We wait

Hope has a big heart. It sounds like swell news and something she comes about honestly -- Mo is nothing if not magnanimous -- but it's probably not.

We returned today to the cardiologist for a follow-up appointment. Of the issues that send Hope once a week to various specialists, her heart defects are the ones that terrify us. We fill notebooks cataloguing her daily feeds and weight, debate the quality of her poop and sleep for hours and absolutely delight discussing her latest smiles, farts and grimaces. But we barely talk about her heart issues because they're that scary.

Or maybe they aren't.

As we learned six weeks ago, Hope has an abnormally thick left ventricle. That's the main pumping chamber of the four that comprise her heart. It's on the mild side of big -- we're talking perhaps 5 millimeters compared to 3 -- but it's definitely measuring larger since her birth. Presumably, that's from overuse. Like a muscle, the heart grows bigger when it's asked to do more. It shouldn't have to do all that much for a little girl who turned 3 months old today.

Oddly enough, Hope's heart appears to be working well. Her blood pressures indicate it's doing a bang-up job distributing blood through her body. The flow through her heart seems fine as well. And the cardiologist today ruled out aortic coarctation, a pinching of a valve like a garden hose that causes blood to flow with increased velocity -- and worried us the most just two months ago.

So, like a lot in life and medicine, we're left with an unknown that's only resolved with time.

If her heart is growing thicker, it appears to be doing it on its own. There's no blockage making it work harder. The tissue itself could be abnormal, a very serious condition known as cardiomyopathy. We don't know now. This could go away, be a lifetime condition managed -- but not fixed -- by medicine, or it could eventually prove fatal.

So we wait.

The unknown is frightening. We're not good at it. But there are worse outcomes. The thickening isn't severe enough to rush us back to the cardiologist soon. We have a follow-up appointment in three months. We left feeling OK. The morning was stressful -- Hope melted down during an echocardiogram, EKG and blood-pressure screenings -- but our big, unspoken fears weren't realized. The tests didn't find more problems to require immediate surgery.

Driving home, we talked about the last few months and years. I launched into an empassioned diatribe of gobbledygook about how this is the defining moment of our lives. It sounded awesome for about 40 seconds. Then, as is my wont, it quickly made no sense.

Mo cut me off: "If anything, this just teaches me once again that we need to savor every day like the gift it is." She's right, of course. I'd be tempted to say she has a big heart if I didn't just learn that isn't always a good thing.

'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.

Reality returns

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I put my parents on a plane back to Maine today. It's been almost 20 years since I left for college and I've perfected the routine: No awkward moments, swallowing tears, blubbering sweet sorrows or speechifying; just give 'em a hug; honk the horn at the curb and hit the gas. I save my tears for later.

They were here for five days and it was great. Both have a great rapport and gentle manner with little Hopesy. I took the time off work and it was nice to soak in the goo-goo ga ga time, celebrate Penguin Awareness Day and pass off a few marathon feedings to good ol' Mom. (Free marriage advice: If you and your spouse want to reconnect for a first night alone in three months, "There Will Be Blood" is not a great date movie. I know. It's shocking.)

But sure as dawn, reality returns. So today it was back to work, and Mo was back for Round Two with the inscrutable Dr. Spitenup, the pediatric gastroenterologist who wears grape-flavored gloves, takes no measurements and dispenses diagnoses through a rapid-fire series of questions that requires us to quantify the problem through percentages. Mo told him Hope still has 71 percent happy days, but falls asleep midway through about 40 percent of her feedings and seems about 17 percent less interested in milk the past two weeks. Dr. Spitenup told Mo that he now realizes Hope has red hair and told her to come back in six weeks.

Good ol' Dr. Spitenup. I wish I could find a dentist like him ('My teeth feel 98 percent clean. See you later!')

Tomorrow, Mo finalizes the last of an exhaustive process of meetings and paperwork to enroll Hope in Early On, a state program for developmentally delayed children that should connect her soon with an occupational therapist to work on feeding and other issues.

Joy

Sometimes, on this blog, I worry we dwell on the doctors and the challenges and not the joys. This short video, shot by my Mom during a five-day visit, does a better job at capturing them than any of my purple prose.

Breakthrough

Change doesn't come easy around these parts. I lived like a college student until I was about 32. Mo still thinks Madonna is cool. And Hope has steadfastly clung to being a 4-pound baby.

For days, we've eagerly weighed her, hoping she would break into into the ranks of the 5-pounders. For days, the digital scale atop her dresser stubbornly read 4 lbs, 15 oz.

Even with our sundry doctors appointments, Hope's biggest issue day-in, day-out is feeding. On paper, it should take about 8 hours of the day. In reality, it's a round-the-clock gig. Every three hours, she gets about 1 1/2 ounces for a solid day of 12 ounces -- the equivalent of a can of Diet Pepsi that we gulp in 5 minutes. Sometimes, we cajole more. Usually, she's not having it.

It takes about 30 minutes of burping, cooing, tickling and soothing to get it down. That's followed by another 30 minutes of holding her upright and playing with her to ensure it doesn't all come back up. Theoretically, it's off to bed before the process repeats. But often, it's back to bed for 15 minutes, wakey-wakey "Me so mad!" for another 15, more cooing and soothing, then back to bed.

So we were happy today when the scale hit 5 pounds. For those keeping track at home, that's 17 ounces in the 80 days since her birth and 18 ounces in the 64 days since she left the hospital (she lost some weight.) That's about 2 ounces a week, which at this rate will have her on Jenny Craig in about 72 years.

Seriously, we're happy to hit this milestone. It's going to be slow going. We know that. We'd just like to jump-start the process a bit to make it easier, so it's back to Dr. Spitenup on Thursday to discuss our options and her progress.

A diagnosis

When Mo's pregnancy first showed signs of trouble -- measuring small, low levels of a certain protein, heart defects -- our reaction was perfectly 21st century normal. First, we cried. Then, we Googled. Mo found a delightful condition -- Williams Syndrome -- that seemed to match all our problems. We told ourselves, "Man, if our daughter has to have a genetic condition, we hope it's this one." Kids with it are mildly retarded, extremely sociable, love to laugh and are excellent musicians. It's like DudleyMoore-Itis.

Few would have similar reactions to Cornelia de Lange Syndrome. It terrifies us if we think about it in its totality: retardation (though some have normal intelligence); speech delays that can last into adolescence and limitations that linger for a lifetime; extreme shortness; behavior problems; digestion issues and a host of health concerns that cause many to die before their parents.

Hope was officially diagnosed with it Tuesday.

Our geneticist, who writes 12 paragraphs explaining that he has nothing against blue, yellow or chartreuse when you tell him he has a nice red tie, surprised us by clinically diagnosing Hope. That means he took a long look at her and made the call after seeing numerous similarities with CdLS kids -- including small hands and feet, a thick head of hair, a button nose, mile-long eyelashes, pointy pinkies and several other markers. We're also following up with an analysis of her blood in a 50/50 bid of zeroing in the faulty gene that caused the condition.

We knew the diagnosis was only a matter of time. In some ways, it's a relief because CdLS has a better support network than some other conditions. But it's still sobering to erase any last vestige of that late-night, "Hey, maybe they got it all wrong" false optimism.

We've been circumspect about CdLS on the blog because Hope lacked a diagnosis and we don't want people to judge her. We refuse to allow CdLS to define our daughter. She's Hope B. Kurth, the little auburn-haired cuddler with Greta Garbo eyelashes and Betty Boop eyes. And like most other genetic conditions, there's a variation of severity. There are signs to indicate Hope has a milder strain. There are others that aren't so encouraging.

The sense we get is things will settle down into some new version of normal after one or two years that are challenging as hell: A gazillion doctors; anxious and fearful nights; and a tricky social adjustment from having strangers say "What a cute preemie" to asking us why our year-old daughter is 10 pounds.

We still don't have any great explanation for why this would happen. Basically, it's one -- perhaps two -- mutated genes among the 28,000 to 120,000 that we all have. Unlike other conditions, CdLS is dominant. That means you can't be an innocent bystander carrier: If you have the gene, you have the condition. Neither Mo nor I do, so Hope's condition was caused when sperm met egg and one or two rebel genes refused to cooperate.

We wish Hope didn't have the condition, but we believe that she is who she's supposed to be. The wacky hippy church we've attended for the past few years calls everyone "individualized expressions of God." That has a nice ring to it with Hope, who I'm sure isn't nearly as angst-ridden about her diagnosis as her parents. That's important: It's not about us. If she's happy, we're happy and to hell with the rest.

So the fears and meditations can wait another day. Hope's most pressing issue today is that she's a baby. And she's doing a fantastic job at being one. Today, she graduated from the finger sandwich to the full-on thumb suck. Sitting on her Mom's knee, after a feeding, Hope looked us in the eye and thought, "Check it out, I'm putting this thumb in my mouth and giving it a suck."

It was wonderful and so gratifying. We laughed, smiled and felt proud. And it made me think about another difference in raising a special-needs child: We may have to wait a bit longer for some of the rewards, but when they come, they're extra-sweet.

Club Meds

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The timing of the weekend may have made us predisposed to being emotional. Or maybe I'm still not used to hanging around specialists' offices with cold and spooky equipment.

Either way, I'm still moved thinking about a defiant little dude who absolutely refused to go into radiology. It was Friday. We were in a waiting room at Beaumont Hospital before Hope's barium swallow test to examine her upper gastrointenstinal system and get a better handle on her feeding issues.

The kid was about 7. He was clearly no stranger to waiting rooms. He wore a cartoon-decorated hospital gown and a serious frown. His mother was trying to cheer him up: "No needles today! Isn't that good? It won't take long this time. You should be happy."

The kid hung his head. His mother kept talking, trying to encourage him. Eventually, he stuck his fingers in his ears and screamed: "Please Momma! Stop talking! Leave me alone! Leave me alone!"

Five minutes later, we were undressing Hope before her 15-minute test. She lay on a massive, movable X-ray machine and sipped milk spiked with sugary barium, a chalky additive that contains enough metal to show up on X-rays and allows doctors to trace the flow of milk. It would have been cool if it wasn't so freaky. Eying a TV monitor, we watched as pillowy puffs of milk eased down her throat. They looked like an inkblot and moved like a tiny jellyfish, shimmying and contorting until settling into her stomach.

We got the test because Hope isn't a world-class feeder and it is standard procedure for CdLS kids. True to form, the results weren't perfect but could have been worse: Hope doesn't have a malrotated bowel, but she does have a mild hernia in her stomach --a hiatal hernia -- that bulges slightly into the opening of her windpipe. It no doubt contributes to her difficulty sometime keeping food down. Sadly, it's a condition more typically associated with overweight, heavy smoking 60 year-olds than 2 1/2 month-old girls.

We're not sure what's next. That's a decision for Dr. Spitenup. The sense I get is that it's not the biggest deal, could be mitigated by Prevacid to minimize the amount of acids that could come back up, but may eventually require surgery if Hope has to go in for something else.

We were were back into the waiting room after comparing Big Ten notes with the doctor who performed the test. That's a requirement for docs delivering bad news: Always follow-up with something trivial, smile, then run like hell. This guy looked at Mo's MSU hat, remarked "I like to look at hats. I am from Wisconsin" and bolted out the door.

The sad little boy was still making his stand in the waiting room. Now, there were two doctors and two nurses standing above him. His head bowed deeper, he refused to look up as they begged "C'mon! What do you say? Let's do this test. It won't take long!"

We dressed Hope, put her in a car seat and walked out the door while the scene still unfolded. I left feeling incredibly sad for the kid -- wondering why he's been the hospital so much to be so adamant, feeling sorry he has to go through that and realizing that we're part of the same club now too.

Mighty Will

Our son, Will, died three years ago today. Like today, it was a sunny and unseasonably warm. Like today, our hearts were heavy.

Will was a good boy who had to endure too much in his 89 days on Earth. He never left the hospital, but made us happy and exposed us to an unconditional love that we didn't know existed and are tapping still today.

Loss is unpredictable. The days you think are going to be the worst often aren't. We are thinking about him a lot, but aren't planning anything special. We try to mark the anniversaries of his birth and survive the anniversaries of his death. Today, we're just holding Hope and bathing in her smiles.

It's true what they say: It gets easier with time, but you are never completely the same. We're happy Will is at peace, but miss him and wish he could've known his beautiful sister who looks less like him every day. So if you think about it today, say a prayer, pause, reflect on something nice, raise a glass or eat an extra scoop of ice cream to our mighty Will.

New Year silliness

We planned on posting a montage today of Hope's latest and greatest smiles. She's become quite the wide-faced jiver of late. Unfortunately, we're having technical difficulties and you'll have to take our word on it.

Instead, here's a goofy video I threw together last week documenting our hell-on-wheels New Years, complete with crazy hats and everything.

It's been a quiet week, which is nice. Hope has a few shots tomorrow and some gastrointestinal tests Friday.

Meet Dr. Spitenup

After weeks of trying, we finally got in Friday to see a pediatric gastroenterologist to address Hope's spitting up, acid reflux, so-so-feeding and begin the process of evaluating her sucking-swallowing-digesting system.

It's a big deal for CdLS kids. Many are on feeding tubes. Malrotated bowels causing blockage, other problems or death are a concern.

Hope's a bit ahead of the curve. She sucks like a champ, but often falls asleep or runs out of energy halfway through feeding. She can be sloppy. We still need to hold her upright for a half-hour after every feed to avoid throw-ups. At eight feedings a day that also take about a half hour each, you start talking about real time after a while. She's been on Prevacid for nearly three weeks. The acid reflux drug has prompted some improvement, but it's no panacea.

We weren't expecting a cure-all from the doctor, whom we'll call Dr. Spitenup, and the appointment proceeded something like this.

Dr. Spitenup: Describe her spitups. Is there blood? Mucus? Would you say her poops are more creamy or slimy? On a sliding scale of sliminess, what number would you give them? What percentage of the day does she stiffen like a board? When she cries mid-feeding, it is a cry of pain, communication or empathy for a world gone wrong? Is she a happy baby? What percentage of the day would you say she is happy? Have you noticed a difference from the Prevacid? What percentage? Would you say 20 percent? No. How about 44? When she spits up, does it come out of her mouth or nose? Nose, huh? That's unusual. Onto her bowel movements ...

Us: Ah. Um. Err. Uh.

Him: OK, let's go over all this. Normal, normal, normal, normal, normal, not-so-normal but nothing to worry about, normal, normal, normal. Even if she had major problems, we wouldn't do much until she is 2. See you in three weeks.

Us: Um. OK.

Hey, normal is good. We'll take it, but we're not quite sure we trust the good doctor's system of ordering no tests and evaluating Hope based on us picking numbers out of thin air. We're not sure what to expect in three weeks: If we say Hope cries 25 percent of the time during feedings rather than 30 does that mean his treatment is working? How is that anything but arbitrary?

Warned that Spitenup was unorthodox but good, we left the appointment feeling 36 percent relieved, 18 percent amused and 46 percent bewildered.

Another in the ranks of Team Hope

Classic good news/other news today at the pediatrician: Hope's weight is proportional for her length. She's tipping the scales now at 4 pounds, 10 ounces and is almost 18 inches long. That might not seem like much, but it's progress: After eight weeks, she's officially outgrown her first outfit. It's a preemie outfit but an outfit nonetheless.

The flip side is that our pediatrician, whom we like, agrees that it could be time to start exploring supplements in hopes of jump-starting some weight gain. And rather than simply prescribe them, he's referred us to a yet another player in Team Hope: A nutritionist.

Already comprised of a pediatrician, geneticist, cardiologist, ear-nose-throat specialist, gastro-intestinal doctor and occupational therapist, Hope's network of providers is beginning to rival the Army of Carthage.

Frankly, it's an enormous weight that falls squarely on Mo's shoulders. After 2 months, Hope's care is now detailed over about 4 files and spreading into a fifth. Navigating the labyrinth of referrals, specialists, insurance, supplemental insurance and the like is dizzying and can become a full-time job. As most who've dealt with doctors and insurance can attest, little can usually be accomplished in one phone call and seemingly simple tasks become Goldberg-esque. Maureen is one hell of a reporter and an adept problem solver, but acknowledges it's maddening. Today, she made about six phone calls to find one nutritionist.

It's all very annoying and can become discouraging. Then Hope does something really cool, like today. After driving alone to the pediatrician for the first time -- and white-knuckling it all the way, I'm sure -- Mo reports that she was in the examining room, minding her own business when Hope decided, "Enough is enough. I'm hungry and bored, so I'm sticking this hand in my mouth."

She'd been leaning in that direction for days, eyeballing her fingers with the same look her father gets near Swedish meatballs. Today, Hope committed to the finger sandwich. It's very nifty because it means she's right on track of a big developmental milestone.

Tonight, we celebrate. Tomorrow, the army masses again for a trip to the GI doctor for a consultation in hopes of making headway of Hope's spitting-up habit, which is improving but persists.