Cicily's first word!!! (or sign for those who make a distinction)

Chris and Cicily were playing last week - a game they play where Chris puts his head down and Cicily pats his head to get him to lift his head up and give her a kiss. Chris stopped for a minute and apparently Cicily was not done! She looked at her hands and with real determination put them together and apart repeatedly, signing "more". Then she would sign more and point to Chris' mouth. So we like to say her first words were "more kisses"!
She's been doing this sign a few times a day since then. I think it actually means more of that specific game, as opposed to more in general- which is quite adorable really.
She's also been "babbling" with her hands a lot in the last few weeks. (That's something Deaf babies who are signed to will do, but I didn't know if we signed to Cicily enough to have her do that, apparently we do, or she's just that smart. :)) She'll look really deliberately at her hands and move them and her fingers pointedly.
It's very exciting to have Cicily be able to communicate some. She's always done a fabulous job communicating with her expressions, but this is an exciting milestone for us all.

Upcoming Surgery - BAHA implant (stage 1)

*Postponed*

In taking with Cicily's craniofacial plastic surgeon (Dr. Singh) last week, we've decided to wait to have her BAHA implant done for another couple years. We are in the process of collecting opinions on the timing for this surgery. The factors in question being improved sound quality and bilateral hearing vs. cranial bone density.


We just scheduled Cicily's next surgery today. She will be having the screws placed for her BAHAs (Bone Anchored Hearing Aid) on Friday Novemer 16. This surgery will be done by Dr. Fucci - a neurotologist (surgeon who specializes in ears, hearing, and skull based surgeries). He will drill into her skull behind her ears and screw in 2 titanium screws on both sides of her head. The screws are each 3 mm long. One screw on each side will be used to wear her BAHA and the other screw on each side will be a "sleeper screw", just hanging out in her skull in case something happens to the working one.
It is an outpatient surgery, so no hospital stay- yay. Hopefully they won't have to shave off too much of her beautiful carefully grown hair to place the screws. (Moms worry about strange things, I know. When your daughter's having a surgery where the surgeon is drilling into her skull mm's away from her brain, should you really be concerned with her hair??? I don't know, but I am!)
After the 4 screws are in place (2 on each side of her head), they will osseointegrate (grow into her bone) for a full year. So next year, Dr. Fucci will do another surgery - stage 2- where he will make an incision on her scalp and attach an abutment to one screw on each side. The BAHA (same hearing aid she wears now on her soft head band) will then be placed on the abutment. And voila- she has functioning directional hearing.
If you want to look at the BAHA, and read more about it, here's the company's website:
http://www.cochlearamericas.com/Products/2013.asp

Journey to being an Oral Eater

While we were on vacation, I gave her a Red Vine to chew on. It's the first real taste she's ever had other than milk, or a tiny bit of something on my spoon. She really liked it and made a great mess!

Performing her own "oral stim" therapy! Using one of her many therapy toys- a vibrating toothbrush We get excited whenever she puts anyting in her mouth!

Cicily's been working on eating through her mouth in earnest this past month. She goes to see a special speech therapist in Phoenix once a week who specializes in feeding. She also has an occupational therapist who comes to our house once a week to work on eating with Cicily. So far she takes little amounts of baby food a couple times a day. When I say "takes" I mean, she sits in her high chair while I make her smile so she opens her mouth, and then I shove the spoon in. And when I say "little amounts" I mean she swallows about 1/2 teaspoon a day, while the rest dribbles down her chin. But, slowly and surely she'll get better at eating by mouth, I'm sure.

Our little game player

Cicily is becoming such a fun game player. She loves to play peek-a-boo! I love this video of her and her Daddy playing one night. (Sorry it's a tad bit dark.) A few days ago she was sitting in her highchair and she'd turn her head to one side and then look at me really quick and smile, then she'd turn it to the other side and look at me really quick and smile, and then the other side, and we kept playing like that for a couple minutes. She's a smart little gal- Sometimes I actually think she's much wiser than most of the adults in her life.

Another reason I love this video is because Cicily is wearing the Passy-Muir Valve on her trach that allows her to "talk" so we can hear her little voice. Love love that voice!

Photo Album - 10 months

An advantage of having a trach! I went to check on Cicily one morning and her face was smashed right into the bumper of her crib.
Cousin Aubrey, Mommy, Cicily, and Maya riding a train during our great vacation in Scottsdale
A cupfull of spoons - what a fabulous toy!! (Our "vacation house" in the background) Maya's been asking lately if Cis can sleep with her in her bed. So one afternoon, we put Cicily down by Maya for a picture and they both loved it.
Yet another sepia sleeper pic - but how precious is that!?!

Photo Album - 9 months

Isabel & Cicily - We left them on the floor to play and in our hotel room and after a matter of minutes they had the contents of Ondria's purse scattered and shared! Lookout Gma and Gpa's playroom in a couple years!
:) Guilty! :)
It was a HOT afternoon, too hot to swim outside, so we swam in Mom and Dad's bathroom!
Love the sepia sleeper pics
Just enjoying a good book on her "therapy step"

What does Cicily have?

Cicily has a craniofacial condition called Treacher Collins Syndrome (TCS). For Cicily this basically means she has a cleft palate, small lower jaw, small ears, and no ear canals. When put that way it actually sounds quite simple! In reality because of these things Cicily sees 12 doctors on a regular basis, has 4 therapists, and will probably have 15-25 or so surgeries. It's absolutely amazing the "domino effect" that happens when one little mutation occurs when a fetus is developing! Cis is such a tough little baby though, and she's so easy going. She handles all of her difficulties so well already, she's certainly up for her challenges!

TCS does not affect cognitive (brain/learning) development or physical development (however she is somewhat physically delayed because she's been so sick).

Cicily's cleft palate (which does not include a cleft lip), is very "wide". She pretty much does not have a "roof of her mouth", you can see her nasal bone by looking into her mouth. This impairs eating and speaking. The current timeframe for reconstructing the cleft is when she is 1 1/2 - 2 years old.

Her small lower jaw is the reason for her trach. As I've learned, our tongues are connected to our lower jaws. Cicily's lower jaw is small and underdeveloped. Therefore, this causes the placement of her tongue to be further back than usual and it rests on top of her airway (trachea, or "windpipe") not allowing air to enter or leave her mouth/nose. Her lower jaw will be grown through a procedure called a jaw distraction (current timeframe: 2-4 yrs old).

Finally Cicily has small ears and no ear canals. To make her appearance more "normal" (although her Mom honestly loves those little ears and will miss them!), she will have ears constructed when she is anywhere from 4-7 years old. Because she does not have ear canals she has a hearing loss and uses bone conduction hearing aids (implanted at 1 yr. old) to help her hear better.

Links for more TCS information:
(please keep in mind TCS has a broad spectrum with ranges from mild to severe, Cicily's case is somewhat moderate)
http://www.treachercollins.org/main.html Amie's (a pediatric med student w/TCS) site about TCS, includes great personal stories and pictures from a variety of people with TCS.
http://www.ccakids.com/Syndrome/Treachers.PDF My favorite comprehensive info on TCS
http://www.faces-cranio.org/Disord/Treacher.htm A good condensed resource on TCS