Goody Hope

Hope got new hearing aid molds recently. They're a bit too big, making her ears jut out. Hope is so self-conscious of the goofy look that she's become adept and whipping them off seconds after we put them in.

Mo's solution: A bonnet that makes Hope look like she's gearing up to share the first Thanksgiving with those kindly Wampanoags or burn witches at the stake along with those other no-goodniks in "The Crucible."

It works. Sort of. Hope is so stunned by the fashion effrontery that she becomes paralyzed with shame. She doesn't grab her hearing aids. But she doesn't do much else either. She just sits there, dumbstruck, thinking, "Why am I wearing this silly hat?"

Too bad for her. She looks too cute to take it off.

Updates galore

In newspapers, you get blamed sometimes for failing to follow through.

Big splash on the front page: "THIS GUY IS A DIRTBAG!" Then, nine months later, the case winds its way through the machinations, memories fade, the news trudges forward, more scandalous dirtbags emerge, follow-ups get fewer and farther between until -- woopsie daisy -- a blurb is printed on page 43B reading: "Allegations of alleged dirtbaggery allegedly could be unfounded, prosecutors allege."

Critics call it a conspiracy by dirtbag newspapers to rake muck. I say it's human nature: Crises and scandal always get more attention than process, minutia and murky outcomes. When the answers aren't always clear-cut or better than you initially feared, it's even easier to forget the initial problem.

This blog is no exception. In that vein, here's some answers to issues that no longer loom as large as they did a few months ago and some that do.

The spitting of blood: Once a weekly nuisance, it's faded considerably in the past few months but hasn't disappeared. For about two months this summer, Hope would wake up crying. We'd rush into her nursery and see her laying next to a pool of dried blood. We did two endoscopies to pinpoint the problem.

Old pal Dr. Spitenup concluded it stems from her hernia and stomach rubbing against her esophagus. His solution: Well, it stinks, but it doesn't bother her, so live with it. We investigated on our own and believe it was exacerbated by supplemental oxygen fed through her nose as she slept. We have a good friend with cystic fibrosis who uses oxygen. She reasoned that the oxygen dries out her nasal passages, leading to blood that Hope swallows, and then eventually spits up.

We stopped using the oxygen at night. Viola. No more blood twice a week. But the issue persists. A few weeks ago, Hope awoke coated in a mammoth pool of blood so old it was black. We think it was from the rubbing of the hernia.

The eyesight: Six months ago, Hope's eyesight was tested as a matter of routine. The opthamalogist, a kindly, geriatric fellow who inhabits a wood-paneled office that looks like a hunting lodge and I'll call Dr. Speakupsonny, found that Hope was extremely near-sighted and would need glasses when she turned 1. We were crushed. We always thought that, given her hearing issues, it was highly unfair she'd have to wear hearing aids and glasses.

We had a follow-up appointment on Election Day. Dr. Speakupsonny found that her vision has improved, but she'll probably still eventually need glasses. We return to the hunting lodge in six months.

The laryngomalacia: Hope wheezes when she breathes because her larynx is floppy. It's a fairly common, and mostly benign, condition that most kids outgrow by about eight months. She hasn't. That's probably because she is so small. At the rate she's growing, it could be an issue for another 2-3 years. This remains a cause of concern because she could have a very difficult time breathing if she catches a bad cold. Once again, this year, she is having the monthly Synagis shot, the uber-expensive shot to ward off RSV (a respiratory virus) that is recommended for preemies. Dr. Frosty also worries that she's expending so many calories breathing that Hope has a harder time gaining weight. There is a surgery to correct the issue. We are exploring it.

The teeth: Six months ago, I foolishly wrote a blog item proclaiming that Hope was teething. She still could be. But there is little evidence of it. Children with CdLS take forever to cut a tooth. The tooth fairy got tired of waiting by the door, checking her watch and tapping her feet, and was last seen speeding away in a used K Car, listening to, of all things, "'99 Luft Balloons."

The heart: Learning my lesson from the teeth, I am declining substantial comment. For about two months, soon after her birth, this was our biggest concern about Hope. Six months ago, we were told the situation looks good. We have another follow-up appointment soon. I ain't saying another word.

The Dice-K: For weeks, Hope's favorite friend in God's green Earth was a teddy bear of Red Sox pitcher Daisuke Matsuzaka, also known in this age of lackluster nicknames as Dice-K. Lulu ate the doll. My sister faithfully mailed a duplicate. Alas, love is fleeting at a tender age and Hope's affections have wandered. Her new love: Big plastic cow on wheels.

The cutie: I am doing fine, thank you very much. And Hope is getting cuter by the day.

Bad dog

Did you hear the one about the dog who thought she needed hearing aids?

No joke. Continuing this month's theme of animal-induced melodrama, the scene was thus: Mo is downstairs; Hope is dozing contentedly, dreaming of ceiling fans; I am reading with the dog at my feet. It's an achingly stereotypical portrait of suburban idyll.

And then: Crunch, goes the dog. Huh? goes the oblivious owner. Crunch goes the dog again.

By the time I look down, the damage is done. Lulu is nibbling on one of the Oticon Vigo BTEs, the sleek, pretty-in-pink hearing aid that we spent the summer battling bureaucrats to acquire. We had them precisely one month.

We were mortified. The aids go in a hard plastic carrying case. They were left somewhere Lulu could find them, which as a friend says, is planet Earth. We were especially embarrassed because we felt like irresponsible ninnies who need to learn everything the hard way.

It went down something like this.





Fortunately, insurance is picking up the cost, and no animals were hurt in the reenactment.

We adopted Lulu as a puppy two years ago this week after listening to "Marley and Me," a tale of another bad dog who's hard not to love. Since that time, Lulu has destroyed our yard, eaten roughly 130 pairs of socks and underwear and ruined our carpet.

But she's tough to stay mad at for very long. Everyone has their thing. Lulu's is eating stuff.

On another level, we think Lulu has prepared us for raising a special-needs daughter, imbuing us with forgiveness, understanding and patience that we were sorely lacking a few years ago. Or maybe that's just a hifalutin way to say we're too lazy for more dog training.

Vision in pink

As promised, here's Hopes fancy new hearing aids, the Oticon Vigo BTE. They're sleek, they're pink and they help her hear. We couldn't ask for much more.

She was fitted for molds this week, the tailor-made rubbery things that form to her ears. They should come in a few weeks. Until then, we have standard molds, which are pretty good, but sometimes emit static because they're not a perfect fit.

We haven't tested her hearing in months, but suspect it's improving. The last time we did, she had a mild-moderate conductive hearing loss. That means that her inner-ear is fundamentally sound, but something is blocking sound from getting to it -- most likely her tiny ear canals. That's fairly common among CdLS kids. Many outgrow it. Our fingers are crossed that Hope will as well.

I'm home with Hopesy today, doing the Mr. Mom thing for the second Thursday in three weeks while Mo works. I dig it. It's a nice change of pace: hanging out, going for walks, playing with my girl while Mo makes the moolah. I could get used to this.

Happy face

This one's for you, Big Mo: Today's entry is all smiling toads, pots o' gold and bales of cotton candy. So stereotypically Irish it's politically incorrect, Mo would rather eat her own eyeballs than think someone was feeling sorry for her.

She gets peeved when I include too many details about bad days on the blog, preferring instead I skim over the ugly parts and accentuate the positive.

So here goes: We won our battle with supplemental insurance and got sleek, small and lighter hearing aids for Hopesy. And they're pink! Photos to come. Hope resumed occupational therapy on Monday. It's not as many hours as we 'd like -- or think she deserves --but Michigan is budget-cut crazy, so we're grateful for her two hours a week of therapy.

She remains the undisputed, No. 1 Cutie.

Whirlwind

Phew. It's been one of those weeks where breathing is a luxury: Hearing aids, a baby blessing ceremony/open house and endoscopy all in the span of four days. At this rate, we're planning to take up stem cell research as a hobby over the long holiday weekend.

Hopesy has emerged from the hubbub like a champ. She was feeling so miserable last week we almost canceled a blessing ceremony we planned at our house on Sunday. We postponed her hearing aids and were about 5 minutes of continuous screams away from taking her to the hospital on Friday, when Dr. Spitenup had a suggestion: Mylanta. Cherry Flavor if possible.

Eureka. It was a magic mood changer.

Despite our worries, she was a doll as dozens of people pawed over her for five hours Sunday. Kids ran buck wild. Our house is too small. We spent far too long cleaning and cooking. And it was easily one of our favorite parties ever. If it could have gone better, we're not sure how.

Tuesday, she got her temporary hearing aids. They're a bit big, but Hope doesn't seem to mind them at all once they're in. We worried about behavior issues and were warned to ease them in gradually, beginning at 10-minute intervals. But she acts like she's been wearing them for years.

We capped the excitement Wednesday with an endoscopy, a surgical procedure that snakes a tube down her throat to try to find what made her so fussy last week. We thought it was reflux. Spitenup, true to form, isn't so sure. The scope didn't find major damage that would indicate a bigger problem.

We're not sure where that leaves us -- or what to make of how much Hope enjoyed sedation. As the drugs kicked in, it was as though all her old favorites -- Dice-K dolls, ceiling fans, balloons -- were tickling her toes. She smiled. She laughed at nothing in particular. She dug the world, then she dug sleep.

And we dig her. It's been one of those weeks of extreme emotion -- up, down, anxiety, fear, frustration and elation -- that, when we finally get a spare second, make us realize that we're very lucky and proud.

No better news

Sometimes, good news comes when you need it most.

The breast-beatings, lamentations and general malaise lasted longer than we expected after last week's ear-tube tumult. We both wish it went better and beat ourselves up for pinning so much on what we should have known was a long-shot.

And it's been one of those weeks with Hope. My worry about disrupting the status quo with surgery proved prescient. The huge smiles, full-body giggles and "Oooh Daddy! You are so Funny" chortles are mostly gone. In their place are much fussiness, tears and apathy about Mom and Dad.

So, as usual, we felt physically ill sitting in the waiting room today for Hope's cardiology appointment. Heart defects are the main reason some CdLS kids don't reach adolescence.

It was a three-month follow-up to check on Hope's left ventricle, the main pumping chamber of the four that comprise the heart. It's a bit big. That's not good.

The fear is that generally chambers are big for a reason: Something is making it work too hard. We worried it is a manageable, but incurable, condition known as cardiomyopathy, which is quite bad.

It turns out that, like a lot with Hope, she may just be doing her own thing.

Her ventricle is a bit bigger than it was three months ago, but proportionately so to the rest of her heart. Blood is flowing fine. Her numbers are great. The heart is beating up a storm. Everything is doing what it should.

"I couldn't deliver any better news today," Hope's cardiologist told us. "If we haven't been tracking her since birth, we probably wouldn't have ever known there was an issue."

We'll see the cardiologist again in six months. After that, maybe once a year or maybe never.

Wow.

Now is normally the time I would pontificate: Let this be a lesson in the vagaries of life, I might say, or Sometimes, your worst fears become pussycats when you stare them in the eye. But I've learned the hard way not to tempt fate by getting too ahead of myself.

So I leave it at wow.

Wow.

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.

Simple procedure

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.

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.

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.

Stupid intuition

Every once in a while, intuition kicks in and whispers in your ear: “Life is about to turn to Hell, buddy. Pay close attention.”

So I did. There's Mo, holding Hope's finger as she's wrapped in an iron blanket and led between two massive industrial cameras. "Ain't Too Proud to Beg" is on the radio and that nice technician who seemed so chatty five minutes ago becomes very quiet as the test concludes.

Intuition is practically screaming now: "The technician knows how bad it is. That's why she's not saying anything. Hope is in rough shape. She needs surgery."

Stupid intuition. You blew it again. Hope is fine. As is her wont, she once again exceeded our worst fears and continues to confound her perplexed parents.

Hope's utter unpredictability -- great one week, terrible the next, acing tests we think she'll fail and vice versa -- may be maddening. But it's also quite endearing. Like many great artists, Hope is inscrutable. The minute she thinks you have her pegged -- boom -- she'll zing you for having the audacity of presuming she's that easy to figure out.

The latest moment came amid tests during 50-hour hospital stay that was alternately stressful and peaceful, exhausting and restful, interesting and dull and worthwhile and worthless.

We arrived home late Saturday afternoon with the exact same plan we had before setting foot in the hospital: Hope is now on eensy amounts of supplemental oxygen piped into her nose through a cannula while she sleeps. It's supposed to help her apnea, and so far, so good.

But getting there was interesting. The carefully mapped plan for the hospital of weaning her to increasingly smaller flows of oxygen and testing whether it makes her lazy quickly faded. She slept on oxygen Thursday night. The next morning, the attending physician walked in and said, "Well, she didn't have any episodes. I guess it works. What do you want to do? Wanna leave?"

The plan in tatters, we opted for another night of observation. Since we had the time, we also had Hope undergo a gastric emptying test -- a two-day affair scheduled to start Wednesday -- to measure how much food she spits up and whether some spills into her lungs.

That test, in the bowels of Beaumont Hospital, consisted of feeding Hope milk laced with a small amount of harmless radioactive material. Similar to a stress test, she was then wheeled under two cameras that took photos of the material every 60 seconds for an hour. It traced how long it took Hope to digest her food, how much was refluxing back up her esophagus and whether any of it was getting into her lungs or aspirating.

The results were a big deal. If she was aspirating, she would have to undergo a surgery to shut off her esophagus. A feeding tube would be installed at the same time. But if the test went well, it's full-steam ahead to put tubes in her ears and trim some of her larynx.

Despite the nagging, ominous warnings of my intuition, the results were tame: Hope has some reflux that comes back about a third of the way up her esophagus; half the contents of her stomach empties in about an hour; and milk doesn't get into her lungs.

Translation: Normal.

Prepared for the worst, we hadn't even considered the possibility. A day later, it's still confusing. For three months, we've based her care on the knowledge she has severe reflux. Some days, she would spit up 4 times if we didn't hold her perfectly upright. Heck, further evidence this morning forced me to change my shirt -- and that doesn't happen often.

"That little girl is becoming pretty good at making liars out of us," Mo said after the test.

If she is, she's making us happy liars. The Inscrutable Kid rides again.

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.

'That's why it's a syndrome'

When we learned Hope had a syndrome, my reaction was typically naive: "As long as she's healthy, I'm OK." Mo was quick to call me out as a dunderhead. "Kids with syndromes aren't healthy. That's why it's a syndrome."

Unfortunately, she's right. There's a gazillion biological explanations to back her up, but kids with syndromes usually have health issues -- or else they wouldn't have syndromes. That's not to say they won't get stable or live healthy lives. They do. But it's sometimes a lot of work and a lot of hassles to get there.

That lesson was reinforced today with Mo's visit to Hope's ear-nose-and-throat specialist. We were hoping he'd green-light surgery for tubes in her ears to help her hear. Her defect is caused primarily by tiny canals, which in part are caused because she herself is so small. The issue is beginning to acquire real urgency. Hope is 3 months old on Wednesday. These are critical months for her development. If she can't hear, she learns slower, talks later and bonds less with us.

Of course, there's a delay. The ENT dude, who is regarded as one of the best in Michigan, first wants to evaluate Hope's sleeping because we suspect apnea. Sometimes, she briefly stops breathing while snoozing, catches herself, then breathes again. It's fairly common among tots. But Hope's could be caused by her floppy larynx, or laryngomalacia. Essentially, the elasticity of the windpipe may cause it to close when she inhales. If it's a severe enough case, it might require minor surgery to nip and tuck her larynx into shape. And since no one likes putting 5-pounders under anesthesia more than necessary, the thinking is he'd do both at once. But first, we need to take Hope into the hospital for an overnight stay to evaluate the extent of her apnea.

Simple? It gets complicated.

The ENT took a peek at Hope's throat and found it is inflamed. That's because food is coming back up her throat because she has a slight hernia. When it does, the acids in the food irritate her throat. That makes it uncomfortable to eat. So she eats less. That slows weight gain for a child with a syndrome where it's always difficult, keeping her -- and her ear canals --tiny. So there's a convoluted line from spitting up to hearing.

Phew. I'm exhausted even typing that sentence.

The next step is a sleep study to determine the extent of the apnea. In a few weeks, we'll spend another night at Beaumont Hospital for more wires and blinking lights. Then presumably she could have surgery in March for the tubes and whatever else.

On a happier note, Big Mo has joined the blogging brigade, joining the Michigan Moms blog on our newspaper's Web site. Check it out. Poor Hopey. It's a good thing this Internet fad will never last. Otherwise, she'd really have it in for us one day.

Surgery?

Quick update: Hope had her appointment today with the ear-nose-throat dude for her hearing deficiency. Generally good news: He wants to put tubes in her ears to open what appears to be a tiny ear canal. He's talking about six weeks, maybe the end of January, for surgery that would require anesthesia. That's a bit scary, but apparently, it's about a 15-minute procedure, which is the most common nowadays on children. The protocol: follow-up checkup in about six weeks to see how she's developing, then probably surgery. If it all goes well, she should be able to hear, which would be abso-freaking awesome. It's tough reading, singing and talking to Hope as much as we do knowing that she probably can't hear us.

Mo also learned a new word during the appointment: Laryngomalacia. That's what the doc thinks is causing her wheezy breathing, rather than tracheomalacia. It's the same general idea: An under-developed floppiness that obstructs her airway, makes it imperative she avoid colds but generally is something she will outgrow.

But instead of coming in the trachea (the windpipe) it comes from the larynx (the voice box.) The doc says he'll take a peek to confirm while she's under for the tubes procedure.

More good news: Mo reports Hope was an absolute trouper during what must have been an annoying morning. She also got her Synagis shot to boost her immune system. Hope cried a bit while the doctor was jamming scopes down her ears, but otherwise did great.

She's been through too much already, but she's already making us hugely proud. It's days like today that remind us that no matter the difficulties (see self-pitying earlier posts), we're incredibly blessed and fortunate to be on this journey with this wonderful little girl.

Not perfect. Not as bad

Mo has said it plenty of times since Hope's birth, and it was true again today: Her health is never perfect, but it's never as bad as we fear.

Concerns about her hearing were confirmed today when we bundled our girl up at 6:30 a.m. and drove 40 minutes on icy streets so audiologists could attach wires and probes to her head. Mo held her for two hours as Hope slept in a cold examining room while soundwaves were pumped through tiny earphones. Using EEGs, the test measured small differences in brainwaves during different pitches and sounds.

Hope flunked an earlier screening in neo-natal intensive care and a similar test found mild hearing deficiencies in her right ear and moderate ones in her left. Today, the results were worse: Moderate in the right; little perceptible reaction in the left. Normal conversation is 60 dB (120 for Mo.) Hope can hear at 80 dB. Hearing loss in adults begins at 15 dB.

It sounds crummy, and it is. But it could have been far worse. Hope's deficiency is a conductive hearing loss. That means the inner-workings of her ear are structurally sound and there's no nerve damage. Instead, something in the outer reaches of her ear mutes sound from reaching inside. It could be malformed or absent tiny bones, fluid or slow development that has kept the eardrum from opening.

The good news is it's treatable. We were referred to yet another specialist (three and counting.) This time, it's an pediatric ear-nose-and-throat dude who will take a harder look Monday. The recourse could be small tubes to open the canal, medicine that would drain fluid. hearing aids or surgery, which isn't likely anytime soon because of her size and age.

The solution, of course, depends on the cause. But none seem entirely permanent. The hope is she could grow out of her problem with a bit of intervention. But I could be making that up. I have a tendency to do that. I am a journalist.

We'd love to say we're surprised, but we're not. CdLS kids often have tiny ear canals that take longer to develop and prompt false positives for deafness.

It takes a lot to startle Hope. That may be because she's cool as a cucumber, but she's also the daughter of two people who aren't known for their meekness. We can scream. We have a barky dog. Our smoke detectors blare so much we take out the batteries about three times a week. The Girlmore Girls chat it up with pretentious dialogue on reruns every day at 5 p.m.

None of it has phases Hope. Curious -- and a bit sadistic -- Mo banged pots and pans together the other day. Hope twitched a bit. Mo thinks.

The silver lining is that it's good to get on top of this stuff early. Master of the obvious statement of the day: Hearing is important. It helps with speech. It helps Hope bond with us. It helps when you order Thai food.

So we need to tackle this soon. Hopefully, we can get a game plan after meeting with the ear-nose-throad dude on Monday.

There's a lot to digest all at once. I liked it better when there was so little report I posted dog videos. When we think about everything Hope faces -- heart, hearing, developmental and physical problems -- it is overwhelming. We have our tears. We feel like hell. We question God. We get mad and snap at each other.

But then we have quiet days like this and we remember why it's tough to feel bad for too long.

Bushed

Powered by GoodWidgets.com

We bought a garage door the other day. Once adorned with retro eagles that Mo insisted I remove when we moved in, it broke every three months. My parents taught me how to deal with these problems: Ignore them until you can't possibly anymore, then apply the cheapest fix and forget it until it breaks again.

After years of adhering to the tradition of turning small problems into expensive ones, we followed the door's lead and broke down. Returning home from work, I was eager to see it.

I made it about three steps into the garage when Mo yelled at me through the side door.

"What are you doing?" she barked. "Get in here! I need a break."

That's life now. Five minutes looking at a door is wasted time. I'm typing with two fingers and trying to coax Hope back asleep. Mo is napping. She's making up for last night Afflicted with what my father-in-law delicately calls "the creeping cruds," I decided I couldn't help with Hope's feeds and needed a bubble-bath and nine hours' sleep to slay whatever ailed me.

Call it what you like. I consider it a valiant sacrifice to shield my daughter from germs that had nothing to do with eating six slices of pizza in 10 minutes.

Like all new parents, we knew this would be exhausting but weren't prepared for how much. From afar, it shouldn't seem so hard: You burp, feed, fuss, coo; change a few diapers, apply some creams now and again and -- voila! -- wile away the next three hours completing jigsaw puzzles or online enrichment courses.

Of course, it's all-consuming and unrelenting. I'm bushed. Mo's bushed. We're learning that raising a child with unique challenges is probably like raising a healthy one but magnified. It starts with the doctors: In addition to a pediatrician, Hope sees a cardiologist, audiologist, geneticist and physical therapist. And the spit-ups and rashes that would normally be scary are petrifying. Mo is now navigating the maze of health care and HMOs. We'd like to add a gastrointestinal specialist to the list to evaluate Hope and address her spit-ups. It's supposed to be standard for CdLS kids. Our network has pediatric GI docs. The next opening is May.

But big deal. It's work. What great in life isn't? And Hope's doing good, home now for three weeks and notching small landmarks. We don't panic anymore when we don't hear her for 3 minutes. She's becoming more emotive every day, sticking out her tongue, fixing her stares and flashing dimples like she was at the county fair. We're not counting her breaths anymore. But we are counting our blessings.

Now, the rest of our lives

So this is it. Home. Alone. Perpetually tired. A dog at your feet, a baby monitor never more than 3 feet away. No burp, wheeze, hiccup, fart or gasp left unexamined. All of them sparking new worries, what-ifs and moments of "I know we shouldn't, but let's just check the Internet this one time for symptoms of whooping cough."

I dropped off my sister and brother, Beth and Steve, at the airport today about 5 a.m. For the first extended time since Hope's birth, we have no house guests. There's no one to run errands, give us another excuse to order take-out or offer advice, reassurances and fresh insights into "Dancing with the Stars."

This is our life now: Me, Mo, two annoying cats, an occasionally behaved dog and a baby who may not need our full attention all 1,440 minutes a day but sure as heck gets it.

It's what we always wanted. And it's great, but scary. Even if Hope didn't have a genetic condition, we'd be fretting now. But she does.

We're coming to believe that caring for a special-needs child is a lot like raising a healthy one. But everything is magnified and you have a lot more doctors' appointments. We have the same fears -- is she getting enough to eat? is that sneeze the start of the Taiwan Flu? -- but less margin for error. Hope is doing great, but she may always have health worries. Kids with CdLS, her presumed condition, have a host of potential problems from feeding and hearing to motor skills and development.

Hope already has seen more doctors than I have in my life and her December is shaping up like a doozy: Cardiologists to follow up on the blood-flow issues in her heart (so far, so good, but intervention probable in her life); audiologists to follow up on her hearing (preliminary tests show she could have a mild hearing deficiency that she may be able to outgrow); physical therapy to tone those arms as well as regular appointments with our pediatrician and geneticist.

It's overwhelming stuff. We have our moments, but we try not to let it consume us. We feel terrible that Hope may have to endure more than other kids, but we are well-practiced in the art of self-help mantra: She is who she is supposed to be. Don't borrow worries. One day at a time. Don't worry about five years any more than parents with healthy children worry about them growing up to be jerks.

They may be cliches. But they're also quite true, and Hope makes all of this so much easier. We fall deeper for the sassy redhead who becomes more expressive (and attitudinal) every day.

And she's doing well. Hope isn't exactly a candidate for Jenny Craig yet, but is finally back to her birth weight. Her funny breathing -- tracheomalacia, the saggy windpipe that eventually goes away -- seems a bit worse in the past two or three days. The doctors say it's nothing to worry about, but we do anyway. She's also feeding well, although I gave the false impression last week that Hope is a breast-feeding champ. She has fed from her mother's breast. Once tremendously. Since then? Eh, not so much. But you should see that kid with a bottle of mother's milk! Chug-a-lug!

We weren't sure about the future of the blog after she got home. The crisis has passed. But the need remains for a place to post updates, keep those who are interested in the loop and minimize redundant conversations.

Plus, it gives us a chance to brag up our girl and share fantastic photos like the ones Steve shot this week.

Ain't she a cutie-patootie?