Friday, April 25, 2008

Bummer

Our biggest fear today was that Hope wouldn't do well with anesthesia during surgery for her ear tubes. By that measure, things went exceedingly well. The girl is tough. She was groggy for much of the afternoon, but seems to be rebounding well.

Unfortunately, today fell short by other measures. The tubes are in. Dr. Frosty emerged after 95 minutes looking like he straggled in from Fallujah and announcing that Hope's ear tubes were his toughest operation yet and her canals are the smallest he's seen.

Ten hours later, he returned to tell us they didn't make much difference in her hearing. One ear, which had severe hearing loss, has slightly improved and is now considered moderate. The other, which was moderate, had no change. That designation sounds better than it is. She still can't regularly hear our voice.

The tests still showed that the inner-workings of Hope's ears are mechanically sound, so she has conductive hearing deficiency: Sound isn't getting into her inner-ear. There's a chance her ear canals could widen as she ages. It's apparently not uncommon for kids with CdLS to flunk hearing tests as newborns and have normal hearing by 2.

So now it's on to getting Hope fit for hearing aids. We hope it isn't as burdensome a process as the tubes. She's a few weeks shy of 6 months and action is paramount.

We're sad, groggy and a bit numb from a long day, but I'm not sure we're surprised. Both Mo and I had sneaking suspicions this may be the result. Still, we allowed the possibility of a quick fix to tantalize and daydreamed about how wonderful it would be if we could take her home to song and mindless chatter that she could hear.

But we know that things are rarely going to be that easy. It stinks. But we're not big wallowers. So we move on and know it could have been worse.

Hope should be home tomorrow. They kept her overnight for observation because Dr. Frosty also stuck some probes down her throat to definitively diagnose her laryngomalacia (floppy airway) and there was some concern about swelling and breathing.

Addendum: Hope did return home on Saturday. She's OK, but cranky. So are we a bit. She may get fitted for temporary hearing aids on Friday, but there's a mountain of paperwork between now and then.

Thursday, April 24, 2008

A thousand points of pride



It's moments like these that make people so deliriously happy and dumbfounded by pride that the only recourse is to slap a "MY KID RULES THE UNIVERSE" bumper sticker on the car.

It came last night. Hope's grandfather was in the other room, watching the Tigers. Hope was on her stomach. Her fanny was in the air, shaking in a manner that is adorable now but may cause scandal in about 10 years. It inched higher and higher, like a tulip blooming in super-speed on the Discovery Channel.

Oomph, went her arms.

Ooops, went her back, swaying like Andy Capp before he gets bonked with a rolling pin.

C'mon Hope! You can do it!


Our faces were on the floor, cheering her on. Encouraged by her coaches, Hope has become a hardcore squirmer of late. She's kicking, flailing her arms, grabbing stuff and rolling from side to side. We both agreed she's on the verge of a breakthrough and kicked ourselves for waiting so long to figure out that, hey, this kid like being on her back.

She hoisted. She grunted. She pushed.


Argh. No luck.


C'mon Hope! Do it for daddy!


Hope regrouped. Her face stared at her play mat with a look of steely determination unseen since pipsqueak gymnast Kerri Strug ignored her aching ankle. I froze in a moment familiar, I'm sure, to parents everywhere: Do I run now and grab the camera to document history at the risk of missing it?

Hope answered the question. She pushed again and -- wowzers -- flipped from her stomach to back. She wore a look of bewilderment and excitement, as though she wasn't quite sure what she did.


We did. Our baby just rolled over.


We beamed. Pride gushed over us. In all fairness, I knew it was coming. Mo reported the big event occurred that morning during physical therapy. This was an encore or proof.


We were ecstatic. We've worked so long, so hard. What more could we ask for?


Do it again! Do it again!


P.S. Thanks to everyone for their thoughts, prayers and good karma about the surgery Friday. We go in at 6:15 a.m. We are nervous.

Sunday, April 20, 2008

Simple procedure

video

Neither Mo nor I want to verbalize it, for fear of hexes, but this could be a huge week for Hope. We got the long-awaited call late last week: The surgery for ear tubes is Friday, followed by a BAER test to determine if they did the trick.

If they do, my oh my, she can hear.

Supposedly, it's a routine, short outpatient operation. She goes in early Friday, is sedated so her head remains perfectly still, undergoes a 15-30 minute procedure to implant the tubes, has the hearing test and should go home that afternoon.

Our pediatric ear-nose-and-throat specialist, Dr. Frosty, has a good track record. This is his bread-and-butter. He's only failed to implant one patient and he got them in on the second try a few months later. But No. 2 is inevitable at some point, and he cautions that Hope's ear canals are tiny.


Like a lot with Hope, we don't want to get ahead of ourselves. We need to temper our nerves, excitement and wide-eyed eagerness with sober realism. There's a chance it won't work, so we'll have to proceed to hearing aids, wait until Hope gets bigger and try again. There's a chance it will work, the tubes might not make a difference and she'll need the aids anyway.


Either way, the odds of improving Hope's hearing are good. She has conductive hearing loss, which means the inner workings of the ear are fine but there's a blockage that prevents sound from reaching them. The tiny canals may be the culprit, but so could wax buildup, fluid or a missing bone.

We're convinced that Hope's hearing has slowly improved since the first BAER test, which revealed she could detect sounds at high pitches or extreme volumes. She startles sometimes. Every once in a while, we'll call her name and she'll turn to us. Then again, one of our smoke detectors is perilously close to our oven. It squawks nearly nightly. Hope doesn't seem to mind.

So we've proceeded as though hearing is an inevitability. We read to her. We sing her songs. We babble about our day and carry on conversations that she likely can't hear. But she sees us moving our lips and pays rapt attention. Hopefully, when those wagging lips begin to produce sounds she can comprehend, she'll put it together and life will be good.

But you never know. Dr. Frosty assures us that kids are resilient and "do fine" after successful surgeries. Still, we wonder about the adjustment. All of a sudden, if things work the way we want, Hope's world will expand exponentially. In addition to sights, smells, touch and taste, she'll have this whole sensation to figure out. Will it be melodious or cacophony? Will it expand her burgeoning sense of wonder or simply overwhelm? Will "Running Away from Mom" sound nearly as cool if she can hear my singing?


Nor are tubes a cure-all. Sometimes, I've deluded myself into believing that our path will suddenly become easier once tubes are implanted. The skies will open. God will rain M&Ms from the sky and remove the brown ones. And I will suddenly have a full head of hair. Big Mo brings me back to Earth, pointing out that hearing is just one part of the puzzle toward communication, which is always a challenge with Cornelia de Lange kids.


Still, we're excited. The benefits are hundreds of times better than any niggling concerns and it's such a relief to finally be doing something after what seemed like months of limbo. So we do what's beginning to come naturally: Take whatever comes as a gift and roll with it.

Thursday, April 17, 2008

Happy days



This is what bliss feels like. Hope's grandma from Maine is in town; the girl gets a double dose of grandmas today; and we're fairly convinced that Wednesday was the happiest day of her life. Thursday was swell too. But Wednesday? Oh. What a day.

Pip is probably the happiest kid on the block when nothing is bugging her. Unfortunately, something often creeps up -- be it her colic, reflux, constipation or parents -- and dashes the good-time vibe.

Wednesday? Nothing. Everything was hunky-von-dory. Mile-high smiles all around. Hope aced her therapy. She went for long walk under 60 degree skies. She became acquainted with the Bumbo seat and didn't freak out.

It's amazing this is the same kid who spent all last weekend crying. We're not complaining.

Wednesday, April 16, 2008

A love reborn?


It's spring, love and possibility abound and Hope's boyfriend -- or a reasonable facsimile thereof -- has come wooing.

As some recall, Hope endured the whirlwind with teddy bear beefcake Daisuke Matsuzaka. Attraction begat infatuation, intoxication and finally shame at being so young and falling so hard, so fast. Like many classic romances, it ended in tragedy.

Lulu ate the doll. Scorning a love she could never understand, she lashed out like Iago or some other Shakespearean scallywag. Or perhaps she was hungry.

Either way, we were crestfallen. Hope's first love was gone. Failures as parents, we scoured the Internet for a replacement. My sister, Beth, answered the call. Another doll was in the mail in two days.

But young love is elastic, especially when it involves broken hearts. And can ooga cha-cha, hubba hubba burning love simply be cloned?

So Dice-K is back. But Hope persevered. Holding plastic rings makes her squeal. Her hands fascinate. There's more to life than hunky dolls. She has moved on.

But oh what a specimen. She tries to resist. But it's Dice-K. She looks askance. She sneaks a peak. He's still got it. Oh my.

Can she trust her heart again? We wait. We wonder, while Lulu lurks.

Sunday, April 13, 2008

Her own course

Our dilly-dallying, meticulous geneticist finally completed the paperwork outlining Hope's diagnosis with Cornelia de Lange Syndrome. It arrived Saturday in an envelope the size of some yellow pages.

Bureaucratically, we need it to buy supplemental insurance for kids with disabilities and enroll in the Children's Hospital of Philadelphia study to pinpoint the gene that went awry to give Hope the syndrome.

Emotionally, it was sobering but unsurprising. The packet listed everything from the arc of her eyebrows to malformed bones and contained such page-turning phrases as "the pinnae are well-formed and normally placed, without thickened helices."

One phrase stuck out: Her development to date is normal.

Granted, the report was based on data from three months ago. But it was still nice to see. We've kept score of milestones the way some folks track thoroughbreds or box scores. We know the day is looming when she'll fall far behind the curve. But so far, to our delight, she's surprising us. She's mostly on track and got her first report card the other day from occupational and physical therapists: Gold stars all around.

Hope's doing nifty things. She's grabbing her binkies, moving them from one hand to another, gazing at her outstretched hand and willing her thumb to her mouth (usually she misses.) We regularly consult the development chart on the refrigerator: Grabs stuff. Reaches out. Likes to play. Controls neck. Check, check, check, check. Son of a gun.

So we were feeling fairly pleased when we left for a play date with a pal born about two months before Hope. We left with our eyes wide open. We're working on a different standard altogether.

Hope's pal, Lanagan Jack, is in the 25th percentile weight-wise. He is 9 pounds heavier than Hope. On paper, they could seem similar socially: Both grab things, are happy on their stomachs, smile at their mommas and poppas and can kinda, sorta prop themselves up with their arms.

But when Lanagan grabs you, you know it. He's a bruiser. When Hope does, it's delicate and slow. He amused himself with toys and whimpered once. Hope cried for two hours.

They're both great, beautiful, wide-eyed joys. But they aren't really peers. It's kind of tough, but it's OK.

We've known since Hopesy's birth that she's moving at her own pace. It's a lesson that's reinforced every time we think we have figured her out and she zings us for the presumption. We suffer no delusions. Things that come naturally for most will always be a challenge for her.

We could wallow, but that's boring and exhausting. So we party twice as hard at Hope's accomplishments. This week, she began kicking up a storm. She's swaying from side to side, reaching for toys across her body and plotting a slow, deliberate but definite course to rolling over.

Tuesday, April 8, 2008

Hear, hear



Good news on the hearing front: We met with out ear-nose-throat dude on Monday, and he green-lighted us for outpatient surgery to put in hearing tubes. We should get a date for the operation within the next 10 days. At the same time, the doc -- a gregarious guy with frosted hair and themed ties -- may trim some of her larnyx to help with her laryngomalacia.

There's no guarantee he can get the tubes in. Her ear canals are tiny. But the doc is optimistic. These surgeries are his bread and butter and he's only had one that wasn't a success. So we're cautiously optimistic. It's wonderful to finally be moving on this front and we'll be so delighted for the day when she can hear us tell her how much we love her.


Sunday, April 6, 2008

Baby noir

video

One of the causes of the blog break was my first stab at baby film noir. Like a lot around here, I'm not sure if it's sheer genius or just dumb.

The back story: We've had partial season tickets to the Detroit Tigers for four years. Opening Day is sacrosanct. Initially, that was because of the majesty of the game, the hope for renewal and the return of spring. Now, it's just a good excuse to drink beer.

This year, it caused a self-induced miniature morality play. Check it out.

A big, short stroll



The sun shone for the first time in eons. The same streets that were so humdrum for months suddenly seemed charming. The mercury kissed 61. What better day to debut the Chicco Cortina Travel System?

That's right. It's not a stroller. It's a "travel system" with four cup holders for the parental units and infants, all-wheel suspension and something called a 5-point harness. The cruise control and heated seats were optional.

We waited for this day since Hope's birth. Pushing your kid down the street in a "travel system" is one of those mundane activities that seem so profound when you have to wait -- and work -- for months to get there.

First, Hopesy was too small. Then, winter descended and our doctor-ordered hibernation during cold and flu season began.

This was huge. One small stroll for Pippi. One giant leap for normalcy.

I kicked the tires and checked the manual for 20 minutes. Mo fussed with blankets before finding exactly the right one for the 15-minute journey. We made it to the end of the driveway before acknowledging the obvious.

"Why are we so nervous?" I asked.

"We just need to live our lives," Mo said.

That's been her mantra for weeks. It's one thing to vow to give your daughter a normal life when it's an abstraction because you can't go anywhere. It's another to actually do so when you can.

Our worries were unspoken: What if we were 10 minutes from home and Hope melted down? What if we hit a bump? What if Lulu went bananas? What if some hillbilly busybody sees our 5-month-old, 6 1/2 pound girl, gets the wrong idea and phones Child Protective Services?


We walked one block, gingerly traversing each sidewalk crack. We walked another block. Then another.

Hope was so concerned she fell asleep.

Like a lot with Pip -- or life, I suppose -- the first time is always the most nerve-wracking, and big fears that can paralyze rarely materialize.

We took the long way home.




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Friday, April 4, 2008

Great girl, bad blogger



Pardon our dereliction from blogging, but the old saw is true: No news is good.


Medically, there's delightfully little to report. Hopesy's cold seems to have run its course. A follow-up visit Thursday with the venerable Dr. Spitenup went so well that we don't have to see him again for a few months. His manner is strange, but we've become grudging fans of the speckled-haired specialist. That's mostly because what he's said so far is true: If Hope is pooping, she's happy. If she's not, ay caramba. Sometimes, life is just that simple.




Socially, Hope is going gangbusters. We marked her fifth month a few days ago with wonder. In some ways, it seems like the months have flown. In others, they've crawled. But there's no denying Hope's progress. She's doing incredible things for a 6 1/2 pound girl, becoming more expressive daily, laughing like the dickens, grabbing everything in sight, removing her beloved binkie from her mouth and plopping it back in, holding up her neck and going to town with tummy-time. She's still mostly where she should be on the growth curve, which is a testament to her and her Mom. She who works with Hope with all the determination and grit of a Hungarian gymnastics coach cajoling a 9-year-old over the pummel horse. Hope's occupational therapist was wowed at her progress Wednesday after a two-week gap.


Bureaucratically, life is slow. We're finally meeting with her ear-nose-throat specialist on Monday in hopes of kick-starting surgery to fit Pippi with hearing tubes. She's at the point now where conductive hearing loss is becoming critical. Six months is about the cut-off point for intervention before it affects development.


The paperwork chain to enroll in the genetic study of parents with multiple children with CdLS is glacial. The point man to coordinate communication between three hospitals is our old friend the geneticist. He's just now submitting reports about her diagnosis that were due three months ago. We've known the doctor for three years. He's kind, warm and a tremendous help. He's also methodical to the point of paralysis. Every day, I suspect he goes to the hospital cafeteria and expounds on the comparative merits of macaroni and cheese vs. tuna sandwiches before handing the cook a six-page memo explaining why he would like a side of cole slaw.


So we wait. And that's OK. Many days, like this morning, it's such a joy to see Hope squirming and giggling that you feel like skipping in air. It's corny. But true. I guess that's parenthood.