Chronic

Chronic... it's the word that reverberates in my head. I can hear it over and over - - much like it's skipping on my old white record player with the penny taped on the needle arm. No matter how hard I try to stop it, it won't stop repeating. When I close my eyes, I can see the word in print. I've read it in countless articles, written it down time and again in notes and have even used it here on the blog a time or two. It is a word - - the word - - that is used to describe so many of Gracie's medical conditions... Chronic Gastritis, Chronically Ill, Chronic Migraines, Chronic Middle Ear Pathology (ear infections), Chronic Pain and last Wednesday, a new one - - Chronic Lung Disease.

I was not, in any way, prepared for the later. I still am coming to grips with Chronic Pain - - that one makes me cry on a regular basis... having only recently come to terms with the fact that Gracie's pain is, indeed, chronic. While I've known that in the back of my mind, it's an entirely different thing to actually use that word and truly comprehend the meaning. Despite the effort I will make until I breathe my last breath, her pain will likely never go away. She copes remarkably well with all that she endures - - I am quite certain that if I were in her shoes, I would not.

There are several well-written and informative articles about pain in CHARGE Syndrome. Some of them can be found in two previous issues of CHARGE Accounts - - Spring 2007 and Summer 2007. Considering we've discussed this topic at length with professionals over the years and the fact that I read those articles when they were published two years ago (more than once), one would think that I'd have already come to grips with the idea as it applies to Gracie. But, when it comes to pain and your child, there's not a lot of room for acceptance.

Then, on the ninth, during Gracie's (ridiculously long) pulmonary clinic appointment, the word was presented again. Perhaps I was easily rattled because we had pulled into the parking garage at the hospital at 2:15 and the nurse practitioner didn't come into the room until well after 5:00. Perhaps it was because when the doctor finally came in (a colleague of our regular doctor), I was freezing from sitting in the over-air conditioned room (Gracie liked it, though), had been trying to keep Gracie occupied and happy for hours (she actually coped with the wait better than I did) and had to use the restroom for over an hour. Perhaps it was because I just thought we were there to have a check-up, get some paperwork signed for school and discuss a few "housekeeping" items. I am not sure it was any of these things or a combination of all of them... but I didn't handle the information well. It was a good thing that the nurse practitioner was in the room the while the doctor was and that she wrote down everything for me... because I missed half of the conversation - - a fact I discovered upon reviewing the notes with the nurse.

While Gracie is not formally diagnosed with Chronic Lung Disease, the doctor feels we have to treat her as having it. (Whatever that means... if it's serious enough to treat her for it, then it might as well be a formal diagnosis). She has primary aspiration - - which means that she aspirates her own saliva - - (as opposed to secondary aspiration which is refluxing, then aspirating). This is not news, of course. In spite of being trached, she continues to micro-aspirate her saliva into her lungs. For over two years, we have treated her with an inhaled antibiotic (a month on, a month off) to ward off the many trach "bugs" that are so common (and that seem to be very attracted to Gracie). Over the past several months, though, I have noticed that on the off months, she is getting more and more symptomatic of the bugs long before the medication is scheduled to restart. I shared this with the team last Wednesday. The consensus is that Gracie isn't developing a resistance to the med, rather she is not getting her lower lobes cleared out enough and is, therefore, harboring that bacteria all the time. The antibiotic, the (extremely proactive) doctor feels, is only masking the symptoms and the continuous micro-aspiration is doing permanent damage to her lungs.

As a result, she's prescribing a new medication regimen and CPT (Chest Physiotherapy - which is vigorous percussion of the chest wall). On months that she's on the antibiotic (which she believes will be through the spring months) she will have a total of six breathing treatments a day. The regimen is: Xopenex (to open her lungs) via nebulizer, followed by CPT (about 20-25 minutes), thorough suctioning to clear out all mucous brought up with the CPT, followed by Pulmicort via the nebulizer and then, finally, the antibiotic med via the nebulizer. Twice a day.

This is a lot... and I am not complaining because I will do whatever needs to be done... though I am really concerned with the amount of time this will take (Gracie's already getting a total of an hour of treatments a day). On the months that she's on the antibiotic, with the new plan, it will now take well over an hour. Twice a day. This on top of school, feedings, therapy, FUN... etc. Luckily, she loves her nebulizer and the CPT. But so far, she's not overjoyed about that hour and fifteen minute stretch at a time. We've tried it... and I absolutely have to give her a break. This is not the optimal way to deliver these meds or the therapy... there is a method to the doctor's madness. But, we also have to live in the real world. For the first few days I was emotional and angry about the whole thing. I had left that office feeling like I was going to be harming my daughter if I wasn't able to fully comply, yet also harming her if I force the full-length treatments twice a day. What I've come to realize, though, is that there are going to be days that I can't get all of it in... that I have to skip a med or a little of the CPT. We have to live in the real world... and we have to live.

And that brings me to the better part of this post... the "Other Chronics" in our lives! For instance, Chronically Amazed and Chronically Surprised!

On Saturday, my very intuitive daughter somehow knew that Mommy was in need of a pick-me-up and during the day's first very lengthy set of breathing treatments and CPT, she started pushing herself into a sit... all by herself... over and OVER!!! She pushed up on all fours, walked her hands up to her knees and pushed back into a sit. She then corrected her positioning, sat there very pleased with her achievement, got herself down and did it all again... at least thirty times! And she has done it many times every single day since!


Then, that night, she cruised from one thing to another for the first time...


And then, as a sweet reward while Preston napped on Sunday, Mommy gave her a well deserved, very luxurious pedicure... complete with a warm-lotion leg and foot massage!


The photo quality isn't great... these were all taken with my cell phone and the lighting was terrible. But they are actual moments captured - - the moments that made me refocus from last Wednesday's appointment and concentrate on all of the Good Chronic in our lives.

Foto Friday vol. 25

I said a couple of months ago that I would post some more of our favorite photos from the shoot Catherine did for us in June. There are so many that we love... so many... that it's truly hard to narrow it down! Here are a handful that bring us such joy.

Water Wheel

Gracie has made-up a new game to play in the bathtub - - a new game with a toy that has been in her tub for years... long before Preston was born! She did this over a month ago for the first time and I haven't seen her do it since... until Friday night. For every bath since then, she has played it. It's not something I encouraged, as I just wanted to wait and see if she'd do it again.

The Game: She removes the top part of the toy (the water reservoir), takes her index finger and spins the water wheel really hard and then stops it. Whatever color is on top, she quickly signs it and then spins again.

She will do this for about 10 minutes. I never interrupt - - only quietly watch in utter fascination. Interestingly enough, the water wheel is the only toy that she doesn't throw out of the tub! All of the others that float around her feet and where she can splash with her hands must be discarded... an obsession she's had for a very long time. Since her "toy removal" regimen is such a good core strength builder, I don't discourage her need to toss everything over the side! She picks most items up with her toes (her big toes work much like opposable thumbs!) and does a toe-toucher sit-up to grab the item with her hands and then does a lateral half sit-up to throw it over the side.
(I am ashamed to say that I DO NOT bear the core strength to imitate this series of maneuvers! She may lack balance and strength in many areas, but she has abs of steel!)

Backyard OT with Mommy (part 3)

We were at it again! This time with paint - - multiple colors - - and the kids used fingers, brushes, sponges... whatever they chose! First and foremost, this was fun. Lots of fun! Folded into all of that fun and tactile work were all kinds of learning opportunities. Disguised lessons - - my favorite way to work with and teach my kids.

We talked (and signed, of course) about the colors, the shapes of the sponges, the way the paint felt, the way the brushes felt, mixing colors and what each combination made (all the way until the combination was a murky brown!).

When they were finished, we inspected their colorful hands. Gracie was fascinated by hers - - staring at them for several minutes. They are pictured below... a work of art all their own.


When it was time to clean the sponges and brushes, we used a bowl of warm water. Thus, we talked about the water - - the color after we rinsed their tools, etc. Then we dumped the bowl and added more warm water to wash off their hands. We added ice and talked about it being cold.


We poured it out (actually, Gracie dumped it!) and we talked about where the water went (see Gracie looking through the bowl) and then how the bowl became a hat for her.


Then we laid the masterpieces out to dry on the lawn... here are a couple of them.
Never once did they realize how much "mommy school" was happening the whole time we painted and played!

Foto Friday vol. 24 - Shop Vac

These were taken through our back windows - - which is why there is a reflection in them. I didn't want to interrupt their fun and knew that if I walked outside with the camera, I would have altered the dynamics!

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!