First Day of 1st Grade

Today was Gracie's first day of the first grade... sigh.
I cannot believe that my little girl is now a big first grader. Perhaps because she didn't get to do the "normal" preschool routine and her "Kindergarten" school year was only a total of a little over four months plus summer school. Or, perhaps (and most likely) it's because I am in a bit of denial about how quickly time passes - - at least in some respects!

She wasn't all that thrilled about school this morning... didn't want any part of getting ready and was downright furious when we took her outside to wait for the bus. (Granted, she slept all of about three hours total all night... ouch for her... ouch for mommy!) But, when she actually saw the bus and got in her seat, she was pretty stinkin' pleased!

Shortly after the bus left, I went over to the school to discuss some things with the school RN and take Gracie's gait trainer. I got to see her happy little face for a brief second as she headed to another classroom for a bit. Her teacher and I also had the chance to talk today (we talked at length Friday when Preston and I took in a bunch of Gracie's supplies) and she's now seen the walking video and a bit of the blog... so she really knows what Little Miss can do! She's got Gracie's number now and plans on pushing her - - and I am happy! :-)

This is when she got home from school and
I asked her if she had a good day!
As happy as she was to be back at school, she was tired! After she cooled down from the ride and being outside, I fed her, gave her a breathing treatment and she took a 45 minute nap.

Then, it was off to PT and OT.
For PT, she wasn't all that excited about walking - - she was tired and, as luck would have it, it was the day that her PT wanted to time her walking certain distances for her assessment report... go figure! She had Gracie do other things instead and we will try the walking again on Thursday.

She was happy in OT, though... and sat up independently for most of the session - - only a few small protests and she was back up.

She practiced putting the wood blocks on the dowels and also threading them. Her favorite thing to do, though, is put her fingers in the holes and have blocks on all five digits!

She put the necklaces as part of OT, has little weights on her wrists and had all the frilly stuff around her, so Ericka, her OT, put the glasses on and I couldn't resist the photo op!

Hopefully, she sleeps better tonight!

Foto Friday vol. 23 - 50/50 Bar

All summer, we'd been freezing "wuice" (water diluted juice) popsicles for Preston. A few weeks ago, our neighbors offered him a yogurt popsicle. Brilliant idea if you haven't thought of this for your kids (or yourself)... which we hadn't!
No mixing with milk or juice required... just open the yogurt of your choice, fill the popsicle mold, freeze and voila! It's like ice cream! Well... it's not like ice cream, really :-), but I have yet to meet a kiddo who doesn't love it - - or love to share it!


(Hint: Yoplait Orange Creme makes great popsicles... they taste like a 50/50 bar! And Trader Joe's Vanilla is delicious, too!)

Pixie Stix - First session of Vital Stim

Several weeks ago, I scheduled Gracie's first appointment for Vital Stim and, as I was making it, I had a fleeting thought of, "That's the anniversary of her first open heart surgery." But it wasn't until the morning of the 25th that I realized there was a real irony in the date that she began this therapy.

The 25th of August, because it was the day of her first open heart surgery, represents a series of events that significantly detoured the path we'd been traveling with our daughter. In the weeks to come, she had four failed attempts at extubation (getting her off the ventilator), an episode of significant oxygen deprivation and repeated raised CO2 levels... all due to a very angry, floppy and, at the time, insurmountably compromised airway. Then came countless tests, procedures, the pacemaker, the trach and all that goes with it.

Her airway has since healed and, though there are structural anomalies within her airway, the reason she remains trached is her inability to swallow her own secretions. August 25, 2004 was the last day that Gracie had a functional swallow of any sort. Five years... to the day... she started Vital Stim. Though I remain cautiously optimistic, I would be lying if I didn't admit that I find great symbolism in this realization. I tried to tell myself that I shouldn't be more hopeful because of the significance of the date, but secretly I was (am). And as I wheeled Gracie into the reception room for outpatient therapy, I realized I was holding my breath.

I was so nervous. Nervous that she'd not even tolerate the electrodes on her neck. Nervous that it would be a negative experience for her and she'd not last the full time. I was nervous for Gracie because I know this is, at least as of today's therapies and medication intervention, our last chance at helping her rehab her swallow. Though I claim to be "cautiously optimistic," I guess my nerves proved that I am hoping beyond hope that something comes of this. My head knows it's a long shot - - and my heart believes in long shots.

As it turns out, my nerves regarding her tolerance of the therapy itself were all for naught. Gracie handled the first session beautifully. Her therapist was great with her and set-up slowly to give Gracie a chance to check it all out. She was curious about the electrodes and tape on her neck, but only gently touched them. As the therapist turned up the electric stim, Gracie would freeze a bit and get this look like, "What's that?" A couple of times, but only for a brief second, she grimaced as the intensity increased and the left side of her face twitched a bit. Best of all, (and guaranteed to make her want to go back again and again), she gets to taste things the whole time! She started with a lollipop (fitting, as we've affectionately called her "Lollie" for years!) and then progressed to a tongue depressor dipped in Orange Pixie Stix! She loved that! She signed "more" and "ready more" throughout the session.

During the three month trial, we will be watching for an improvement with the number of times she requires suctioning during a session (and later, at home), the amount of times she coughs and secretions come out her trach, her ability to swallow as we increase the "management" difficulty with the types of tastes she tries and she could possibly have a repeat swallow study. At the end of the three months, it will be determined how/whether to proceed.

And though the skies didn't part and Gracie didn't miraculously swallow everything in her mouth, she did have many audible and visual swallows during the session. One, in particular, was done with great timing and coordination in relation to her tasting of the Pixie Stix. Her therapist was completely impressed with her tolerance and was happy with the oral motor activity she saw along with the swallowing attempts during the stim. So, as I continually say regarding so many things with Miss Gracie, these are baby steps... but they are steps, indeed!

Saturday School

In an effort to keep Gracie moving forward (or at least not regressing) during the summer, I have been working with her in a structured setting for at least an hour or two daily. (The exception is the day her nurse, Nicole, works. That day is Gracie's day of fun... she spends at least three hours out at the park and loves it. And, she expects to dictate the day!) Some days when we work, she ignores me and "checks out," as we call it, and we keep trying to re-visit the structured learning activities. Other times, she's a signing machine - - communicating all she can about what she sees as we are working on a lesson.

Saturday, my little signing machine was in rare form and, let me tell you, it was FUN! She worked so hard and signed (unprompted) up a storm - - telling me colors of certain items, naming pictures, counting and recognizing numbers 1-5 easily and repeatedly. She matched items in one of her file folder activities and signed several words in two different books we read. She was "on" - - in a big way - - and I couldn't get enough of her!




We sign "strawberry" as "red fruit" not "berry" and "grapes" in this picture looks a bit more like "rash," but she's always signed it all the way up her arm!
She's also far more interested in taking off the numbers than she is in matching the numbers to their column... it's good OT work, I guess, but not the intended task!

Preston, of course, is always underfoot, which can make teaching Gracie difficult, as he always wants to do the things I am doing with her. This is fine, except that he often barges in and takes over while she's trying to process the task. I'm not complaining, of course... he's so stinkin' smart and I wouldn't have it any other way. I just need to find him a preschool... I can't keep up! He needs structure and to learn to WAIT and follow directions!

For instance, in the structured circle-time part of TOTnastics, he has the order memorized and is about 45 seconds ahead of the rest of the group - - this despite my repeated pleas to follow the teacher's instructions and wait! He does the same thing when we do certain lessons at home. And, even though I know he's only two and this may be typical, I wonder a bit if he's already bored! What to do... what to do?!

We also had recess... Bubbles!
Bubbles are a guaranteed "happy maker" in this house! After "Mommy School," I got out the kids' bubble machine (a stellar shared birthday gift from their Great Uncle Bob and Great Aunt Elaine... affectionately known as GUB and GAE!) and we were all happily entertained for over an hour.
Gracie, of course, stripped out of her clothes immediately upon getting out of her chair. Any tips for keeping my little exhibitionist clothed would be greatly appreciated! Thankfully, she still hasn't tried this anywhere other than in homes of friends and family. And, I know it looks a little barbaric that she's scooting on rough, patchy grass (she does concrete, too!), but if it hurt her, she wouldn't do it!

Yesterday, all our troubles seemed so far away!

For three Fridays in a row, Preston has missed his beloved TOTnastics class. The last two Fridays were because we didn't have Nicole working with Gracie, so when Nicole arrived yesterday morning, he knew he was finally going back to his class.

What he didn't yet know, was that I had secretly packed the tote bag full of towels, sunscreen, sand toys and lunch... and we were going to spend a couple of hours at the beach afterward!

He was so excited for class when we left the house! (He can and does, by the way, give me directions from the main road outside our neighborhood all the way to the gym!) As he climbed out of the car, he glanced in the back and saw the tote bag and said, "Go to beach, Mama." And, after thoroughly enjoying his return to TOTnastics, we did!


We spent an hour playing in the sand, tossing rocks into his water-filled buckets, eating lunch and then finally, despite the unseasonably cold weather, he took his shirt off and was ready to run and play in the water. I have no pics of the later, given that he was so wild and crazy, I couldn't even blink for the entire hour he played in the water!

It was so much fun... from 10 'til 3, we didn't have a single care in the world!

Foto Friday vol. 22 - A quick trip north

Last week, the Flock drove north and stayed with Nana and Papa for a few days. We actually had to leave our house while some mold/mildew repair work was done on the home we are renting. (Thankfully, the work was successful and the mold issue not severe enough to warrant us moving out... a prospect that's been hanging over our heads for the past month. Though our lease isn't up until November, we just renewed for another year. It's nice to know we're "settled" here for a full school year, but it's still weird for me to not know for sure where we'll be living in 13 months. I am a planner and like being settled!)

We had a 450 mile journey and left Sunday evening. This is the perfect time to travel with two kids and a dog... the fewest stops and no traffic. It was nice for John and me because we actually had some uninterrupted time for conversation... something that rarely occurs amongst the chaos at home! We arrived in the wee hours of Monday morning and that whole day was sort of a lost cause for both kids and their parents! We were tired! Gracie didn't feel well at all on Monday and spent most of the day asleep in her diaper. The highlights of the day - a yummy breakfast, a yummy dinner and just hanging out with Nana and Papa. (Somehow, we managed to bust out the camera only once while at their house!)

On Tuesday, we had a couple of appointments, but to make it fun for the kids, we sandwiched the appointments with two different park outings. The first was to a park that was fully accessible for Gracie. She really loved it and it was early enough in the day that it wasn't too hot to swing. Ironically, we'd never been to that park even though it is just a handful of miles from our old house. After the appointments, we took them to one of the sprinkler parks... and they LOVED it just as much as they used to! Once Gracie dried off and heated back up, though, she was miserable. So, back to the air conditioned house we went!




On Wednesday, we visited with long-time friends. It was so nice... like a mini-reunion at my college friend Becky's house. Amy, her twin sister and also my college friend, was there with her three girls and their older sister, Jenny, and her three kids were there, too, as were the girls' parents who have been such amazing friends and supporters to us over the years. (Amongst so many generous, kind-hearted things over the years, their dad, David, was a constant presence in the PICU five years ago. He regularly brought boxes of baked goods to keep the PICU nurses, doctors and therapists happy during Gracie's three month stay!)

Upon our arrival, Gracie quickly stripped off her shirt and then, later, took a nap! This made Auntie Barecca (her name is Rebecca... she goes by Becky... and I have always lovingly called her Barecca) so happy because she knew Gracie was truly comfy at her house... a miracle when just a year and a half ago, she would have panicked by simply being in her house. Here are a few highlights of the day... (Unfortunately, no postable pics of Miss Gracie and John managed to steer clear of the cameral the whole day!)


Sadly, our girls night planned for that evening got scrapped when I ended up with a sore throat and fever. Yep... sick, again... for the umpteenth time this year. So, we left Thursday morning for a very long journey home. Thankfully, the kids and John managed to avoid it. I am on strong antibiotics for the sinus infection that's now developed. Hopefully, these drugs will also kick the lingering cough I've had for weeks on end.

While we enjoyed our trip, we will never travel that far for that short of a visit again! And, one thing we learned while being in the heat up north - - we have become very accustomed (er... spoiled) to the cooler beach weather and cannot imagine living in that 100+ degree heat again, even though that was home for seven years. It feels like home here, rather than there. And Gracie, especially, was so happy to get home to the milder days where she can be outside any time of the day and better regulate her temperature. We miss family and friends terribly, but guys, your summer weather stinks! :-)

Head, Shoulders, Knees & Toes (Really... Ears, Throat, Heart & Head)

Audiology
In late July, Gracie had a very long, thorough audiological evaluation... again. With our move late last year, this new group is her sixth audiology group to try and get to the bottom of her hearing loss and the best way to aid her.

At our first visit in April, they did a booth test.
The report reads, "The results indicated a profound hearing loss, bilaterally."

Four sedated ABR tests over Gracie's lifetime show "a profound sensorineural hearing loss, bilaterally."

However, booth tests and social hearing behaviors up until late 2005 showed (sporadic) moderate hearing loss in the right ear and a severe-profound hearing loss in the left ear.
Additionally, we still notice sporadic "hearing behaviors" where Gracie will turn to sound that we know she's hearing - - not feeling. The interesting thing is that these are both high and low tones... which doesn't make much sense given her ABR results over her lifetime.

When we went to the CHARGE Syndrome Conference in 2005, we learned about Auditory Neuropathy and we were convinced at the time that Gracie had this. With Auditory Neuropathy (AN), a patient might have hearing, but sounds is like an out of tune AM station. There are tests that can be done to diagnose this, but Gracie never was a clear-cut case (she's not "clear cut" on anything!) so we never got a confirmed diagnosis... only "suspicions" of AN.

She has, as is often very typical of kids with CHARGE, "a history of chronic middle ear pathology." This is, I am thoroughly convinced, due to the fact that her ears are so "wide open" that her saliva drains out her ears when she has tubes. Her left ear is much worse... it literally pours out on her "junkier" days.

So, back to the appointment in July. This time... a non-sedated ABR was done to rule out and/or confirm Auditory Neuropathy/Dys-Synchrony. "Today's results indicate a profound hearing loss, possibly mixed, bilaterally, with concerns about Auditory Neuropathy in the left ear." So, again, not definitive, but AN pops up again and this time it's actually documented.

In a nutshell, Gracie is profoundly deaf... and even if she weren't, her left ear would cause great difficulty in discerning the sounds due to the AN. And now we are left (yet again) with the big issue of how to aid her... whether she can be aided... whether she'd benefit from a cochlear implant. This is not the first time we've discussed an implant. It's also not the first time that we've struggled with whether it's the right decision. While so many are "pro" implant, I continue struggling with the idea.

Over the years, with certain audiology groups (not our current), it has often felt a bit like, "they have great insurance, what's the harm in giving it a shot?" situation. While I know this isn't entirely true, I also know that there are so many things pointing to the "not implanting" column that no one can really refute. They can only say that there is "a possibility she could, with intensive training, access sound." The "possibility" is what makes me sleepless, stressed and ties my stomach in knots.

Gracie isn't (and has never been) a true aid wearer. She can't wear her BTEs (Behind The Ear aids) because of constant fluid in (or draining out of) her ears. She wears a BC (Bone Conduction Aid) during structured activities while sitting, but will NOT wear it any other time. In theory, a bone conduction aid shouldn't really be helping her (as her loss is primarily sensorineural, not conductive). She doesn't seem to mind the BC aid in those structured activities, but I have always questioned whether she just enjoys the vibro-tactile input rather than gaining any sense of sound from the aid. The fact that she doesn't seem to benefit from the BTEs when she can wear them (which is almost never) is a huge issue. We don't know if she'd even benefit from those aids because she constantly has a fluid problems. What would lead anyone to believe that a child who isn't an aid wearer would be a candidate for an implant? Furthermore, do we believe that Gracie can be trained/taught to use the implant even if it worked...? It's not a matter of just turning it on and "voila!" It's an intensive, complicated process. Does she have the processing skills and cognitive skills to use it? We know she's a smart little cookie, but we also realize how delayed she is and how affected she is by her language processing issues.

Another concern is her facial palsy. Gracie's cranial nerve involvement is so complex... she's "classic" CHARGE... no swallow at all, right-side facial palsy, all inner ear issues, no true blink on either side, her nerves are so small/narrow in some areas that they can't actually confirm presence or completion on a CT. Thus, "opening her up" for a look-see isn't all that appealing to us. Then there is the risk of damaging the good facial nerve on her left side - - a risk that is so frightening to me... she'd have bilateral palsy and lose all facial expression.

As I consider everything, it feels like moving forward with the surgery would be doing something to Gracie rather than something for her.
But, there is still the big, yet very unlikely, "What if it works?" that won't let me be...

Vital Stim
For years, we've tried to get Gracie cleared for Vital Stim. This is a swallow rehabilitation therapy using electric stimulation. Previously, the pacemaker company and her cardiologist weren't comfortable with her receiving the therapy as there wasn't much data. And even if we'd gotten their approval, there weren't any Speech Pathologists in our area who were certified or willing to become certified. They all stated that there wasn't enough data regarding the use of Vital Stim in pediatrics.

When Gracie had her latest swallow study done in the spring, I mentioned the therapy to the speech pathologist who informed me that many of the SP's at CHOC (Children's Hospital Orange County) were certified in administering Vital Stim. She also mentioned that she was fairly certain there were other patients with pacemakers. So, my phone calls started and... now... after nearly four years of hoping and trying... Gracie is approved by the pacemaker company, her cardiologist and our insurance for Vital Stim! She was put on the waiting list and now has her first session next Tuesday.

We are cautiously optimistic (because we like believing there is a miracle left for her swallow), but are also realistic. Vital Stim has not had a great track record for kids with CHARGE Syndrome. There are some successes out there, but most of those kids have a lesser cranial nerve involvement than Gracie.

This is (as of today's technology and medicine) our last remaining effort to work on her swallow. We have tried everything... even Botox into her salivary glands... without success.
Our goal is for her to be able to better manage her secretions... and swallow them enough to avoid aspirating. A great success would be if she'd have enough ability to swallow that she'd tolerate a PMV (Passy Muir Valve - a speaking valve worn over the trach). As of right now, she's not able to wear one due to the "bubble bath" of secretions that sits in the back of her throat and blocks the ability to visualize her vocal cords in a nasal endoscopy. An even greater success with Vital Stim would be that she'd have a chance at decannulation (getting her trach out).

Cardiology
She had a routine check-up and a pacemaker interrogation. Turns out Gracie is using up her battery life on the new generator much faster than initially anticipated. The battery life is estimated at 3.5 years... less than half of what we'd expected. She is completely pacer dependent and this, coupled with the additional work her heart does because of her mitral valve regurgitation, is causing her to use up the battery quickly. She will eventually have to have an artificial valve placed, but we are hoping to do that once she's older and bigger, thus minimizing the need for multiple valve replacements as she grows. She will be on Coumadin (a blood thinner) and require monthly labs once this surgery occurs. Our hope now will be that we can replace her generator and the leads at the same time, rather than having to have an additional surgery a year later to replace the leads.

We go back for another echo in November to check the mitral valve regurgitation. The hope is that it remains stable and her heart doesn't look enlarged from the extra work it does despite the pacemaker.

Neurology
Gracie had an appointment with her new neurologist a couple of weeks ago. Yes - - another new one because the one we saw when we moved here has left the practice. Thankfully, I really like the new one, so it all worked out fine. Unfortunately, though, the appointment was incorrectly scheduled as a return-patient (only 15 minutes instead of 45 - - and my opinion, when it comes to our "complicated" kids, 15 minutes isn't sufficient for a return-patient anyway), so we have to go back again to give the doctor time to get to know Gracie and her history better. I was just grateful that he wanted to take that time.

Good news: He wrote the prescriptions for her new wheelchair and walker :-)
Yay for mobility and independence!

Next medical update just around the corner, will include: Dentist, Vital Stim, GI, Pulmonology, Behavioral Pediatician... it never, ever ends!

Gracie's School Projects - Summer

Since they can't hang on the fridge or wall forever... and since I can't store every single thing in a box in the attic...

Foto Friday vol. 21 - Down at the Park

We ♥ the park!

I've had these pics set aside for a video montage for a while now... most of them were taken in May and June during our many outings to our neighborhood parks and school playgrounds.