Encouragement

The past week or so around these parts have been filled with "avast, aghast, pity poor us" posts. All the facts are true -- tough questions from strangers, a bad sleep study, strange doctors -- but in sum, they may give the impression that times are tough. They're not. Hope is doing great.

She's been in physical and occupational therapy for more than a month and has responded well. I have to be careful what I say and avoid giving either therapist clever nicknames because they're readers. But it's safe to report Hope is a promising pupil. We worried about how she'd handle 30 minutes of stimulation, but so far, she has exceeded our expectations.

They come to our house on alternate Wednesdays and work on things like neck control, balance, tummy time and building muscle. Sometimes, that's through bouncing her on exercise balls or laying her on her tummy. Other exercises involve toys with bright lights. Eventually, therapy will expand to off-site visits for a group therapy session on Thursdays.

Two observations from the physical therapist have delighted us.

I see Hope running one day.

and

Socially, Hope is where she's supposed to be or even a bit advanced.

I think Mo almost cried when she heard both. Too often, our expectations have had to shift downward. It's great to have high hopes.




P.S.: The uber-cool photos above are from my brother, Steve. That's my sister, Beth, and Hope being enthralled by a Daisuke Matsuzaka doll. Steve's a great shutterbug. Thanks, dude.

P.P.S: Happy birthdays to two of our favorite ladies: Mo's Leap Year sissy, Molly, and my dear ol' Momito. We love you guys oodles.

The conversation

We're cautiously emerging from the protective yolk of winter. It's an "oooh, it's so cold, big toenail in the water, arms folded across our chest" emergence. But it's a step away from hibernation.

That's fantastic. It's what we've waited for all winter. It's also an adjustment.

As we mentioned in the beginning of December, Hope's floppy windpipe, or laryngomalacia, makes it imperative that she avoid catching colds. That could complicate breathing and hospitalize her -- and meant staying inside during flu season except doctors' appointments. So it's been three months of (a) staring intently into each others' eyes and whispering "No, I love you more" (b) sharing conversations and dreams so soul-stirring they'd make even the Sisterhood of the Traveling Pants gag and (c) watching a lot of TV.

Amazingly, that gets old after 100 days.

So we're ramping up our bravery and venturing into the outside world. About a month ago, we took up Mo's parents, Clem and the Big Beef, on their babysitting offers. Then, last weekend, we sneaked out Hope out for short spurts: Furniture shopping; small errands; church; the post office.

It's nerve-wracking. We worry about Hope catching something. We fear she is going to wake up screaming. We move with caution, knowing the conversation lurks with every stranger.

"She's so tiny!"

Mo's heard it for months at doctors' offices. I'm just starting to encounter it. It's nice that it's often accompanied with "She's so adorable." But it soon leads to awkward conversations. Neither Mo nor us quite know how to respond. Hope is tiny. But she's also gorgeous, a snuggle-puppy, sensitive soul and, like her Dad, an occasional grouch. Her size isn't who she is.

But that's usually where the conversation goes.

"She is just so tiny! I'd be afraid to touch her!"

We know people take their cues about Hope from us. Like most parents, we love the chance to brag up our kid. We'd just rather not do so at La-Z-Boy -- or launch a conversation about genetics while juggling a can of peas and the car seat.

On the other hand, we don't want to seem rude. So we've tried the simple ("Yes, she is tiny!") and the subtle ("That's how she's supposed to be!) We've also mulled the evasive ("She is small. And she has lovely eyes!) and the sarcastic ("Really? Are you sure?")

Alright, that last one is me.

The next question is inevitable: "Is she a newborn?"

Its variant-- "Is she a preemie?" -- is easier. Technically, Hope was born at 37 weeks. Morally, answering in the affirmative is a bit misleading.

So Mo usually smiles and explains that Hope is almost four months old and has health issues that make her small. That usually ends the conversation, which is understandable but also sad.

So we're searching for the perfect rejoiner. It needs to be one that conveys (a) how much we love our daughter (b) how she makes us proud (c) acknowledges that we realize she doesn't look like babies her age and (d) politely signals that we'd rather not elaborate for the postal clerk.

We've already determined that Hope will have as normal a childhood as possible, so it's a conversation we'll need to get used to. Like a lot with Hope, it's an adjustment, but worth it.

Happy Sunday

For once, I'm tired of my own words. Here are some pix.

Lancelot's larynx

As if we needed a reminder of how unusual life has become, the moment came last night. A nurse spent an hour measuring Hope's head, marked it with red dots from a wax pencil, affixed a dozen electrodes and leads and finished with the piece de resistance -- wrapping her entire head and neck in a gauze mask to keep the wires from crossing.

It was tough to watch. Her little head was covered in wires. The gauze left her face exposed but covered her head and neck. My mind raced: This is freaking wrong, you shouldn't be 3 1/2 months and have a head full of electrodes to measure brain activity. She looked pathetic, but I couldn't decide if she looked more like a Depression-era football player or an onion.

Mo settled the debate.

"Hello my little Lancelot. Have you found your place at the Round Table?"


That was the one moment of levity in an otherwise long night during Hope's long-awaited sleep study. We don't get the results for a week, but we doubt they'll be good.

The study, which kept her in a surreally spooky clinic overnight, used electrodes, belts and other gizmos to measure the quality of Hope's sleep, especially her breathing and apnea. Those are the brief interludes when she stops breathing, catches herself and thinks "Hey! I'm not breathing!"

The fear is Hope's acid reflux is causing a blockage in her throat. Or perhaps her floppy airway, or laryngomalacia, closes during deep sleeps and suspends her breathing. Either way, we need to figure it out. Surgery is in her near future for ear tubes to help her hear, and her apnea could be cured by a surgical tweak during the same operation.

The study was about a mile from Beaumont Hospital in a non-descript office building that appeared otherwise vacant. It was all so strange. We got there about 7:30 p.m., had wait for 10 minutes to be buzzed in, then were led into a basement. We were ushered into a room that sort of looked like a hotel, except one with a camera that moved when you did and a loudspeaker from which unseen nurses and a neurologist could bark commands.

Big Brother was watching.

The study videotaped eight hours of sleeping, as well as brain activity, breaths through Hope's nose and the elevation of her chest and stomach as she inhaled and exhaled. A doctor will evaluate the results and make recommendations.

They might not be pretty. Hospital rules only allowed one parent in the room through the night. I stuck around 90 minutes, then hightailed it home. The ladies returned about 6:30 a.m. It was an especially bad night. Hope woke up about six times, was given supplemental oxygen through her nose because she wasn't breathing well and threw up all her milk about 4 a.m. Mo didn't get any sleep.

We're tempted to make excuses. The place was weird. The heating system was loud. How would you sleep if you were covered in wires and belts and videotaped? It was just one night. Swell questions, we know. But they're not going to change the results.

We have a lot of fears about what's next. We worry the cure may involve more complicated surgery or supplemental oxygen at night. But the sight of Hope covered in a spaghetti-strand tangle of wires reminds us yet again that we're way past the point of fear. We need to buck up and do what's best for her.

A rival for Hope's smiles

Lately, we've deluded ourselves into thinking we're getting the hang of this parenting thing. Our daughter digs us. She looks straight into our eyes, laughs at our jokes and smiles like she just won a pie-eating contest whenever we're nearby. We are so cool.

We learned how we really rank today when we met a rival for her affections, something so fascinating and endlessly amusing that we can't hope to compete.

Hope has discovered the ceiling fan. We may never win her back.

We will hold her, tickle her belly, coo and goo. She'll like it OK. Then, it happens: Off, over our shoulder, she will spot it a few feet away. Her eyes widen. Her mouth slackens. She succumbs to its allure.

Game over.

Hope is transfixed. And who can blame her? Sometimes, it goes fast. Sometimes, slow. Always, round and round. That's tall company.

Hope rewards the fan with full-body, giggling delight smiles the likes of which are far more euphoric than the "Hee, hee! OK, I see you already" grins she bestows on us.

Like a lot we've learned so far in parenting, it's a bit humbling. Hope's happiness is a primary concern. Besides health, it's what we want most, so we'll take it however it comes. But a fan? A clock maybe. A blinking alarm clock for sure. But a stinking fan?

Full disclosure moment: We don't even rank #2 behind the fan. That distinction goes to lights. They are bright and full of, well, light. Hope will smile in our arms, full of mirth and glee. We will congratulate ourselves on developing such a rapport with our sweet princess, then realize she's looked past us for 10 minutes and is enjoying a special moment with a lightbulb.

Again, it's tough to argue. Lights help you see. But for those keeping score at home, Hope's true loves are in order: Fans; lights; that special lady who birthed her from her loins, feeds her eight times a day, comforts her amid the sorrows, lavishes her with hugs and cleans our her eye boogers; and that dork with the video camera.

We had a moment of pause when we worried unnatural fascination with fans was a sign of a developmental delay. Then, we discovered page upon page online of perplexed parents of newborns who also can't compete with rotating blades.

So we'll take it. If Hope is happy, we're happy. And hey, Lulu still thinks we're pretty cool. Then again, she eats her own vomit.

Valentine

Last year, Mo surprised me by taking me to White Castle for Valentine's Day. It was fantastic: They closed the restaurant a few hours for reservation-only service that included white tablecloths, sparkling apple cider, tiny cheeseburgers, red roses and a heaping side of l-u-v.

No White Castle this year. Bummer. Hope's still a bit on the small side and we're still in the throes of flu season. We're slowly removing the protective yolk around her, but plopping her for an hour inside a greasy burger joint still falls a shade outside our comfort zone.

So it's a picnic on the living room floor, befitting our season of hibernation. I will stare deeply into Mo's eyes. She will tell me to brush my teeth.


Seriously, it's a happy day. I'm lucky to have a wonderful wife whose patience, strength, intelligence and humor continue to amaze and humble me. Hope has been an absolute joy after a rough stretch for a few weks. She's very active, bright-eyed, attentive and smiling like crazy. Lulu's no slouch either. I have great ladies in my life. The cats are another story.

A few notes about collaboration

We have a few arguments in this house about blog entries. I write 'em. Mo edits 'em. Initially, Mo felt jealous, claiming I spent more time with the blog than her. She dropped that when I started spending more time with her.

Lately, the friction stems from our philosophical differences as journalists. I want to tell rip-roaring yarns that entertain, enlighten, provoke and even tickle the heart. She keeps insisting they be factually accurate.

Whatever, lady.

Honestly, we walk a tight line. Our first priority is protecting Hope. There are some scary possibilities with CdLS that don't need to be announced and prejudice anyone's view of her. But we also have an opportunity to spread the word about a syndrome most don't know exist.

So there are many chats about how much to say, when and how. I usually err toward letting it rip, brutal honesty and endless chin scratching that paints me in the best possible light. Mo prefers a judicious, "We went to the doctor today and here is what happened" approach. At times, the chasm proved so great that I threatened to (a) Start a secret, alternative blog using pseudonyms and a made-up condition where I could finally be free to vent inner demons and philosophize (b) Kick her off the blog about her daughter or (c) Withhold kisses until she is a nicer editor who lets me write what I want.

Her response: "Whatever, loser."

That's my lady!

We've bandied about compromises. Mo doesn't want to alternate entries. I suggested we approach the blog like an annotated study Bible: I'd set down divine prose and she'd make persnickety notes in adjoining, red-font paragraphs to offer her claim to the "truth."

Until then, we're stuck as collaborators, in more ways than one and must make it work. In that spirit, here's the latest, in prose to make Mo proud.

Monday, Mo took Hope to the eye doctor for a routine examination. Hope is doing well. She can see well. She does not have cataracts. Her eyes were dilated. She tracks motion well. The doctor is a nice old man. He gave tips on how to massage the balls of Hope's eyes to ease what appears to be a blockage in her tear ducts causing excess eye boogers. We will seem him again in a few months, but so far, so good!

Last week, Hope began occupational therapy. She will have occupational or physical therapy every Wednesday at our house and, when she is big enough, will go to group therapy Thursdays with other tots. It is part of Michigan's Early On intervention effort for special-needs children.

The therapists work on such things as "tummy time," neck control, feeding and eventually crawling and arm control. One of the exercises involves a helium balloon. You put it near her to encourage eye tracking and playing with her arms. She had a jolly good time Monday night with a Valentine's Day balloon. We held her, she giggled and followed as it gently bobbed and swayed. She reached for it repeatedly, but didn't quite bat it with her hands. Oh well. A good time was had by all. We also worked on an exercise with a rubber pilates ball. She lays on it with her tummy and we gently roll her. That encourages neck control. She did a good job.

Our sleep study last week was postponed until Feb. 19 from Friday. Other than therapy on Wednesday, it is a quiet week.

Over and out.

Guinea pigs

(Hope, meet Gavin. Five and a half pound baby, meet nine pound baby. Slobbering ensues.)

We're becoming guinea pigs. It's nothing to aspire to. If you think about it too hard, it's glum reminder of what rare, freaky straits we find ourselves in. But it could do a lot of good.

Even though Hope was diagnosed a few weeks ago with Cornelia de Lange Syndrome, we are proceeding with blood tests to confirm it -- a process that could pinpoint the specific gene that went awry to give the syndrome to Hope and Will.

We're enlisting in a study by Children's Hospital of Philadelphia of families with multiple CdLS children. Hope's blood first will be analyzed in hopes of finding the gene. If it is, they will follow with blood from Will that we banked when he died.

We've had chats about entering the study. On the surface, it seems redundant. We instinctively want to protect Hope. Drawing her blood to map her genomes and sharing those results with the medical world seems like the ultimate intrusion.

But the potential benefits outweigh those qualms. Foremost, it could further understanding of a condition we wish didn't exist. It also could assist us if we roll the dice with future pregnancies.

Research is moving swiftly, but it's still mostly a small-scale operation run largely from Children's Hospital of Philadelphia. There's simply not that many kids out there; it's thought to affect 1 in 10,000-100,000 live births.

And there are far fewer families with more than one CdLS child. Supposedly, 1-2 percent have multiple children with the syndrome. But it's a bit like lightning striking twice. Caused by one gene mutating and not forming correctly during conception, CdLS is a dominant condition. That means if you have the gene, you have CdLS. Neither Mo nor I do.

There's a complicated explanation for why this would happen twice called germline mosaicism that involves a problem during meiosos, the process of cell division in sexual reproduction.

That's a tough concept to wrap your head around. Mo summed it up best when she said, "One of us is kicking out some messed up sperms or eggs."

The tests to find the gene are being run by Dr. Ian Krantz of Children's Hospital. He is one of two scientists in the past few years to isolate the cause of CdLS to three genes among the 30,000 or so we all have. One or the other is all it takes to cause CdLS: All are located on the fifth chromosome of the 46 that form life.

The test is hardly a sure thing. Common syndromes like Down Syndrome are easy to confirm at a microscopic level because they involve an entire missing or extra chromosome. Kids with CdLS have the correct number of chromosomes, but one bad gene within one of the 46.

The difference is like looking at a house from a satellite. Finding Down is like noticing it's missing a wall. Finding CdLS is like peering through the window, going around a wall, opening a door and noticing the missing sock under the bed.

And the results may not give us much comfort. The tests are able to find the gene only about half the time. When they do, they usually find a gene linked to classic CdLS, a more severe variety associated with greater retardation. There's a small chance of finding a gene that causes the milder strain.

Failure to find the gene means nothing. Hope still has CdLS.

But the payoff could be great. We'd like to have another child. Everything we've read or heard leads us to believe that children with CdLS do far better with siblings they can mimic and learn from. And it would help us too. We still crave those rich, mundane passages like soccer practice, spelling bees and junior proms that we may not get to experience with Hope.

Finding the gene could help our odds. Our crash course in genetics has taught us that, no matter how bad the condition, the worst odds of recurrence are 50/50. Ours are presumably more than 2 percent and less than 50.

Giggles

The fussbudget is still fussy. But beyond patience, another skill we may need as neophyte parents is perspective. Flipping through some recent video from last week, I came across these. She looks pretty happy here. So maybe, in the blur of the baby fog, it only seems like she's crying every waking moment. Maybe we should heed our own advice and not hinge all our expectations on the outcome of her latest feed. But like all wisdom, it's sometimes easier said than done.

Patience for the fussbudget

It's been one of those weeks that I'm sure all parents endure: The lovable little fussbudget whose smiles you cherish learns that she can torture you by denying them.

Atop everything, Hope seems to have a cold. We think. Otherwise, her wake-up-in-the-morning, look-you-in-the-eye and begin wailing uncontrollably routine is just downright cruel.

It's 40 minutes following that moment, after wiggling, bopping, tickling and cooing produce only more tears, that make you realize that talk of unconditional love is hollow without patience.

Neither of us have it in any great supply. Mo has way more. I start with the best intentions. Eventually, "There, there, Sweetie" deteriorates to "What's wrong?" and then "Dagnabbit, Hope!" The worst is this feeling of helplessness, inadequacy and failure to do something as simple as comfort a child. It cuts to your core: Self-worth cultivated for decades evaporates at the realizaion you can't do something that apes have accomplished for millenia. Even Britney can soothe her children (or at least hire someone to.) You are a loser.

The problems lately seem increasingly bad during feedings. Sometimes, we put the nipple to Hope's mouth and she begins to scream. We think it's because her vocal chords are swollen because they're irritated from spitting up so much.


Initially, we thought Hope was developing colic. That now seems unlikely, in part because she seems a bit old to get it now. Also, her episodes don't seem that predictable. We're hoping it's a one-two-three whammy of the inflammation of her floppy, immature windpipe (exacerbated by reflux) and a run-of-the-mill cold. Unfortunately, cold medicines are too dangerous, so we got the green-light today to increase the dose of Prevacid to reduce the acids that discomfort her. The medicine doesn't prevent spitting up. It neutralizes her stomach acids that irritate her when she spits up.



Hopefully, that will work. Until then, it's more waiting, worrying and mental pretzels, trying to shove fear into the crevices of your brain and assuring yourself this isn't the start of the boogie-woogie flu or Guinea Worm Disease.


Mo and I hashed out our fears and frustrations yesterday. It was a tough chat, admitting your failures and acknowledging that sometimes we feel completely over our heads. I beated my breasts in agony for 40 minutes. Mo summed up our charge: "It's patience. We need to learn some. That's all there is to it." So we will. Pronto.