New gear

Cis gets to wear this helmet with chin strap for the next week to close her open bite. The distractors are growing her mandible at a downward angle, so this chin cup and the elastic tension will mold the soft new bone she's making to more of a straight out angle. This is pretty standard with this type of jaw distraction device. (However, usually they put elastics on teeth or a chin strap on a headband. Cicily's helmet is an experimental way to keep the chin strap in place on a toddler.) In the first day alone, I saw huge improvement- her mouth had closed a lot!

For anyone wondering, a real doctor did put this chin strap contraption together. Dr. Glick is the craniofacial orthodontist working with Cicily. We like her a lot! (of course! I don't really mention the very few doctors we don't care for on here :)) The chin cup is a Dr. Scholl's pad held in place with ortho elastics. Ingenious - and it's working!! After I added the flowers to the helmet, I talked to Chris' Dad and he mentioned it looking like Chris' football helmets- darn, I should have put a BYU sticker on there!
Cicily doesn't love the gear, but she's of course a huge trooper (Chris thinks a storm trooper ;))and got used to it after 4-5 hours. During her first fitfull nap after we got home from Dr. Glick's office though, I was nervous.

This is our last week turning screws and having Cicily wear any outer gear. Her screw extensions should be removed Tues. and the helmet also. Keep our fingers crossed! We're almost into bone consolidation (hardening for those who don't speak jaw distraction)!!!

Half Done

From the x-rays of Cicilys jaw on Wed. it looks like the distractors are turned 1/2 of the full length. This means we are 1/2 way to completion of turning the screws!! If all goes as planned we will continue turning 3 times a day and then Dr. Singh will remove the external arms (the portion of the device that hangs down from her chin) on Aug. 5.
The turning is becoming painful for Cicily. I try to turn while she is in a deep sleep for at least the night time turn, but when she's awake it takes Chris holding her arms/entertaining her in order to get them turned correctly. So turning is not the exciting occurrence that it was in the beginning; I think of it more as torture three times a day. It is worth it though. We can see a HUGE difference in the position of her mandible. Her chin looks like Jay Leno's to us! And, I can now see her uvula for the very first time. Something most people take for granted, but I've never been so excited to see a uvula- what a great thing!
From the laryngoscopy Dr. Mancuso saw improvement in her airway. He didn't have to thrust her tongue off of her trachea like before. He is optimistic that if we can achieve the full 30 mm of mandibular growth, then Cicily could be decannulated. So, we're a few major, precarious steps away from that still, but the optimism is nice.

I was attempting to get a picture of Cicily NOT smiling to post as a progress shot. She was not complying- she smiled for every single picture I took that day! She makes this all so much easier. This picture was taken Monday (5 days ago).

Surgery #6 - Laryngoscopy

Wow, is it only #6?!? It seems like we should have about 10 under our belts by now. :)
Tomorrow, July 22, Cicily will have another laryngoscopy to evaluate her airway. This is a "no big deal" surgery. Dr. Mancuso (ENT) will take a camera on a thin cable and put it down her trach hole and also through her mouth into her trachea. This will allow him to see how open her airway is now. Hopefully he can give us an idea of how many more mms we need to distract Cicily's jaw for her to able to breath without a trach.

Jaw Distraction X-ray

This is one of the skull films that were taken today. After a pin turning/device/x-ray complete befuddle, Cicily ended up with this x-ray which shows both sides turned approximately the same distance. Phew, major relief!

Essentially Tues we went in for weekly x-rays and the length of separation in the bone on both sides was only about 4mm instead of the 11mm it should have been. Today we went back in and the Rt side was closed up to 2mm and the left side was separated about 10mm. WHAT happened??? Dr. Singh called the doctors she did her fellowship with in Philadelphia who invented the device Cicily is using and they said it could only be an error in turning the screws. Thankfully Dr. Singh knows me well and figured I must be turning them correctly as I've told her I believe I am. So she turned the Rt side 15 times in her office to compensate for the asymmetry we somehow had. Then we took more x-rays (above).

Don't worry if you don't understand this post at all. I don't understand what could have possibly happened, and Dr. Singh is equally as puzzled by it all. The main thing is- good news, the distractors are both now turned approximately 1/3 of the way to the complete 30 mm. YAY!

(The two circular shapes showing by Cicily's bottom teeth are the buttons on the dress she had on. The big metal thing on the top side of her cranium is my ring and you can also see my fingers overlaying. The rest - screws, rod, trach and all are things that are a part of Cicily right now.)

Jaw Distraction details

This will be a somewhat detailed (not comprehensive as I'm Mom, not Dr.!) description of the mandibular distraction osteogenesis that Cicily is undergoing right now. This is Cicily's first jaw distraction and she is 21 months old. The distraction is being done with the goal of moving her jaw down and out so she may be decannulated and make it easier for her to learn to swallow. Dr. Singh is the craniofacial plastic surgeon doing the distraction. The device is a Synthes internal distractor. I turn the "screws" on the device 3 times a day moving 1/3 mm per turn for a total of 1 mm per day. The device will extend a maximum of 30 mm, therefore if we need to, we will distract 30 mm by turning the screws for 30 days.

Device description (in Mom terms): The mandible was broken on either side and then 2 metal plates were screwed into position on either side of the breaks with 4 tiny (3ish mm long) metal screws. A long rod is connected to these plates that extends from her ears to her chin. All this is inside her mouth, between her gums and cheeks. Then 1 1/2 inch flexible screws hang down on the outside of her jaw on either side of her trach. These screws are what we attach the screwdriver to and turn each day.

Living through jaw distraction: The distraction device is very delicate and therefore Cicily (or anyone else- including x-ray technicians!) cannot turn them (except designated 3 turns/day), pull them, or fall on them. Dr. Singh was quite insistant that any of these things could lead to device failure and ruin the distraction. Since Cicily is a wobbly toddler, she has constant 24/7 supervision to ensure she does not twist, fall on, bump, or pull her device. I stay with Cicily all day- we play lots of song games (patty cake, monkeys, etc.), read books, and do light physical and occupational therapy playtime. I also keep watch while she is napping as her habit is to stand up in her crib until she falls asleep and then roll over on her face while she's sleeping, neither of which she should do right now. Chris and Grandma DeEtte have taken turns staying up with Cicily at night to watch for these same sleep habits, give her medicine, suction, monitor oxygen levels, and ensure her trach stays humidified.

So far the distraction process is going better than I expected. Although we are nervous the space in her device is not the 11 mm is should be now, but we will discuss that more Fri with Dr. Singh. I have also heard recently that the screws become more painful to turn at the end of distraction, which I'm glad to know but sad because I was thrilled that Cicily seemed completely unaffected when I turned the screws. This process and time in our lives would not be as calm and relatively easy as it is without our amazing family and friends who have done everything from praying, sending positive thoughts, bringing dinners, watching Maya, and expressing love and concern. Thank you all so very much.

Jaw Distraction Surgery - Phase I

The surgery to place the distraction device went well. (Minus a trach mishap which ended in Cicily having to be intubated through her trach stoma for 18 hours and subsequent drugs to sedate and paralyze her.) But, overall Dr. Singh was pleased with how she was able to place the distractors. Cicily had her usual fighting angry time the first 36 hours after surgery, but is now home and sleeping peacefully in her own bed!
I want to post pictures for those who are interested and hopefully to help other families get a better idea of what distraction can be like. Friendly warning though- if you are extremely faint hearted, you may not want to look closely.

The anesthesiologist let me take Cicily to the OR this time and help her fall asleep happier. She was nervous about the "bunny gown" until I smiled at her and signed, "silly Mom" and that made her smile. I think she still sensed something was up though.

A few hours post op.

Still intubated and paralyzed. If there's one thing I could possibly dislike more than the trach- it's the ET tube (being intubated)!

About 24 hours post op.

Finally awake and returned to the trach so she can have a good snuggle before zonking out again. Quite a bit of swelling in her face.

36 hours post op.

A good look at the distractor "screws/keychains/beard" as we fondly have been calling them. The sore on her lips is from cranking her mouth open so wide to position the plates and screws.

48 hours post op.

In the car on our way home from the hospital!!!

48 hours post op.

Home again and smiling. Can you get any more tough and pleasant than that?!
Certainly not back to her normal Cicily self, but MUCH better than yesterday!