Tuesday proved to be a crazy day. I need to back up slightly first.
Sometime in the last few days Murphy stepped on a lego (a constant hazard around here) and cut the bottom of his foot. Tom washed it and put a bandaid on it, and I forgot all about it. Until Monday night, when Murphy started complaining about it (or Tom saw some drainage on his sock, or something. I know, ewwww, right?) So, we took a look and said, "Hello, Infection!" There was pus (such a yucky word), his foot was swollen and there was a red line creeping up his foot starting at the site. Ugh. Of course, he didn't have school on Monday and I took them to the mall to play - but did he say anything then - like when the doctor's office was open? No.
So we are supposed to leave the house at 9am the next morning, and I have no idea when we'll be back. I didn't think he could go another whole day without antibiotics. So, as soon as the office opened (8:30) Tuesday morning, I called his doc's office and they said to bring him in asap and they would take him and get him back out the door to try to help accommodate our schedule. They are so awesome.
Tom left and went to get Murphy from school while I stayed home to feed Harlie and pack the car. Despite our major efforts, by the time we put gas in the car and dropped the prescription off at the pharmacy, it was 9:30 by the time we were on the road.
More than an hour later (we were in Newport News by then) I switched out a movie for Harlie. And in doing so, I noticed that the floor looked pretty bare. Then I asked, "Where's Harlie's suction machine?"
WE LEFT HARLIE'S SUCTION MACHINE AT HOME!!!!
As the magnitude of this error sunk in, Tom started being funny (our go-to plan for very stressful times). And then I laughed and cried at the same time. Oh, so many emotions! We thought about our last thoughts as we were leaving the house. Tom said, "Don't forget your Diet Coke!" Thank God we remembered that! Whew!
And then I remembered plugging it in to charge because somehow we forgot to do that the night before. So, I plugged it in until it was time to go. While I was loading the car I thought about putting it in the car then, but then thought, "no, what if we need it before we get in the car?" I didn't want it to be in the driveway while I was in the house because that was too far.
That freaking suction machine has been at Harlie's side every minute of her life (practically) since the day she first left the hospital.
What if she had a plug? Well, she's certainly had them - but they've not been emergent so far. But I know our luck. The day we don't have the suction machine IS the day we're going to need it. Like really need it. And since it helps keep her airway open - the thought that I don't have it around is not a good one.
We were WAY too far to turn around. And she has a much stronger cough now. And we didn't have a choice. We were just going to have to be without it and hope for the best. I thought if I make it to old age, it'll be a miracle.
So, we get to the hospital and see an ENT first. He said that Harlie's ear is full of fluid, her eardrum is retracted (meaning pressure!) and of course, it's all scarred (nothing new there). Dang it! But, it's not infected. Of course she just had her last dose of antibiotics the night before. So, who knows what will happen now. So, to her - it sounds like she's underwater. Which would explain why we haven't seen an improvement since the blood clot came out a few weeks ago. Which reminds me, I don't think I ever told you about that. Ugh.
Okay - real quick... you might remember in this post I talked about how the pediatrician looked in Harlie's ear and then it started bleeding? It bled off and on for several days. Then a few days later she started pointing to her ear and signing "broken" which she does when the hearing aid battery has died. But, the aid was working fine. So, clearly something was amiss. I called her local ENT and they worked us in. The doc pulled out a HUGE blood clot that was blocking over 80% of her canal. No wonder she couldn't hear! And that's been in there everyday since before school started!
We thought for sure we'd see a turnaround in her listening and participation at school. But, after a few days, it seemed to get even worse! So, the fluid that's in there now would explain that. The ENT that we just saw yesterday said that if it doesn't clear up in two weeks, we should get her another tube. Ugh. It looks like I should really look into a bone anchored hearing aid (BAHA) for her. At least that would give us some back up that wouldn't be affected by fluid or blood, or whatever.
After we saw the ENT, her plastic surgeon came in. We spent more time with him yesterday than we ever have. He looked at her CT scan. I need to figure out how to get a photo of some of the images so I can show you. Her jaw is crazy. Unfortunately, my Mac computer won't read the disc. I'll see if Tom can work on it for me.
Anyway, he talked about what he needed to do. Her ramus is in a horizontal position instead of vertical, like in this picture:
And she's missing the condyle on her right. And I think there's something wrong with the coronoid process, but don't quote me on that.
It's really hard to picture the ramus in a horizontal position. But, her jaw looks nothing like that picture. She has no angle at all, really. But he said he could add one in when she's older. Oh goody, more surgeries! And her bottom teeth are way off from her top teeth. Although you don't need a CT scan to see that! And clearly, the two sides of her jaw are completely different. Her last reconstruction was done in December 2009. The bone graft on the left side took just fine. But the one on the right got infected and had to be removed. This left her jaw even more asymmetrical than before.
Basically, he's going to have to cut up her jaw into several pieces and then put them back together again in a better way. If you've ever had any kind of dental work done at all - I'm sure you can only imagine the pain when recovering from this kind of surgery. It is truly horrifying.
The good news is that he thinks she has more bone to work with this time around. I guess the bone has gotten thicker as she's gotten older. So, hopefully that will help a lot. And he feels confident he can make the changes necessary for this surgery to be successful. But, we've thought that each time and here we are.
The bad news is that plastic surgeons (especially reconstructive for anatomy defects) are like artists. Each patient is completely different. The results vary widely and cannot always be predicted. And since her structure is abnormal to begin with, it makes it almost impossible to know what to expect. All these are my words by the way - not his. It's just my take on it after all these years.
And he said that with Goldenhar Syndrome, not only is the bony structure affected - but so is the soft tissue. And if the soft tissue won't stretch or accommodate the changes made to the bony structure, then the results might not be favorable. The soft tissue can actually force the bone to retract or move by the constant pressure. Which is why it takes a year to know if the surgery was successful. The agony!
He said that he could do the surgery whenever we are comfortable.
After talking it through, we decided that we would shoot for May, 2012. Since her jaw has to be wired shut for 9 weeks, if we waited till after school let out for the summer, we would run a huge risk of her not being able to start school again in the fall. Since she's had 2 out of 3 bone grafts get infected and require additional hospital time, we would rather be safe than sorry. And Tom was thinking that it would be better for her to miss the last month of her first time through kindergarten, than miss her first month of real kindergarten.
So, if we are lucky, and all goes the way we want it to, we would be hoping for decannulation (getting the trach out) sometime in the summer of 2013. So, she would be 7 and starting the first grade (assuming she does kindergarten twice, and is able to progress to first grade).
It's really overwhelming to think she'll be almost 7 and still be trached. I had no idea it would be like this. And what if this surgery isn't successful? I can't even think about it.
Which brings me to my struggle with this whole mess. I was thinking that we should get some other opinions. I just thought that we owe it to Harlie and to ourselves to make sure that we are making the best decision on this. So, I spoke with her ENT in DC about it several months ago. He gave me the name of a surgeon in Jacksonville, Florida. I e-mailed him today. And he replied, and in his reply he "strongly recommended" that I reach out to another surgeon in Miami.
And his recommendation got me thinking that I could probably reach out to a dozen plastic surgeons and that I would still be in the same situation. How will I know who's the right one? So, thinking things through....
1. If I went to a different surgeon, they would be going into her jaw with no prior experience as to what worked or didn't work in the two previous surgeries.
2. I do believe that no matter who performs the surgery - the same issues are present (the question about the soft tissue, bone grafts, results, etc.).
3. I have to have a facility that has a cardiac anesthesiologist and can support her cardiac issues (or at least get her to DC if needed).
4. If we travel far away for surgery (like Miami or Boston) how do we do the follow up appointments? And what if she gets another infection like before? What if time is of the essence???
5. I've already seen two other plastic surgeons (in addition to her current one) and neither of them were candidates to perform surgery, in my opinion. Both of them only perform jaw distraction - not jaw reconstruction. Her current surgeon does both procedures. And it is very clear from her CT scan that jaw distraction is NOT an option for her. Period. Her jaw needs WAY more than just lengthening. It needs full reconstruction. That's just the way it is. And both of those surgeons were trying to tell me that distraction can be done in almost every case. Whatever. I just don't believe that. And to be fair, they had not seen her CT scan when they said that. But, the fact that they would say that without seeing her CT scan is concerning, if you ask me.
6. I am very comfortable with this surgeon. I think he's a great person, as well as a surgeon. But, there is a part of me that says we have ONE more shot to get this right. It HAS to work this time. But are more opinions going to help or confuse? I don't know. But I do know that the thought of something going wrong and having to travel great distances to see her surgeon is VERY SCARY (considering how awful our last experience was, which lead to this).
If this surgery is successful, our lives will change dramatically. Without the trach, Harlie could learn to speak at a much faster pace. Her voice would be stronger. And she would be able to make sound with a lot less effort - which would greatly speed up the process.
Eating could be a lot easier. I have to say that after seeing her CT scan - I have to wonder how much the alignment of her teeth affects her ability/desire to eat by mouth. Her bottom teeth fall to the right and are no where near lining up with her top teeth. So, as I sit here and make my jaw go to the right and then try to swallow, it's damn near impossible! Try it. I don't know how in the world she does it!!! And now I feel like a total horrible mother for making her!
And forget chewing!!! When is she going to be able to learn to chew??? I really don't see how chewing is going to be an option at all - until her teeth line up somewhat. And the thought of going more and more years with no chewing makes me want to barf. Can you imagine the impact of that socially? Not to mention YEARS and YEARS of more pureeing food for her!!! Oh brother. I have to stop thinking about that. For real.
So, I guess you could say I have a lot on my mind. As much as I want this surgery to happen, I dread it. The thought of her waking up afterwards and looking me with that look. The look that says, "What did you let them do to me???" I just have to hope that the next seven months will provide some growth in maturity and focus to allow us to try to talk to her about it so she'll know what's happening. Or at least know more than she usually does before a surgery.
It makes my heart hurt to know what lies ahead of her. Until then, we'll laugh as much as possible.
Oh, and just in case you were wondering, we did just fine without the suction machine. We didn't need to use it once. And what a difference it's absence made to our load when walking around the hospital! And all those times I take it into the grocery store, or Target or wherever? Maybe we can lighten our load a wee bit? I think if it's in the car and we can get to it quickly if needed, it would be fine to not carry it all the time.
Well, that's it. It's super late and this post is super long. As always, thanks for reading!
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