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.