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!

Happy Birthday, Daddy!

Happy 35th Birthday to the greatest Daddy and husband in the whole world!

We love you VERY much!

Gracie, Preston and their Mommy! :-)

Concerning GI issues

For the past 18+ months, Gracie has dealt with persistent diarhea, a low-grade fever and the inability to tolerate her feedings.
The fever is an odd thing - - not sure if we can really call it that now as it just seems that she has "regulated" a bit. Up until she was about 3 1/2 yrs, her temperature ran pretty low - - about two and a half degrees below normal. She inexplicably began running just above normal summer '06 and it never went back. Her "new" baseline temp used to be a fever for her. Several of her doctors had previously told us that her abnormally functioning hypothalamus (which regulates your body temperature) would not ever change. She has had lab after lab ordered and nothing explains the cause... it's been "written off" by mostly everyone as her "new baseline temp", but it seems to me an odd coincidence that it occurred at the same time her bowel issues increased.
She has had persistent recurrent diarhea pretty much since August 2006... and it has only improved slightly while on one med. No results in labs explain the problem... none of her regular medications explain the problem and her formula should not be the problem. So... it's a mystery. Between this issue and the inability to tolerate normal volume via her feeding pump, we are struggling to keep her weight on.
As an infant and toddler, she dealt with cyclical abdominal migraines - - these transitioned to actual migraines as she got older. However, anytime Gracie isn't feeling well it manifests in her stomach. It's difficult to explain what happens and it's really torturous to watch how uncomfortable she is and how helpless we are, unable to give her real relief. There are times when her stomach is so sensitive that we can turn on her feeding pump at a rate of 15ml/hr (that's half an ounce over an hour) and she will react by retching. When this happens, we can sometimes give her ibuprofen and it will do the trick. Most of the time, however, we have to stop feeding her for a few hours and then start back up with just Pedialyte and move to a mix of half formula, half pedialyte and then work our way back to full formula. This usually takes 24 hours. If we go too fast, she gets worse and we have to start all over. She has lost more than 3 pounds and is now below 30 pounds. The weight loss coupled with the inability to get all her calories all the time has led to a set-back in her gross motor development. She is just now getting back to the level she was at in her gait-trainer (walker) in the Spring of '06.
Last month she had a Gastric Emptying Study that was less than impressive, according to her doctor. We fed her just one ounce of formula mixed with small amount of radioactive dye and for an hour, a machine took pictures of her gut to watch the digestion progress. (Needless to say, this was not a pleasant experience for her. Because sedation slows down motility, it is not used in this type of study. So, Gracie was wide awake and strapped to a table for an hour with a machine just inches from her face and body. She was terrified. John sat at her feet, giving her joint compressions & massage and I sat where she could see me, signing to her, suctioning her every couple of minutes and trying to help her understand that we couldn't go home yet.) This is not the first time she's had this type of study - - but the last one was before she had her trach, so she was only about 15 months old... all of them showed similar results.
Also in the last year or so, her GI doctor has taken three "peeks" at her stomach and bowels and has also biopsied the inflamed tissues all through her digestive tract. She keeps telling us that it doesn't look good in there... that we have work to do to get her better, but there is no real "plan of action" and we are feeling a little lost. We feed her the most elemental formula that is made - - it is broken down so that the gut has to do the least amount of work to digest it. This has not helped. We have tried numerous medications... to no avail. So, now her doctor wants to take another look and do a full colonoscopy. We told her "no, not yet." We want to give some other, more natural options a try. We wonder if her digestive tract actually needs to do more work. We might be wrong... but it can't be any worse than the multitude of medications and procedures that have done nothing to rectify the situation. So I am researching blended diets ("real" ingredients blended down to pass through her feeding tube) and hope to give this method a shot by the first of March.
Please keep your fingers crossed.

Last week in review

Last week was yet another busy week in our house.
Monday
Monday was Gracie's IEP (Individualized Education Program). This was not her annual, which will be April 18th, rather a parent-requested meeting to discuss transitioning her to a classroom environment. For those not familiar with the process, in special education, once a child turns three, they move from the local infant program (which provides services from birth through age three - - in Gracie's case, Deaf & Hard of Hearing Services, OT, PT, Vision Services and Music Therapy) to the preschool program through the local school district. Due to her precarious health, Gracie has always been on "Home Hospital" services, which means that because she is too ill to attend a class, her services are all in our home - - very similar to the infant program in that respect. She receives three hours a week of preschool instruction, one hour of Deaf & Hard of Hearing Instruction, OT, PT, Speech twice a month (for assistive technology consultation - - not actually "speech"), audiological services once a month (this is new after Monday's IEP) and vision services twice a month. So, now we are talking about the transition process. This wasn't our first meeting... we tried this last spring and, in fact, wrote it exactly the same as what was written on Monday... and it was not accepted by the "powers that be" in the district. I asked why it worked this time and not previously... there wasn't a "real" answer.
What we proposed was for Gracie to keep her existing home & hospital program and have social visits to a Preschool setting where she could get used to being around other kids and the completely new setting (the lighting, the kids darting about, the things in the classroom, etc.) We also requested that she be able to visit the county Deaf & Hard of Hearing Preschool on a social basis so that she could be around other kids who use sign as their method of communication. While the district agreed to the former, it appears that the later is going to take some time due to the logistics of crossing over from a district program to a county program. We aren't convinced that either setting is the right class for her, but want her to have the opportunity to be in a classroom setting, see other kids signing (because she is only around adults - - the only kids she's ever seen signing are on her DVDs) and just be around other kids without losing her services. Typically, the district's standpoint is that if a child is healthy to make social visits, then they are healthy enough to attend school. Our contention is that Gracie's sensory issues and life experiences do not put her in the norm and they need to think outside the box. New environments (or even familiar environments that aren't home) can cause her a great deal of stress and anxiety... which can easily transcend into an actual illness. It's so important that she have an opportunity to slowly move into a classroom setting without losing other services - - if she lasts five minutes in the classroom at first, without physically shutting down, that will be a positive in our eyes. If we move too quickly, we could cause her enough stress that could make it almost impossible to get her back to that particular setting.
So... for now, we are trying the Special Day Class through the district, which happens to be at the elementary school just around the corner. We will make social visits as Gracie tolerates and do our best to build up her stamina so that we will have a better idea of how to proceed in her transition plan come the annual IEP in April. Coming to an agreement at that time, we are certain, is going to be a challenge.
Tuesday
Preston's 9 month check-up! He's 28 1/4 inches and 20 lbs, 11.5 oz. Right in the middle... just as he's been from the get-go. His feet and hands are huge, though, and if he grows into those, he's going to be a big boy! Clean bill of health... always a blessing.
Wednesday
Alice, Gracie's Deaf & Hard of Hearing teacher, brought yet another headband for us to try with Gracie's hearing aid. Gracie wasn't feeling super spiffy while she was here, but later that night she wore her aid for 58 straight minutes. She adjusted it a couple of times, moving the bone-conductor piece, but not once did she take it off or protest. Clearly, she is getting some sort of benefit from the aid, as Miss Gracie is absolutely NOT one to leave something on her head "just because."
Thursday
Gracie had an appointment with her Pulmonologist - - her lungs are on "auto pilot" right now - - to quote Dr. Perez!
This is, of course, great news... and not unexpected. Since infancy, Gracie has only had pneumonia one time... from a cold that turned ugly when her heart was in failure in summer of 2006. Her lungs couldn't do their job because her heart wasn't doing it's job. So... this was good news... and... we didn't even get a hand-slap for the snow day! Ha!
In the afternoon, when I went to put her hearing aid on, I discovered it wasn't working. The battery was changed twice... still, no vibration and a strange feedback/echoing sound. Four-letter words ran through my mind in a frenzy... as we are just finally having success and now it's out of commission. And, it's the end of the week with a three-day weekend approaching... so the likelihood of getting it fixed fast is slim to none. UGH.
Friday
Gracie had an appointment with her Opthamologist. We expected this one to be a quick appointment... but her Dr. wanted to dilate her eyes. Then, what should have been a 20-minute wait after the dilation turned into an hour and 20-minutes. Needless to say, the kids were not very happy and thus, neither were their parents!
What we learned is that Gracie would now benefit from glasses due to nearsightedness and an astigmatism... if she'll keep them on. She is incredibly visually social and attentive, so perhaps this will be a piece of cake. She tried on these cool frames from Solobambini that are soft and have no hinges or screws - - very cool. She looked SO stinkin' cute and she knew it! :-)
Those will be ordered next week... and we'll see how it goes (no pun intended!). I do feel strongly, though, that if she will tolerate only the glasses or the hearing aid and not both, the hearing aid will have to win for now. It's imperative that we keep her interested in listening... no matter what she is actually hearing... it's a skill she needs to practice all the time.

Happy Valentine's Day!

From our "Sweet Hearts" to you...

Happy Valentine's Day!

Snow Day

Had to share these photos... we had a big day in the Swann house December 27th...
We ignored all Doctor's orders, as you sometimes have to in order to live a little... and took the kids to the snow. (Against doctor's orders for Gracie because she doesn't wear anything on her trach to protect her airway/lungs. She's pretty healthy in the lung department, but the extreme cold is not good for her since there is nothing protecting her lungs through the trach.)

Since we only have to drive about 40 minutes this time of year, we thought we'd give it a shot. (Often, new experiences can be terrifying and stressful for her, so we always take into account how long she'll be really upset/sick from the stress.) Perhaps things are starting to change for her... perhaps it's a combination of getting older and Preston's influence... whatever it is, we'll take it! She became very excited as the color outside turned completely white.

Once we got outside, she was happy, then unsure, then happy! It was too cute! Preston, of course, was game for the whole thing!
So... a big "baby book" day for both kids... the snow was a first for both of them! And, with all the storms recently, we'll be going back again!

We wished you a Merry Christmas!

Although we didn't get our Christmas wishes out to everyone again this year, our family and friends, as always, were on our minds. It's hard to believe that yet another year has passed by. It's hard to know where to start. Perhaps the most obvious change is the best place... we have (as many of you know) added a little Swann to our family! Preston James is our busy, happy boy. If we had known how easily he'd fit in and how much joy he'd bring, we'd have had him a long time ago! :-) Gracie might not so readily agree to that one! She absolutely adored him when he was born... he was small and still! While she likes him now, she's not fond at all of his fast hands that constantly attack her hair and her feeding tube! We cannot turn our backs for a second when he's awake. He has (they both do) all of these great toys and he's most interested in the tubing on Gracie's suction machine, her feeding tube and the wheels on her IV pole that holds her feeding pump. Go figure. He's already pulling up to a stand and trying to cruise furniture... and he just turned 8 months on Jan 9th. He's really an easy baby to take care of... as long as he's free to roam and play. He loves his Daddy (who doesn't love his Daddy? He's so much fun!) and especially enjoys his "boy" time with him. Rough-housing is the name of this baby's game! It doesn't take much to make him laugh right out loud! You can see a little bit of mischief in this picture... and his red cheeks from drooling so much! He just cut two teeth a few weeks ago.
Gracie is appropriately jealous of him which is so great to see... and it's funny... toys that she hasn't cared about in years, if ever, are now being played with because she saw Preston with them! If his hands aren't free to ambush her, she does seem to like him still... but long gone are the early days when they'd lie next to each other cuddling and she could stare at him for an hour!
The last 18 months have been pretty tough for Miss Gracie... there's been a lot going on with her health. From July of 2006 thru December, she was hospitalized five times. Not a good year. Since we were in the hospital during Christmas in 2006, we didn't get our "annual" update and Christmas card out then, either... Have I mentioned that time flies?
Gracie had her second (third counting her pacemaker placement) open-heart surgery in December of 2006. That was actually a breeze for her... especially compared to the first two. She handled it very well and healed quickly - - and having the trach made her airway access so easy. What's troubled her for the better part of 18 months or so is a series of GI issues that have made her quite ill - - that coupled with constant ear infections and a low grade fever for months and months. We are finally now starting to get a true handle on them (knock on wood) and are on the way to transitioning her to a classroom environment. (Cross your fingers! We have to work w/ the school district to come up with an agreeable transition process...) That's going to take time and a lot of patience, as new environments can cause her so much stress. She's so loving and social... it's just a tragedy that she's going to be five in April and hasn't really ever had the chance to interact with other kids other than in the waiting room at the doctors' offices. Hopefully, this is the last year that I have to say that we think the difficult health years are behind us... hopefully, they really are and she can start living a more normal childhood. She is, despite all she goes through, a miraculously happy little girl. She's so incredibly affectionate, she has a great sense of humor, she's strong-willed and SO stubborn! I have NO idea where she could have gotten that! :-)

Welcome to the Flock of Swanns Blog!

Pumpkins in January...?

Well... I am finally going to give this a shot. Inspired by many families we know in the CHARGE Syndrome world who use this medium to keep up with family and friends, I am going to become - - or attempt to become - - a Blogger.

Besides being a "public journal" and a way to keep everyone up to date on the latest news with us, especially the kids, it will satisfy my need to "talk it out" - - even if it ends up only being with myself and the keyboard! And... instead of me drowning your email with photo updates, you can check in here at your leisure and catch up on us! Another selfish plus is that I will have an electronic journal of events (if I can keep it up!) and since I haven't done any work on Gracie's scrapbook since she was born (but who had time, right?!), I obviously haven't started Preston's either.

So, check back often... maybe not too often... til I get the hang of it! :-)
After all, I started trying to get this up and running in the fall (thus the photo) and am just now getting back to it!

Blog with you soon!