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

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.

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.

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

Words we never thought we'd hear

Cicily had her laryngoscopy yesterday.  She came out of anesthesia very well (we're attributing that to a new trick - the drug fentanyl).  I was surprised when I went to recovery to see her and she had a much larger sized trach in place.  I just thought, I guess her airway is still small and we'll have to try a distraction again sooner rather than later.  Surprisingly, I wasn't too horrified at the thought, just pretty calm.  Then Dr. Mancuso came and explained how surgery went.  The granuloma (scar tissue in her trachea) was growing on a curve and with the curve of her trachea he did not want to risk cutting it with a laser and cutting through the trachea.  So in January or February, after a round of antibiotics, he will do another excision of granuloma, but this time do it the old fashioned way with a scalpel.  He was apologetic that he couldn't get it done with the laser, but we didn't care at all because he then gave us some GREAT news.  

Her airway has improved remarkably.  Dr. Mancuso said it was a typical airway.  I was in complete shock!  I honestly always thought she'd have a small airway no matter how many distractions we did.  I pressed him on it further and he said an intubation would be no more difficult on Cicily than on any typical pediatric patient.  

I cried on and off all day long.  Happy tears of course!  My baby girl shouldn't have to struggle to breath anymore!  All the prayers and the hard work of her jaw distraction have worked in a most impressive way.  

After the granuloma excision surgery in Jan or Feb, Cis will have the decannulation study done to see if she can breath without her trach in all situations.  

Cis and Maya on a happy afternoon a couple weeks ago.

laundry & scar tips

Along with all the detailed new medical knowledge I've acquired being Cicily's Mom, I've also learned some useful everyday tips I thought I'd share.

#1 Laundry Blood Tip
We've had a lot of blood on clothes around our house in the past couple months, but not a single blood stain! Here's the secret: salt. Pour it directly onto fresh blood or two day old blood and soak it in cold water, wash the clothing, and blood will be gone. We actually had a salt solution in our washing machine every day during the month of July.

#2 Scar Tip
Growing up I always put vitamin E on my scars to make them dissappear. Dr. Singh also recommended this practice and added massaging the scar to the regimen. I bought a bottle of vitamin E capsules and 3-4 times a day I poke one with a pin and massage a little into Cicily's jd scars. We'll see if it's just as magic as the salt in making things dissappear. :)

Jaw Distraction #1, Surgery #7 in the history books!

Cicily went in yesterday to have her distraction device removed. It went very well. Dr. Singh removed everything completely internally (via small incisions inside her mouth) so Cicily will only have two small scars where her pins were sticking out. Yes, Dr. Singh is a surgical marvel! She also said Cicily's bone looked good and her bite is closed in the back of her mouth and only slightly open in the very front (3-4mms). So Cicily has an underbite, which is exactly what we wanted so she could have some room to grow into her jaw.

Overall the distraction was a huge success. What a relief!!!

We want to thank everyone again for helping us. This was certainly a "village" effort. We have such amazing family and friends, extremely talented doctors and have been so blessed. Thank you very much to all of you and to Dr. Singh and Dr. Glick.

Cicily has done quite well since surgery. When she woke up enough in recovery, she started signing "out", "home", "all done", and trying to take out her own I.V. We've decided she equates the I.V. coming out with leaving the hospital! She got so frustrated after signing and trying to leave, she held her breath until she passed out (2 times in fact!). So we were very glad Dr. Singh thought she would do ok coming home. Cicily slept very well last night in her bed and has been up and playing today.

Here are pictures of Cicily post surgery. Once again, I pull no punches, so don't look if you can't handle blood.

Last night at home - about 4 hours post-op The pin sites were left open a little so they could drain. And drain they did! I tried cleaning her up before bed, but she screamed and almost passed out again, so I decided a little blood wouldn't hurt for one night.

Today - looking pretty good! Quite a lot of swelling in her cheeks. It reminds us of 9 month old Cicily. And yes, the right side of her face is not moving much. A little disconcerting, but should go away in a few days.

X-ray before and after

Cicily hates the x-rays, so she always screams and has an open mouth, which makes it difficult to tell what her jaw actually looks like! But, you can note how the mandible is further out in the second slide than in the first and you can see how the distractors are slid all the way to the bottom of the distractor body. Comparing both profiles is pretty amazing. Look in a straight line from her forehead to her chin- quite an improvement!

Also of interest is the difference in Cicily's right and left middle ears. In the first slide you can see a small opening of an ear canal (it's in the middle of the darker white area on the bottom of the cranium-where the ear is). The second slide is Cicily's left side which has complete canal atresia and all you can see is the same darker white area without any ear canal opening.

Final jaw distraction surgery scheduled

*This will be the last surgery for this jaw distraction, however as Cicily grows, most likely her jaw will not grow enough and she will need additional jaw distractions. *

On Monday September 29, Cicily will have the distraction device removed from her jaw. Dr. Singh will make a small cut in her cheek, closer to hear ears than her chin, to unscrew the distraction rod. Then the device will be removed from her bones and mouth. This will be an overnight stay at St. Joseph's Hospital. Cicily will then wear the helmet for another 1-2 weeks (yup- another) and then this jaw distraction will be over and done with! Very much looking forward to this surgery as it means no more wound cleaning twice a day!!!

Why the helmet??

Wide open mouth! This picture was taken during the first 2 weeks wearing the helmet. Cicily was not closing her mouth at all when the helmet was off during this time.
4 weeks of helmet and chin strap. Cicily now closes her mouth quite frequently, when her tongue is not in the way (which we're working on after talking to Dr. Glick on the consequences of tongue posture).
Side view after 4 weeks in the helmet. Her mouth is open, but look at the jaw angle. It no longer goes straight down.

She wears the helmet for 23 hours every day. We're on the 5th week now. It hasn't been the greatest 5 weeks. Cicily does quite well wearing the helmet once it's on actually. However, she cries and screams when it comes off and when it goes back on. We also had a dehydration episode that improved with a lot of water and Chris running to get some Gatorade at 3 am. We'll have another little celebration when we're done with the helmet in a few weeks!

It's another few weeks of keeping the end goal firmly locked in perspective. (The end goal being that Cicily will not only have a larger airway from growing her jaw approx. 1 inch while turning the screws, she will also be able to talk and eat better if her mouth can close and she can get decent tooth alignment.)

Distractor guide book

I just found the "technique guide " for the distraction device being used for Cicily. Here is a link if anyone else really wants to know more about this distraction process from a technical aspect. It does have great pictures so you can really get a feel for what the device looks like internally. Dr. Singh showed us these pictures before the first distraction surgery, but it's nice to leisurely read through the "guide".
http://products.synthes.com/KYOCMF/Product%20Support%20Materials/Technique%20Guides/CMF/MXTGCMFDistractionJ7415A.pdf

Have I mentioned what an amazing surgeon Dr. Singh is?!! I'm so glad she practices in the valley. And I'm glad I was only responsible for turning the extensions! (Especially because I didn't even do that very well.)

Anyway, maybe this guide book can help another family looking into jaw distraction and wanting to know more details.

JD update

No more screws!!! They stopped turning yesterday afternoon and I felt like I'd won the lottery! 30 days, 30mms - done. Dr. Singh took them off this morning. So, Cis still has the rods internally, but no more dangling jewelry hanging from her chin!
We do have new orders for the helmet though. It'll have to be worn for probably the whole consolidation phase (about 6 weeks). But, that should allow her teeth to meet and her jaw to angle straight out as opposed to sloping down like it is now. We have high hopes she won't have a Jay Leno chin after all! We'll see. :)
Cicily no longer needs 24 hour monitoring and she's already taking advantage of her renewed freedom and getting into bathroom cupboards. Once again, we rejoice at the messes of our toddler!

A work in progress -Amazing!

I wanted to put up some progress shots because I was shocked when I looked at Cicily's before pictures this morning!!! I can't believe what an amazing difference this jaw distraction has made so far. I'm trying to remind myself that all babies grow and change in appearance because I kind of miss Cicily's "before" face. After the surgery to remove all the hardware in Sept, we'll post some real before and afters. We're still working on raising Cis' jaw up a little more so we'll see how that turns out.
*Dr. Singh's plan has always been to "overcorrect" Cis' jaw so she'll have an underbite (her lower jaw will stick out more than her upper). This will allow Cicily more of a chance for losing the trach, and allow her to grow into her jaw over the years and hopefully need less future distractions.

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!

Jaw Distraction surgery scheduled

Cicily will have her first jaw distraction surgery on Tuesday July 1. Her craniofacial plastic surgeon Dr. S will surgically brake her mandible and then place screws into the bone. For more details on the surgery, look at the previous post on jaw distraction on this blog. http://cicilyspage.blogspot.com/2008/02/more-good-news-jaw-distraction.html
We're planning on an overnight stay at the hospital and then back home the next day. We begin turning screws 4 days after the device is placed. The device Dr. S decided would work best for Cicily right now is actually an internal jaw distraction device. This means only a flexible one inch metal piece will stick out of her skin just below her ears. I just looked for pictures online of this type of distraction device, but didn't find any. I did see this blog on the first page of my search results though, so hopefully the pictures we'll be posting in the next few months will help someone else!