I have been working on this post since our pre-op days in Boston a couple of weeks ago. But, to be honest, this one is going to be a really hard one, so writing about it hasn't been easy. I found the four days of pre-ops particularly exhausting, both mentally and physically. Somehow, we managed to still laugh and have a little fun. But, I can tell how my spirit was by the lack of photos.
Anyway, if you're interested in the nitty gritty of what's about to go down, here it is in all it's glory. Proceed at your own risk.
Here was our schedule:
Monday: 9am flight to Boston, Cardiology at 2:30 (EKG, Pacemaker check)
Tuesday: Echocardiogram at 11am, then Dr. Lee (ENT) at 1pm
Wednesday: Off - New England Aquarium
Thursday: Dr. Resnick (plastic surgeon) at 10:30am, Pre-Op at 12:30, 9pm flight back to Richmond
Today is Wednesday, February 12 and it is our day off from appointments, which we are enjoying. We (Caylee, me and Harlie) flew up Monday morning, February 10.
We had five rolling cases (one suitcase with clothes, etc. for each of us, one large suitcase for all of Harlie's medical equipment, formula, etc., and her portable oxygen concentrator), Caylee has a backpack, I had my bag, and Harlie had her suction machine bag. It was a lot of hard work for two adults rolling her wheelchair AND five rolling cases. We had Harlie hold on to her little suitcase (red one), Caylee pushed Harlie with one hand, pulled her suitcase (purple) with her other hand, and I put two matching large suitcases (green and blue) together and rolled them with one hand and rolled the portable oxygen concentrator (black) in the other. The most challenging part of rolling all these cases was getting in/out of elevators (which you have to do a lot when you have a child in a wheelchair). Most of the time the wheels would get stuck in the crack in the floor and the doors would start to close while we were still trying to get everything in/out - and this was while people were looking at us like we were CRAZY.
It was exhausting. And I could never have done this alone.
Once in Boston, we ordered an UberXL, and it was still a game of tetris to fit her wheelchair in with all of our stuff - plus us. Haha! We ubered to the hospital because it was too early to check in to our AirB&B. We checked our luggage in at the hospital (this is something a hospital offers when they know so many patients travel from far away) and went to find a place to sit in the cafeteria. Our appointment was at 2:30, but we got to the hospital at 11am.
The cafeteria seating area is quite small for such a large hospital. And even though it wasn't quite lunch time, every table was full. There were several tables where only one person was sitting down, on their phone, with no food. I am not normally like this, but I was exhausted and went up to an employee of the hospital who was taking up a table while on his phone. I asked him if he would mind letting us have the table. He left. I didn't even feel bad, that is how tired I was. After a few minutes, Tom called to tell me that the Air B&B contacted him to let him know that we could check in if we wanted. So, we went back and got our luggage and got another UberXL (this one wasn't big enough) and we SQUEEZED ourselves in. Ugh. So tiring. We unloaded at the apartment and had to carry all the stuff (including her wheelchair) down one flight of stairs. OMG. I cannot tell you how tiring all of this moving around was.
The apartment is awesome and perfect for our stay (except the flight of stairs which is a real pain with the wheelchair). No, there is no elevator. The owner of the AirB&B contacted Tom last week and told him that the people who were in this unit wanted to stay longer and asked if we would be willing to take a unit on the 3rd floor instead. While we wanted to help out (we could very well be in that same situation next month) there is NO way I could have handled carrying all this stuff up/down three flights of stairs. No way. So, I guess those people had to move upstairs. I feel bad, but... just no.
It rained all day Monday and Tuesday. It is less than a mile's walk to the hospital from our apartment. We decided to walk anyway. I brought an umbrella and Harlie held it over herself.
The cardiology appointment on Monday went okay. She had an EKG, and a pacemaker check. While waiting I fell asleep in the chair! I told Caylee I would pay her a million dollars if she went downstairs and got us coffee. She did it for free. While sitting there, feeling so tired, I thought I just don't know how I'm going to do this again. I don't know how I'm going to do another stressful, difficult, hospital stay. My God, how many more are in our future? I am only human - Harlie is only human - how much more can we do? And the last two surgeries resulted in three months in the hospital. Having those two recoveries as my most recent experiences and memories are proving very challenging for me. If you see me out and about and think, "Oh, she's totally fine" I'm sorry to say that you are wrong. I'm working really hard to keep my shit to myself. So, what you're seeing is a fuck ton of effort paying off. You're welcome.
Anyway, they scheduled an echocardiogram for Tuesday. We left close to 5pm, I think. We walked to Trader Joe's and I forgot how freaking insanely busy that place is! We bought a few things like coffee creamer, eggs, and wine. Lots of wine. Then we hit CVS and then walked back to the apartment. My arms felt like they were going to fall off. Wine is heavy and we were walking. It was an exhausting day. Not sure if I mentioned how tiring it was. Haha.
We went back to cardiology Tuesday, and got her echo done. Echos used to be so difficult for her. She would cry and fuss, which I never fully understood because it was like the least painful thing she had ever done. But, I am happy to say that phase is behind us and she was perfectly cooperative and the tech spoke directly to her and she did what he asked. Hallelujah!
After that, we headed across the street to meet the ENT, Dr. Lee. Last month, Harlie had a DLB (direct laryngoscopy and bronchoscopy - aka - scope of her airway) in DC with her ENT, Dr. Preciado. He told me that Dr. Lee had already contacted him about Harlie. I was impressed. Although, the fact that docs are discussing my kiddo two months before a surgery because she is so complicated is not lost on me.
Dr. Lee spent a long time with us. We went over her history and what our concerns were for next month. Hard to believe it is is only a little over 3 weeks away! He said that if this jaw distraction doesn't get her tongue base out of her airway enough, he could do surgery on her tongue and remove part of the base.
For real.
It is called a glossectomy. I'm just going to hope it doesn't come to that.
He put a camera down through her nose to look at her upper airway. This was hard on Harlie, but she was a champ and tried so hard not to fight it. I know it wasn't comfortable, but she managed. We saw her epiglottis. Hers stands up (because her tongue base is too far back towards her throat), but it is supposed to lay down.
At one point he asked when her surgery was. I answered March 6. He looked at his calendar on his phone and said, "I'm in Waltham that day. Can you do it March 5?" I was like, what? I explained that getting Dr. Resnick and Dr. Padwa available on the same day was a challenge and we scheduled this back in the summer. Oh my gosh, don't tell me you have to be there, too. I didn't say that last sentence out loud, of course. I mean, if it is best for Harlie, then... but please dear God tell me that's not the case. Thankfully, he thought it over and said it was way more important for him to see her during her recovery and before she gets discharged. He said when they remove the hardware a few months down the road, he would like to do a scope at the same time so he can see what her airway looks like. Sounds like a good plan to me. Whew!
He agreed that we should know pretty quickly whether this distraction surgery is successful. When they remove the hardware, theoretically, she should be able to breathe around the trach. So, we'll see... that's several months away from now.
After that was over, we headed back to the apartment to relax until dinner.
Today (Wednesday) we are headed to the New England Aquarium. Harlie has already looked up the Boston Children's Museum on her tablet (I don't know how she did that) and has said she really wants to go there. Unfortunately, I just can't let her go where a lot of kids go and have touched everything. I just can't risk it. I told her we didn't have time. I thought she would fuss, but she just said, "Okay, maybe next time we are in Boston?" Sometimes, her NOT fussing is worse. So, I said, "Yes, maybe when we come back for surgery we can go the day before." But I'm not sure I can let her do it then either. Ugh.
Thursday, February 13
So, yesterday we went to the aquarium. It was fun. She loves that place. And they are so nice - they let people with wheelchairs pay inside vs. standing in line outside. And they let her in for free. I sent a text to a friend of mine that gets my humor and said, "They let her in free because she's in a wheelchair. Its finally paying off!" I crack myself up.
Anyway, look how happy Harlie is to go there.
She took photos with her tablet of everything so she could look at them later. She is so funny.
The aquarium was perfect - it was cold and rainy out and there was practically no one there. She got up from her chair a few times and walked around.
This next picture was at the top of the big tank that runs down the center of the building (down 3 flights, I think). If you look, you can see her waving at me from the other side of the tank.
There was something about the gusto in her wave that got me. Like she was all in - not holding anything back. That is how she is - no shame, no apologies. She lives life the way she wants, she likes what she likes and she doesn't care how other people view her. And she lives in the moment - forget about the hospital - I'm in the aquarium right now!
I wish I could be more like her.
Just because I loved this guy and he was posing for me...
Today (Thursday) we meet with one of her plastic surgeons, Dr. Resnick. Then we go and do the hospital pre-op stuff. Then we should be done. Unfortunately, we have to check out of this apartment and take all of our luggage with us to the hospital. We will store it there for the day until we are ready to go to the airport.
It is now 4pm and we are finally done with all the pre-op stuff and we are already at the airport. I asked if we could get on the earlier flight (6pm) because waiting here till 9pm sounds like it could kill me. I couldn't believe our luck! There were three seats TOGETHER in row 2 on the 6pm flight. YAY!! We were so happy.
So, about that jaw distraction surgery...
Our appointment with Dr. Resnick went well. He showed us the surgical plan. Back in the summer, it was more like a brainstorming session since they had just seen the CT scans right before our appointment. They have had a few months now to put together an actual plan. He said that the 3D printed model of her jaw, etc. is in production. They ordered custom pieces to be placed in/around her jaw and skull. Once everything is in, he will make sure it all fits on the model first so they can work out some kinks before putting them on Harlie. Fascinating stuff, really. Probably more fascinating when it is someone else's kid. Haha. But, I am appreciating all the new stuff they can do to help give her the best chance at a successful outcome.
For those interested, basically, here is the overall plan:
The picture on the left is her jaw now. The picture on the right is what they hope to achieve. They want to move her jaw forward, down and up. He described it as "turning a corner."
The bone highlighted in blue/green is her fibula bone they put in her jaw back in 2012. He said it is a thin bone and we are asking a lot of it with this surgery. I think he said a potential risk is that the bone could react by shrinking a little. I'm not sure I totally understand this. But, regardless, we can only hope that it can do what we need it to.
They will install some hardware, some will be under the skin, and some will be outside the skin. Here is a picture of the cutting guides and her jaw. The holes on the cutting guides are there to give the surgeons several options of where to screw it into her jaw. The cutting guides are there for them to cut her jaw on each side.
Here is the metal that they will attach after cutting her jaw. The holes give the surgeons options for attaching them to her jaw. He said he will cut away the unused metal. Then, the device will have some kind of screw thing that will come out in two places on each side (see arrows). One will move the jaw forward and one will move it down.
I think he said that in her last jaw distraction surgery (done in 2013) they used a device that attempted to move in both directions at the same time. They don't think that will work for her now (maybe no one else, either, I don't remember). So they have one area that moves the jaw down and one that moves it forward.
We will attach a tool to the metal sticking out and with each turn of the screw, we will pull apart the bone where it was cut. The bone will grow to heal, and each day we will basically re-break it and force new bone to grow each day, adding length to her jaw. I think our goal is like 12mm.
The chicken foot device is there to force the growth of her jaw in a forward direction only. We do not want the bone to be allowed to move backwards. So, this device keeps her bone stable. The chicken foot part will go into her skull somewhere above her ears, and metal will come down into the other set of metal. The rods you see attaching the chicken foot parts to the metal in her jaw will be on the outside of her face.
There will be two places where we have to turn the screws on each side (so four places total). He said he is expecting that we will have to turn the screws for two weeks. Then, we just let everything heal, while all the hardware stays in place.
Then, if all is going well, we will return to Boston eight weeks after surgery for them to remove all the hardware.
There is one thing we all have to think over and decide before surgery. Dr. Resnick said that the chicken foot device has a built in moving point, which allows her to move her mouth (open and close it). Unfortunately, this movement negatively impacts the end result, if you will. Allowing the movement might not get us as much growth as we would like. So, we might have the option to prevent the movement, and if we do that, we might be able to have a better end result. This would mean she would have very limited movement (or none) of her mouth for eight weeks.
She has had her jaw wired shut before - twice. And she lived for months (probably a year) with her jaw fused in one position. She has a secure airway with the trach and she is g-tube fed. So, I'm thinking she will be able to handle limited mobility of her jaw for eight weeks. I just hate the thought of putting her through all of this and not getting the BEST possible outcome we can get. So, we are all going to think it over (her docs and Tom and I) and make a decision before surgery.
So, after the hardware is removed, he would like to get another CT scan about five to six months after surgery (around August). They will use the CT scan to create custom joints for her jaw. It takes about four to six months to fabricate them. So, basically we are looking at doing some sort of joint replacement surgery in about a year. They can't do it at the same time because her skin is too tight. They need to stretch her skin gradually, so it doesn't fight the movement forward. Crazy stuff.
After that appointment, we headed to do the hospital pre-op stuff (admissions, anesthesia, etc.) I had to answer a bazillion questions (again) and then we were done. We walked to the Squealing Pig for a late lunch. Then we walked back to the hospital to get our luggage and head to the airport.
All in all, I am not looking forward to the next few months. We are in the "going up the first hill of the roller coaster" phase, one slow, agonizing click at a time. I really feel like this is the worst part. Once surgery happens, then I'm dealing with whatever happens, as it happens. Surgery is 11 days away now, and we have to do everything we can to keep her healthy.
Oh, I forgot to mention that in the lobby of Boston Children's Hospital, they had a beam they are going to use in a new building of the hospital. They were asking kids to sign it. Pretty cute.
Oh, another thing, while we were in Boston, every night after dinner, Caylee and I watched an episode of The Pharmicist on Netflix. There are four episodes. It is really good. I highly recommend it.
Well, that's it for this post. As always, thanks for reading and for caring. I appreciate it more than you know.
Much love,
Christy xo
