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.

Four weeks worth of Foto Friday - The Long Lost Flock

Returning after yet another extended break in blogging. Super busy - - as usual - - but more tired and foggy brained. We have a lot of "stuff" on our minds and hearts (on top of the usual - - and too much to delve into here) and it has been making the days seem even longer and, thus, the fatigue that is routine somehow feels much heavier.

I should probably start where I left off - - and that was when everyone was sick. It turned around quickly for Preston. For Gracie, it took some weird twists and turns, leaving me to wonder if she had her own "ick" vs. the virus that the rest of us shared. And, to add some "excitement" to our days, she has been having (about every two weeks) a fever that lasts a handful of hours and then is just gone... poof. Each time, the fever goes up a bit and this past Saturday she had one that was as high as 104.4 (her baseline is just under 100) and made her very itchy - - not surprising, due to the fact that she was burning up. Then, around 2:30 in the morning, it broke completely and she was back to normal. We aren't certain of the cause, but my gut is telling me that it's a neurological thing, rather than an infection. Needless to say we are taking note of everything to share with the doctors. Anyway, she's back to "normal" with the exception of the fever thing and has had some really good days this week.

I finally went to the doctor for my persistent cough and was given an inhaler and told to take allergy meds again. So far, not much help. The cough is an annoying tickle that makes me grunt and groan if I try to stifle it... very attractive. Otherwise, I feel fine. As for John, he was the luckiest of all of us and was spared the horrible ick, only having gotten a very mild version. At least someone was spared!

Gracie's summer school ended on Wednesday - - she doesn't "get" it yet that she won't be going back to school for a month. I am in panic mode trying to think of ways to spice up my "Mommy School" routine, as she had grown quite bored with me by the time she finally started school here in April! Thankfully, her wonderful aid sent home some of her materials so that I can keep up with some of the things she's been working on. And, even though I am completely bummed that summer school is over (mostly because it's so hard to help Gracie understand why she's not going when she's begging to go in the mornings!), I am so grateful that during this insane economy and horrible budget crisis, there was a five-week summer program for her to attend four days a week for just under 4 hours. Very grateful.

The bus situation eventually worked out, thanks to the dedication of her aid and the RN at the school. But we aren't sure what will happen in the fall. Both individuals, sadly... very sadly... might not be working in the same capacity next year due to budget cuts. If this is the case, especially with her aid, then the bus will not be an option until I am comfortable (and Gracie's comfortable) with her new aid. In the meantime, I am trying not to think about it... as losing her health aid (who also serves as her instructional aid and who has dedicated herself to taking more ASL classes aside from what the school provides her...) would be tragic at this juncture. She has earned Gracie's trust and they have built a really good rapport. Her aid understands her, can read her, respects her health issues and her chronic discomfort, yet pushes her whenever possible. And, she's not afraid to ask me questions to make sure she's picking up on Gracie's cues correctly. Losing her would be tragic... a huge adjustment... like taking steps backward and starting the transition to school all over again.

We have had some fun amidst the "heavy fog" I mentioned before, and that is what I'll share now as I ease back into bloggerland and catch up on four weeks of missed "Foto Fridays!"

Nana and Papa's Visit
Nana and Papa visited us the second week of July.
Papa is finally home from his year-long service in Afghanistan.
(It's hard to see in the dark picture, but the kids colored on the banner we hung for Papa!)
It was wonderful seeing them even though we were all still feeling a bit funky.
We just hung out - - had breakfast at the beach and let Preston play while Gracie was at school, they went to Gracie's therapy to watch the "walking wonder" in person, they went to TOTnastics with Preston to watch the "climbing, somersaulting, head standing, jumping, balancing wonder," they went on walks with us in the evenings. It was so nice to just "be" with them.
(As a side note, Preston actually runs the entire "walk" most nights... the longest was 1 1/4 miles!!! I clocked it the next day! I don't have that kind of stamina!)

One day after Gracie got home from school, Papa took us on the Joint Forces Training Base so the kids could look at the old helicopters. Preston LOVED every minute of that!

Preston doin' his thing at TOTnastics

Gracie showing off her triceps! Ha!
She's actually "sneaking" her way back down from sitting up!

Chop Those Locks
The kids got haircuts again... Preston twice. John took him to a butcher barber, who was supposed to give him a trim in the front and shorten the back a bit. Instead, he chopped-off his hair - - DRY! It was so uneven and horrible... and I will admit, without shame, that I did shed a little mama-tear over it. Granted, I know it's just hair, but I also knew that if Preston had gotten ahold of the scissors himself, he'd have done a better job! Anyway, I let it grow out for two weeks, and then Nicole (Gracie's nurse on Fridays) helped me take both kids in for proper cuts.
And, just like I shared back in the spring, my girl is such a girl! She loved every minute of being pampered! Nicole and I were cracking up at her!

Sigh... I just adore her expression as she checks herself out in the mirror!

Little Boy of Summer


Snail Mail - A parcel of fun from dear friends
My long-time, dear friend, Becky, and her two adorable daughters, Abby and Emma, sent Gracie and Preston a package of fun. They loved opening it... and they really loved what they found inside! :-)
For Gracie, Glitter Lava... slimy, squishy and oh-so-much fun!
For Preston, a Curious George coloring book and Caillou computer game!
And, special, sweet notes from Auntie Becky! :-) Thank you!!!

Gracie did share with Preston, although he didn't dive right in the way she did!
It's hard to believe that just last year we had an entire goal written for this kiddo to tolerate five novel sensory tasks!

He loves coloring - - for about five minutes!
He's all about sharpening the crayons or organizing them in the box!
He can't decide if he's a lefty or righty! I don't care either way, but I wish he'd figure out his preference soon! He bats, throws and eats better lefty... we'll see!

Ugh...

This is what our house looks like so far today... ugh. Lights out, blinds drawn shut to keep out the sunshine, thermometer, stethoscope and nebulizer working overtime.
I guess it was inevitable. There is something running rampant in the community... and since I am still nursing a relentless cough from two weeks ago and since John got a mild version of my "ick," I guess it's no wonder that the kids finally gave in and got it full force.

He: 100.2 (Tylenol on board) with a stuffy nose, cough that sounds like a small seal and clingy (and cuddly... I like that part) as can be. Saw the doctor yesterday and have antibiotics on stand-by if he gets worse. Otherwise, lots of rest, fluids and nose blowing. (Suctioning with Gracie's machine, actually. Thankfully, he doesn't mind this and recognizes the benefits. He's even tried to do it himself!)

She: 101.4 (Tylenol on board) with a stuffy nose, cough and stuffy trach, still raging ear infection and a visit to the doctor in store. She's not yet cuddly, rather wanting her space and sleeping on and off (since she didn't do that last night or the night before). I've given regular breathing treatments since yesterday after school to keep her lungs nice and open... but she probably needs an antibiotic sooner than later.

Switch Hitter

Attention MLB Recruiters (National League):
He's a southpaw who can switch hit!
Post your offer for the 2030 season (his momma is going to make him finish college first) under comments and if we are interested, we'll have our people call your people! HA!!!!

(Video taken June 13, 2009)

Backyard OT with Mommy (part 2)

Oh... the simple pleasures of a bowl of cornstarch and water! Gracie could mess with this for an entire afternoon... and I could easily watch her! These were taken in May, but we do this about every ten days or so... it never gets old!

She loves getting goopy (Preston does not! That's his hand holding the spoon... and that's as close as he got!) and we find all kinds of ways to make it more entertaining - - as well as challenging from a fine motor perspective. I will make the mixture really firm at the bottom of the bowl and then add water. She ends up sticking her little hands into makeshift quicksand and gets her fingers stuck at the bottom. It's a lot of work for her to pull her fingers free - - and this is good for strengthening, plus it cracks her up! Using a spoons to stir and scoop - - and adding in miscellaneous objects/ingredients) also provide great fun along with strengthening hands, arms and fingers... and coordination.

Fun... with a purpose!
(A two-fold purpose, actually! Good OT... and good OCD management! She can't strip naked when her hands are immersed in this mess!)

The Fourth... Ouchies and Cupcakes

He's okay.
I, on the other hand, am trying to recover.
Preston and I went outside in the morning to do our normal "doggie poopie pick-up" in the back yard. He ran to the grass to start taking inventory (he likes to tell me where it all is...) and I stopped on the far end of the patio to fight a battle with a spider and it's web. Using the big broom as my weapon, I didn't hear Preston come up right behind me. I took a big sweep backwards right as he ran up to me... and wham... impact.

The most sickening feeling overcame my stomach. The sound of the broom handle and his eye meeting along with the sound of his screams and sight of him shaking in pain will take a long time to leave my mind. He's okay... I talked to the advice nurse at the hospital and am doing and watching for all the right things. He is seeing normally... and we will continue to monitor that carefully for a while. He took a mighty blow and will surely have quite a shiner to show for it. My mommy heart is scarred, though, for being the cause of this accident. Not so happy morning of the fourth.


Little Miss was not dressed for very long today. She has a raging ear infection in her left ear and spent half of the afternoon asleep in a diaper under the ceiling fan. But, while she was dressed and awake, I took these pics! The hat that Gracie's wearing was a project at school, as are the decorations I taped to the mirror behind the kids.
(Thanks, again, for the adorable outfit, Clayton Family!)

How to eat a cupcake...and how to recover from a battle with a broomstick handle.

Thankfully, Gracie rallied a bit in the evening and when John got home from work we all went for a walk. We ended up meeting some very nice people a few streets away and they invited us to watch fireworks. It was really a nice, unexpected ending to a rather dreary (but for the cupcakes!) Fourth of July. Preston befriended a sweet nine year-old who was so patient with him and Gracie was very content sitting in the stroller (out of the smoke line), signing "colors" and "lights" over and over.

Foto Friday vol. 19

Sigh... This was Monday morning... Gracie's first day of summer school and first bus ride... and much harder for Mommy than her first day of school without me observing. Much harder.

Having Gracie take the bus was not an easy decision. But the fact is, with me taking her and picking her up each day, Preston and I were spending almost two hours in the car just for that purpose. Hardly fair to him... and with a list of "to-do's" as long as mine, not exactly the most effective use of my time. Since the summer school campus is a bit farther than her regular school, summer seemed like a good time to give the bus a trial run.

Her health aid rides with her, so she is with a trusted person and in good hands. We've had a full week on the bus now (no school on Fridays) and I am not yet sold on the idea. It could be because this is the first week where school has finally seen some of the "real" Gracie issues with feedings and excessive secretions. Their "honeymoon period" is over and that has been a little more stressful for all of us. Gracie does appear to like the bus and her aid says she does fine. It's me... I am not so fond of the rush to give report on her status in the morning and receive a report on how her day went. It feels chaotic and every morning I feel like I've forgotten to do something important. Plus, I am missing what had been my favorite part of the day - - watching her face through the rear view mirror as we pulled into the drop-off circle and seeing her light up with excitement.

I will give it time. It has, after all, only been a week and we are all trying to iron out the kinks! The great part about the bus is that Preston is getting a lot more quality time with me during the morning.

Thanks for the great idea, Hannah! :-)
This kept Preston busy for a long time! He loved it!
Although, you can see that his favorite brush is the broom! He likes clearing his canvas!

As I cleared the table after dinner that same night, I looked outside and saw this sweetness... they don't even know I took this!

Pigtails and Art... Body Art, that is!
Check out my girl coloring her arm!
This is one of her favorite activities!

"Momma... find me!" I hear Preston calling from his room.
"Okay, Bubby! Here I come!"
I walked in his room, saw him in his toy box, grabbed my camera and took these hurried and blurry (but fun!) pictures of him in his new hide and seek spot!

Preston with his Auntie NuNu (John's sister, Kristen) at the beach playground.
She came to see the kids (and us) this week!