Good Luck, John!

On Monday John started his 27 weeks at the California Highway Patrol Academy.
This is a big career change and is one that was several years in the making. I am so proud of him... and equally excited for him.
Since we met (12 years ago) John has spoken of a career in this field. For various reasons, the timing wasn't right and once Gracie was born, many plans changed. We did what made the most sense for our family - - John worked part-time (to keep his fabulous union health benefits) and I worked full-time to financially support our family. It worked really well for us on many levels, and while we were always grateful for the great insurance he had and the flexibility of his employer (and mine!), John was never satisfied professionally. During the last few years, he looked into other careers that seemed interesting and appeared to be an easier transition for our family, but he always came back to law enforcement - - specifically, the CHP.
Timing is everything and interestingly enough, several things happened at once to align John's stars - - the biggest of which were that Gracie was approaching the maximum dollar amount through John's policy and he was approaching the maximum age to apply for the CHP. If Gracie's insurance was running out, then there was nothing really holding him back from making the change... so he went for it. The interview process (just to be offered a spot at the Academy) is rigorous and takes many months. It was, obviously, a big decision for our family - - one that was carefully thought out. The Academy is a 27 week, live-in, military based program. He gets "liberty" on the weekends and Wednesday evenings (not all). Luckily, the only academy training center in the state is in West Sacramento, so he's close enough to come home during those liberties (provided he doesn't have too much studying to do). Two and a half years of curriculum are squeezed into that time. It's intense to say the least. But, after all things were considered, the right decision for John and our family was for him to pursue his dreams. It's his turn... his time... and he deserves it.
So, I have stopped working full-time and my "PIC" (Partner in "Crime", Partner in Children, Partner in Chores, etc.) is GONE! I do have help... my mom and Jim are always a phone-call away (as they've always been) and Judy, Gracie's nurse, is here during the day (until she moves away in July).
And, quite fitting... as John dove in to the rigors of Academy life on Monday morning, I dove in to life without my "PIC" by driving down to Stanford to take Gracie to a GI appointment. Nana came with us and Papa stayed home to look after Preston... thank goodness for both of them!
* * * * * * * * * * * * * * * * * * * * * * * *
The photo was taken as he rushed out the door Monday.
I will be keeping a little journal of photos of John during this time... as with the intense physical training they do, I am sure that he will be morphing back into a "twenty-something" - ha!

Like Father, Like Son

This is John at 11 months...

And this is Preston at 11 months... in Daddy's shirt!

Here are some more shots trying to get a "matched pose..." which was impossible!

Preston the "Present"

Not to be outdone on his sister's birthday, Preston tries to steal Gracie's show
by crawling into one of her giftbags! John promptly put the bag upright and out emerged the little gift below!

In case you were wondering... yes, he knows he's a funny little guy!

This had Gracie grinning up a storm!

Peek-a-boo!

Gracie's Birthday Evening

After her big day and a somewhat rough afternoon, our birthday girl rallied for a little party time at home. We opened gifts, hung out and then sang and signed all over again... this time with a very special cake made by her sweet Daddy. :-)

The crown had a repeat performance at home!

"Wow, Daddy! You made that just for me?!"

Daddy helped with this wish... I'll bet it was a good one! :)

Yep... She definitely likes playing with food better at home
than at school!

What a BIG day for our BIG girl of FIVE!!!

A school party for Gracie

On her actual Birthday, Gracie was invited to the Deaf and Hard of Hearing Preschool (DHOH Preschool) to attend class and have a birthday party. (This is the same class we've been visiting and it's taught by the DHOH teacher who comes to our home to work with Gracie.)
This day was a big milestone for her- - and a dream come true for John and me as her parents. There are so many "regular" things that we dream of for her... and this day was a big one... a birthday party at school with little friends, cupcakes and oodles of attention all for her.

Check out her crown made by her teacher
and teacher's assistant! :-)

Her "crowning moment" as everyone signed and sang "Happy
Birthday."

Mommy helped make a wish and blow out candles.

Happy Birthday, Gracie!!! :-) Soak up the attention,
girlie... it's all about YOU!

Um... can someone help me out here, please?!

"The chocolate is pretty good, but I think I am done... will
you be signing/singing to me again? I liked that part best! "

It was truly fantastic... and I still get emotional thinking about it. It's the very first real party she's ever had, as her health and stress tolerance have just not made it possible until now. Granted, she had a pretty significant recovery time from her big morning, but she got to have the big morning and that's all we hoped for!

Happy 5th Birthday, Gracie Girl!

Happy Birthday, Sweet Girl!

You are a big girl of FIVE now!

We are so proud of you!

With all our love,

Mommy, Daddy and Preston

....................................................................

Dearest Sweet Gracelyn,

I just can't believe that our Gracie Girl is five years old today. In some ways it seems like just yesterday that Daddy and I were a few minutes late to my induction appointment in order to shamelessly catch the last few minutes of American Idol!

Your birth certainly didn't go as planned... I was induced because your due date of April 2nd passed by and soon April 15th was upon us and you were showing no signs of wanting to leave your cozy place inside me! After hours and hours of labor, you were in distress and I had a fever, so it was off to the operating room for a c-section... and then you were here.

From the moment you entered this world and struggled to take your first breath, you defined "fighter" and continue to do so. Daddy went with you to the nursery and as I went off to recovery, I could see the worry in the faces of all our family.

I only caught a glimpse of you for a quick moment as the doctors worked feverishly to help you breath, so when Daddy came into the recovery room over an hour later, I asked him, "What does she look like?" While I was overcome with fear and sadness for the struggles you were facing and imagining tubes and machines helping you, your amazing Daddy said only this, "She looks like our daughter - - she is beautiful."

The next several hours, days and weeks were terrifying, overwhelming and exhausting - - constantly learning more about what this thing called CHARGE Syndrome was and how you were affected by it. The one thing that gave us peace was sitting in the NICU and looking into your beautiful blue eyes and seeing only love looking back. It's that same love, only now grown larger than life, that I see when I look into your eyes now, five years later.

Your struggles have been too many... your fights harder than many others... there have been more times than I care to remember that we feared we'd never see today. But it seems you, my brave girl, have never questioned those fights, and instead have only fought them.

You have never once ceased to amaze Daddy and me. Some say it's a testament to us as your parents that you have the spirit and disposition you do given all that you've been through... but we know that is just who you are. You came into this world like that and we've just been here to bear witness to the amazing things that you've accomplished in spite of all that you've endured.

You are, without question, the bravest, strongest and most resilient person I know. And, even though you are just five years old, you undoubtedly know more about "fight" and perseverance than people who have lived 80 years.

My card to you today reads, "Ask me what beauty is, and I will show you my daughter."
There are not words to describe how privileged, honored and humbled I am to be your mommy and to bear witness to the miracles that happen through you, around you and because of you. You are an inspiration, a teacher and a blessing - and I will forever wonder how I got so lucky to be your mommy.
Happy Birthday, Gracelyn Marie.
I love you with all my heart,
Mommy

Finally... results from procedures on 3/28... another LONG post!

Gracie had a Colonoscopy, EGD and Bronchosopy on 3/28.
That same day we received preliminary results - - the doctors' observations - - and then had to wait for all of the lab results to obtain answers. As some of you know, due to the preliminary findings that day, we chose not to update the blog until we had definitive answers... we were worried, scared and given all the symptoms she's been having for such a long time, we felt that putting partial info on our blog would only tend to worry others and/or encourage a Google search that would then incite more worry. We just needed to wait it out privately.
Her GI doc found a very irritated stomach and bowel - - which we've known about for a long while now. Gracie has active gastritis (inflammation of the gastric mucosa of the stomach) and small bowel lymphoid hyperplasia (swollen lymph nodes throughout her entire small bowel). In fact, what appeared in a previous study to be a large polyp was, in fact, a very inflamed lymph node. Several biopsies were done and we are happy to report that the tissues biopsied do NOT have any abnormal cells - - no intestinal lymphoma. This is WONDERFUL news. Although we knew in our hearts that this wasn't her diagnosis, it was hard to ignore the similarity in the symptoms and we only grew more nervous as time went on and we waited.
We still have no real answers as to the cause, but are clearly thrilled to have ruled out the one thing that loomed in our minds. We will be changing her feedings to an amino acid based formula that we must create from a pure amino acid base... I will learn more on Monday. She is already on the purest amino-acid based powder formula on the market, but it has corn product, which may be the culprit if she's allergic. However, all of the formulas she's been on in her life have had a corn derivative... so we'll see.
Other findings from that day:

• Her upper airway is a mess (we knew this)... damaged from reflux and it appears structural damage from the repeated intubations several years ago (prior to the trach) and her own structural anomalies from birth. We have already been referred to a new ENT at Stanford who is more experienced in difficult airway issues and will pursue that once the GI issues are more stable.
• Her lungs look GREAT! Despite the horrible reflux that she has and the inability to coordinate a functional swallow, Gracie is not aspirating at all! There were absolutely NO lipid (fat) cells found in her lungs. This is fantastic news!
• Her stomach is raw inside... with linear ulcers... active gastritis.
• We are waiting on more info from additional labs that her GI doc sent out... Gracie has always shown low albumin levels (even at points of near dehydration). Gracie's nutrition is constantly in a compromised state... so we don't know if her albumin levels are low because of that or an inability to properly absorb protein. http://www.labtestsonline.org/understanding/analytes/albumin/test.html
• We are also waiting for results of a pancreatic elastase test (determines the function of enzymes in the pancreas)

So... we wait for more info and in the meantime will make small changes one by one to rule things out. I stressed to her doc tonight that this is affecting every part of Gracie's life - - she knows that and is determined to help Gracie. Compromised nutrition affects her development and strength both physically and mentally; her discomfort affects her physically and emotionally. When she's uncomfortable, her secretions increase which increases her need to be suctioned and when she can't manage her secretions herself, she becomes distressed. It's a vicious cycle. And, the last place she wants to be when she's not feeling well is at school... which is where she needs to be to grow and learn and have little friends like kids should. I am truly all consumed with finding a resolution for her. It's literally on my mind every moment... constantly trying to troubleshoot, theorize, diagnose and cure.

Home for Easter Evening

Our little princess... happy on Easter morning after a sponge bath as I propped her up in bed only to have her scoot down again! She tends to be depressed in the hospital (who can blame her?) and isn't interested in much at all. Thus, these smiles and her sporadic interactions with her docs and nurses during this stay were a welcome break from her preference of lying facing away from the nurses station and covering her left eye to shield the light and, it seems, the world in general.
Gracie with her IV Bunny generously given by Child Life at the hospital. They also brought Gracie a very cute Easter Basket... donated by a thoughtful individual at our local Nordstrom.

(Our hospital, Sutter Memorial, relies solely on unsolicited donations to provide gifts, toys, projects, etc. to the countless sick children they care for. Every child in the hospital received a very special Easter Basket... every child.)

Our two sweet bunnies.

(Yes... Preston had to endure the ears to continue years of tradition started by Sophie and Gracie! He doesn't look any worse for the wear, though, does he?!)

"Whatchya got in yours, Gracie?"

Not having read the directions to our new camera, it took several tries running back and forth to get this shot of the four of us with Nana and Papa! Some of them are so blurry because we were all laughing hysterically and trying to keep the bunny ears on the kids!

What a difference just a few hours can make... we much prefer this photo to the first two! After a long soak in the bathtub to wash off the hospital stay, Gracie was thrilled to be home... but her location change didn't stop her from scooting right down in the chair!

We weren't discharged until Sunday following less than adequate renal panel results on Saturday morning. She finally bounced back into normal ranges on Sunday, but then we were left with the debate of whether to actually go home. Her GI doc was trying to arrange for her colonoscopy on Monday (with a bronchoscopy to be done by an intensivist to rule out an internal granuloma at her trach site... suspected from the massive amount bleeding she experienced while inpatient) and it seemed rather cruel to send her home to come back the very next day for a new IV and certainly some psychological trauma due to the rapid return. When it was confirmed with her doc that it would most likely not occur Monday, we happily came home. Now we are looking at the possibility of Friday for the procedures. I will be calling frequently until this is set in stone... we still have no conclusive answers to all that ails her and this is not acceptable.

On another happy note, Mr. Preston took a little step between John and my mom. I actually got it on video... so once I figure out how to edit and post it... you can catch it here! :-)

***Updated 3/26/08*** Gracie is scheduled to have a Colonoscopy, Esophagoduodenoscopy (EGD) and Bronchoscopy on Friday, 3/28/08.

Gracie in the hospital

Well, I haven't done very well with this so far... with no updates since February... and now not-so-good news. Miss Gracie was admitted on Thursday morning due to her GI issues. She is doing well and should be able to go home Saturday and then will return for an outpatient colonoscopy (and who knows what else) next week.

I previously posted about our intent to start a blended diet... that never got off the ground. Unfortunately, since Gracie has such a difficult time tolerating her feeds, it was really impossible to make a safe attempt at it... too much risk with getting her off balance with minerals, etc. We did try to bolus her really small volumes (feed her a set volume by gravity over a short period of time - - this simulates a more "natural" feeding regimen as opposed to her continuous feeds) but this did not work well either.

She had a busy week - - starting with a wonderful visit with our friends Mike and Natalie and their three kids on Sunday, which she not only tolerated but had a great time. Then Tuesday she had a two-hour visit to the DHOH (Deaf and Hard of Hearing) preschool class... a HUGE success. But... at the same time as all this "wonderful" she began having a hard time tolerating feedings and grew more and more quiet as the week progressed. (Interestingly enough, she was OFF her feeds during those great parts of her day...)
By Wednesday afternoon, she was having such a hard time tolerating her feeds that we dropped her to straight Pedialyte and that still made her uncomfortable. Wednesday night she spiked a high fever and we knew that she was on the verge of dehydration... so we called her GI doc and she was admitted.

On Thursday evening she had an X-ray that showed a strange loop in her small bowel... this did not show on a film she had in the fall. So, instead of a colonoscopy on Friday as planned, her doc wanted to have an upper GI study w/ small bowel follow-through. (Barium gets injected into her G-tube and then a series of films (X-ray and ultrasound) are taken to watch the progress.) Unlike the Gastric Emptying Study, Gracie did not have to remain on the table the entire time... thank goodness! The entire process took nearly three hours today and she was an amazing little trooper. Lots of waiting - - back and forth from her KidKart to the table.

The study was not conclusive... there weren't any true blockages or thickening in the small bowel as feared from the oddities in the film from Thursday. The study did reveal a hiatal hernia, a possible polyp (which was not a surprise as she has presented with a couple of tiny polyps in the past) and some sort of small bowel follow-through defect - - this was determined when the barium simply stopped moving through the bowel over an hour into the study and the only thing that made it push through was when more barium was injected to push it along. This leaves me wondering if she is constantly having her feedings get "stuck" and thus, never feels good and is super sensitive to even the tiniest of volumes going into her tummy.

So... the plan is now to have a colonoscopy and to take a look at the hernia next week. (Because unfortunately, the anesthesiologist on call this weekend doesn't do "elective" procedures on the weekend... DON'T get me started on how I feel about that... so instead of getting it done while she's already got an IV in and is here... we will take her home and bring her back for yet another poke... I hate that.)

Clearly, the hernia won't cause the diarrhea... but it would definitely contribute to feeding discomfort. The diarrhea will be addressed by sending a sample to the state lab for a very thorough work-up as the state lab can run tests for all the strange bugs, whereas the hospital lab only runs the "standard" tests - - all of which have previously been negative.

I will update when there is news. Please cross your fingers that we can get to the bottom of this for her... it's been such a battle for well over a year, and because it makes her feel so awful, it truly has become dehabilitating... and now she has lost a total of five pounds from her highest weight.

On a happier note... Gracie has handled this stay exceptionally well. Many smiles... some attempts at signing - - even with only one working hand due to the IV... a much better tolerated stay than any in the past. We are very proud of her:)
Also, coming to the hospital is always like a reunion of sorts. Since we haven't been in (other than out-patient procedures) since December 2006, it was great fun to catch-up with our hospital family. Many got to see/meet Preston for the first time - - as I brought him in for a bit today to see where we are - - and especially to see Gracie. I stayed home with him last night while John stayed with Gracie. This morning, Preston was very quiet - - which is unusual - - and seemed to be looking for his Daddy and his sister. Too sweet.

The mystery that is Gracie's Hearing

Gracie's hearing continues to perplex us all.

Many of you have emailed me about her hearing after seeing her with an aid on again... so here is the scoop in a (believe it or not!) abbreviated version. (I started this post a couple of weeks ago... now that I have time sitting here at Gracie's hospital bedside, I have a little time to catch-up.)

She has been seen by four different audiological groups... all have been left stumped by her presentation. As an infant and toddler, her ABRs (Auditory Brainstem Response test) showed a severe to profound loss. Behaviorally (tests in the booth and her social/behavioral responses at home) showed a moderate to severe loss.

When she had her PE tubes in her ears, she had a constant drainage problem - - and because she is so open on the left side, we would even see the color of things she tasted come out her ear. She constantly was on eardrops for the drainage/infection issue and over time we started noticing a decrease in her hearing behavior. Then she got pretty sick with her GI issues and heart problems in summer of '06 and by spring of '07, it seemed she wasn't hearing anything at all. And she wouldn't even consider putting on her bone conduction aid.

In September, we took her to the California Ear Institue to try and get to the bottom of her hearing situation. They have a great CT machine that you can sit in the seat and in about 90 seconds it gets the shots they need. Unfortunately, it was too scary for Gracie and she wasn't still enough. After explaining her history and what we've been observing, the audiologists decided not to put her in the booth and it was recommended we have a new ABR, OAE, ASSR and CT done - - all under sedation. So that's what we did - - and at the end of it all, the audiologists came out and told us that there was absolutely no brain response at all... TOTAL DEAFNESS... given her recent hearing behavior, we weren't floored by these results, but were a little surprised that there wasn't at least some brain response. They also told us to give up on the hearing aids because clearly, there was no point. Gracie had a CT to determine the presence of the auditory nerves and the cochlea. Unfortunately, since she is "structurally complicated" and her nerves are very small, the CT was inconclusive and an MRI was recommended. Well... Gracie has a pacemaker and thus, cannot have an MRI. So, our only option in regard to giving a cochlear implant a try was to have the surgery and they would find out in the OR whether or not the nerves were present/viable. We assume they are present since we had previously witnessed hearing behaviors, but felt that at this point, we weren't entirely comfortable taking the surgical route with no guarantees of success and if she really did have some hearing potential, that could be destroyed with the operation. Plus, our guts were telling us that all that fluid was causing an additional conductive problem... that the loss might not be complete or permanent. The team at CEI noted the fluid in their reports, but insisted that the fluid wouldn't have resulted in a complete lack of brain response. (I should note that Gracie is neurologically complex and her brain structure is not normal... we reiterated this to them and wondered if it's possible that this fact would affect the results... but they were confident in the results.)

In November, we requested the ENT take out her left tube (the right had come out on its own in the spring). He did... and patched the eardrum. At the same time, thanks to the persistence and help of Gracie's DHOH (Deaf & Hard of Hearing) teacher, the district approved an evaluation with an educational audiologist. She came in December for a few visits... right around the same time that we were certain that Gracie was turning to some of Preston's shrieking (after having thought it for a while, now we really felt certain). Katie, the audiologist, brought a nifty machine (the name of which slips my mind) that has a piece that looks a little like a giant stethoscope head at one end and has a microphone attached to the other end of the box. The "stethoscope" piece was placed on different areas of Gracie's head and body and then various sounds were made - - voices, banging of a drum, etc. The "stethoscope" piece provides vibro-tactile stimulation in response to the sound. Gracie loved it... so we started trials with her bone-conduction aid again and had positive responses/behaviors from her.
Once we made what seemed to be real progress, the aid broke and we were without it for over two weeks. So... now we have essentially started over with her auditory training.

We are still in limbo on this issue... so complex is our girl!