Update

Charlotte has been doing really well lately. We just had a follow up yesterday and here are her stats:

Weight: 9.8 kilos (21.6 pounds)
{15%tile for actual age/ 50%tile for adjusted age}

Height: 72 cm (28 inches)
{2%tile for actual age/ not on the char for adjusted age}

Head Circumference: 42 cm
{not on chart for actual or adjusted age)

Head: Charlotte has what's called "microcephaly", meaning, her head is more than 2 standard deviations from the average head for age and sex. More specifically, Charlotte has micoencephaly, which is a small brain, thus accounting for the small head. {We know this because Charlotte suffered a pretty severe stroke in the cerebellum, leaving very little brain matter in that space} However, luckily for Charlotte, she doesn't appear to have a small head, so it's all good. Her pediatrician just wanted it to be an official diagnosis. Yay for more labels. :)

Hearing: A new one, right? Charlotte's had a bit of a "regression" in the vocal department as of late. Prior to our move, she was making vowel sounds, and the occasional consonant/vowel sound. Lately, it's only been noises, which is good, because it means she can manipulate the vocal cord, but bad because she's not making any progress verbally. We want to check her hearing to make sure this is not a factor in her speech delay. Right now, we're only going for the standard hearing test, but if she still has a vocal delay in say, four to six months, we'll have to go for a sedated hearing test.

Lungs: Doing well! We're weaning her from some of her diuretics, and so far, so good {knock on wood}. She's been consistently off of oxygen for over a month now, and we're thrilled to see how stable she has been.

GI: Still the same. J-tube feedings 18 hours/day. We're hoping that soon (in the next few months) we can start working towards G-tube feedings, and then possibly condensing those feedings so that they are at specific times (called bolus feedings) rather than the continuous feeding. The hope is that we can eventually get her to "eat" during regular meal times, and then start weaning those tube feedings so she can be hungry enough to eat by mouth. Basically, we've got a lot of therapy in our future. And I'm not just talking OT :)

Developmental: We've started with our new therapists here in California. We're seeing Occupational Therapy twice a week, Physical Therapy twice a week and Speech Therapy once a week (maybe adding another one on? who knows). Charlotte has started to crawl since we've moved her, and we're thrilled with her progress. She still has a problem with rolling over from her back to her front, so if she ends up on her back, she just pushes herself backward around on the floor. The back of her head will never have hair on it :) She is, however, transitioning from crawling to sitting, and from sitting to crawling. It's not elegant, but it gets the job done, and you can tell how excited she is about all of it.

ENT: The ENT team here has pretty much taken over the role of managing Charlotte's Sleep Apnea. Right now, that's probably her biggest struggle. Last night, she had 10 desats, a few into the 70's. She hasn't needed to be resuscitated in almost two months, but she does need repositioning and stim to bring her out of some of the more significant desats. She's starting to tolerate her BiPAP mask less and less, and honestly, I just don't know what to do for the poor girl. We have an appointment the beginning of August with the Airway clinic (pulmonology and ENT together) so hopefully they will have some better ideas about how to help her sleep. We still don't have a real reason why she's having the central apneas, and for that matter, we don't have a real reason as to why she's obstructing. We're mostly hoping at this point that she'll grow out of it. But that can take years (if it ever happens at all) and kids kinda need to sleep :)

Overall, Charlotte is happy. She's playing a lot more now that she's mobile, and its awesome to see her and Caleb play together so well. Basically, I just love this gal. She's a keeper (in case you were wondering!)

An Update

I haven't written a post like this in awhile, but for recording keeping sake, here's Charlotte Amalie's latest medical run-down:

Weight: a whopping 7.8 kilos (17.5 pounds)

Length: 63.5 cm (25 inches)

Eyes: Her left pupil still dilates differently than her right (scaring nurses who meet her for the first time, and neurologists) but we now know that the eye is related to the paralyzed vocal cord. We have our next follow up in April, and hopefully she'll still have a clean bill of health.

Vocal Cords: Left is still paralyzed, but she's making great improvement with how well she is compensating with the right vocal cord. We've started actual speech therapy (not just feeding therapy with the speech therapist) and we're slowly introducing sign language. She will be re-scoped in March

Lungs: Charlotte fluctuates from needing 1/8 to 1/4 O2, depending on her heart rate and her oxygen sats. She needs 1/4 a night about half the time, but can almost always be weaned down to an eighth by morning. She's still taking her diuretics to keep her lungs dry, and also has a few inhalers that keep her open and breathing. At her last pulmonary appointment they said she is currently better than she ever has been. Awesome! We're thinking that after RSV season, we might just be able to kick this oxygen. So maybe around May? We sure hope so!

GI: Charlotte's now on 20 hour continuous feeds. She's doing so so so SO much better on the continuous feeding than she was on bolus feeding. We have an appointment in two weeks to meet with the Complex Care Center at CHOP and we'll decide then if we want to start condensing her feeds again or if we're just going to roll with the continuous feeds. We believe that if she can just make it another 9 months or so, she'll be developmentally much more capable of handling the reflux and such. So we'll decide if we just keep plugging along for another 9 months or if we try to handle the reflux now. Handling the reflux now means another "event" is possible and she's more uncomfortable, but it's much more physiologically "normal" for her to have bolus feeds than it is for the continuous feeds. The continuous feeds help her reflux, but they stunt her gut and stomach development. So yeah, flip a coin. Any suggestions?

Developmental: Charlotte is capable of rolling over, but I wouldn't say she's rolling over. She's done it a few times, but it's not a daily occasion.  She's also capable of sitting for 20 seconds or so. These are our goals to master by HER FIRST BIRTHDAY (holy dang, it's a month away!).  Cognitively she's doing well, tracking and interacting and playing a lot more than she used to. It's amazing how quickly skills develop. We're also hoping that by her first birthday she'll be passing toys from one hand to another and that she'll be able to reach out and grab something more efficiently. Currently she reaches for things, but she has a bit of a hard time actually grasping things in an efficient manner. We're working on it :)

Medicines: Daily: Aldactone 2xday: Prilosec, Diuril, Flovent, KCL 4xday: Erythromycin PRN: Albuterol, mylecon, tylenol

Notice how I didn't label anything Oral or Feeding? Yeah, that's because she's not doing it. She'll put her hands in her mouth, and she's much less sensitive in regards to her gag reflex, but still nothing by mouth. Another goal for her first birthday, I suppose.

And that's our girl. Medically speaking, of course.

Milk Scan

We got the results from the milk scan this morning and they told us, well, pretty much nothing. Her gastric emptying time is within normal limits and it showed several episodes of reflux. No big announcements there.

So we're waiting to hear back from the GI department to see if they want us to go for a scope to check out her esophagus or if we'll just wait and see how things play out once she has her sleep study.

Hurry up and wait: the story of Charlotte's life.

Literally.

GI and Pulmonary Appointments

Charlotte has had a busy week. Yesterday she meet with the GI department at CHOP and today she had a follow up with the Pulmonologist, had a milk scan, and was evaluated for a sleep study. Phew. I don't know how she keeps up with herself... did that make sense? Must be the lack of sleep.

Anyway.

The GI suggestions depend a lot on the results from the milk scan. Charlotte's been having some problems with her feedings, and has a difficult time keeping her oxygen saturations up while sleeping.

I'm going to ramble here with a bit of an explanation, so those who want to can skip it.

When Charlotte had her g-tube placed, she also had a nissen fundoplication done as well. The nissen procedure takes the top of the stomach and wraps it around the esophagus, creating a valve that only allows things to come from the mouth into the stomach, and not allowing things from the stomach to flow up to the mouth. Vomiting, burping, spitting up, etc are not "possible" for Charlotte. {the quotes indicate that anything is possible in real life}. Since part of Charlotte's stomach is wrapped around the esophagus, there isn't as much space in her belly for food. So she can only take about 2 ounces of formula at once. (A 10 month old typically takes about 6 ounces at a time). So Charlotte gets 2 ounces 4 times a day, and then, to make up for the missing calories and fluid, she is fed continuously at night for ten hours. Charlotte has been having an increasingly difficult time with these nighttime feedings. She's been gagging and retching so much that she often turns blue, and cannot breathe, which tends to be an important thing to do. Luckily we have nursing and they have fancy things like ambu bags to keep her breathing.

So far, we've changed her fluid volume, spread her feedings out so they take longer (she's fed via a pump that feeds at a certain rate since she can't stretch her stomach fast enough to take all the volume at once), and added special bags that should help her vent (she can't burp, so the air has to be vented from her belly). So far we've seen some improvement, but not enough to be thrilled. Actually, not even enough to be comfortable with the current status quo.

The surgery team (who placed her g-tube and performed the fundoplication) and the GI team are currently suggesting that we change Charlotte to a GJ tube. Her current feeding tube feeds right into the stomach, while the GJ tube would feed into her stomach during the day, and into her intestines during her night time feedings. We're not totally on board with this suggestion, as we feel it takes care of her symptoms and doesn't address the problem behind it. Of course, that's a problem in and of itself, since we have NO idea what that problem actually is.

So we're waiting it out. We'll get the results from the milk maybe tomorrow, but probably next week. From this scan we'll find out if her stomach is emptying too fast (called dumping syndrome) or if it is emptying too slowly (called delayed gastric emptying). Either of these problems could causing the gagging/retching episodes that we have been witnessing. We'd treat dumping with fiber (tricky, huh?) and delayed gastric emptying would be treated with a motility agent (usually an antibiotic).

If it isn't one of those, it could be that her esophagus is narrowed. At that point, we'll work on treatments for that.

Clear as mud, eh?

Pulmonary wrap up will have to come in another post. But you knew that was going to happen, right?

Uncooperative

We had an upper GI/ G-tube test done yesterday. Basically they insert Barium into Charlotte's belly via the G-tube and see where the barium goes through an x-ray technique called fluoroscopy. Fluoro is a continuous x-ray (kinda like if you had x-ray vision) so you can see all the stuff moving around on the screen.

Charlotte was an angel and performed beautifully during the exam, except for the fact that we were trying to see what her insides look like when she is gagging and refluxing. So just to be ornery, she was totally fine. Once the doctor was certain she didn't have any problems with her nissen, he turned off the fluoro and we placed her back in the stroller and got ready to leave.

Pretty much the second the doctor left the radiation suite, Charlotte started gagging and choking.

Nice.

Way to work with us kid.

The upper GI did show that her nissen is intact. We still don't know why she's having such a difficult time with her feedings, even those delivered via the tube. We have a swallow study (yay for more radiation!) on Friday. The upper GI gave us an image of what happens when food is already in the stomach, the swallow study will give us image of what happens while food is going from the mouth to the stomach.

We also have an endoscopy scheduled for the 17th of December, which will give us a better idea of the actual structure of her throat and vocal cords (which has a lot to do with how well she swallows).