20 Months Old!

Abby hasn't been weighed or measured in a couple of weeks, so I can't actually do my usual report. This is awesome because it means she hasn't had very many appointments outside of therapy recently. Woohoo! 

She is learning new signs all the time, while we learn to navigate the early-onset of the terrible twos. It's been a doozy. I'd hoped that maybe we'd just get to, you know, skip it. No such luck, lol. Oh well.  We've been trying to combat the whining and tantruming by getting out and enjoying the nice weather. There are so many neat things to do around here and I hope that Abby is enjoying everything as much as I am!

We found out recently that Abby is going to need tubes placed in her ears. Despite not having many ear infections, she's had fluid built up in her ears for months now and it's got to go. Because she can't be sedated here in San Antonio, we'll be treking out to Houston at the end of the month to have TCH get everything rolling for the procedure. We've gotten to avoid anything surgical for so long now that I'm really not looking forward to it, but apparently she is probably hearing things as though she were underwater, so I know it's for the best.

Tube-feeds are going great! She is only getting Real Food Blends and water through the tube during the day and a little formula and water overnight. Eventually we're hoping to get rid of the overnight feed and have her receive all of her nutrition during the day, but I think we're still a few months out from that kind of volume tolerance.

Abby's gait-trainer arrived last month and she's been doing her little ski-pole scootchy-scootch all over the house. She is not at all interested in using her feet reciprocally to walk right now, but hopefully when her SMOs finally come in, she'll gain some confidence in her standing and walking! 

I think that's all I have to report right now!  We had a really nice, low-key Easter, and spent the end of last month celebrating Jameson's birthday. I'm looking forward to some more beautiful days and new experiences before the heat starts ramping up!

19 Months Old!

Abby is 19 months old today! She's right around 30 inches long and just under 20 pounds!

This past month has been a doozy. Abby finally got past the respiratory viruses she'd been battling, only to come down with the stomach bugs that landed her in the hospital. She has bounced back from her bout of dehydration well. The diarrhea has finally begun to improve and we've started reintroducing the Real Food Blends to her diet. Transitions in Aberdeen's feeding schedule always need to made really slowly, so it will be a bit before we get back to where she was pre-stomach bugs. She did lose a little weight during everything, so we're playing a bit of a catch-up game right now. She got a thumbs up from TCH cardiology that all looks unchanged post-illness, and they actually decided to keep her off diuretics (they were stopped while inpatient) to see how she does. One more med down. Yay!

For some reason the universe has decided it's time for Abby's two-year molars to come in. Nevermind the fact that her one-year molars are each only partially through, or that she doesn't have her canines yet, ALL FOUR of her second molars are busting through. Whyyyyy???? Our happy happy girl is having a rough time. Maybe it's the teeth, maybe it's just the age, but woof has she started throwing tantrums. Aside from the few weeks post OHS, when she started having seizures, she has never been a screamy baby. But, oh, the screaming is here. Poor girl. 

The one upside to all this teething has been some improvement in Abby's oral aversion issues. She's tolerating a lot more food in and around her mouth without gagging or freaking out. She still doesn't seem to be swallowing much of anything, but it still feels like a step in the right direction. I definitely think she's tasting more than she has previously, and maybe that will help get her more interested in trying different flavors in the future!

We are still waiting on Abby's gait-trainer, but it sounds like it may be arriving in the next couple of weeks! With Abby having just been measured for her new ankle braces, we might be seeing a big jump in her mobility here soon, which would be so exciting!!!

We've also started working with Abby on signing! She has "want" down, and now we just need to give her some nouns so we can figure out what exactly it is that she wants, lol. She knows "eat" and "drink" as well - though it's usually a response to what she's seeing us doing as opposed to a request for herself. It's really neat to see her catching on to our prompting now though, and even more amazing when she starts using a sign unprompted! 

We have to make a trip back to ENT this month to address some persistent fluid behind Aberdeen's eardrums, but other than that, I think this next month should be focused mostly on therapies and dietary - fingers crossed!

Inpatient in Texas

Last Sunday, Aberdeen started exhibiting signs of having a stomach virus. We fiddled with her feeds to try to keep her hydrated, and while she did ok throughout the week, by Saturday it was clear that something was very wrong. Her breathing had become quite rapid and she was having trouble staying awake. 

We took her to the emergency room and were told fairly quickly that she would need to be admitted. Lots of labs were run and she was found to have astrovirus (a fairly common stomach virus) and EPEC (something that generally shows up in third-world countries...huh?). They also found some haziness on her chest X-Ray, suggesting either mild pneumonia or atelectasis (lung collapse). She was started on fluids to combat dehydration and antibiotics for the pneumonia/EPEC and moved to the peds floor. Dehydration is of particular concern for her due to her heart condition. If her heart gets too dried out, her risk of going into heart failure increases greatly. We did our best throughout the week, but ultimately, it wasn't something we could get ahead of at home.

She had a rough day yesterday, and just wasn't herself at all - super lethargic and unengaged. We heard some conflicting things about the previous night's chest X-Ray, but after review, they decided the initial assessment was correct. They weaned her IV fluids to Pedialyte through her G-tube, which she handled well, despite the ongoing diarrhea. 

We started her on a slow continuous feed of formula around noon today, gave her a break this evening, and are attempting her normal overnight feed tonight. She definitely perked up this afternoon and was acting much more like herself, thank goodness. I worry that we're moving too fast on her feed advancement and we'll see a return of her vomiting, but maybe she'll surprise me. 

Depending on how she does tonight, we'll continue to work on getting her back to her home feeding plan tomorrow. Hopefully we'll see an improvement in the diarrhea situation before being discharged. I really don't want to go home just to end up back here in a couple days. 

Praying she can kick all of this yuck soon and get back to being her smiley self full-time!

Sunday afternoon

Monday afternoon :)

Wednesday morning update: 

Abby is back on her home feeding regimen (back to formula though, no blended diet right now). Her diarrhea is still terrible, but since she's otherwise tolerating her feeds and acting fine, they're talking about letting us leave today. We'd have to follow up with her ped tomorrow and cardiologist on Friday. Honestly, I'm not sure how i feel about leaving with her gut still being such a mess. We'll see. I think she would appreciate an uninterrupted nap!

After getting a nice bed bath yesterday.

Girl likes her naps!

Wednesday afternoon update:

We're heading home with plans to follow-up with various folks over the next few days!

18 Months Old!

Aberdeen is 18 months old today! A year-and-a-half!!! So impressed with this kid. She has had a lot of extra appointments lately and has been handling them amazingly well. In the past couple of weeks she has seen the craniofacial team at TCH, neurology, pulmonology, GI and nutrition, had an EEG, and had her 18 month check today. That's in addition to speech, OT, and PT! She has another nutrition appointment and her monthly Synagis injection this week, as well as an unsedated MRI and cardiology follow-up next week. Yeesh! 

With all of these appointments, we've made a few changes here and there. We're working on transitioning Abby to a blended diet through her g-tube. This means REAL FOOD! After consulting with the nutritionist that works with GI, we've decided to start with a commercial product called Real Food Blends, but eventually may cook and blend up her food ourselves. It's going to be a slow process, but we love the idea of her getting whole-food nutrition instead of formula (since she's never tolerated formula very well). Hopefully this change will also help push Abby past the growth plateau she's hit.

From a cardiology standpoint, we're holding steady for now. We heard back with Cincinnati's opinion on her heart status, and while they noted considerable narrowing of her left ventricular outflow, they agreed with TCH's treatment plan. So, we'll keep monitoring her closely, and managing with beta-blockers, but hold off on any surgical talk for the time being.

With heart stuff being relatively uneventful right now, the pendulum has swung back to concern about Aberdeen's head. The craniofacial team definitely thinks she'll need another surgery to release the re-fused coronal sutures in Abby's skull, but nobody seems too keen on putting Abby through any sort of sedation currently (anesthesia and cardiomyopathy is tricky business). Neurology has requested imaging to make sure everything looks ok brain-wise, so we did an EEG yesterday and will attempt an MRI next week. Hoping things are looking ok on that front and we can continue putting off cranial surgery.

Abby's been doing lots of fun new things this month! She has started picking up her feet reciprocally and walking while we hold her hands! Her PT has decided it's time for ankle braces (SMOs), and we hope this will help her find her balance while standing! She has demonstrated knowledge of many new words: head, nose, mouth, ears, eyes, foot, sock, book, and ball. She's actively opening and closing her hands in response to Twinkle Twinkle Little Star (and Baa Baa Black Sheep, which makes me super proud, since they have almost the exact same melody), she's discovered and plays with her shadow, and has taken some masterful selfies!

On a broader level, this month is awareness month for Noonan Syndrome, congenital heart defects, and feeding tubes! We love inviting others into our little world of the medically complex! A few facts:

• Noonan Syndrome is variably expressed, it does not affect any two individuals identically. Some have very few medical complications, some have many. Some have intellectual delays, some have none. Some have 'characteristic' facial features, while others don't. It is diagnosed through the identification of certain genetic mutations (i.e. PTPN11, SOS1, RIT1, RAF1, KRAS, etc) or the suspicion of a yet-undiscovered genetic mutation based on presentation and symptoms.

• Congenital Heart Defects affect nearly 1 in 100 live births and there are over 40 different types of CHDs. Many CHDs require surgical correction, but even with surgery, individuals may continue to struggle with feeding issues, developmental delays, and other complications long after correction. Though treatment of CHDs is one of costliest issues facing our hospitals, research toward causes and prevention is grossly underfunded.

• Feeding Tubes are used by roughly half a million people in the United States for hundreds of different reasons such as chromosomal and genetic conditions, neurological conditions, connective tissue disorders, craniofacial conditions, diseases, heart conditions, and so on. Some feeding tubes feed directly into an individual's stomach (NG, G-tubes) while others bypass the stomach into the intestines (ND, NJ, G/J, and J-tubes). Diets for tube-fed people vary from broken-down, elemental formulas to breastmilk, to blenderized table food depending on the individual's conditions and needs.

We invite you to take a moment and light a candle tonight in memory of all of our little heart angels gone too soon.

17 Months Old and 1 Year Seizure Free!!!

Aberdeen is 17 months old today! She is so much fun right now, loves playing games with us, and is usually happy despite lots of teeth coming in - she's up to 10 now! She continues to hover right around 20lbs, is 29.5in long, and has gone a full year FREE of seizures (as far as we know)!!! I'm so thankful that the Cincinnati neurology folks got her seizure activity under control as quickly as they did, and that it has continued to stay under control without ever needing to adjust her meds. I'm hoping that her new neurologist will be similarly pleased and open to discussing a possible wean. We've been told that it's standard to wait until a patient had been seizure-free for two years before weaning meds, but since her seizure activity was thought to have been caused by a brain bleed that has since resolved, we think it might be reasonable to try a wean at this point. We'll meet with neuro toward the end of this month and see what they say!

This week also marked the end of over five months of treatment for a persistent GI issue. It was never really more than a nuisance, so I didn't talk about it, but I'm super glad to be done with another med! 

Speaking of meds, it has been interesting to discover lately that our friends and some family had no idea how many meds Abby was on. I guess I've never really talked about it on here, because it's just our normal day-to-day, but I suppose it is kind of interesting: she's on 7-8 different meds (depending on the day), with 15-20 total doses throughout the day. Her med regimen is just one of the reasons we are incredibly thankful for her g-tube. Even if she were a fantastic oral eater, getting 20 doses of meds a day into a 17 month old would be WORK! As it is, Aberdeen still takes nothing by mouth, but even if she someday starts eating like a champ, we'll keep that tube for meds!!!

Though she may not be eating yet, Abby is tolerating some oral input (like teeth brushing) much better than she used to! Even into October, she would gag and scream and puke every time I attempted to brush her teeth. But thanks to lots of work, and a great speech therapist, she has made a ton of progress. She will bring food to her mouth now, and even bite down on it sometimes. After that, she kind of freaks out and either gags or spits out the food, but it's a huge step in the right direction. Yay! We're also starting to work on some signing! Ironically, the signs she's picked up on are "eat" and "drink" :P.

OT and PT continue to go well. Abby actually enjoys standing and is working really hard at pulling up. With this new-found interest, she is starting to try to pull to sitting from laying down as well. Transitions have always been a huge challenge, so we're thrilled that she's showing interest without prompting! They've been taking her in the pool the last couple of weeks to work on those transitions, which has been really fun and different! She's still working hard in the gait-trainer, and we're trying to get her to use her feet reciprocally, though she would rather use a technique something akin to cross-country skiing.

Things have calmed way down on a cardiological front. An additional echo at TCH showed no change from the level of outflow obstruction we'd seen prior to our move, so we're finally starting to accept that things aren't quite as dire as we were led to believe throughout October and November. We're still waiting for the second opinion from Cinci to confirm what TCH is saying, but with how great Aberdeen is doing right now, I expect they'll say that there's been no change. Whew. Assuming Cinci comes back with the conclusion we think they will, we'll stop seeing her local cardiologist and only have her followed at TCH.

Abby's been dealing with a nice series of minor winter illnesses, including her first ear infection and a cold, but they haven't slowed her down! We've been putting her on CPAP during her naps (in addition to her normal night time routine), just to give her a little break during the day. Hopefully we can avoid anything more serious as we are coming up on the 1 year point from her last long inpatient stay!!!

In other news, we had a really wonderful and low-key holiday season. We got to see lots of friends and family, and just generally were able to relax and enjoy our sweet girl at home. It was fantastic. 

The next month will include a trip to Houston for a neurosurgery follow-up, as well as local follow-ups with neurology, GI, and pulmonary. Hopefully we'll get Abby's home gait-trainer and continue thriving on all fronts! I can't believe next month Abby will be a year-and-a-half old!

 (I would just like to note that Blogger changes the font of my posts according to its whim, and not at all to my liking.)