The blog lives

Contrary to my lonely heart proclamation in November that we'd do better with the blog, we clearly failed. But it's a new year, a time for grand proclamations and terrific intentions. Hey, anything is possible.

The holidays were lovely as always. Hope survived a nasty one-two punch of seasonal maladies shortly before Christmas. First, she caught a virulent stomach flu that made the rounds of Mo's family faster than the Japanese Bullet Train pulling into Osaka. This holiday will be remembered in the family annals as "Oh Yeah, That Time Everyone Was Barfing."

We feared Hope would have to spend Christmas in the hospital. She lost nearly 2 pounds through dehydration, which is the equivalent of about 20 pounds for a 15-pounder, and generally had the temperament of a damp dish towel. She did spend about three hours in the ER on Christmas Eve Eve, but perked up with the nourishing drops of IV fluid and we were discharged.

But her bout with the Ralph Macchios was only half the problem.

The clues, as they are wont to do, emerged gradually. No. 1: Hope screamed for four hours straight two nights in a row. No. 2: She would only sleep in the darkest, coldest part of the house. No. 3: She liked standing in front of the freezer. No. 4: Her eye began to cake.

Naturally, I read the signs, used my advanced powers of deduction and solved the caper: I think she's hungry. Her wails are those of a famished child confronted with a cruel paradox: She wants to eat, but cannot, because she has the John O'Hurleys.

Fortunately, I'm married to someone who may have married a lummox, but isn't one herself. Mo took Hope to the eye doctor. She had a corneal ulcer. Those are bad. They are formed when an abrasion to the cornea becomes infected. Somehow, Hope must've scratched her eye. Untreated, they can cause permanent loss of sight. If nothing else, we feared Hope might have to take a cue from Jack Sparrow and wear an eyepatch for Christmas.

But doubly fortunate, we caught it in time and, after about a week, the nasty milky spot in her iris has disappeared.

We missed most of the local festivities, quarantining Hope, but she rebounded in time to fly with us to Connecticut for Round Two of the Yuletide Hootenanny: Seven days with my family. Hope regained her weight and her spirits. We ate. We played too many not nearly enough games of Monopoly. We hit all sorts of touristy, geeky yet thoroughly entertaining historical sites in Hartford (who knew?) and generally had a merry, merry time.

Too merry to document, in fact, but not too merry to steal a video from my dear ol' Mom documenting Hope's continued efforts to walk. She's still got a ways to go, but she finds it all tremendously entertaining. And so do we.

Yuck face

We're now into Day Six of the "Whatever It It, It's Sure Got Her Sick" mini-crisis, hopeful for some serious, double-whammy, reverse-mojo "announce it on the blog and the opposite is sure to happen" luck.

After waffling, we took Hopesy to the Emergency Room about 3 a.m. Wednesday. She stayed for about 10 hours, felt a little better, and has since settled into her new, troublesome routine: Serious lethargy, a lot of sleeping, little if no appetite and the all around blahs.

Poor Mo has called the doctor about six times, we went back to the hospital Friday afternoon for blood work and are trying our darndest to avoid dehydration. But it seems like Hope is stuck in a bit of a vicious cycle: The more she feels bad, the less she eats, the less she eats ...

We're hoping it'll run its course soon. Some nasty stomach thing has been making the rounds that usually lasts about 48 hours. With her, we double that and cross our fingers. The docs say she should emerge by the end of the weekend. Still, we fret and worry. It's tough seeing our little spitfire mope around like a sack o' moldy potatoes.

Update: Hope went back to the ER on Saturday afternoon, not long after my lame attempt at harnessing the reverse mojo of the blog failed miserably. She was admitted late Saturday night and probably will be here at least another day or so.

Cardiac Christmas

Hope saw the two most important people yesterday: Santa and her cardiologist. She was ho-ho-hum about Santa, but kicked, giggled and waved her arms for the cardiologist. The girl's got some suspect priorities.

But maybe she was on to something. The cardiology appointment is a biggie. We've worried about it for weeks, in our familiar way. First, we deny and refuse to discuss. Then, as it approaches, we feel sick to our stomachs and paralyzed by dread until we're in the waiting room thinking, "Oh God, we're going to spend Christmas in the hospital."

It's not the ideal strategy, but so far, so good.

And it sort of works: Hope's appointment went well. Her heart is still a bit abnormal. She has a narrowing of her aorta, which could be suggestive of a mild coarctation. Some blood appears to be flowing a wee bit faster than it should, which could lead to other problems down the road. But not now. Her numbers are great. Her heart is working as it should. There's no change from seven months ago.

In short, good news. We don't have to see the cardiologist again for nine months.

Merry Christmas indeed. Phew.

Decision day

Today is D-Day with Dr. Spitenup, when we throw ourselves at his quirky mercy and see if Hope needs surgery to perform a fundoplication and install a feeding tube.

We pushed up an appointment set for late October when Hope added a new trick to her repertoire: Projectile vomiting. It's eased, but there was a week or so when she'd make like "The Exorcist" twice daily.

Eleven months into being parents of a special need child, we've had to re-learn worry. It's a tricky balancing act. We can't rush to the hospital every time she thew up old blood. But we shouldn't get used to it, either.

A fundoplication, which wraps part of the stomach around the esophagus, could help with her reflux. A feeding tube would allow us to continually pump calories when she sleeps to fatten her up.

When Spitenup broached the fundoplication and feeding tube a month ago, we were nervous and scared.
We've since slowly warmed to the idea and, after a summer of Hope spitting up blood and negligible weight gain, we've concluded it may even be overdue.

Ideally, we'd like to coordinate surgeries with our ear-nose-and throat dude, Dr. Frosty. He wants to trim part of her windpipe -- the larynx -- to fix her heavy breathing. He thinks part of her problem with weight gain is because she burns too many calories breathing.

But we shall see. Spitenup has a way of confounding us.

Retroactive ugh

It's Sunday morning. The coffee is strong. Mo's at church, getting religion. And enough days have passed to put the ordeal of Tuesday's endoscopy in perspective.

I wrote a scathing, gut-wrenching, "Avast ye! Accursed world!" entry on Wednesday morning, after Hope was up all night vomiting and wailing. Like shopping hungry, blogging angry probably is not the best idea.

The endoscopy, as we feared, was no fun. We highly recommend other pursuits of leisure.

The procedure involved sedating her and worming a small camera down her throat to peek at her stomach and provide clues for her occasional bleeding. That took about 20 minutes, but we were in the hospital for about six hours dealing with one indignity after another.

Two veins were blown in her head trying to get an IV for sedation. Finally, one was put through her groin, which is every bit as nasty as it sounds. She briefly stopped breathing while under. Nurses "bagged" her, or pumped air into her lungs. A gung-ho physician even tried to intubate her, working another tube down her throat in a fruitless attempt to supply a steady stream of oxygen into her lungs.

The results from Dr. Spitenup: Pretty much what we thought. One fold of her stomach is irritated from a hiatal hernia, the rubbing of the stomach and the esophagus. That's likely the source of the old blood that Hope was vomiting about once a week earlier this summer. Spitenup says the irritation shouldn't hurt her. We're not so sure.

There's little recourse beyond surgery to fix the hernia. The problem isn't bad enough yet, though, so we're forced to grin and bear it.

Initially, we were annoyed and anguished. Hope was put through the ringer to confirm what was suspected, but fix nothing. We kicked ourselves as bad advocates for her care, second-guessing the decision to go through with the endoscopy.

After a few days, we're more even-keeled. Hope has rebounded well from the procedure, which we acknowledge is necessary to confirm the problem before intervention.

And as always, we're still conflicted about Spitenup. We're sore he surprised us with his decision to go ahead with the endoscopy over a holiday weekend after indicating it may not be necessary. His bedside manner alternates between non-existent and strange (After the procedure, he approached us in the waiting area, motioned us inside and offered a barely audible grunt.)

No doubt, Spitenup is a stinker. But he's also been right about everything so far.

And we know our angst at him is window-dressing. The real source of frustration, such as it is, is seeing something hurt your baby and not being able to fix it.

On again

Our pal, Dr. Spitenup, continues to confound. Hope's poop tested negative for old blood Friday. We thought that would trigger more monitoring. Instead, Spitenup threw a curve and wants to go ahead with the endoscopy sceduled for this Tuesday.

We're not thrilled. Hope has been in such a giddy mood lately we'd think she was on goofballs if we didn't know better. We're anxious about messing with that mojo, but eager to try to learn more about the source of her intermittent bloody up-chucking.

Like most with CdLS, Hope still struggles with feeding, reflux and digestion. We've stuffed her like foie de gras. She's taken to solid foods -- particularly oatmeal and peas -- like Mikey to Life Cereal. Even so, her slow weight gain has slowed even further. She's put on only about a pound over the summer. Our goal of having her hit 10 pounds by 12 months seems unattainable.

If the endoscopy can provide some answers, we'd be thrilled. Our fear is the opposite: We'll subject her to tremendous discomfort and learn nothing. The alternative, of course, is inertia and that does no one's Breck girl any good.

Speaking of which, we're not being modest about Hope's supposed debut in the CdLS Foundation's monthly newsletter. We've heard rumors for a week that her photo graces the pages, but we're stumped.

We play it cool around the mail lady, but peep out the windows in anticipation whenever she's in the neighborhood. Super-duper telekinetic powers to the contrary, our copy has yet to arrive. But prepare for some serious chest-thumping, "Yeah, that's our girlie" when it does.

Poop collectors

One terrific thing about parenthood is the intensity of the experiences. Joy, wonder, pride, worry and love are redefined in ways so transcendent you question whether you fully experienced them before.

Then there's the little stuff, like poop.

I thought of that this morning when I applied a Popsicle stick ever so delicately to Hope's diaper and smeared it on a small sample strip. Moderation was key. Wielding a metaphor like few can, our gastroenterologist, Dr. Spitenup, warned, "Just a little will do. Don't put it on there like a prostitute putting on makeup."

Oh, Dr. Spitenup, how we've missed you.

Like Chuck Berry, zoologists or those guys in "Jurassic Park," we're collecting poop this week. Even the package is creepy: We wipe the poop on a piece of cardboard with three little pockets for each days' sample. Each package is labeled, "Hemoccult, The World Leader in Occult Blood Testing."

Insert joke here about "Rosemary's Baby." But if you're going to test occult blood, it's nice to have the world leader.

Anyway, the hope is to get to the bottom of Hope's sporadic old-blood belching episodes. They were weekly occurrences in early summer and have since tapered, but haven't completely gone away.

Spitenup's hunch remains that as Hope inches closer to sitting up -- she is getting there but has a ways to go -- she is using more of her stomach muscles and irritating her hiatal hernia. She is scheduled for another endoscopy Tuesday. That's a terrible out-patient procedure she underwent in spring that requires sedation and snakes a scope down her throat with an attached camera. It throws her for a loop for about three days.

If there's no blood in the poop, we can probably avoid the prospect and keep monitoring the situation. Dr. Spitenup doesn't like to be rushed.

We find out Friday. Mo is dropping off the specimens on her way to -- ahem, ahem -- work.

That's right. She made the monumental decision to return two days a week, starting today. It hasn't been easy or without its share of Spitenup-worthy gut wrenching. Mo went on bed rest several weeks before Hope was born, so it's been nearly 11 months since she worked. She worries about getting back into the grind. We're anxious about leaving Hope in the hands of others.

Ultimately, we think it's the right decision for her and for us. The nice thing about working two days is that, no matter the stress, tomorrow is always Friday or the weekend.

We found a nanny nearby to watch Hopesy on Thursday. She has experience watching a child with a similar syndrome, so that is comforting. Fridays, Hope is whooping it up with super-nanny, Granny Clem. I'm not sure who is looking forward to it more.

Covering the bases

In journalism, this is called burying the lead. In comedy, it's called a delayed punchline. Either way, it goes something like this:

Mo: You really should post a blog update saying Hope is feeling better.

Me: Uh, OK. (Thinking to myself: "Damn, every time we announce Hope is doing well, something bad happens.")

We're not sure this qualifies as good, bad or neither, but two hours after posting about Hope's triumph over Roseola, an envelope arrived from Beaumont Hospital's Genetics Department. Inside was the long-awaited results of her genetics testing.

The short answer: Hope has a mutated gene typically associated with classic Cornelia de Lange Syndrome.

That is the more severe variant of the syndrome that poses more challenges.

Using microarray analysis, a high-tech look at microscopic spots of DNA, researchers at the University of Chicago analyzed one gene. It's called NIPBL. The test essentially checks to see if the sequencing and building blocks of the gene are as they should be.

Attached to the letter were the laboratory results in big bold, upper-case letters is a word we've seen too often and still hurts to see: ABNORMAL.

It's complicated stuff, but the Cliff's Notes version is that genes are recipes for making proteins that, in the formation of life, tell the gooey blob that becomes a person how many amino acids to string together. At first blush, it seems cruel and capricious that one gene among 20,000-40,000 could have such a profound effect: everything from small stature and arched eyebrows to retardation and limb abnormalities.

If she had to have a mutated gene, why couldn't it be the one that makes cilantro taste spicy?

It's not that simple. NIPBL is an important gene. It's something of a regulator that tells other genes how many amino acids to produce. It's not a perfect analogy, but it's almost like a bad Christmas tree light: One blown fuse can dim the entire line.

The other gene associated with CdLS is called SMC1A. Those who have that mutation tend to have a milder form of CdLS and fewer challenges. It's sometimes so mild that parents may not suspect something is wrong until their child is 3 or 4 or older.

We always knew that wasn't Hope. We've debated the severity of her condition -- and still do -- but knew she isn't mild. Geneticists have a checklist for CdLS. Hope has at least a dozen of the indicators.

So we suspected the news was coming, but there's still comfort in uncertainty and a maybe, just maybe sliver of buoyancy that our hunches could be wrong.

They weren't, but the letter brought some relief as well. Our game plan all along to undergoing the test was two-fold: To help the understanding of recurrence of CdLS by participating in a Children's Hospital of Philadelphia study of parents with multiply affected children; and to help ourselves with future pregnancies to maximize our chances of a child without the syndrome.

That could means more tests, perhaps of Will's banked blood and perhaps ours as well. The hope is that by isolating the gene, a fertilized egg could be tested for that gene's mutation before implantation. It's an oddly sterile and slightly creepy concept, but such are the hands we're dealt.

And they're not bad hands at all.

I was sad when I read the letter. We have a better sense of Hope's future than we did yesterday, and it can be scary in its totality. But microarray analysis is a crap shoot: In the best cases with CdLS, they're only able to discover a mutated gene 50 percent of the time. And failure to isolate the mutation changes nothing. It doesn't mean children aren't classic or mild. It simply means they couldn't find the mutation.

In that sense, we're relieved.

I was worried about Mo's reaction. She sighed, whispered "that sucks," and then came home and held our baby.

Hope laughed and smiled. We don't know what the future portends. There's still a range of abilities in classic CdLS. After a few months of stridently checking the development chart, we're now focused on what she can do, not what she can't. And Hope is doing so much and making us so happy that it's impossible not to be optimistic. We named her Hope for a reason.

Mo re-read the letter. It was from one of our favorite doctors. We like him so much because he gives us such great material. Straight from Central Casting as the intrepid, nerdy scientist, he's big on footnotes and thoroughness.

Delivering the news we wish we didn't have to hear, he closed a letter dated Aug. 13 with "With my very best wishes for the Holidays and New Year."

"Well," Mo said, folding the letter. "I'm glad he made sure to cover that base."

Arrivederci Roseola

Sound the fuglehorns and cue the choir: Our girl is back. Some 10 days after a nasty bout of Roseola, Hope seems to have finally licked the mighty bug. She's been rebounding all week, but seemed to finally return to her own Friday, playing with toys and smiling her patented billboard-sized grins.

Hope's also mastering a nifty new trick: Grabbing her oatmeal spoon and jamming it into her mouth with roughly the same vigor and grace as her Mom attacking a box of Cheez-Its. It's great to see.

We're not completely out of the woods. Are we ever? After a month sabbatical, she spat up more old blood overnight, a sticky, thick glob that at first blush could pass for motor oil. Our old friend, the mercurial Dr. Spitenup, wants another endoscopy in early September to see what's what. He's been pestering us about it for about a month, when she was spitting up old blood with greater frequency. We were reluctant because it's requires her to go under and throws her off kilter for about three days. But it's probably a good idea.

A gazillion apologies for being such a lackluster blogger of late. It's summertime and we seem to be in a perpetual state of frenzy. Mo's busy preparing for her sister's wedding next weekend and lining up a nanny to watch Hopesy one day a week when she returns to work part-time in a few weeks. I've been entrenched in the newspaper's coverage of the never-ending train wreck/soap opera/police blotter/political intrigue/funny pages that is Detroit City Hall.

Hopefully, things will return soon to some state of normal. Until then, it's great to see our girl smiling again.

Sluggish return

Form held true this week. So much for the perfect homecoming.

Hope and Mo arrived Tuesday. There were kisses, Welcome Home signs and lots of sleep. Initially, that seemed understandable. Their trek home from Maine began at 4 a.m. and spanned two flights.

When Hope slept all day Wednesday as well, we worried. When her temperature reached 102, we knew something was wrong. She was listless. She didn't make eye contact. She would sort of wake up for a half-hour, gulp some food, and then fall asleep halfway through.

We phoned the doctor on Thursday and went in on Friday. He seems to think she has a viral and respiratory infection. He didn't seem altogether concerned -- it's the summer sick season -- and prescribed Albuterol to relax airway muscles and increase air flow into her lungs.

Her fever broke today, but it was replaced by a splotchy rash that began on her neck and spread to her back. It may be the virus expunging itself, but it also seems consistent with Roseola, a fairly common and quite contagious virus. Our doctor downplayed the possibility she caught it on the planes, but it seems like a good bet. She's a bit better today, but still pretty sleepy. We go back to the doctor on Monday.

She's had a few bouts of colds, but we've never seen her so completely listless and uninterested. It's tough to see your little one so out of it. She was a super trouper in Maine, in great spirits and charming strangers. I had a hunch she would have a tough time adjusting to home after almost 20 days away, but this was a bit scary.

Hope seems to be turning the corner a bit. She was eating like a champ today and making a run at grabbing the glasses from Mo's face.

We're happy she's home. We'll be happier when she's all the way back.


(thanks for pix, pop)

Fed up, Spitenup

The more I learn about Cornelia de Lange Syndrome, the more I realize the health issues keep on coming.

We're reminded of that again this morning after Hope spat up another batch of old blood. It's becoming a weekly ritual. Last Friday, we were at the movies. My mother and sister babysat. Mo ducked out, placed the obligatory call for reassurance and heard: "Well, she soaked her shirt and bed with what looks like blood and bile ... But she seemed really happy afterward."

She's had about five episodes, each about a week apart, since an endoscopy in late May showed no major damage to her esophagus from reflux. She underwent that procedure -- a scope snaked down her throat -- because we found a few drops of old blood. These are geysers.

The mercurial Dr. Spitenup is on the case. He examined the blood, pontificated and analyzed before arriving at his diagnosis: Hey, it happens.

For months, Hope has moved toward sitting on her own, crunching her abdominal muscles like Jack Lalanne. She's not quite there, but Spitenup thinks the pressure is exacerbating her hialtal hernia, a mostly benign condition in which her stomach pushes into her esophagus. He doesn't think it's a major issue unless it happens more frequently.

Our answer: Uh-unh.

We're pressing Spitenup yet again for better answers. It's taken us months to grow accustomed to his strange ways. We've come to respect him because, so far, he's been spot on -- even if his bedside manner is straight out of 20 Questions or a police interrogation.

But this is different. If he can't make this stop, pronto, we're going elsewhere. Among all of life's nuisances, spitting up old blood doesn't strike us as something to get used to or shrug off.

Seeing hints

Apparently, our girl's been sending us unsubtle hints.

Hope has a new obsession. Dice-K is yesterday's news. Ceiling fans are passe. Eyeglasses are all the rage. Hope leans over, snatches them off Mo's face and obsessively fiddles and diddles. More! More! Four times is never enough. She digs it so that Mo has had to teach Hope a new word to stop: No.

We're not wild about it, but Hope may soon be able to play with her own glasses.

A routine eye appointment diagnosed Hope as very near-sighted. The opthamologist thinks Hope may need glasses in a few months, when she turns 1. We were floored. Vision problems are common with CdLS kids, but Hope's focus on toys -- and earlier tests -- fooled us.

The toughest news is the kind you don't expect. Glasses aren't the end of the world, but the thought of our little girl in them at such a young age made us sad. Logistically, we're not sure how she could even wear glasses and hearing aides at the same time.

It could be worse: Tests showed equally bad vision in both eyes. That means she's not favoring one eye over the other. If she was, vision in the weak eye could continue to deteriorate.

We have some time to think about it, because we're not due back for five months. She'll be re-tested before we proceed on anything. So we move forward, shove the worries into the recesses of our minds and hope that form holds true and issues with our girl aren't as bad as they initially seem.

Mystery solved?

We like to think we're clever people. I almost solved a Suduko once. Mo kicks some serious patootey at Scrabble. We're both self-aware enough to know that few things can make you feel so dumb, so fast as child-rearing.

We've been in fits over Hope's mystery ailment. She has a bad cough. She's running a low-grade fever. Her diaper rash looks like the surface of Mars. She drools like Marmaduke eying the mailman.

Last week, we took Hope to the pediatrician. The diagnosis: Who knows? Maybe she has an ear infection. The treatment: 10 days of bubble-gum flavored antibiotics.

The drool, two-pack-a-day cough, diaper rash and boogies persisted.

Next came Internet research. Is it allergies? Do we need to find a good home for Lulu or -- horrors -- actually start cleaning the house?

We were onto the rock 'n' roll pneumonia and the croup before Mo put it all together.

Bad rashes. Coughing. Drooling. Irritability. Mo stuck her hand in Hope's mouth.

Son of a gun, we think our girl is teething.

One of the dangers, I think, of having a child with a fairly exotic genetic condition is that sometimes we skip over the simple causes for her discomfort.

But it's sometimes complicated stuff: We got the tests back from Hope's endoscopy a few weeks back to measure her reflux and determine why she spat up blood. Once again, the inscrutable Dr. Spitenup threw us for a loop. The results showed mild inflammation of the area near where the stomach and esophagus meet.

Translation: A bit abnormal but not the horror show we expected. So, once again, we've dodged our worst fears. And for that, we're happy.

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.

Mojo interrupted

We should have seen this coming from Mars. We broke every difficultly learned lesson. We messed with mojo, scorned superstition and jinxed our juju.

Never, never, never tempt the Fates so brazenly as to post a video online to the tune of "Happy Baby."

Since it's been up, she's been anything but. Like a moldie oldie, all her Greatest Hits are back: Inconsolable tears, fussiness, whining and reflux.

For the first day or so, we thought it was cyclic. Then, we worried about a cold or allergies. When she spat up what appeared to be old blood yesterday, we knew it was time to call upon our old friend, the gastroenterologist, Dr. Spitenup.

He shined a special light today on the evidence -- a dirty bib -- and confirmed our fears. It's blood. She's not just depressed about the cancellation of "Gilmore Girls." There's a serious rum-tum-tum in her tummy and throat. So it's time to take a look and get serious, once again, about her nemesis, reflux.

She's scheduled for an out-patient endoscopy next Wednesday at our home away from home, the Pediatric Intensive Care Unit at Beaumont Hospital. She will be sedated. Spitenup will snake a scope down her scope and assess damage to her esophagus. If it's isolated, we'll likely stay the course of twice-daily treatments of Prevacid. If it's not, we'll have to get more aggressive and perhaps try more medicine.

Quick lesson about reflux: It's the bane of kids with CdLS. The stomach shoots liquid up the throat. Sometimes, it is spat out the nose or mouth. More often. it is swallowed. It's heartburn and majorly annoying. Aside from surgery, it isn't solved but managed with medicines like Prevacid that neutralize acids and prevent corrosion of the esophagus and mouth.

We have come to respect Spitenup and his mysterious ways, but his conservative, stay-the-course approach sometimes rankles. We're bummed to return to the hospital -- even for just an afternoon -- for the third time in as many months. We're sadder still that the baby who was so happy a week ago is so miserable. But we're glad that Spitenup finally seems to be taking us seriously. For too long, we've stuck with wait-and-see. It's time to act.

And this time, we've absolutely, positively, cross-our-heart, rub-a-rabbits-foot, find-a-four-leaf-clover learned our lesson. No more sweeping declarations announcing the glorious state of Hope to the world.

From now on, it's strictly facts ... unless of course, the reverse-reverse-mojo rules apply and the mere act of stating that she's miserable leads to a dramatic turn in her temperament.

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.