Fundraising, surgery and a helmet

A gigantic thank you to friends and family near and far for another successful Walk for Will and Hope. This year, we drew about 75-80 walkers for a picture-perfect 2-mile stroll. The weather was unseasonably warm. The leaves were magnificent. The company was terrific. Hope and Oscar were in fine spirits. We really couldn't have asked for much more.

We're happy to report we raised another $4,500 for the Cornelia de Lange Syndrome Foundation, pushing our two-year total past $10,000. It means so much to us that we could do this to honor Hope and Will, who would have turned 6 five days after the walk. It's hard to believe it's been that long. We miss you, pal.

It's also hard to believe it's been 18 days since the walk. A whole lot has happened since then. About five days after the walk -- two days after we put my parents on a plane -- Hope had her supraglottoplasty surgery. That's the surgery to correct her laryngomalacia, a floppiness of her airway that made her seem like she was wheezing or snoring when she breathed.

Laryngomalacia is fairly common and benign among newborns. Most outgrow it in about 12-24 months. But most aren't nearly 3 and still about 16 pounds. We worried Hope's breathing was compromised by the floppiness of her airway and that she had a tougher time getting over colds because of it.

The surgery involved cutting floppy skin from her windpipe that caused the wheezing. It required anesthesia and putting an oxygen tube down her throat during the procedure. So naturally, we were freaked out. We were doubly freaked when we decided on the eve of surgery to read medical studies about what could go wrong. Never a great idea.

Hope was a champ. She left the hospital that afternoon, was a total pill that night, but bounced back to her old goofy, sweet self the next day. Her breathing improved immediately. It's odd not to be able to hear Hope from 50 paces anymore, but wonderful.

Both Oscar and Hope are doing great. We realize how infrequently we've updated the blog when we look back and realize what we've neglected to mention.

Hope started school in September. I drop her off about 9 and Mo picks her up about 3. We were dreading it. But Hope seems to be doing great. In a little more than a month, she's begun potty training and is weaning herself off bottles. It's five days a week and Hope is usually pretty exhausted -- no naps for little girls -- but she's been really happy since she started.

Oscar got a cranial helmet to correct a flat spot on the back of his head about three days before the walk. We were bummed, of course, that he'll need to wear it 23+ hours a day for the next 2-4 months. He may look like Spaceman Spiff, but he is utterly unbothered. His attitude seems to be: Hey dudes! I'm wearin' a helmet!

It's ironic, after nearly three years of fielding questions about Hope's size or her glasses, to answer questions about Oscar's helmet. The other day, in the Post Office, a clerk asked Mo if it was a fashion accessory. A few weeks ago, a girl at a toy store pointed at Oscar and screamed: Look! A baby in a helmet! Her embarrassed mother found us later and tripped over herself apologizing. I told my daughter, some kids are just born sick. We don't make fun of them.

Lordy, lordy, lady. Quit while you're behind.

There's a whole lot more, but not enough time. We're busy making cupcakes and preparing for Hope's third birthday on Halloween. She's a fairy princess. Oscar is a monkey. We think they look pretty cute. But neither seems thrilled by the prospect.

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.

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.

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.

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.

Back to the hospital

Hope is going into the hospital in the next day or two for 48 hours of observation. It's nerve-wracking, but we're trying not to get too freaked out.

The dreaded results of the sleep study returned. We knew they would be bad. They were worse than we feared: Severe obstructive sleep apnea marked by an alarming number of episodes of suspended or shallow breathing.

We quibble with the severity of the findings. We know our daughter, have witnessed her episodes and have already ranted about the wisdom of putting 47 wires on an infant in a strange room and telling her to sleep tight.

But we don't disagree with the conclusion. She has apnea and we need to do something.

Like everything else with Hope, the solution is complicated. Ultimately, she could still get some of her larnyx trimmed during her surgery for ear tubes. There's some thought that her laryngomalacia or floppy airway may close while she's sleeping and cause the episodes. Or perhaps it could be her reflux and milk is coming back up while she's dreaming about frogs and flapjacks. Or maybe there's another cause altogether.

Either way, we need to figure it out, so we're glad in a way that things are moving quickly. Left untreated, infant apnea can lead to all manner of woe. At the very least, it makes her crankier than her father with five O's and two E's in Scrabble.

We're in the process of evaluating the apnea. Separate tests are scheduled Tuesday to help determine the extent of her reflux. In the meantime, her pediatrician recommended Hope receive supplemental oxygen at home while she sleeps. This would consist of a tank and a small strip between her upper lip and nose that would provide a little boost if necessary.

We were set to proceed with the plan until we met yesterday with the neurologist who monitored Hope's sleep study. His caveat is that not everyone does well with oxygen. Some get lazy, let the supplemental stuff do the work for them and problems worsen.

So we're off to the hospital so doctors can evaluate how Hope reacts to the oxygen. She'll be hooked up to a few more tubes and it undoubtedly will be a major stress for all those involved.

We know this is the right thing for her. Of course we wish we weren't in this position. But our fanciful days of delusions have long since faded. We know this isn't the last time she'll be in the hospital over the next couple years while we stabilize her health. It stinks. But it's true.

The timing is a major bummer. I was ready to fly solo this weekend while Mo went Up North for her annual Women's Weekend retreat, where she laughs and bonds with old friends with hot tubs, large glasses of wine and nonstop talk about how they couldn't possibly live another day without their hunky fellows.

Mo has looked forward to it for months, first worrying she'd miss the festivities, slowly warming to the idea and finally lining up baby-sitting reinforcements in case I wasn't up to the task. It's soul nourishment and she'll miss it. I feel terrible for her. She could use a break.

But life is different now. Not worse. Not browbeating, fists to the sky in grief. More challenging, sure, but also more rewarding because it includes the Hopester.

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.

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.

'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.