This kid is going to LOVE roller coasters.
Good afternoon, everyone! Shabbat Shalom! I just had to say hello and be grateful for a moment. The Butterfly Fund recently posted LeahBug’s Amazon wishlist, and several people we’ve never met have sent Leah gifts, including some new diapers and covers (because she’s outgrowing the old ones), a sweet outfit, a toy, some books, and medical supplies – AND somebody sent her the ARK Z-vibe, a therapy tool aimed at oral stimulation to help with speech and feeding. We are super-psyched to start working with this as it comes highly recommended by other parents of children with Down Syndrome. Just need to do some reading first as there are specific exercises that help with particular issues.
This past week has been a reminder of how truly awesome people are. Not that we needed a reminder – we are grateful every day for your support and generosity and it continues to amaze us.
Whew! How did I manage to go so long without blogging? Here is what has been going on in LeahLand:
-Leah had a dilation last week and made a little friend in the hospital. A little girl (maybe 6 or 7?) had been staring at LeahBug in the waiting room, and she was called in a while before we were. When we went in, we could hear her crying that she wanted to leave and go home. I wanted to bring Leah down to her little “cubicle” to say hello, but I didn’t want to bother them. Gran, being more gregarious than Mama Lisa, brought LeahBug down and introduced her. The little girl perked right up, and asked about the baby, and we didn’t her her cry again after that. We waved at her and she waved back as they rolled her off for surgery, and we saw her being wheeled out afterward to go home and her mom said she had asked how the little baby was doing. 🙂 Very glad our sweet Bug was able to bring a little sunshine to another little girl’s otherwise yucky day.
-We celebrated our first World Down Syndrome Day as mommies with our friends Jess, Toni and Wesley. Wesley is 20 years old and has Down Syndrome, and he just graduated from a cool program at UCLA for students with developmental disabilities. What a great model for our little BabyBruin! Mama Jess made a beautiful Shabbat dinner and Wesley held Leah for the first time! Pics below.
-Leah will not be having a Nissen fundoplication next week after all. They will be repairing her diaphragmatic hernia and putting her little colon back where it belongs. I do think that is causing her some discomfort, and it seems to be affecting her little poops too – usually she has one every day but lately it’s been every few days. Regarding the fundoplication, there is some speculation that the reflux may not be the primary cause of the stubborn stricture, and a Nissen is a huge step to take if there’s a pretty good chance it won’t work. Instead, they will continue with the dilations for a few more months, and if they don’t work then we may be looking at a surgical fix; it’s possible that the scar tissue is just too severe to be repaired. That is the main reason they aren’t doing the Nissen – if Leah needs another surgery to deal with the stricture, they need to preserve as much of the esophagus as possible, and a Nissen would create more scar tissue and damage to the area and would give them less native esophagus to work with. So they will hold off on that procedure for now.
-Leah had an echocardiogram and the cardiologist still feels confident that Leah does not need heart surgery right now. Again, the jury is still out on whether she will need it later down the line (like, in a couple of years) – she does have the increased blood flow to the lungs, and the VSD has not improved. But he doesn’t feel that immediate intervention is indicated.
-LeahBug had her very first play date with a couple of other babies who live within our general area and also have Down Syndrome! They have to be the region’s three cutest babies. I’m pretty sure of it. Pics below.
So, that’s what’s what! Let’s break out the pictures!
We got out of the hospital on March 14. It was Pi Day. After a long and difficult day of waiting with a hungry baby, we felt we deserved to celebrate Pi Day with pie. Well. Really, it was Leah who deserved pie. Someday we’ll be able to give her pie!
Later that day… we went to meet with Sue of Binkeez For Comfort, who sent Leah the sweet polka-dotted blanket you always see with Leah when she goes to the hospital. Recently they made a special lovebug Binkeez in honor of Leah (pictured above), and we will be delivering some to the children in the PICU just as soon as the blankets arrive. Sue delivered Leah’s special Binkeez by hand as she was already in Los Angeles to visit Children’s Hospital LA for another special delivery. Leah loves to stare at the cute bugs on this special blanket! Thank you, Binkeez! Sue took this sweet picture when we met her at the hospital. Then we had a nice visit – we went to get frozen yogurt, and then we drove Sue back to her hotel via the “scenic route” (i.e. Hollyweird Blvd!). Such a fun day!
The next day, Leah celebrated her very first Purim! It is customary for children to dress up as the characters in the story, so Leah dressed up as Queen Esther. Cabbage Patch Queen Esther.
She was in a much better mood than she was the last time she wore this dress… it fits her a heck of a lot better, too!
Things have been pretty hectic around here, so I’ve fallen behind on pictures. I’m working on a photoblast, I promise!
Just a quick “diary” paragraph and then I promise I’ll get to what you’re really waiting for: the surgeon’s update.
On Leah’s 10-month birthday (March 8), we took her to the pier so she could see her first sunset. She slept through it, of course, but she did wake up in time to see all the pretty colors, and it was her first time seeing the ocean, so that’s pretty cool. That day I also painted her little toenails like ladybugs while she was sleeping. I’ve gotta have my fun, too! The following day we went out to dinner with our dear friend Faith and her family and some friends for her birthday, and LeahBug met her little friends Storm, Cady and Iris for the first time. Miss Cady brought Leah a little ladybug finger puppet. Very sweet. This past week we also visited a bit with Leah’s buddy Ry Ry, his mama Auntie Z (Mama’s BFF), and his dad “Bob.” (Not really, but I couldn’t resist). In the waiting room at OT the other day, we ran into one of Leah’s old NICU roommates and her mommy. I don’t think Leah’s seen so many other kids since she was in NICU! In other news, Leah’s g-tube popped out twice (uneventfully, thankfully), and she continues to make wishful noms when she watches us eat. So sad and yet so cute. And finally, LeahBug experienced (read: slept through) her second earthquake. This one was a bit larger than the last one she didn’t feel; the last one was around 3.0 or so and was in the middle of the day, and this was a 4.4 magnitude rocker at 6:25 AM. Sooner or later she will actually notice an earthquake, but I’m in no rush for a quake large enough to register with her!!
If any of that didn’t make sense, you can blame it on the fact that I was up at 6:25AM looking up the magnitude of the earthquake that woke me up.
Okay – now, what you’ve been waiting for. Yes, Leah is going to have another surgery. She will continue weekly dilations on March 20 and 27, and will go in April 3 for a Nissen fundoplication and a hernia repair.
As it turns out, it appears Leah actually has two hernias: a paraesophageal hiatal hernia (likely a result of her initial esophageal repair), and a Morgagni hernia, a type of congenital diaphragmatic hernia (CDH) that makes up about 2% of all CDH cases (which themselves occur in less than 5 out of every 10,000 births). In this case it’s a lucky thing that Leah has the more rare form. In many cases, congenital diaphragmatic hernias create problems with fetal development, as the intruding organs in the chest cavity can impede lung growth. Overall, CDH has a mortality rate of about 40-62% of live births, the primary reason being that the lungs have not been permitted to develop fully due to the presence of other organs in the chest cavity. So we are very grateful that Leah’s CDH is the type that has a better prognosis.
Nonetheless… it needs to be repaired. I mean… the kid has a loop of colon in her chest cavity. Really.
The big concern right now is Leah’s acid reflux, which appears to be the cause of much of the scar tissue in the esophagus. Leah’s surgeon wants to avoid having to redo the esophageal repair; he said if it didn’t work the first time, that doesn’t bode well for a second repair. He feels that a Nissen fundoplication is the best way to keep her reflux at bay. (Here is a longer and more technical article.) It’s always been on the table as a future option – I just kind of thought the future would be a little bit further down the line. But here it is.
Leah also saw her cardiologist today and he remains confident that Leah does not need heart surgery in the immediate future. He says the jury is still out on whether she will need it in a couple of years. She does have increased blood flow to the lungs, and the VSD has not decreased in size – but it hasn’t increased either. If it doesn’t improve then Leah will need heart surgery eventually. Let’s hope her heart patches itself up! Go BabyBug Heart!
Pictures soon, I promise!
When we arrived at the hospital at 6:45AM yesterday (Thursday), we expected to be home around 2PM. It’s now 8:47PM on Friday night and we are awaiting discharge from Peds.
Leah’s procedure took a bit longer yesterday – I think she was in the OR almost 2 hours or so – because they injected steroids into her esophagus to help with the inflammation and scarring, in the hope that it would keep the stricture from continuing to tighten. Prior to the procedure, Leah’s temp was a TINY bit high (98.8) and she sounded a TINY bit junky, but since she hasn’t been sick, we went ahead with the procedure.
Afterward, Leah was super-fussy, as usual, but this time they wouldn’t let us feed her until she was transferred from the recovery area to the discharge area. When she arrived at the discharge area she was feeling a little bit warm and her temp was about 100.3. They were getting ready to discharge us when I noticed that she seemed to be retracting a little bit – using her abdominal muscles to breathe. They called the anesthesiologist over to look at her and he suggested that she might be agitated from being hungry, so we could try feeding her and seeing if that helped with her breathing. She was also sounding a little junky (in her lungs), and was having a tiny bit of wheezing on exhalation (I wouldn’t call it stridor but it was going in that direction). We fed her and tilted her neck up at the doctor’s suggestion, and that seemed to help with the retractions, but after a while they started again, and her fever went up to 100.6 (don’t worry – that’s as high as it got). After a long chunk of time had passed, she was still pulling a bit to breathe and still sounding a little icky and still had the mild fever, so with all of those little things plus the fact of this being the first steroidal injection, they decided to keep her overnight for observation and also to do a chest x-ray to see if aspiration was causing the “junky” sounds from her lungs.
Well. The peds unit was short on rooms at the time, so Leah wound up having her chest x-ray in the discharge area. We spent a good several hours in the discharge area waiting for a room to open up on “the floor” (as they call Peds). While we were waiting, Leah’s GI doctor came by to discuss the x-ray results with us. Her lungs looked fine, but the radiologist had seen something suspicious in the upper GI area and wanted to run another x-ray in case it was just a poor film. So we waited another couple of hours, and they took the second x-ray just before they brought her up to Peds. For reference’s sake, Leah’s surgery was at 8:30AM, she got out about 10:45AM or so, and Leah was rolled up to Peds at around 5PM. We did a lot of waiting yesterday!
We had a pretty mellow evening and got to see some familiar faces; Leah’s RT had treated her many times in PICU, and her overnight nurse had also cared for her several times. It wasn’t a bad night. They stopped Leah’s feed at 4AM, with the plan being to run a contrast study some time around or shortly after 10:00AM to determine what was going on in Leah’s chest – the second x-ray was not particularly helpful in determining whether something was wrong.
Well. 10:00AM turned into 1:30PM, which turned into 2:30PM, which turned into 3PM. I went to radiology with Leah and kept her entertained during the breaks; they had her ingest contrast, so they had to take breaks to give the dye time to move through her bowels. For most of the study they didn’t see anything wrong, but in the very last “take,” they finally saw what they had been looking for. Leah has a diaphragmatic hernia, which means that a segment of her bowel is poking through her diaphragm.
That’s about all I can tell you right now. We have many questions, particularly because this is usually a congenital defect that is detected either prenatally or immediately after birth when the newborn goes into respiratory distress. It is also possible that this is an acquired diaphragmatic hernia caused by trauma to the area, e.g. abdominal surgery (because yeah, that happened!). We really have no answers right now beyond the diagnosis. They are sending us home tonight since Leah is stable and relatively asymptomatic, and we will see Leah’s surgeon in the clinic on Monday to discuss plans going forward. The repair is surgical, and the big question is whether they do the repair now or wait.
So. Now, we go home!
Sorry it’s been so long – busy week! Quick update – Leah had another dilation tomorrow (Thursday), and they will inject a steroid into her esophagus to help ease the inflammation and keep the stricture from tightening after dilations. They will keep her on the weekly schedule for the time being.
We also had an interesting discovery in speech therapy today – Leah has not been turning when she hears sounds and voices, but there seems to be a difference depending on what side the noise is on. She turned to investigate a rattle shaken on her right side even while she completely ignored it when it was on the left. In PICU all the bells and alarms were on the left – and the left side is also the side the ENT could not see. So we still don’t know what is going on, but it is something.
So! Some medical updates for you:
-I mentioned in an earlier post that Leah has mild obstructive sleep apnea. The ENT reminded me that she also has some central apnea, which occurs when the brain simply doesn’t send adequate signals to breathe. Treatment remains the same, but I want to make sure I’m being accurate since I’m sure I’ll use this blog for reference later on.
-Speaking of the ENT, Leah had a miserable appointment the other day. She had to get her little ears scraped out because she had a lot of wax, and she had her little nose scoped. NO FUN. I know it was much worse for her, but I’m not gonna lie – it was pretty freaking awful having to hold her still while she screamed and sobbed uncontrollably on my lap. They don’t have an exam table in there, so I had to hold her still (with the help of one of the nurses). Her little tears were rolling all the way down to her neck. Poor Bunny. She is due for an audiology test later this month; we’ve had some concerns about her hearing since she is still not responding to noises from sources outside her direct light of sight. I still think it’s more an issue of attentiveness since she was in the ICU for so long and it’s possible she has learned to tune things out, but at the same time it’s important to rule out hearing issues since many kids with Down Syndrome do have ear problems. And you could probably hit a gong behind this kid and she wouldn’t turn around to see what’s up. The doc was only able to see into one ear canal (although this is the first time ANYBODY has been able to see anything at all), and she said Leah’s eardrum activity looks good and she didn’t see fluid. No word on the other ear. Too itty-bitty!
-Our little chunky monkey hit 13.5 lbs today!
-The big update: Leah had a dilation today, and while the last dilation took her esophagus from 9mm to 10mm, this procedure had the doctor re-dilating her from below 5mm back up to 8mm. He doesn’t know exactly how narrow it was, but he knows it was less than 5mm because he couldn’t get the scope through. So we’re essentially back to square one. Her next procedure is scheduled for next Thursday, but the surgeons and the GI will need to confer and decide what they want to do next, because there is not much sense in continuing to do regular dilations if the stricture is just going to tighten again. The GI discussed perhaps injecting steroids into the stricture, but apparently this requires special equipment that may or may not be available at this hospital, particularly before next week. He also discussed the possibility of the surgeons needing to operate on the esophagus again, as well as the potential for a fundoplication, a surgical procedure that curbs reflux. The fundoplication has always been on the table as a possible necessity in the future – it just seems like maybe the future has arrived a little sooner than we thought. But I don’t want to jump the gun – the surgeons and the GI haven’t yet conferred, so all I can do is speculate.
And on that note… smile! It’s picture time!