Raise your Hand

If you read our blog, you know someone with a rare disease.  Sadly, chances are Batten, and Celia, aren't the only names that come to mind.  One in ten people nationwide have a rare disease.  That's likely three of your old second grade classmates, two of your former little league teammates, a few members of your graduating class, a couple of your colleagues, several of your fellow church congregants, someone in your family.  That means these diseases are, as Celia's G'Ro says, "not rare enough."
Today is World Rare Disease Day, sponsored by NORD, the National Organization for Rare Disorders.  If you'll take a moment to follow the link and click on "Raise Your Hand" on the right sidebar of the Rare Disease Day homepage, Lundbeck Inc.* will donate one dollar to NORD's general research fund. 
Rare diseases get little notice.  Some organizations rely on celebrities to put fame behind their cause, but most rare disorders remain unheard of by many Americans.  Drug companies don't have financial incentive to develop medications to treat them.  For those afflicted, a simple diagnosis can take years, and there are often very few experts to turn to, scant treatment options, and no one near who understands what they're going through.  By following the link above, you can help fund research, drive policy and develop educational programs that could make a difference for some of the 30 million Americans (75% of whom are children) affected by one of 7,000 rare diseases.  

Children like Celia can't raise their hands.  Will you raise yours for them?
Thank you.
JEB
Statistics from NORD's website.  Visit to find out more.
Photo by Amy Parrish
*Lundbeck Inc., a pharmaceutical company based in Deerfield, Illinois, is dedicated to improving the lives of patients affected by rare diseases for which few, if any, effective treatments are available. Since November 2008, the company has brought three products to market for the treatment of rare diseases for which there are currently no other U.S. FDA–approved therapies.

Gusto

With gusto, he does everything.
He crashes cars on the coffee table and performs acrobatic maneuvers in his ottoman dismount.
He drinks milk like the Jeep guzzles gasoline and gobbles food as if famine may soon sweep through the house.
He carries important things from room to room, like balls and vacuum hose attachments, in his hands or in his mouth, and offers to share only his least favorite items.
He waves wildly at friends and strangers alike with the kind of exuberant greeting that makes everyone glad to be acknowledged.
He initiates games of peek-a-boo and supervises phone calling and channel changing.
He demonstrates fierce opposition to the word "No."
He does everything he can, and tries everything he can't, with gusto.

This is who I am

"This ain't who I want to be... it's just who I am."

These lyrics rang true one recent night while I was driving Cel up to Dublin to stay with her Great-Aunts. It was snowing, turning a twenty minute drive into forty. She was screaming, making forty minutes feel like eighty. So I just turned up the music. My best coping mechanism has been detachment.  Understand that, unlike a normal crying child who might be comforted with a hug or calmed with a sweet smile and soft kiss, holding Celia sometimes makes it worse. So I detach. Well, first I medicate her and then I detach.
Her current needs weren’t part of my fathering paradigm. Most days I don't feel like her father - just her nurse.  And most days that is the easy part.
I’ve had to learn how to be a different kind of dad. This detour wasn't on my parenting map. Have you ever looked down at your GPS and realized you are off course? You're on some road that, according to the terabytes of data compiled to map the earth, seems not to exist. All you see is the little triangle pointing up and the line marking your path behind. No landmarks ahead. No turn by turn from here. All the thing can tell you is where you are. I'm there, and it's not where I wanted to be.

 

This is where I want to be, who I want to be...

I'm not the father I wanted to be, thought I would be, but maybe that’s part of what being a dad means.
Andy

Close to Home

 with Hubert and Helga Seifert at Spagio's "Celebration of Wine, Food, and Dining with the Stars"

We've long been fans of Spagio, a restaurant just a few blocks from our house. In fact, Spagio may have been one of the factors that drew us to this neighborhood. Well, Spagio and Staufs coffee shop and the charming architecture of the old homes and several within-walking-distance bars and the stellar school district and a couple great pizza joints... But really, we love Spagio.  It's one of those love-at-first-bite kind of places.  When we moved to Grandview, we'd walk over to have dinner there, and walk home hours later. During warmer months, we'd stroll over midafternoon and enjoy the fare and the weather from the patio. Celia joined us at Spagio several times, feeding herself bits of buttered bread and begging for bites of our entrees. We're not able to go on a regular basis any more, but last night was a special occasion.  For thirteen years, Chef Hubert has been hosting some of the world's finest chefs for an event that raises funds for Nationwide Children's Hospital Hospice.

Last night we tasted braised beef cheeks and enjoyed duck dumplings, we savored sea scallops in oxtail black truffle sauce and devoured hand painted chocolates. We sipped wine from Oregon and Argentina, from California and Australia. We oohed and aahed over the raffle prizes and the silent auction items. And we marveled at the generous gestures of so many people.
Celia has been under the care of Hospice for almost a year now, and we can't imagine not having their help. It was important for us to thank Chef Hubert in person. Near the end of the night, we were able to get most of our story out before the tears came and Chef Hubert hugged us close and kissed our cheeks.  He invited us to sit at the table with all the chefs, where we were able to thank them too -- for traveling, for sacrificing their time, for dedicating their talents. We told them about Celia, about the corona of red rings that sits atop a beautiful body betrayed.  We sang and toasted and laughed.  Choruses of Take Me Home, Country Roads blended with German cheers and conversations in Italian as well as Chef Handke's plated delicacies blended in taste.  As we left, well after the event was officially over, we parted with the words, "We'll see you next year."  Unfortunately, there will be more children, more families, who will need the services of NCH Hospice. But the chefs know that, and as families like ours prepare to say goodbye to our children, the world-renowned chefs will converge down the street to prepare another feast, reminding us of the significance of every child, of their lives and of their loss.
JEB

Usurped

Before we even had children, Andy and I fretted over the volume of primary colored plastic things that tend to crowd so many kid friendly homes. We worried that our little house could be overrun.  And now, even with a firm toy limit, I'm afraid most days our house appears to be decorated in a style that can only be described as a blend of Contemporary Little Tikes and Early American Fisher Price with a hint of traditional wooden puzzle piece and a splash of rustic naked baby doll.  What we hadn't entirely anticipated were all the "toys" that aren't really toys at all.  The brown paper bag, the wooden spoon, the empty yogurt tub and the butt paste tube, these and other junk items capture his attention and clutter our living quarters too.
And then there are the books.

Books in baskets, books on shelves, but mostly books on the floor. In every room.  Now let me be clear, we will never have too many books.  If we trudged through books and were smothered by books and had books spilling from the windows I would not complain about having too many books.  We read all the books.  We read them cover to cover, front to back and back to front, skipping some pages and reading others over and over.  Amid the primary colored plastic things and the bits of recycled untoys, dog hair piles up.  Dirty laundry runs rampant and dishes don't get clean.  Because now he brings me books.  So I stop scrubbing the potatoes and we read.  He brings me books and I leave the bedsheets in a heap and we read.  My books sit abandoned, magazines backlog, e-mails are left without response, because we're reading.
And then at night, after he's tucked into bed, I find books under the sofa and behind the armoire, in the bath tub and on the dog's pillow, books hijacking square footage and confiscating precious real estate, books taking over the house. And I know these are signs of a day well spent.  I pick them up and I catch myself thinking there is no home decor I'd find more appealing, nothing with which I'd rather be sharing my space.  And I know there is no way I'd rather be spending my time.
JEB

Four

This morning has not gone smoothly.  We've been up for several hours and none of them have been pleasant. But yesterday we enjoyed some family time, the four of us together in this part of our forever.

JEB

Learning

We're continually astounded by Tucker's developments.  We've learned not to take any new skill for granted, and so we sit and watch, or chase after and observe.  And later we say things like "Did you know he could do that?" and we wonder aloud "When are babies supposed to learn this?" and we marvel proudly "Look how he figured that out!"  Much of what he does now is seen through what feel like the eyes of a first time parent.
Tucker seems to have good instincts and makes quick connections, he has a burgeoning curiosity and budding confidence.  He grasps the concept of "in", putting shapes in the sorter, putting his food in our mouths, putting our car keys in his ball popper.  He learned, some time ago now, that when he rolls his ball under the couch, he can watch for it to come out on the other side.  And that when we shut the door from the kitchen to the basement, he can peer underneath it and follow us to the bottom with his eyes.

When he'd like to be picked up, he pulls our hands down and positions them under his armpits, a silent but effective request.  He has developed, as many babies do, his own version of sign language.  He uses his hands to guide ours in the sign -- his method is quite logical, given that we've spent months using our hands to guide his hands to make signs. When we ask him to clap he puts his hands on ours and pushes them together like he is the teacher in the relationship.  And more often than not, he is.
JEB

Object of Affection

Cherubs are said to go about the business of helping humans fall in love.  She qualifies.

JEB

Eclipsed

I loved my family and my friends and dogs and dolphins and rabbits.  I loved rainbows and Smurfette and soccer. I loved music, diet Mountain Dew, new shoes, and most of the time I loved my sister. I loved the color red, a good book, clean sheets. I loved the beach, a bubble bath, brownies and ice cream.
And then I loved a boy. When we said our vows, I was smitten.  Ohmygoodness I loved the man I got to marry, my all-the-time valentine, and I've adored him more ever since. Unwavering, unyielding love.

And now I love my children.  I love my children with all my heart, from the deepest place I own.  With them, I’ve learned to love all the way.  All the way.
My love for anything else has been eclipsed, just a pale light in comparison.

JEB

Little Brother

Dear Tucker,

I'm not sure that you'll grow up thinking of yourself as a little brother.  Of course we'll remind you that you had an older sister, that she was smart and beautiful and that she'll always watch over you.
I am sure that I will never forget how much you loved her. 
You look for her from room to room, peeking under blankets you recognize as hers, climbing on chairs and into her bed in search.  You make the "s" sound when you see her, for "sister" or for "Celia."  You hold her hand and kiss her forehead, you share your blocks with her and show her books.  When we don't allow you to touch her (because she's sleeping, or because it hurts when you pull her hair) you throw big fits.  You stick out your bottom lip and you whine and throw yourself to the floor.  I wonder if you know your time with her is limited.
You love your sister more than bears love honey, and more than any other expressions I can recall.  I wanted to record your love for her here, but despite an adequate facility with language and despite several attempts to explain it, I can't find the words to describe how big your love for her is right now. When it comes down to it though, the words I don't use may be just as important as the ones I do.  Because when I see how much you love your sister, I know it's not something I'll need to explain to you.  I know it's something you'll never forget either.
Your big sister is lucky to have a little brother like you.
Love,
Mama

Promise

Last fall we promised to post about Cardthartic's Book Loft event for BDSRA, and we're finally following through. Cardthartic's new website is up, and although we're not sure the launch is official until next week, we think the new site looks fabulous.

 photo by Amy Parrish

When this photo was taken, her exterior belied an extraordinary problem. But Cardthartic saw promise. Cardthartic saw her light. They saw a faerie and they organized an event that allowed so many Celia supporters to show love and to share compassion.  Over $4,400 was raised for BDSRA last September, money that allowed the continuation of promising research aimed at finding a cure.

But that's not all.  Cardthartic promised to donate all proceeds from the sale of Celia's card, anywhere and forever, to BDSRA.  It says so right on the back of the card.  If you haven't seen one yet, and you'd like your very own, please visit Cardthartic's new website, where you'll be able to view and order Celia's card (Little Reminder #329) and many other cards that likely capture exactly what you want to say.
It's evident that the folks at Cardthartic understand that problems to be solved aren't as important as people to be loved.  But the promise of an eventual cure for Batten, of solving the enzyme problem, seems more deliverable with every donation... and the big one from Cardthartic's Book Loft event means ever so much.
JEB

Honey Moon

Twelve moons have swelled since he joined us here, eleven months ago.

He is so thick with sweetness, spending time with him is intoxicating.  His gooey goodness precludes sobriety.

You'd think he hung our moon.  
JEB

Bowl Day

We don't own a sled.  So we improvised with a bowl.  Our idea was met with some skepticism.

The Pits

Although we don't want to keep lamenting her horrible illness or forcing the findable joy, there is nothing else.  There are no anecdotes to recount with pride, no sweet pictures of perfection to share.  She has a disease and, sadly, the disease has her.  The pain of her being here, the pain of the idea of her not being here, both shoot to the pit of our stomachs.  So we let the sadness visit and we cling to the treasures, we live breath by breath and laugh by laugh and tear by tear.  And we try to remember that our worst is not the worst.

JEB

By the Numbers

Five days without a computer, because he knocked it on the floor. And then a fistful of cash to fix it.
Nine teeth, four on top, five on the bottom. And always a smile that splits his face in two.
One new word, considered official now. "Mama," he speaks.  And it leaves me with zero words, unspeakable joy.
Eight steps, entirely unassisted, all in an effort to get to his sister.  And then too many slobbery kisses to count. 
Ten months old, our baby boy.  And a gazillion prayers of thanksgiving, give or take a few.
JEB

Fine

We're fine.  Computerless, indefinitely, but fine. 

 

Can the internet keep internetting without regular glimpses of this guy?