Today is Leah’s 6-month birthday!

Not a lot to update but I feel like we should write something before certain beloved family members who shall not be named start texting and emailing to ask where their blog update is. 🙂 <3

Leah is still on the vent, as you know. We had a really rough day/night on Wednesday, and the nurses/docs woke me up three times during the night, twice to clear the room for chest x-rays and once to let me know that they were re-intubating. I’m told I was so knocked out that I fell asleep as soon as I confirmed that I understood what they were doing and then slept through the entire intubation, which did not happen in the OR as I had assumed when they told me the anesthesiologist was doing it – he (or she?) came up here. It was a long night. Thankfully she has been a lot more comfortable these past few days, and today has even been awake a bit, albeit stoned and groggy and moderately pissed off whenever somebody lifts her up for a second. For a little while she was even sucking on the breathing tube and trying to taste the plastic bridge holding it in place. That’s our Bug! We are hoping they will be able to extubate early next week, but after seeing what Leah went through last time we want to make sure it happens when she is ready!!!

We had another little bit of excitement last night and this morning when we had to move rooms briefly so that the facilities people could repair a massive crack in our window. We noticed and reported it a few weeks ago, but it’s gradually been growing and has gained maybe 6 inches just in the past week or week and a half. Don’t ask me how a crack showed up in an upper-story window. It’s not like there are kids in the street playing baseball. I guess maybe with all the construction going on, the vibrations could be causing cracks. Apparently there have been others recently as well. So we switched to the room next door and had to take EVERYTHING down as if we were leaving. That was a weird experience. Everything was totally backwards in the other room. I was so turned around and am so relieved to be back in the room we’re used to. I know it’s a little thing but sometimes the little things matter!!!

Pictures:

Sleeping Beauty

There’s a lot going on here!

“‘sup, Mamas?”

More Sleeping Beauty

SO! Here is our longer update of yesterday’s events.

All things considered, Leah had a pretty good morning yesterday. She did spend a good half hour scolding us for not serving breakfast, but I’d be pretty mad if I had to skip a meal too. Wait. Ha. Let’s make that Mandy. Mandy would be pretty mad if she had to skip a meal too. (I seriously wrote that without thinking.) Considering that sometimes she starts getting angry when her food is not EARLY, I’d say things could have been a lot worse. She was generally pretty smiley for most of the morning. It’s like she knew it was going to be a good day. She fell asleep before they came in to prep her for transport to the OR, so she slept through most of the trip and was overall very calm. The last time we went down to the OR with Leah she started crying once we got downstairs. This time she was pretty chill. She is such a good baby. We were definitely more agitated than the baby was!

After they rolled Leah away at around 1PM, we met my dad, Mandy’s mom and cousin Beth in the waiting room. The surgical team’s nurse practitioner had told us that they should know within about an hour and a half how they would be proceeding, so it was really that first major period of uncertainty that we knew would be the hardest. We couldn’t stay in the waiting room. That room is just too tense and anxiety-filled. So instead the five of us had a nice lunch at a nearby cafe and then killed a good 30-40 minutes browsing in a little shop across the street. It was the perfect distraction, although I’m not sure my dad enjoyed it quite as much as the four ladies did. 🙂 Then Beth and I headed back to RMH for a little bit and the rest of the group went back to the hospital to wait for news.

It turned out to be more like two hours before we heard any news, but the news we got at that point was worth it: They told Mandy that they were pretty sure they were going to be able to do the primary repair. OH EM GEE. That is not the news we expected, but it was damned good news! We’ll take it! When Beth and I got back to the hospital, we all waited for them to bring Leah back. Shortly after we arrived, the nurse told us they would be bringing Leah up in about 40 minutes. That made us pretty nervous – or at least it made ME nervous. They had told us the Foker procedure would be pretty quick, a few hours, but that if they did the primary repair it could be as many as five hours. At this point it had barely even been three hours. I was sure they’d had to resort to the Foker procedure. At around 4PM the surgeons came in with big smiles on their faces and gave us a thumbs up. They were able to connect the pouches! Holy moly! Good news! They showed us a picture (yes, in full color) of the two pouches before they stretched them out, and then explained how the procedure had gone. They were very pleased with the outcome. Everybody was very pleased with the outcome. “Very pleased” is like, the understatement of the century. Yeah. Leah returned to the room about half an hour later. Yay!

So the pouches are connected. There is a good amount of tension on them because they really had to stretch them to get them together, but they said they have ways to distribute the tension so that it’s less likely to tear. Leah has to be sedated and paralyzed for at least three days – probably until Monday – because if she moves she could tear the sutures. In about a week they will run a contrast study to confirm that everything is still intact and that there have not been any tears, leaks or strictures. At that point, if all goes well, they will be able to begin the next step of Leah’s recovery: teaching Leah how to feed orally. As far as I know, her release from the hospital is still anticipated to be at least a month, as she has to recover and be monitored now and also once she begins to feed. But yesterday was a huge step in the right direction.

As I mentioned, the biggest concerns in the coming days are tears and leaks. Another major concern is pneumonia. The last major concern right now is that Leah needs to stay paralyzed and unconscious for the next few days. Children with Down Syndrome can have a high tolerance for pain medications and sedatives. Last night Leah tried to open her eyes a couple of times when she knew we were next to the bed. They have already had to increase her dose of Fentanyl and also add another sedative on top of that. The paralytic is administered every hour, and I’m told that as she gets closer to the end of the hour she starts stirring a little bit. Today’s nurse said the sedatives and paralytic are barely doing their job. She will need a lot of monitoring over the next few days to make sure that she doesn’t wake up. There is a chance that they could have to raise the dose again (and again) and eventually her body could become addicted to the sedatives, which would mean she could go through withdrawal when it is time for them to stop. We’re hoping that because she will be under for such a short period of time we won’t need to get to that point. Let’s hope not!

That’s about all the news we have at this point. I just want to take this opportunity to thank you SO MUCH for your comments and messages and texts and voicemails and other words of encouragement yesterday, for changing your profile pics to support Leah, and for all of the positive thoughts and prayers and energy being sent in Leah’s direction not just yesterday but throughout her hospitalization. (Also for the delicious sandwiches for dinner. You know who you are – thank you!!!!)

All of you really made yesterday SO MUCH EASIER to get through. It was such a comfort to wake up and see our kid trending in my newsfeed. There was a lot of, “OH, look what X posted! Awwww, look at this text from Y! Awww, did you see Z’s comment?” going back and forth between Mandy and me. Also… I may or may not have had my nose buried in my phone through most of lunch, most of the walk to and from the restaurant and RMH, most of the rest of the wait for BabyPie to get out of surgery, most of the time they spent setting her up once she was back, when we couldn’t be underfoot… I’m just saying. Your constant support yesterday kept us from going totally crazy obsessing over every minute we didn’t hear from the docs. And I KNOW that Leah felt all that love being sent out to her. I bet she even felt it in the morning. Maybe that’s why she was smiling so much. 🙂

Okay… pictures! There are not too many, but here you go! BTW, Aunti Staci has been spearheading the t-shirt project and we should have the shirts by the end of the weekend!! So excited for that. We will keep you posted and you’ll hear from us soon if you requested a shirt.

This is the smallest surgical gown they had. It almost fits her.

Leah slept through all of the prep!

This is what LeahBug looked like yesterday afternoon when they brought her back. I don’t think she had this many wires and tubes even when she was first born!

A little gift from our friend Jennie. It brought a big smile to our faces after a really difficult day. Thank you, Jennie!

BabyBug this morning. She’s so beautiful even with all these tubes attached.

Leah’s surgery is in roughly ELEVEN HOURS.

The past few days have been a nice combination of crazy and calm. Leah had lots of visits from friends and family, and missed out on seeing other friends and family because people keep getting sick. Very sad. Get better, people! On Monday and Tuesday we had visits from Gran and a few friends. Wednesday we saw another friend, and Grandma Barbie and Grandpa Bill brought Grandpa Mannie to visit. Grandpa Bill helped Mommy Mandy give BabyBug a bath. That turned into an adventure when it was discovered – as Grandpa Bill carried the bathtub from the crib to the sink to spill it out – that the plug in the bottom of the bathtub was not in place. Need the floor mopped? Not anymore!

Later on Wednesday, cousins Beth and Erin came to visit and Erin serenaded a knocked-out BabyBug by playing some songs on the guitar. Whew! But that’s not all! Last night, Leah met one of her cousins (in Georgia!) on Skype, and this afternoon Leah met her great-uncle (Grandma Barbie’s brother) and great-aunt on Facetime. They live in New York! BabyBug has friends and family all over the place! <3

So that’s the crazy. Today was the calm – we had NO visitors at all. Well – no visitors except for the nursies and staff who came around this afternoon to “reverse trick-or-treat,” a.k.a. to dress up and bring candy to the kids (and parents). If you thought the giraffe outfit was Leah’s Halloween costume, you clearly have not met us yet. Oh no. Just scroll down.

In other random news:

– Leah is one step closer to surgery this evening; they inserted the IVs about two hours ago. They called somebody up from NICU to do it because her veins are SO FREAKING TINY. She got that from Mommy as well. She is sound asleep now – they wore her out!

– It is confirmed that Leah has her Grandma Barbie’s eyebrows. I just thought you would like to know. I am just starting to see a little bit of me in her, too. Up to now I’ve only been able to see Grandpa Bill and Aunti Staci. And a little bit of Mommy Mandy in the nose.

– On a more serious note… Once again we have been blown away by people’s generosity and kindheartedness. We learned that an email went out earlier today to the community at our family’s old temple, and for the whole day we’ve seen donations coming in on the GoFundMe page. We are so grateful for people’s generosity and willingness to help out in difficult times. I try not to spend too much time on the blog harping on financials, but I’ll say that it hasn’t been easy dealing with the stress of lost income, unexpected expenses, and a whole host of other factors to worry about going forward (not least being Leah’s care once we get home). I don’t know how we can adequately express how much your contributions have helped us these past few months – financially, mentally, emotionally. You haven’t just kept us afloat – you have kept us sane and in one piece, and have allowed us to focus our energies on our little girl. We can’t thank you all enough for that. But we’ll try.

– Regarding tomorrow’s surgery: They will take her down to the OR around noon. The procedure could be anywhere from three to five hours. We won’t know until tomorrow. There is a lot we won’t know until tomorrow. We will do our best to keep you posted. In the mean time, thank you, dear blog readers, for keeping me distracted tonight while my wife and baby sleep. Trying not to be a nervous wreck.

Behold: Photos!

Yeah, this happened. The PICU nurse leader was kind enough to indulge my eccentric request and allowed us to put Leah in an isolette so we could take this picture and show you how our little Bug has grown! Look at her chubby-chub thighs!!! Remember those chicken-legs of summer days past?

“Um, Mama? I’m a big girl now. What is this about?”

Our toesies, however, are still pretty tiny.

A preview of our new Halloween outfit. “Mama, it’s not Halloween yet! You’re SO SILLY!”

Have you ever seen such a look of scorn on an infant? This is probably after the skirt-pants went on.

Yep. More scorn. “Mama. Really? Really?”

AIR KISSIES!

“OMG, please tell me I’m not going to be seeeeeen in this skiiiiirt!”

“I guess the leg warmers aren’t so bad!”

“Oh hai, guys! It’s Halloween MONTH!”

We love baby leg warmers!

“Whatchoo doin’ up there, Mama?”

“NOMNOMNOMNUMMYFINGERS!”

Sweet face

Sweet Sleepy Bug apparently can’t sleep unless she has all of her friends with her.

BabyBug and I started playing a new game the other night: “Smile or Grimace.” It’s pretty fun. Mama won, and by won, I mean got lots of smiles. This, however, is probably a grimace.

Bobblehead Baby practicing neck control!

“Are we done yet?”

I love how such a slight change in position and expression makes all the difference in attitude. In the first picture she looks so disgusted by the outfit and by the second picture she’s like, hand on the hip, knee sticking out, going, “When do I start getting an allowance? Can I wear eye shadow yet? I wanna go to the mall!”

I know this is blurry but I love it SO FREAKING MUCH that I included it anyway.

“Sup, Mama?” Our friend D stopped by for a visit on Monday morning!

“Oh hai, I don’t have enough shirts of my own. I think I’ll have your shirt too.”

“Tada!”

I’MTHEKINGOFTHEWORLD on Mama’s lap.

“Ummmm, Mama, I don’t know how to tell you this, but… I think this lady was already here today.”

“Well hello!” Our friend D’s identical twin sister M came by later in the evening. In case you hadn’t already figured that out.

Nomming on Mommy’s finger during dinner. Tastes like milk!

Our friend Dee came to visit on Tuesday evening. Thank you for bringing more clothes! Leah says, “OH HAI, RUBY’S MOMMY!!! Thanks SO MUCH for getting my Mamas hooked on baby leg warmers! It’s all your fault!”

“Hi, Arleta! I think I’ll melt in your arms. K, thanks. ZZZZZZZZZZZZZZZZ.”

“Hey, Facebook! You need another baby giraffe? Here I am!”

“It’s hard being so cute.”

“Hi world! I’m upside down!”

Two, two, two giraffes in one picture! BabyBug is hypnotized by her friends on the mobile.

The costume you’ve been waiting for. A legitimate, for realz LADYBUG. Costume.

“Oh Mamas, you silly!”

Look at the feet!!!

Look at the antennae!!!

Look how cute!!!

Do ladybugs have tongues?

“SILLY MAMAS UPSIDE DOWN!”

Nope – silly BABY upside down!

She started off sweet and happy, but it didn’t last long.

She just needed to nom on the Mommy Ladybug’s shoulder.

Best family portrait ever?

Smooooooooshy cheeks!

Mommy’s shoulder wasn’t filling enough so she went for Mommy’s cheek.

Mommy decided it was only fair to steal a Leah-cheek in return.

Sleepy LadyBug

Um…. if the Unibomber was a ladybug?

Ladybug with a flower crocheted by Mommy!

Ladybug with a flower and a caterpillar crocheted by Mommy! Plus the jingle-bug!

Ladybug with the flower and the caterpillar crocheted by Mommy, plus the jingle-bug, plus the LadyLoveyBug, plus a MAMA! Whew!

Upside-down baby with, you know, stuff made out of yarn ‘n’ stuff.

Leah just woke up from her nap and doesn’t understand why Mommy now has scary things floating over her head.

Best family portrait ever? Yuh huh.

BIG YAWN right in Mama’s face.

Mama makes a comfortable lounge chair.

I think the Mamas might have been the most excited for Halloween!

Tinkerbell Pants and Halloween leg warmers! We are all about high fashion.

Twinsies.

“Oh hai Mama! I love you!” I love you too, BabyBug!

She was so talkative. I kept getting little coos. She has the sweetest little voice!

We suggested that people post this picture as their Facebook profile pic tomorrow to show support for LeahBug during her surgery. I made a square one after I noticed that people were having trouble with Facebook Mobile. Apparently it thinks it’s Instagram and only allows square profile photos? Who knew? Ah well.

Okay. I should sleep now. Thanks again for giving me something to think about other than sutures and pouches and tension (oh my).

Leah’s surgery is in 15 days.

Our little Bug was full of spitballs today. At one point this evening she even hacked up the water that had been used to flush her tube some 15 minutes earlier. She also had a bunch of pretty rough feeds – I don’t remember whether or not I’ve already mentioned this, but for the past few weeks, Leah has been having coughing and gagging episodes at nearly every meal (except sometimes when she is asleep during the feed). She is also hacking spitballs more frequently now that she has discovered nommable fingers; I think the holes are getting stuck to her throat more frequently (although someone once claimed erroneously that this was impossible…). I’m dreading Leah’s surgery day, but at the same time, it cannot come quickly enough. In the mean time, I am grateful for awesome [with an exception] nurses. (Yeah, every once in a while I reread that entry and my blood boils all over again.)

I’ve found myself explaining Leah’s procedure pretty frequently lately, so I want to take a minute to let you know what it is that they’re planning to do – at least, according to my untrained understanding of what will happen.

As you likely know, Leah has esophageal atresia (EA), which means that her esophagus and her stomach do not connect. In most cases of EA, there is also a tracheoesophageal fistula, which means that a part of the esophagus is growing into the trachea. From what I understand, this is typically a more urgent repair since it involves the trachea. Usually these repairs are done shortly after birth – like, within a few days, if that. From the drawings and diagrams that I’ve seen, though, most often what happens is that the proximal esophagus (the top part) ends in a pouch, and the distal esophagus (the part that connects to the stomach) grows into the trachea, which means that it’s long enough to connect to the proximal pouch, but it’s connected to the wrong structure.

Here is a diagram:

(from this website.)

What I just described is illustrated in the first drawing. Leah has the condition illustrated in the second drawing – a far more uncommon form of the condition. Although this drawing is second in the above diagram, it is referred to as “Type A” or “pure” esophageal atresia. It says 9% of cases, but 7-8% is the figure I usually see. In this condition, the proximal and distal portions of the esophagus each end in a pouch, without any fistula into the trachea, and there is a gap between the two pouches. Sometimes the gap is just past the point where it just could be connected on the spot, and sometimes it is very, very long. My understanding is that a gap larger than 3 vertebral bodies is considered a long gap. Leah’s first study showed a gap of 4 to 5 vertebral bodies, and the second study showed a gap of 5-6 vertebral bodies.

So here’s how it will work:

On November 1 they will take our sweet BabyBug into the OR and take a look at the gap, live and in-person, for the first time. From her surgeon’s description it sounded like they might be doing this laparoscopically, but I wouldn’t swear to it. I know he said they’d be using a scope to examine the pouches but I don’t know if that’s in addition to more substantial cutting. The surgeons will assess the actual distance of the gap and see whether they think it’s possible to do a primary anastomosis, which means they just connect the two ends directly. The odds are pretty small that this will be possible, but the surgeon seemed to think Leah would gain some esophagus length once they actually get in there and see how far the ends will reach.

In the more likely event that the surgeons cannot connect the two ends directly, they will perform the Foker procedure. The surgeons will place sutures and a clip at the end of each pouch. The sutures will be brought out of the chest at a slant, with the distal esophagus sutures pointing diagonally upward and the proximal esophagus sutures pointing diagonally downward. Leah will be completely immobilized (sedated and paralyzed) for a period of three to twelve days while the surgeons gradually put tension on the sutures to stretch the pouches but also stimulate them to grow. I think the clips allow the surgeons to take x-rays to see whether the ends are growing toward one another and if so, by how much. Leah must be paralyzed and sedated throughout the entire procedure, because if she moves even a tiny bit it could jeopardize the repair.

If the procedure works, ultimately the surgeons will go back into the OR and connect the two ends of the esophagus, which should be long enough to overlap slightly. At that point, Leah will begin her recovery, during which time she will be monitored vigilantly to watch for leaks and strictures (where the surgical site narrows), and will receive therapy to teach her how to swallow and feed by mouth. She will come home with the g-tube and continue her therapies and keep learning how to feed by mouth, albeit with vigilant monitoring since she is at constant risk of stricture and choking.

If the procedure does not work or if they have to abandon it for some other reason, the surgeons will perform an esophagostomy (also known as a “spit fistula”), in which they bring the proximal esophagus through the neck so that Leah’s saliva (and whatever else she swallows) drains externally into a gauze pad. After a short recovery period they will send Leah home until she is about a year old, at which point we will revisit some of the more traditional approaches to repairing long-gap esophageal atresia, each of which comes along with its own complications. The reason they don’t do the esophagostomy in the first place is that the Foker procedure generally doesn’t work once there has been any other surgical intervention, and that includes the esophagostomy. Since the Foker procedure is the only one that uses the child’s actual esophagus to repair the gap, that is the ideal procedure in many cases. But sometimes it doesn’t work.

I wish I could tell you what percentage of these procedures wind up being successful, but the fact is this just doesn’t really happen very often. I think I posted my little number-crunching exercise on Facebook but not on the blog, so take a look to put things in perspective:

On a completely different note… I’ve been thinking a lot lately about how much things are going to change once Leah’s surgery begins. I’ve mentioned that Leah’s room is like a brightly-colored bubble compared to the rest of the PICU. That changes when Leah’s surgery starts. The wall-o-mermaids stays, but her toys and books (and probably most of her clothes) will likely need to go back to Ronald McDonald House to make room for the ventilator and whatever other machines they will have supporting our little sweetheart.

We’ve gotten spoiled these past two months with our deceptively healthy-looking Bug, and will have to dig down and remember how things were when Leah was first born. I was talking with Leah’s nurse today about what we can expect in the days following her surgery, if they do proceed with the Foker procedure. She said that we need to minimize stimulation as much as possible because the more Leah gets stimulated, the more she will try to wake up from her sedation and the more likely she will be to try to move, which could cause the procedure to fail. Basically… this is the ICU and Leah will be receiving intensive care and needs to be regarded as such. We are going to double-check everything with the surgeons, but I think the general idea is that we are not just hanging out in this room all day visiting with friends and family during that time. To the extent that visitors are allowed at all, they will need to spend most of their time in the waiting room, with brief visits to see the baby, as was the case when she was first born. I doubt they will allow more than two people in the room at a time with the extra equipment that will be in here. I asked if we could read to her during her procedure, but it sounded like that would likely be too much stimulation and could cause her to try to wake up. It’s going to be a quiet week. :-/ In the time leading up to Leah’s surgery, Mandy and I plan to make sure that at least one of us is here every night, largely because she has been hacking up spitballs so frequently and you can’t always hear those from the nurses’ station. I’m not sure yet whether we should sleep in the room while Leah is sedated. It may cause too much commotion.

Argh. Okay. I think I’ve said what I needed to say so that I can try going to sleep without my mind running on its hamster wheel. I started this post at midnight, so technically today is the 17th – our anniversary. Mandy and I are celebrating eleven years together. Whew.

I leave you with this face: