Coming to the End of JD#4: Removal Plus a Lot of Other Stuff

Can't believe I didn't post for the last 3 weeks since we left Cincinnati!  I pretty much went right back to work once we got home, so the days flew by, but we came back on Thursday (7/30) for the final surgery for this Jaw Distraction (JD#4).  It went very smoothly, took just four hours even though Dr. Gordon did a lot of "stuff" in this surgery including:

1. Removing her distractors (the headgear she's been wearing or Rigid Extraction Device aka, "RED" device, even though it's purple!)
2. Rhinoplasty - basically the cuts he made in the first surgery cut her nose bone and maybe her septum so it was laying very flat on her face and was rather distorted.  He stood it back up, trimmed some of the new bone and shaped it to have a little bit of turn up at the end the way most little kid's noses do.
3. Genioglossus - you can look this up if you want more details, but this is a chin advancement by using her existing bone and moving it forward.  She didn't have much of a chin before, so this is a big change - I'm hoping functionally it will help her in controlling her saliva (drooling) better.
4. "Spackling" - this is Dr. Gordon's very technical description of what he does to add a bone paste in areas that need some additional bone and contouring - so he "spackled" her cheekbones   under her eyes and some on her chin and not sure where else.
5. Shaping her jaw - I didn't get to much detail on what he did, but basically all the new bone is still soft (think about a piece of gum that you stretch out to make it long) and he can bend it a bit to give her jaw line more of a natural curve.  I can't easily see what he did, but that's what he said he was gonna do.
6. Removal of her "bubble" on her lower left jaw - this was a skin pedicle from her scapula bone graft (when she was 5 and he took part of her shoulder blade to create the missing jaw bone, called the ramus).  This graft included some blood vessels and soft tissue and to easily see that it all survived in the new location, they take some skin with it and if the skin stays healthy and pink, then you know everything else is doing well too.  But, the skin bubble doesn't look great and finally after three years, he has removed it.  He stitched the site closed with internal stitches and then crazy glue for the outer layer to minimize scaring.  The scar is right on her jaw line, so hopefully even if there is a scar it will be just under her jaw as she continues to grow.  
7. "Debride" teeth - Faith has had some teeth that were identified by the dentist and orthodontist back home to come out, but since Dr. G can't "pull" teeth he just aggressively cleaned them and they unfortunately fell out.  ;)  One was a baby tooth that was hanging behind her new adult front tooth and has been loose for over a year but just hadn't fallen out on it's own yet, another was an adult tooth that came in very badly - down in the bottom of her front lip - not much hope we could have ever moved it into a better location and we weren't succeeding in keeping it clean, so it had to go.  I was surprised there was a third tooth in the container too, but it has no root, so I guess it was a baby tooth also. I'm not sure where it came from yet, but will ask him later if I remember.

Coming out of surgery was a bit difficult, but not quite to the same extent as the last time.  Because all the work he was doing was in her mouth and nose, the blood all drains right down her airway and they have to keep suctioning it out through her trach.  It took about 2 hours for them to bring me back to see her, which for me is worse than waiting for the surgery to be done because I know she is awake and I feel guilty not being there.  The nurses said once I came back she finally calmed down which them made the amount of suctioning go down significantly.  I guess I need to start getting pushy about wanting to be called back as soon as possible in the future.  The good thing about this Children's Hospital and probably many others, is that they really want to know what makes it easiest on the child - i remember they used to skip taking her BP when she was small because it upset her too much.  They used to let me be the one to start her anesthesia when she was little because she handled it better.  But they don't like bringing parents back to the PACU (Post Anesthesia Care Unit) when the kid is bleeding a bit uncontrollably and they don't have a plan yet for where they are going to be admitted.  I have to be a little pushier to let them know she will do better if I get back there sooner and I'm not one of those pushy parents who would care if they don't have a plan yet (see the irony, you have to be pushy and say you're not a pushy parent - guess it's all about balance!!)

Anyway once I got back there I ended up getting put to work, Faith only wants me to do anything to her, so when they wanted to change her to a cuffed trach again she wanted me to do it.  Dr. Gordon got called back to work again too after he had gone home because she was having issues and they needed her primary Dr to give them directions about how he wanted it to be handled.  I felt bad, but at least I got another chance to talk with him about next steps.  He had mentioned right after the surgery that he might want to wire her jaw shut and after he left and I was sitting in the waiting room for 2 hours I didn't really know what he had said.  I was dreading the thought that he might send her home again with her jaw wired shut (she is dying to EAT and TALK again!!), but instead he clarified that it would just be for a few days before she would go home.

We ended up going to the PICU again due to the bleeding and I spent the night getting up every 20 or so to suction her trach out.  It's so exhausting to do that all through the night.  I am so grateful that even though she has had a trach for 8 years the last 6 of those years have been mostly easy except for a few colds and her surgeries.  I know we will be right back to that in a few more days, but right now I'm a bit sleep deprived!!

We have been having a great day today - graduated out of the PICU and over to the Complex Airway unit where she will stay for another day or two and then she'll be discharged (unless the wiring thing happens, then I'm not sure how long we'll stay).  She got her catheter out this afternoon and started her feedings, but she is still sleeping almost non-stop.  After getting her all settled down after the move I decided to head to the Ronald McDonald House for a break where I'm eating and typing this, but it's time to go take a nap!!

I'll add a picture later today - I'm having issues getting it from my phone to my laptop - limited internet connectivity.

Will try to update again soon.

Mid Point check (Post Op 3 weeks)

We saw Dr. Gordon yesterday and got a good progress report. He thought everything is looking good and just wants us to turn her pins for one more week, plus I have to do some hardware adjustments which I felt super uncomfortable doing last time, but I did it then and I watched his residents do it while she was in the hospital after this last surgery, so I guess I can do it again at home rather than setting up another OR appointment. After we are done turning the pins we let it sit for a week to stabalize and then come back for her final surgery to remove the halo/RED. He plans to do a number of other things during that surgery as well including fixing her nose (rhinoplasty), removing the skin bubble (pedicle free flap) from her left jaw that was placed there during her scapula bone graft 3 years ago, shaping her jaw, doing a chin advancement (genioglossis) and adding some bone paste under her eyes (I think he calls it spackling - basically adding some bone paste, which I'm guessing is using some cadaver bone/powder - I'll get more details before hand but I've heard about this from him before and I believe Chloe had it with her last surgery).

It's sounds like a lot in one surgery because it is a lot! He says its about a 4 hour surgery and she will be in the hospital for 2-3 days afterwards, so I'm working with his scheduling assistant to set it up. He said it will might be a weekend because he didn't think he has a 4 hr opening in that timeframe. That's just the way he is, working all the time.

Once i confirm the dates I will provide another update including new pics. The change is huge right now, but some of that is just the way it's being pulled forward right now.

We are finally driving home. It's been a long trip and we are anxious to get home. It was good to take the time off to spend with Faith and it was great seeing friends in NY and my family in VA. It's never enough time to visit with each person, but I love being back home and it was great having my mom's family (Debbie, Bobby, Carol, Tresa, Richard, Sean and Tanner) there too. So glad our dates worked with the family reunion! Faith and Anthony both loved the campfires and the fireworks!!

All for now, thanks for reading!

2 weeks into Jaw Distraction

Quick update to add some comparison pictures from her before shot to 2 weeks post surgery.  We are in Virginia this week but too busy to do a long post today.  Will try to write an update soon, but the pics below say more than I can write...

Post Op Days 1 - 4

So hard to find time to write or to even make phone calls to let everyone know how she is doing, I started this post late last night and realized I had to stop and try to sleep and it's taken me till 10pm tonight to start again.  I've relied a bit on Facebook and text messaging from my phone to at least give a few updates.  But I want to keep up her blog because it's a better place to journal, I can write more details, post pictures I wouldn't put on Facebook and it's a keepsake for Faith to know what she went through someday when she grows up and wants to know more.  Probably too much!!

Seeing Faith after surgery was difficult.  They finally let me come back to the PACU (forgot what it stands for, but basically it's the recovery area for same day surgery) to see her and she was upset and trying to talk but I couldn't understand anything.  She was in pain and it was not a pretty sight (skip to the next paragraph if you don't have a strong stomach for this stuff) - Dr. Gordon didn't cut her skin anywhere except a small hole under her chin, but he was able to get to her upper jaw/cheek bones through her mouth and by pulling open/down her eyelids.  I'm grateful she won't have external scars, but it is really gross.  She was bleeding from every opening - her eyes were the worst, her nose, and mouth.  They tried to clean her up before I saw her, but I don't think there was really a way to get her clean for any length of time - I've been seeing it for the last three days.  Luckily the bleeding pretty much stopped yesterday (Saturday).

I'm going to post pictures in reverse order here to try to minimize some of the shock, you can decide when you have seen enough!!

POST-OP DAY 4: Sunday, 6/21 - Father's Day

Happy Father's Day to Fred from Anthony and Faith!  Not the best way to celebrate, but as a dad, you just do what you gotta do for your kids. Faith was a little cranky this morning, so she wasn't very sweet to Daddy.  Luckily she wrote him a nice Father's day card before we left Denver and she did hand it to him this morning.

Faith managed to sit up by herself today too.  She likes to hold her head and then pull herself up - helping her is definitely off limits right now.  She doesn't trust that anyone else can help her without bumping or going too fast or doing something that would make it hurt more than it already does. She also managed to slide out of the bed and into my lap so the nurse could change her sweaty sheets and give her a clean gown.

Towards the end of the day today old friends from Colorado, Keshena, Dennis, Aidan, Colin and Evan, who moved to Columbus OH awhile back drove down to visit us.  Faith was happy to see them and they brought her lot's of gifts.  I didn't get a picture of all of us, but a cute picture of the boys and Anthony together...

POST-OP DAY 2 or 3 (it's all a blur now):  6/20 or 21, The swelling has gone down substantially and she was starting to be able to open her left eye.  You can see she looks somewhat comfortable and is watching TV.  The beautiful quilt on the bed was a gift from the Ronald McDonald House when we arrived on Tuesday - some wonderful volunteer couldn't have put together a prettier collection of prints for this.  So nice to have something pretty to look at!

POST-OP DAY 1:  This is in the PICU the day after surgery and just after being taken off the ventilator and put on a cool mist humidifier.  We fluctuated between room air and oxygen depending on her blood oxygen saturation level.  It was interesting that while she was in the PICU all the doctors, residents, nurses, respiratory therapists do rounds outside of the kids rooms and invite the parents to join as well.  Fred noticed that it looked like what my teams do at work when we do daily stand-ups.  He is exactly right, the team goes around and talks about the kids latest stats and the goals for the day.  

POST-OP - Day 0 - Wednesday 6/17, this is after we got settled into her PICU room they allow Anthony to come back and see her fro about 15 minutes.  I love this picture, because he did exactly the same thing the first time he ever saw Faith after she was born and he was only 2.5 yrs old!!  She is a bloody sight in this pic, but Anthony managed to ignore it all and just give her a little comfort.

All for tonight.  We are really hoping Faith will get discharged tomorrow, but need to just wait and see how it goes tomorrow.

Goodnight,
Robin

Surgery Day - June 17

So today is Faith's 4th big jaw surgery.
Here's the history...

2 yrs old - first jaw distraction - gained a good couple centimeters and she was able to really start talking and wearing a passy muir valve (one way speaking valve).

5 yrs old - scapula free flap graft - took part of her shoulder blade and some surrounding tissue to build up her left lower mandible where no bone was from birth.  Gained some additional airway.

6 yrs old - second jaw distraction - gained another couple centimeters of growth.  She was able to be capped all day following surgery, but failed a sleep study to be a candidate for decanulation (removal of her trach tube).

8 yrs old - third jaw distraction plus LeFort III - starts today.  Will turn pins for 3-4 weeks and adjust as needed before finishing and removing hardware to solidify.

On top of these major surgeries she's had a lot of smaller surgeries including being trached when she was just 4 hours old, gastrostomy tube and Nissen Fundoplication at 10 days old, bone anchored hearings aids around 2 yrs old plus a couple hiatal hernia repairs (that failed) thrown in the mix.

I've lost track of what number this is, but bottom line, it's been a lot!! It's always hard, but we get through it.  She cried going back this time, I don't remember her doing that before so it made me want to cry too.  I held it back until we turned to walk away.  :(

She did have some fun initially playing UNO with Anthony while the nurse collected all her pre-op info, but once it was time to get the gown on and even with some Versed medicine (an amnesiac that usually makes her a little goofy) she started to get scared.  I'm surprised she hasn't been a mess for weeks, I sure started to lose it last week when I found out everything Dr. Gordon was planning to do.

Basically this time he decided to do both her lower jaw and her upper jaw.  But the upper jaw is way more involved.  There are three basic types of upper jaw surgeries - classified as a LeFort I, II or III depending on where they make the cuts.  Dr. Gordon said he was doing something in between a II and III.  If you Google the LeFort II and III you will see why I got upset - it's just so extensive - cuts through her nose and around her eyes it's just gross and an awful lot.

On the good side though, Fred's parents found this YouTube video (https://www.youtube.com/watch?v=GnP12pX1T9s) about a young girl Melissa who had a LeFort III mid-face advancement and the before and after change is really impressive.  But as a parent who has grown to love their child's face just the way it is, you really don't want it to change!!

She went back into surgery today around 9:30 am, and Dr. Robin Cotton, a really well-known ENT did her bronchoscopy and then came to show us the results (pictures).  Basically, they cannot see past her epiglottis when she is resting, but they are able to see her vocal cords once they manipulate her jaw forward and pass the endoscopic tube down her throat.  Hopefully when he does it again at the end of the distraction it will look significantly better.

After that Dr. Gordon was able to get started.  He finished around 3:50 and came to talk to us - said it went really well, he had thought she would need to have a blood transfusion during surgery, but she did not - said she only lost about 100 ccs of blood during the whole thing.

It's 6:00 and I still haven't gotten to go see her.  I had to start asking what the delay was and learned she was having some issues with her oxygen level.  Blood drainage was going down her airway and so they needed to take care of that by putting a cuffed trach in (see here to learn what a cuffed trach is: http://trachs.com/cuffed-and-cuffless-tracheostomy-tubes/).  This will prevent the blood from being able to go down her airway and into her lungs. Anyway, they finally called me back to see her...

4 hours later ...

Okay, we got to see her and she is doing good... well that might be an overstatement, but she is sleeping now with a fair amount of help from pain killers (she's on a continuous fentanyl drip) and she is on a ventilator for the night.  I hope she comes off of it in the morning, but we have to see how long it takes to wean her oxygen (she's at 40% right now and needs to get back to room air).

They just offered me a sleep room and since she's very drugged up I think I will go take advantage of it instead of trying to sleep here in the hospital room with her.  Fred and Anthony are back at the Ronald McDonald House for the night and even though it's right next door I would rather be here with her overnight.

I will upload a couple post-surgery pictures of her tomorrow.

Goodnight and thank you all for all the great text and Facebook messages, means a lot!!

Robin

Before Surgery Pics

Took these pics yesterday. So this will be the comparison when she is all done!

Before: forward facing

 Before: Left Side (scapula graft/worse side)

Before: Right Side (good side)

Close up of her teeth!!

On Our Way to Cincinnati!

Hit the highway at 9:24 am, thought we were gonna start before 8, but not being on a strict schedule and being able to take lots more than you can when you're flying caused a bit of a delay! Kids are happy in the backseat watching a movie on the DVD player, so we're off to an easy start!

The plan is to drive ~ 9-10 hrs to Kansas City today. I thought I found a great hotel with a water park in it, until I discovered that they close at 6 pm on Sundays and are closed all day on Mondays and read some crummy reviews about the hotel too. Oh well we'll just sleep and get back on the road in the morning!

On Monday we are driving to Indianapolis, about 2 hrs from Cincy. Our niece, Meghan, hooked us up with some discounted rates, but couldn't find anything in Cincy for Monday night , so figured it would be a nice way to break up the driving to stop in Indianapolis.

On Tuesday we go straight to the hospital to see Dr. Gordon and have him explain his surgical plan for Wednesday. On the phone Friday his nurse told me it will be both the lower and the upper jaw. I learned a lot about the upper jaw surgery years ago, called a LeFort II, but she said he is doing some something between a LeFort II and III and I realized I had no idea what the difference was.
I looked it up after getting off the phone and it just means the cut the bone in a different place. It kinda shook me up, it involves her eye sockets and the bridge of her nose. I almost want to say no. I'm having to trust him more than ever. Thankfully thus is his fourth time working on Faith and we've always had good results in the past. He also said for us to expect her to be in the hospital for 5-7 days. That's a LOT longer than the 1-3 nights I was thinking it would be.

We are staying at the Ronald McDonald House from Tuesday - Sunday, but now Faith probably won't get to spend more than the first night there. It still will be much easier for us as we can walk back and forth from the hospital anytime to trade shifts or go shower or get a bite to eat.

I will try to post a little every day or two to keep everyone up to date. Thanks for reading!!

Robin

Good morning Faith😊😀