New Teeth

I told Cicily to show me her teeth so I could get a picture of them. I'd say she obeyed quite nicely!

Here are some pictures of Cicily's new front teeth. (Compare these 2 pics with the ones from Cicily's first day of school to see the teeth difference.) Dr. Carter put crowns on her 4 top front teeth last Friday in surgery. They look so good. We were expecting more "chicklet-like" teeth, and these really look natural. Once again, we got a great doctor who did a great job with our girl! Sadly she still seems sensitive to cold foods, but we'll see how that ends up.

Surgery coming soon

Cicily will be having her next surgery on Friday January 29th. This surgery will be entirely for dental work. She'll be under general anesthesia in the OR and the dentist will be doing all the dental work she needs done all in one shot.
Dental work being done:
Crowns on all four of her top front teeth. Those four teeth are small and grew in without enamel and decalcified. This means they've been very sensitive to cold foods. Hopefully the crowns will protect the teeth from cavities, make them less sensitive, and make Cis' smile even cuter.
Seal her molars. This protects them from decay.
Do a good deap cleaning
Fill any potential cavities she may have.
So it should be a fairly easy, relatively painless surgery.

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.

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.

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.

Next surgery

Rescheduled again for Mon. Dec. 8th. This time due to hospital PICU space. (third time's the charm I hope!)

Rescheduled for Mon. Dec. 1st.

Nevermind - This surgery did not happen today. Insurance issues are mostly to blame. Hopefully we can reschedule it for next Mon???

Cicily will go in for surgery on November 17th to remove a granuloma that has grown in her trachea. A granuloma is a little ball of scar tissue. In this case, it's caused by the trach irritating the tissue around it and Dr. Mancuso will simply cut and cauterize it right off. He said it's quite straight forward. Dr. Mancuso will also perform another laryngoscopy and then we'll proceed one of 3 ways:
1. Cicily's airway will appear adequate and she will have her trach capped in the PICU overnight to see how she tolerates breathing without a trach in all different circumstances.
2 . Cicily's airway will still appear too small from the laryngoscopy and her trach will still be necessary and we'll come home.
3. Cicily's tonsils will be removed to create a larger airway (and get them out of the way so they cannot cause future trouble) and he'll see that her airway is adequate enough to cap her trach for a decannulation trial at a later date when she's recovered from her tonsilectomy (I'm assuming January).
We're hoping for scenario #3, eventhough it means she'd still have her trach until January at the soonest and till she's much older at the latest. I'm nervous about Cicily transitioning from a trach to no trach during the sick winter months anyway. I will update on the 17th or 19th!

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!!!

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!

More good news - jaw distraction

At Cicily's appointment with her craniofacial plastic surgeon, Dr. S, on Tuesday we learned that the jaw distraction (mandibular distraction osteogenesis) is a go!! Sometimes the jaw bones or joints (temporal mandibular joint) aren't developed enough to distract a jaw. So before they can distract, they need to graft bone and let it heal. Dr. S said Cicily's bones are all very small and underdeveloped (hypoplastic) but that they should be able to withstand a distraction and grow well. YAY!!!

How does jaw distraction work? Here is what I've gathered: There is an initial surgery where the lower jaw (mandible) is cut on each side and the distraction device is put into place, which consists of metal rods and screws. Then the patient goes home and the parents turn the screws on the device typically once a day for 2-3 weeks. This moves the bones out and down, leaving a gap in the bone where it was cut. For the next 2 months the device stays in place so that new bone can grow in and fill in the gap. Thus lengthening and growing the jaw bone. Then 3 months after placing them, another surgery is done to remove the distractors. Distraction is done - Happy Day!
To see an awesome video explanation, go to http://www.pedsent.com/surgery/swfs/distraction/distraction.htm Click on "How distraction works"

So, with Cicily now they send out her CT scan to have a silicone 3D model made of her head (I'm picturing beauty school practice heads). Once Dr. S gets the model, she'll take the distraction device she thinks will work best and actually do a "practice surgery" where she cuts the model and places the distractor rods and pins and then turns the pins to see what kind of movement she can get with that device and a model of Cicily's jaw. Then, if it works out well, we're ready to distract. Surgery will probably be scheduled for April. And the hope for this procedure is that it will make eating easier and create an adequate airway and make the trach unnecessary.

CT scan

This is a lateral (side) view of Cicily's head. This is one slice of the 3D CT scan she had done in the hospital. You can see her lower jaw is quite recessed. If you think about how your teeth match up vertically, her teeth don't at all. On her mandible (lower jaw) her molars are back almost to where your throat should be. 'What are they going to do about that' (as an infamous pediatrician asked me when Cis was 2 mos old) - mandibular distraction osteogenesis (aka: 3 months of hell for hopefully a HUGE payoff!)

The Perfect Palate!

I'm not sure that it's "perfect", but it sounded good for a title. Cicily's palate is healing beautifully. She still has stitches dangling down all over, but no holes! We saw Dr. S yesterday and she said it looks great and she should be past the point of developing any fistulas (little holes). So, the surgery obviously went perfectly, and honestly it's all been much better than I ever hoped for. I have to say, Cicily and our family have been blessed with a lot of best case scenarios lately. Cis has to go through a lot of crap, but she is certainly blessed a lot along the way.
One HUGE benefit of having a palate is that now Cicily has normal drool!!!! No more "bubbles". We only use the "bubble cloth" (cute crocheted burp cloths) once a day or so instead of 5 times an hour! I figure she must have had nasal drainage going down into her mouth and mixing with saliva, creating thick bubbles. This benefit was not expected and it's great! The bubbles actually didn't bother me that much, but now that they're gone, it's nice to have them gone!

Upcoming Surgery - Cleft Palate Repair (Palatoplasty)

After months of calls, we've finally succeeded in getting Cicily's next surgery scheduled. She will have a cleft palate repair and another laryngoscopy/bronchoscopy done on Tuesday Jan. 22 at Phoenix Children's.

Cicily's craniofacial plastic surgeon, Dr. Singh will be repairing her palate. Dr. Mancuso, Cicily's ENT will be performing the laryngoscopy/bronchoscopy to evaluate if her airway has improved at all.

As previously mentioned, Cicily's cleft palate is very wide. (She really has no roof of her mouth- you can see her nasal bone- it actually looks really "normal" if you didn't know what a palate should look like!) Dr. Singh will basically have to work magic to take enough existing tissue from her palate and stretch it, pull it all together and stitch it up to give her a roof to her mouth. This is pretty much a "one shot" deal. If the new palate forms any fistulas (holes), then it will be harder, if not impossible to repair it another time due to scar tissue being nearly impossible to stretch and work with. Therefore, our family would greatly appreciate prayers for Cicily and her doctors.

Surgery Report
The surgery went quite well and was much shorter than the expected 3 hours. Although, when you're waiting in a surgical waiting area, it's never short! Dr. Singh said she was able to get plenty of tissue for her palate. She did not expect to have any fistulas form. She did say there was a good chance of having to elongate her palate with a velopharyngeal flap (yeah, I had no clue what it was 6 months ago either - Wikipedia's a great friend!). But, that wouldn't happen until she's 4-5 and only if her speech is sounding too nasally. We'd just lump it in with another surgery, I'm sure she'll be having one around that time! Cicily's mouth looks completely different inside, her palate is so LOW and it's FULL of stitches. The day after surgery she kept moving her tongue all around trying to figure it all out.

The laryngoscopy showed that her airway has improved some more, but not phenomenally more. So, Wed we had a 3D CT scan done (a detailed x-ray of her whole head) and Dr. Singh will evaluate that to talk to us about good timing for a jaw distraction process.

Cicily's been an absolute champ of course. We stayed in the hospital one night and came home the next day. She's hardly required any pain medication, I keep giving it to her "just in case". Today Cis just wanted to play like normal with the exception of wanting Mommy snuggles a little more frequently. She learned really quickly how to play straight-armed, and it's hardly bothered her to wear her arm restraints.

Thanks so very much to everyone praying for and thinking of Cicily.

Day after surgery.

I removed her arm splints for some patty-cake playing. She started signing patty-cake in the pre-op room and remembered it through her sedation and operation until the next day!

(I was going to say the picture looks worse than it really was, but it doesn't, in some ways it actually looks better. ) BTW- if anyone is in need of a hospital gown, we were allowed to keep this one, in addition to 2 new stuffed animals Cicily's beautiful personality scored!

Upcoming Surgery - BAHA implant (stage 1)

*Postponed*

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


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

Upcoming Surgery - Laryngoscopy

Cicily will be having her 3rd surgery on July 3rd. Her ENT (ear, nose, and throat dr.) will be doing a larynogoscopy/bronchoscopy to evaluate her airway. This is an outpatient procedure where he passes a scope in her mouth and down into her trachea to see how things have changed in the past 8 months. He performed this same procedure to evaluate the need for a trach right before he did her tracheostomy. We have interesting pictures that he took with the scope showing her tongue sitting right on top of her trachea. We're hoping this time when he looks her tongue won't be blocking her airway as much!

Surgery Findings
Cicily had her sugery on Tuesday. She went in quite happy, but came out kicking and screaming!! I forgot how mad she can get when she's upset- she's such a determined fighter, so much so that she got dehydrated from all the commotion! The surgery was very quick and her surgeon, Dr. Mancuso, said her airway has improved 60% from when she was 1 week old. So this is incredibly good news! She'll still have her trach for a while, but only time and patience will tell how long. Thanks ever so much to everyone who has prayed for Cicily.