticky it is.  And Tucker loves to twist hair - mine, his own, his sister's - between his fingers, to yank on it and chew it.  We talk about  how the strands feel coarse or fine, are curly or straight.  Tucker loves to pet the dog and to "pat the bunny" and to bury his face in cushy pillows and cozy blankets, and we talk about how soft all those things feel.  Tucker is also really quite interested in my lip gloss, the green rubber C.O.Bigelow tube.  He passes it from hand to hand and gnaws on each end, and we mostly ignore him, not sure what to make of his affinity for makeup...
As interested as Tucker is in textures though, I thought he might like to play with snow.  I thought wrong:




This time of year prompts me to look back, which makes me feel a bit nostalgic, makes me yearn for the good things of days past. Celia’s fleshy thighs have disappeared, along with too many other things that shouldn't have gone with them. And Tucker's turned from baby into toddler at a rather astonishing rate. More subtle transformations have happened right before my very eyes, and were it not for reminiscing over photographs I’m not sure I would have noticed so much. Through the photographs we’re able to watch the children change and grow. Through the children, and through the trials and through these words, we've found ourselves growing.
2009 could become known as The Year of the Batten Disease Diagnosis, but it's also the year Tucker was born. These things, among others, made 2009 feel like a year layered with more loss and love than the prior thirty years combined.  2009 was difficult, but we came out stronger and wiser, perhaps with less money and definitely with fewer hours of sleep, but we we made it, all four of us survived to see the dawn of a new decade. We'll take the new year one day at a time though, not in the stereotypical, cliched sense, but literally moment by moment.
I wish 2010 offered more optimism.  There don't seem to be many signs pointing toward great things, but instead of looking too far ahead, instead of worrying about what's to come, we'll try to embrace the ride.  When I'm filled with greed that makes me wish I could know the future, I'll look to the One in the driver's seat and for confidence in the course. And then I'll check the rearview mirror again :)

            Celia 3.2008 and 1.2009