Cicily's blended diet recipe

I've been asked many times for a sample recipe of what we feed to Cicily through her g-tube. We've been doing a blended diet (blending all normal food) for Cicily since she was a year old. Cis had a feeding therapist when she was a baby that always told me to keep her eating habits as "normal as possible". Feeding her real food just seemed like an extension of that and it was something that I just felt would be really good for her, so we do it. I've never counted calories for Cicily, because I don't count calories for my older daughter either. Some days Cicily gets more calories, some days less. What I've done is taken the FDA guidelines for a child Cicily's age and created a healthy recipe to blend and feed her. I change and increase the ingredients as she grows. When she was in the hospital in July I finally had a dietician look at her recipe and she ran the numbers and said it looked great.
Here's the recipe, this feeds Cicily for one day. As always, feel free to leave your own suggestions or questions.
1 1/2 -2 C. grains (barley, oats, rice, wheat, millet, quinoa, etc.) I do raw with 2 C. water in the Vitamix and blend for 3 or so minutes. I used to used the equivalent of already cooked grains.
2-3 servings of veggies (whatever we have around on that day)
2-3 whole fruits
2 T flaxseeds
2-3 t. olive oil, coconut oil, or any other oil
1 C milk
1 cooked egg, 1 serving of cooked meat we ate that day, or 10 almonds
1 t. salt
Sometimes I add 1/2 C plain yogurt, spices, beans, or whatever else I feel like feeding her
I first blend the grains, meat and water together for a few minutes, in the VitaMix blender, then add the milk and everything else and blend for another few minutes. With each meal I also give her 2-3 oz milk or water (to rinse out the bottle I used to measure her feeding).

My sister- the aspiring respiratory ther./nurse/doctor

This is Maya and her patient Lucy. The other day Lucy was coughing and Maya calmly, but quickly ran over to grab the face mask we use to do CPT on Cicily. (CPT is when you take a mask , or cupped hand and just thump on a person's chest to clear their lungs. We frequently thump Cis' chest when she's coughing.) Maya proceeded to pick up Lucy, lay her down and begin CPT. Treatment was successful -Lucy stopped coughing.

I suppose Maya could be aspiring to be a respiratory therapist or, even better, a Mom to her own wonderful child like Cicily. We've already started wondering when we'd feel comfortable leaving Maya and Cicily alone at home. Let's see, Maya can already replace an HME, suction a trach, call 911, run a feeding pump, and perform CPT when necessary. We'll start working on trach changes. ;)

Preschool Girl

First day of school

What is that smile all about?? I could not get a real smile out of her that morning. Maybe she was just too excited about school.

Last week the preschool had a carnival. Cicily got her face painted!

She always wears all of her "daytime gear" to school: hearing aids, glasses, orthotics, and trach and g-tube of course.

Cicily started preschool last month. She goes to a district-run preschool about a mile away from our house. We were pretty sure we only wanted to send her if she could have her own nurse with her in class. However, after meeting all the faculty at this preschool we felt very comfortable sending Cicily without her own nurse. There is a great nurse at school that helps 3 of the children who have trachs. She goes from classroom to classroom to make sure they're safe and don't need suctioning. Cicily's teacher is fabulous!!! She signs to Cicily, sings with the class, is very easy-going and even suctions Cicily's trach! Cicily has physical, occupational, and speech therapy at the school. I love her PT, she's always giving us new suggestions and doing new things to help Cis learn to walk. Cis' OT is one that worked with her when she was one year old, she's been a favorite OT, so we're glad to have her back! And her ST has worked a lot with kids with a hearing loss and I've been impressed with her plans so far.

I went to school with Cis the very first day to make sure everyone was comfortable with the trach and to see it all in action. Everything seemed to go well, so she's been going on her own ever since. She cried when I dropped her off for the first week, but now she goes with no problem. She's always so excited to tell her Daddy about what she did at school when he gets home. We're very glad she likes it and is gaining a lot of new knowledge, social learning, and therapy skills.

Hippotherapy

What is that you may ask -as we did - some kind of therapy employing hippogriffs? It's actually therapy on a horse. Cicily does her hippotherapy with an experienced, caring physical therapist named Rachel and a patient pony named Ruby. I could explain hippotherapy, but this is a short, great explanation already written, (plus I'm really not a hippotherapy expert yet). http://www.americanequestrian.com/hippotherapy.htm It basically just makes sense in an intrinsic sort of way - you sit on a horse that moves and your brain figures out better how to move. It is more expensive than other therapies, because we have to pay the stable costs. We sort of count it as Cicily's "extra-curricular" activity for now, since we can't exactly pay for dance or singing lessons for her, so she gets horse time (which she probably loves better anyway).

Cicily LOVES seeing all the horses and petting Ruby! Enjoy some pictures of Cicily enjoying her Ruby time.

Signing "walk" to let Ruby know to get the fun started.

After each lap around the arena, Cicily gets to climb off Ruby and tickle her nose. It's definitely Cis' favorite part of the whole deal.

Telling Ruby thank you.

Mommy's favorite part of hippotherapy.

I Love You Gramps

Cicily and her Great Grandpa Vern Watkins. Cicily was sitting with Gramps at her birthday party and her nurse Kelly snapped this priceless picture of Cis signing "I love you" to Gramps. Cicily has always been very fond of her Gramps. He always delights Cicily with his realistic bird calls. She smiles huge, makes her delighted sigh, and signs "bird". I don't think I've ever seen Cis sign I love you quite that well either. A truly dear picture to capture a dear relationship.

Three Years!!!

Cicily giving kisses to her new baby. Maya gave her the doll and it is her favorite!!

Opening presents, while getting a breathing treatment and a g-tube feeding - all in a morning's routine - even on her birthday.
Cis' new HUGE horsey Grandma and Grandpa gave her.
Maya and A.J.'s hiding spot from which to jump up and yell, "Surprise Cicily, Happy Birthday!!"
Birthday party- opening presents from cousins.
Dad helping Cicily get some of the booty from the pinata, before it's all spoken for.

Cicily turned 3 years old last week! What an adventurous 3 years it has been! Cicily's packed so much into her 3 little years - too much in some ways and not nearly enough in other ways. I'm not sure how many times I've rejoiced that she was able to be here to celebrate her birthday with us. The night before her birthday as I sang to her before bed, I was crying thankful tears that we get to see her smile on her 3rd birthday.

The morning of Cicily's birthday, she opened presents. She got lots of birthday wishes on her special day. The next day we had a surprise birthday party for her. All her grandparents, and most of her cousins, aunts, and uncles celebrated with us!

Cleaning a Toilet

Cicily was a little quiet and I hadn't seen her for a few minutes, so I asked Maya to go see what she was up to. Maya comes back, laughing and says, "Mom you're going to want to see this, come on." I followed her to the bathroom and this is what I found.

How many Moms get the chance to feel sincere joy at a scene like this??? I love it for a few reasons: 1. She somehow reached all those things and manipulated them appropriately. 2. She knows what to do with a toilet brush and toilet paper. I've never taught her that. We know she's cognitively on target, but I always love seeing proof! 3. She was LOVING it! Loving cleaning a toilet.

My Nurse

This is my very own nurse Kelly. She comes to play with me everyday except for Wednsdays and Saturdays. She also takes my temperature and listens to me breathe. I love playing with Kelly everyday, I wasn't ever nervous around her like I am some new people. I like that she wears glasses like me! Kelly is learning all about me and my little extras, today she even changed my trachy. Hopefully she can go to school with me in October to keep me safe. Thanks for being in my life Kelly!

White matter specialist appointment update

We took Cicily to see Dr. Bonkowsky in Salt Lake City this week. He is a PhD/MD who researches white matter diseases (leukodystrophies - I'm going to have to get used to spelling that one!) He confirmed that Cicily does have hypomyelination (an absence of the white matter/ myelin coating her brain). He also was somewhat surprised to see her abilities given her limited white matter. From what I understand (which is not a lot, as I'm learning yet another set of medical jargon), hypomyelination is caused by an underlying leukodystrophy. According to Dr. Bonkowsky, 30-50% of leukodystrophies are undiagnosable at this point and none of them are treatable/curable. The United Leukodystrophy Foundation has some good information: http://www.ulf.org/ Cicily's leukodystrophy is "static" though, meaning she should not get progressively more severe.
Dr. Bonkowsky suggested 5 more tests in addition to the other ones her doctors here have already done to identify the leukodystrophy or what causes her hypomyelination. The good doc also enrolled Cis into his research pool to use her case in future research and inform us if there are any new developments.

So Cicily has "Treacher-Collins - like syndrome" and a currently unknown leukodystrophy. It's a novel combination and Cicily will show us all how they affect one another and what she can do inspite of it all.

Glasses Girl!

I just want Cicily to be normal

"NO! I just want Cicily to be normal." That's what Maya said when I told her that Cicily was going to get to wear glasses. I was quite surprised at these strong feelings. First of all, I hate using the word "normal". Second, glasses are pretty common! Third, trach, hearing aids, eating through a tube - not exactly "NORMAL"!
So Maya and I had a discussion on how lots of people wear glasses and it helps them see and Cicily is really excited about wearing glasses. (She actually is, we tried them on at the store and she was loving it! Though it may have been all the helpful people surrounding and oogling her more than the glasses.) It sort of seemed by what Maya was saying that she just likes Cicily the way she is and worried glasses would change her Cicily-ness or something. But, by the end of the conversation Maya was saying she wanted glasses too. I told her maybe Cis could share.
Tune in in a few weeks to see a picture of Cicily sporting glasses in all her normalness.

No longer the "hated foe"

In the past I've referred to Cicily's trach as the "fondest ally and hated foe". I honestly no longer feel that way. I'm so glad she was able to have it out for 2 wonderful months. But I have to say I am honestly so happy to have it back! Now I can suction all that junk out in the morning, and not have to coax her to cough and hope she's just working it all out every day. I can hear her raspy breathing and stick a catheter straight down the tube! I think she'll end up being more healthy this winter with a trach than she would have without it. Who am I kidding, she wouldn't be here this winter without it. And that is the biggest reason I am 100% ok about the trach for now - it will keep my Cicily alive.
It's also weird, when I heard Cicily laugh for the first time hearing her voice I cried. I loved hearing her voice every time she laughed with her trach out. In fact, she was just getting really good at it and really loud with her babbling and talking too. But, the first time I heard her laugh after her trach went back in, it was like seeing an old friend again. It didn't make me sad like I thought it would, it made me smile.
I'm also relieved each time she cries really hard because now I know with her trach back, she'll calm down and breath. Without her trach she'd cry really hard and turn purple. It's such a relief. Maybe the new perspective is just all the prayers you've all been saying for Cicily and our family?? If it is, keep them coming!

And for now I want to say - Welcome back trach, you are no longer my hated foe, but my ally. Thank you for allowing my little girl to hold on to her gift of life.

THANK YOU!!!

If you're one of Cicily's wonderful doctors, nurses, therapists, RTs, MAs, surgical schedulers, audiologists, case managers, etc. then look for one of these cards in your mailbox soon!

We've wanted to send out a special thank you like this to let all the special professionals in Cicily's life know how much we appreciate all they've done for her. The plan was to wait until she was walking, decannulated, talking, and/or eating and then to send out a "thank you - see how all your hard work has paid off" card. BUT, Cicily's life isn't like that. Her life is just hard and things don't come quickly or easily for her. BUT, Cicily just keeps smiling and enjoying her life anyway and all those professionals have helped her to do that.

We thank them and we thank all of you for your concern, love, and prayers!

Special thanks to Aunt Ondria for creating the adorable card!!

The 12 day hospital stay

So after Cicily was stable, they did a chest x-ray to determine if the ET (intubation) tube was in the correct place. The x-ray showed some cloudiness in her left lung, indicating pneumonia. So Cicily was on the ventilator, intubated, and sedated for 5 full days while the pneumonia spread to the other lung and then went away. While she was intubated, she had some special visitors:

Maya could actually come visit this time as the hospital doesn't have age restrictions for visitors in the summer. Cicily has a shirt on that her cousins all signed. What a great get well card!

One of our favorite families! It'll be interesting to see if Jerome is happy or sad that Cicily has her "necklace" back.

On Tuesday July 7th (almost exactly 2 months after it was removed), Cicily had her tracheostomy re-placed. We would have liked to give Cicily a real chance to learn how to handle her secretions without a trach and without being sick. She never really was 100% healthy the whole 2 months her trach was out. However, her episodes just got too severe and her chances were running out. Now we'll have to figure out what works to thin her secretions and she'll have to have her trach capped more and figure out how to cough and breath and not get scared with it all. (Damn that lack of white matter!) It still seems that the trach will be a temporary thing in Cis' life. Chris and I are actually quite relieved that it's back. It's funny, as much fun as it was to hear Cicily's voice when she laughed, I was also happy to hear her trach laugh once again.

The day after her tracheostomy, Cicily woke up from sedation in pain and flailing wildly. She had become addicted to the drug fentanyl that had kept her sedated for the past 5 days. It's an opiate drug, like heroin. So, like a heroin addict, Cicily had to be put on a detox regimen. She was given decreasing doses of the drug methadone to get her over the addiction symptoms.

The above picture was taken the day she woke up, the only time we saw her smile for the next 4 days. It's the first time I can think of in her life when Cicily hasn't just bounced back quickly from a surgery or illness with a smile - it was disconcerting.

Maya was of course the first to get Cicily to smile again! She even got a laugh out of her. One morning we brought Cis' dog Lucy for a visit. That plus Maya won lots of smiles!

After 12 days in the hospital, Cicily was discharged. Because of the addiction and not moving for almost 2 weeks, Cicily was still unable to sit up on her own or even hold up her head. The night she came home, I ran out and bought her this chair she could lounge in and completely recover in.

Cicily has been SO happy to be home. She's been smiling almost non-stop. I think she's happy to be alive and healthy.

The Episode to end all episodes - Life and Death

On Friday July 3rd at 5 am Cicily had a couple big coughs. Chris and I woke up and Chris ran into Cis' room (I walked). Chris got her out of her bed, and she had already started gasping for breath. She was having another "episode" that we've seen so frequently in the past 2 months, with each one getting more severe. Cicily hadn't had an episode for over 2 weeks, but here it was again, so we began our standard treatment. I held Cicily on her rocking chair while Chris got her albuterol breathing treatment, oxygen, and humidifier. I held all 3 of those tubes to Cis' mouth and sang her songs to calm her while Chris suctioned her mouth and throat with a 14 french catheter.
We and Cis' doctors have theorized that she has these episodes because she's only used to coughing up junk into her throat and then out the trach it comes and with no trach as a trap door, the secretions get stuck in her throat and she gets scared and begins gasping. It's almost as much of a mental panic as it is her airway being plugged up by secretions.
After a bit of the usual treatment, it was clear Cicily was not bouncing back like normal. Chris left the room to call 911. By the time he got the phone and was back in the room, Cis had stopped breathing completely. We put her on the floor and Chris started giving her breaths with the ambu bag. I felt her chest and couldn't feel her heart beating. So after a second of both of us freaking out knowing we had to start CPR, we collected ourselves and I began chest compressions with Chris giving breaths. After about 10-20 compressions I started to feel Cis' little heart start to beat under my hands. So we stopped CPR and by this time the fire department was here. The ambulance came quickly after. They proceeded to intubate Cis and attempted to give her an IV (our carpet still bears the blood stain). Then we were off to the hospital in the ambulance. The nearest hospital to us is a heart hospital for older people. That's where Cis was taken because they had to "stabilize" her before they could transfer her to her normal hospital.

Cicily stable in the PICU at her "home hospital"

Chris and I both thought Cicily wasn't going to make it past this episode. When Chris went to ask our neighbor to stay in the house with Maya before we left in the ambulance, Chris told him he thought this was it. At one point before the CPR I put my hand on Chris' arm and said it's ok. I was very calm with the idea that if it was Cicily's time to go then she'd at least be free of her ill-functioning body and she could go chase after some ducks with her great-grandmas. I'm incredibly happy that Cicily did not want to leave us though. She amazes me at how gracefully and joyfully she accepts her calling on this earth.

After a horrible new "episode" on Friday, Cicily has been in the hospital and now has her trach back in. It's mostly due to her white matter issues, her "TCS airway issues" are perfectly fine. Discouraging, but she wants to live and we want her to keep making our family and others smile. The complete story will be written out at a later date.

A new detective in the Case of the Missing Myelin

We asked Cicily's neurologist #4 if he could provide us with a list of neurologists who specialize in white matter issues. He kindly gave us a list of 3 doctors who research white matter (myelin). One was in Salt Lake City, one in Maryland, and one in DC. We checked into all of them and what research papers they've written. We're quite excited because they all have a strong interest in white matter diseases. We decided we'd start closest to home, and see if we could get Cicily in to see the doctor in Salt Lake City.
So in July we'll be heading up to Utah. The doctor we're going to see is an MD and PhD. He uses zebra fish to study white matter. We're hoping he will get interested enough in Cicily to study her white matter, or lack therof rather.
We've decided to stay a few extra days and make a vacation out of the trip. That means two of our last three vacations have been planned for appointments with doctors. Good thing we have doctors to see, or we'd never get a vacation!

Random Photo: I gave Cis a tangelo from our tree that wasn't going to ripen. She popped it into her mouth and thought she was so funny!

Cicily's 1st ambulance ride

I guess to live 2 1/2 years with a trach and never have an ambulance ride or trip to the ER is pretty good luck. So, it's a bit of a surprise that Cicily's first ride in an ambulance to the ER came 1 month after she was decannulated.
Short story: Cicily coughed up too much junk and couldn't swallow or spit it all out, and it was blocking her airway. She wasn't getting enough air, and Chris called 911. Ride in an ambulance and 2 day stay in the hospital.
Long story: Cicily has been sick on and off (mostly on) since a few weeks pre-decannulation. She's done amazingly well during the day at coughing and swallowing her minimal secretions. However, at night or in the mornings, she coughs up lots of thick crap and can't do anything with it. (We're not sure how much this trouble swallowing has to do with her brain issues and how much is related to not eating. Unfortunately, it's probably a combination.) So last Wed night she'd been sleeping and coughing, but doing fine. Then I went in to change her diaper right before I went to bed. As soon as I put her flat on the floor and got her diaper off, she coughed and started fighting to breathe. Chris and I took turns suctioning her mouth with a Yankeur, giving her back blows (infant Heimlich), providing supplemental oxygen, taking her outside (sometimes the change calms her), and singing comforting songs. Usually these tricks work, and Cis calms down and starts to breathe normally. Tonight though she kept getting more and more scared and breathing less and less. After a few minutes (who really knows how long time passes as you watch your child turn various shades of white, purple, and blue) Chris called 911.
The fire truck arrived first. They proceeded to give her an O2 mask, ask lots of questions, hook her up to a pulse ox, and take her blood pressure. I asked about performing an emergency tracheostomy (I thought perhaps her tracheal granuloma had regrown and blocked her trachea). When the captain looked at me like I was insane, I told them to get us to the hospital immediately. (I hope I wasn't too rude, that's another thing you're not too concerned about as you watch your baby's color change.) They were concerned about getting a good blood pressure reading, but then the ambulance came and we quickly got on the gurney and headed for the hospital. In the ambulance they gave her oxygen and an albuterol treatment and that seemed to help some. In the ER the nurse (who was VERY good, by the way), suctioned Cicily's mouth with a 14 french catheter and got a TON of junk out of her throat. Then the RT set up a cool mist and oxygen mask, then gave her a steroid breathing treatment. After that she finally was with it enough to pull off her mask (she hates them). A few minutes later she was pulling away the tube I was holding up to her mouth and giving it to her Grandma Sue to use. What child wakes up in the ER after nearly dying and starts playing around and laughing with her grandma?? That would be our Cicily.
So then she spent the night in the PICU and the next day and night too. It was all very uneventful. We did get a full lesson from the RT in how to deep suction orally and they gave us a supply of 14 french catheters to take home. Our tiny 8 french catheters would be worthless in these situations. We now have an "emergency box" that we keep by her bed with anything we may need for future episodes.
A tip for parents with kids with trachs: I know there are tons of things that can happen to our kids and if we let ourselves, we could worry to the point of being incapacitated. But, we never thought we'd need to deep suction after decannulation and apparently nobody else did either. If I could do it again though, I'd definitely have someone teach us the technique and have larger catheters at home before Cicily came home without a trach. Ah the things we learn the hard way!

Decannulation Party

An event 2 1/2 years in the making! We had Cicily's decannulation party a couple weeks ago. The two main events on the agenda were Cicily wearing a turtle neck and being completely submerged under water for the first time. She actually got to do both at the same time as her swimming suit was a turtle neck! We invited all our family and a couple friends to celebrate with us. Cicily really liked having her face in the water. She'd sit in her tube and dunk her face in every now and again.
I couldn't help but swim in the pool and cry as I thought of last summer swimming with Cicily with hardware protruding from her chin and all the incredible things she's endured to be able to swim and put her head in the water. It was a joyous day!!!

Cis in her decannulation party swimsuit with her Grandma Sue

What's a 3-letter word that made Cicily lose her voice?

DOG

Cicily loves dogs so much, and without a trach we didn't have any more good excuses to give her sister Maya about why we couldn't get one. So, a week after decannulation, we got a puppy! The night we went to pick up the dog, Cicily squealed ALL night! Then the next day when she woke up and saw her puppy, she squealed even more. She literally used her voice more than ever and got so hoarse you could hardly hear the squeals anymore. Cicily and Maya love the puppy so much, we're glad we gave in! Her name is Lucy and she's a Shih tzu. We're hoping she will be a good comforting buddy for Cis when she has surgeries when she's older.

Trach Free

A playful Cis - a few hours post decannulation

Wow, I've been thinking about writing this post for 2 years! Cicily was decannulated (trach out) yesterday May 2nd. A good day!!! She was in the PICU Friday night with her trach capped (we'd also capped at home for a few days during the day). Saturday morning Dr. Mancuso came into her room and I (Mom) got to do the honors of pulling out her trach for the last time. It was glorious to not have to put a new trach back in and strangle her while I tightened the tie. Cicily didn't even flinch or anything as she went from using her trach to breathing entirely out of her nose and mouth. She was watching all the medical personnel in her room like a hawk, making sure they weren't about to pounce on her I think. The rest of the day Cis was happy, especially when we'd ask where her trach is and she'd reach up to put her finger in her trach tie and it wasn't there! She's made the transition much better than I thought she would. The first 5 or so times she coughed she'd cry a bit, but then she got used to it. I expected to have a few days of her being out of sorts with her brand new breathing pattern, so she's pleasantly surprised me. The trach stoma (opening into her neck where the trach went) was actually mostly closed up after only 2 hours of the trach being out. Dr. Mancuso expected it would close quickly because her trach size has always been so small (3.0 neo) and she just had surgery done on it a month ago.

Last night before I left the hospital and let Chris take the night shift, Cicily was breathing so rattly and I wanted to suction her so bad! It was a weird feeling to hear her needing to cough or be suctioned and not be able to do anything about it. She had a great night though and didn't have any major oxygen desaturations. She does still cough and cry when she sleeps, but that's the only time the change seems to bother her.

Right now I'm listening to her voice as she cries herself to sleep - what a glorious sound! Yesterday she was laying on me trying to take a nap in the hospital and had been crying. As she settled in to sleep, she started making those sobbing hiccupy noises like, "huhuhu " and I could hear them!!! And then she started making sleep-talk sounds as she drifted off to sleep and I layed there and cried happy tears.

For those who are concerned about the aesthetics of Cicily's neck, sometime in the next few years Dr. Mancuso will revise her trach scar to leave a nice neat horizontal line on her neck that can barely be seen.

Mom untying Cicily's trach tie for the last time. Cis was still in her jammies for the big event.

And it's out!!! Look at that bare neck! It's so kissable.

The only picture we have of her trach stoma. It closed so quickly! A thorough, unobstructed neck washing for the first time.

"Yeah"

"Yeah" - It's Cicily's favorite word. It's the only word she consistently says all the time, and never signs. She loves to say, "yeah". Whenever she's asked if she wants to play with something, go somewhere, she usually answers with a, "yeah". If she's asked if she wants some food, most of the time there is an odd absence of her standard happy, "yeah" and we have to say, "you can sign 'no' Cis".
Chris commented a few weeks ago that Cicily's yeah is a great demonstration of her personality. Most 2 year olds can't get enough of the word no, and here's our sweetie living it up being a "yeah" girl. She's pretty much interested in trying anything new and she's happy almost all the time (exceptions are when she's in pain, when she's told no, and when Maya cries).
I recently pulled out all of Maya's old clothes that will fit Cicily for the coming spring, summer, and fall. It was a difficult thing for me to see all the cute outfits Maya wore at 2 years old and remember how when she wore these clothes she could run, speak in paragrahps, count to 10 in English and Spanish, knew what all the letters said, etc. I had to say a prayer because I wanted my sad, bitter feelings knowing those things may be a few years off for Cicily to go away. I did feel better and realized that both Maya and Cicily will get to the same destination, they're just on different paths. And I'm happy to have 2 girls on such different paths. I told Chris about it when he came home and he reminded me of what a friend also told me recently. -Cicily is an extremely happy girl. She doesn't care much about all she can't do yet. She tries so hard, and does SO well with what she has. When all is said and done, what more does a parent want than to know that their children are happy? I love our happy girl that makes so many people happy and is always ready with a willing, "yeah".

Outlined path to living trach-free

Problem #1. A jaw that is too small
Fix - 3 month jaw distraction surgery

2. A granuloma grows in the trachea
Fix - surgery to excise the granuloma

3. Granuloma surgery is cancelled numerous times
Fix -reschedule and be patient

4. Infection inhibits granuloma surgery from being completed
Fix - reschedule, wait, antibiotics like crazy

5. A hole develops in trachea from granuloma surgery
Fix - wait 10-14 days for hole to heal

*new 6. Contracts pneumonia 10 days post-op
Fix - hospital stay with IV antibiotics, home antibiotics for 2 weeks and then cap again
Seriously, now it just feels like a comedy of errors or something like that! I am feeling more patient now though.

So Cicily's tracheal scar tissue removal surgery went well. Dr. Mancuso excised all the granuloma and stitched her little neck back up. Then she went to the PICU and her trach was capped for 24 hours. During the 24 hours of capping she was breathing EXCELLENTLY!!! Her oxygen saturations were completely normal while she was sleeping, eating, playing, everything.

There was a minor snag in the plan however. There is a leak in Cicily's trachea from surgery. When her trach was capped and there was more pressure to get air from her lungs to her nose/mouth, the air would leak out of the hole in her trachea and fill up her cheeks. She looked like a swollen balloon! So we took off the cap, swelling went down and Cicily came home from the hospital. Now we have to wait 10-14 days to re-cap and make sure she doesn't become a balloon again, and then we can pull the trach.

Mom's note: I'm very excited about Cicily breathing so well with her trach plugged. It's more than I expected from her at 2 years old. I can't describe the thrilling feeling knowing she could start to cry loudly, talk better, splash in the bathtub, not have to cough all the time. . . .
I'm also freaked out about not being able to suction the stuff she coughs up, hearing her breath differently, and other things. I don't know how to describe what a mixture of emotions it is.
When Cicily was in the hospital with her trach plugged, we made plans Tuesday night to pull out her trach Wed morning. Chris stayed with her Tues night and I went home to sleep. I'm in bed, almost out when the phone rings and Chris tells me it's not going to happen Wed. morning. I was tired, angry, sad. We have a plan in place now for decannulation and I feel good about it. I do feel like it will happen soon most of the time. Sometimes though, I feel like there have been so many curve-balls thrown that I don't know what to expect next. I try to focus on being grateful that her jaw distraction went so well and that she is capable of breathing without her trach now.

Fun times before and after surgery

Mom and Cicily playing in pre-op. They gave us the toy room this time around. Cicily was well entertained for the 2 hour or so wait!
Dad and Cicily driving around the pre-op floor. These cars are a staple of the pre-surgery routine. Cicily loves doing her rounds in her car!

Later that night, both of Cicily's Grandmas and Grandpas came to visit her in the PICU. She was SO excited to see them, it really cheered her up! And they even brought presents to keep her entertained during her hospital stay.

Here she is playing the pillow game made up by Grandmas and Cicily! Grandmas make everything better!!

Grandpa Norm and Cis playing away!
Happy girl with her Grandma DeEtte!

Surgery #9

Cicily is scheduled for an excision of granuloma and a spinal tap on Monday 30th. Dr. Mancuso will flip out a little ball of scar tissue in her trachea and cut it out, then Dr. Black (anesthesiologist) will perform a spinal tap to see if anything shows up to solve the mobility mystery. Sounds easy enough - we'll see. Then the optimistic plan if all goes perfectly is for Cis to stay in the PICU for 3 days capping and pulling out her trach and she'll come home trachless. I'm cautious as this trach process has not gone anywhere near perfectly for the last 5 months and I'm tired of getting my hopes up. Prayers, as always are greatly appreciated and are always helpful.

Sporting Orthotics (not braces)

Cicily is finally (after waiting 2 months for insurance) wearing orthotics on her legs to help her stability. They are technically AFOs, ankle foot orthotics. Maya may correct you as she did her aunt if you try to call them braces. Are you thinking what I think everyday when I put them on, "how much more gear can you put on a little body??" The plus side - she does get to wear expensive skater shoes that I'm sure someone thinks are cool.

Cicily is not a fan of the AFOs. She hasn't learned how to maneuver and crawl in them yet, so they actually limit her mobility at this point. I'm sure she'll get it sometime, I just hope it's soon! They do really help her gait though when we hold her hands and she walks. They also help her legs stay at a better angle when she stands at the couch and make her more stable.

Don't worry, the walking equipment plans aren't over yet. In addition to AFOs she may also get to sport a spandex compression suit to help her stability, and not just an old-lady walker, but a super walker called a gait trainer! Of course none of it sounds like a great time to me, but if it is a step in her gaining the ability to walk, I'm for it. Hell, if it'd help, I'd put her in a bubble of pickle juice all day!!!

White Matter - why it matters

So we had 2 more neurology appointments this week and found out a little more info. Apparently Cicily has no white matter. I asked the doctor how she's able to do anything at all without any white matter and he basically said what they see on the MRI doesn't really add up with what Cicily is doing (average mental capabilities, crawling, signing, etc.). The other neurologist said that newborns can't do anything but flail around and cry because they don't have any white matter in their brains. So basically Cis should act like a newborn; but she doesn't?!? We're of course, not sure why or what this means, but we'll gladly accept it.
It is also clear that Cicily never developed myelination. Her status can't really deteriorate, because as Chris put it, you can't get any worse than zero!
I also wanted to make clear that hypomyelination does not occur with Treacher Collins Syndrome - ever (that anyone knows about). However, we've always been skeptical that Cis has TCS as her genetic tests come back without the TCS gene change and her facial appearance is slightly different from classic TCS. The 3 geneticists we've seen feel TCS is still the best fit for her though, and we agree. I've wondered if Cicily's body may have some protein issues that affect both her TCS characteristics and her hypomyelination, as they both can be caused by proteins not working correctly. We're still doing the spinal tap on the 30th and the neuro-genetics docs are getting back to us on what tests they want to do - genetic, metabolic, fatty acid chains were mentioned.
We are very glad to be working with good neurologists and a geneticist who all seem to be very intrigued by Cis' mysterious, unheard of combination of characteristics. So far they are all doing their research to try and solve the puzzle. We hope they continue to be interested! Chris hopes Cis makes it into some medical journals as "patient A".

Why is Cicily not walking?

We may have found part of the answer to why Cicily is so "wobbly" and has irregular eye movements (nystagmus). Cicily had a 2nd MRI of her brain last week which showed she has hypomyelination of white matter - it's the coating on the brain which helps get signals to the places they need to go. It's developed between 0-3 years old. So in Cicily's first MRI at 1 year old, the lack of white matter wasn't very significant, but now at 28 months old, it was very notable.
We still do not know why she is so deficient in white matter. Apparently it happens with certain genetic diseases or metabolic disorders. One of her neurologists has ordered a spinal tap to test her spinal fluid for various metabolic disorders and other cool, unusual things. So, we're anxiously awaiting the spinal tap and hoping it provides further answers. We're especially hoping the final diagnosis is treatable.
We'll continue to work with Cicily's 2nd and 3rd neurologists and see a 4th soon as they all seem to have different areas of expertise and hopefully together they can figure out our adorable, enigmatic little lady.
I was at first very nervous and upset about this new finding as it's affects can be pretty devastating. I actually feel good about it now though and have faith that in some way the many blessings Cicily has received will be realized. As I said before she was born, she'll grow up and be just fine, I just worry about what she'll have to go through to be fine. (I didn't have a tiny inkling of how much she would have to go through.)

HAPPY VALENTINE'S DAY!!!

Cicily got a box of chocolates for Valentines today. Chris and I were outside for 3 mins. discussing where to plant our new passion fruit vine and walked back in the house to see Cicily in the midst of smashed chocolates all over the carpet and herself! She thought she was so cool, and so did we!!!

Going Bilateral

Cicily got her new BAHA last week!!! She's now the proud wearer of two hearing aids on her headband- one on each side. We've been working on getting this additional BAHA since last May, so we're very excited!
Theoretically this gives her a higher hearing threshold and some directional hearing. All I know is now she can hear me when I'm suctioning her trach and when I whisper to her at church. In fact, Cicily hears better than I do now! At her hearing test this past Tuesday she heard a very low tone that I didn't even hear. (So, good that Cis hears SO well, and kinda bad that I hear less than my girl with a hearing loss!) :)

Jaw Distraction Before & Afters

I realize it's a little late, but we now have pictures to compare Cicily's jaw from before her jaw distraction surgeries, to after her jaw distraction. I like to use her trach vent as a "yardstick". The difference is truly amazing! She has a much bigger jaw. She also has much more hair! One grew easily and naturally; and one grew with the incredible expertise of doctors, and care of Mom, Dad, Grandma, family and friends.
Before pics were taken July 2008
After pics were taken Jan 2009