Wednesday, January 27, 2010

Good stuff

Maile got her report card yesterday---and came home with the kindergarten version of Honor Roll! How cool is that? We had some issues the first nine weeks of school due to all of us slacking when it came to learning her sight words. But we busted those out this time around and we are all so proud of her. She got a ribbon, a certificate, a pencil and a free meal from Sonic. I'm so glad she loves school and learning--I really hope it sticks!

Her sixth birthday is about a month and a half away and I'm already trying to figure out what to do. I've talked to her about it and I think we're going to attempt to put together a small dress-up tea party--girls only. I have some good (and thrifty!) ideas, I just hope I have the time and energy to put them into effect. I'm excited, though. It's important for me to make sure she feels special all the time and to do as much as I can for her. I'm so terrified of her one day looking back with resentment for whatever reason. I never want her to feel like we never did enough for her or thinking that we were not there for her enough. Is that crazy?


Kale had his occupational therapy evaluation today and it went really well. The OT he will have is super nice and he warmed up to her pretty quick. She agreed that he has a few things we need to work on and promised that by his second birthday, he'll be diving into his cake (he wanted nothing to do with his first birthday cake)! She's going to help us with all of his feeding issues, help him to feed himself, help him with "messy play" (he does not like to get anything on his hands), help him to transition to a sippy cup and help us with figuring out how to get him cooperating during bathtime (which is a complete nightmare, oh my god...he is STRONG!). Basically any daily activity that is difficult because of his visual impairment or because of setbacks due to surgery, she is going to help us with. I don't know yet when we'll start meeting with her or how often, but I'm looking forward to it. Even though it means he'll have to meet with someone different at least four days a week (he already sees his TVI twice a week and his ITDS once a week), it's okay because he's finally going to be getting the help he needs. And so are we.

Tuesday, January 26, 2010

When will it end?

Kale had a follow up appointment yesterday in Birmingham. It's looking like the skin on his right eye is starting to wear thin--which has Dr. Scruggs concerned that the expander is pushing through like it did on the left side. Awesome. So she said she'll be in touch with us in a week or so and will probably have Kale back in a few weeks to remove the expander and put in a conformer. As for his left eye, it's okay. For now.

So many things are bothering me right now. His left eye looks good--to us--but to others, it apparently looks awful. I won't go into that right now, but it bothers me. I'm doing what I can to take care of it, but nothing seems to work. I'll leave it at that.

All we've been told since Kale was born that without the help of conformers/expanders his eye sockets won't grow correctly which in turn could cause the rest of his facial structures to grow incorrectly. But then I read from actual parents of children with bilateral anophthalmia/microphthalmia who say they chose to not have their kids undergo surgery or wear any type of prosthetic and they never had issues with any of that happening. The thing is, I don't know necessarily how this works with Kale since we've just found out his actual diagnosis is cryptophthalmos--the microphthalmia being a result of it.

But I can't help it. I just wish I could hear someone tell me that Kale doesn't need to go through all these damn surgeries. I just want someone to tell me that he's going to be fine and that all of this is pointless. Because that's how it effing feels right now. If this right expander of his is in fact doing what the left one did, then we have to start almost all the way over. And I just don't think I can allow that. He is 16 months old now. And all this crap he's had to go through has hindered some of his development. He might actually be feeding himself right now if it weren't for these surgeries. He might actually sleep normally. He might be walking already. But all of these things are not happening and I blame it on the surgeries. Tomorrow he has an occupational therapy evaluation--hopefully some of this can be addressed. But I'm sure it'll all come back to the fact that if it weren't for him going through all of this, he'd be right where he needs to be.

We ask his doctor questions and she tells us as much as she can, but she's new to this whole cryptophthalmos herself. She tells us that she consults with other doctors near and far to get second opinions and we greatly appreciate it. But I don't want Kale to be anyone's guinea pig. I don't necessarily feel that way...yet. But I'm worried that I will eventually.

I don't know. I guess I'm just having a hard time right now. It's pretty lonely right now because there really isn't anyone who can give us any real advice, there's really nobody for us to relate to. It's a pretty awful feeling. I hope it passes.

Thursday, January 21, 2010


A month or so ago, Kale's doctor at UAB had mentioned to us that Kale's diagnosis included something called Cryptophthalmos (hidden eye)--a rare congenital anomaly in which the skin is continuous over the eyeball with absence of eyelids (simplest definition I could find courtesy of Wikipedia). Dr. Scruggs briefly went over it but I'm pretty sure she was telling us this after one of his surgeries so some of the information kind of went in one ear and out the other.

Since then I have done all the research I could to find out more. Unfortunately, there isn't much out there that isn't associated with something called Fraser Syndrome (which I was pretty sure Kale didn't have). So I had e-mailed Dr. Scruggs a week or so ago with a bunch of questions that I have been meaning to ask (but somehow always forget to ask). She had contacted me and let me know that she received the e-mail and was doing some research and would respond once she had all the correct information--cryptophthalmos is apparently exceedingly rare (supposedly only a little over 100 reported cases, around 30 of them isolated--crazy, huh?) and certainly not anything she has ever dealt with.

Well, today I finally got her e-mail. Kale has what is called isolated complete bilateral cryptophthalmos, which is, (quoting from an article Dr. Scruggs quoted from) "Failure of formation of the lid folds and globe results in skin extending from the brow to the cheek without identifiable adnexal [eyelid] structures.  Ultrasound may identify a vestigial ocular structure or cyst within the socket." 

Because his is not associated with any syndrome (that we know of--at least not with Fraser Syndrome), his crypto is isolated. 

There are 3 different types of cryptophthalmos: complete, incomplete or partial, or abortive. Dr. Scruggs sent me info on all types and his is the complete type (which I described a bit ago). 

One of my questions was whether his microphthalmia was a result of the cryptophthalmos or the other way around. She said that if an eyelid is not properly formed, then the globe (eyeball) will be abnormal as well. So, the microphthalmia is a result of the cryptophthalmos.

How does cryptophthalmos happen? Here's what Dr. Scruggs said: 
"From what I have read, cryptophthalmos results b/c of an early embryologic failure of development of the eyelid folds.  Development of the eye is totally dependent on having a normal eyelid, so if lid development fails in the embryo, the globe cannot develop normally.  I do not know why Kale has a small (microphthalmic) eye on one side and a full-sized (but still abnormal) eye on the other (for which I do not have a term--there was no cornea, only sclera)."

So. There you have it. Kale's diagnosis is actually Isolated Complete Bilateral Cryptophthalmos (with Microphthalmia). I don't know if he's still considered as having bilateral microphthalmia--she's saying his left eye is full-sized (but abnormal), but we've been told that while it appears to be normal in size, it is still microphthalmic. 

It is nice to finally know everything. I always just thought Kale's microphthalmia was more severe than others--and we've certainly had issue after issue with his lack of eyelids. Turns out it's just a more difficult situation with him because of something else entirely. Not that it's a good thing, but it makes it a little easier to understand. For me, anyway.

Wednesday, January 6, 2010

It's been a long December...

...and there's reason to believe that maybe this year will be better than the last.

Someone reminded me of that song recently and boy, does it fit.

2009 was rough--and so far 2010 isn't looking so hot, either. Another person suggested that we're just getting all the bad parts out of the way now so we can enjoy the rest of the year. I'm not holding my breath on that one, though.

Kale's surgery last month really took a toll on us. A week after having his last set of expanders put in, his left eye started to do some real funky stuff. A large crusty scab-like thing covered his entire eye and we had no clue why. We went back and forth with his doctor via telephone and e-mail, sending pictures and trying to figure out what the hell was going on. She had him on antibiotics in case it was an infection (although she didn't think that's what it was) and we just kind of had to sit and wait it out until his next appointment which was this past Monday (this wasn't because his doctor wasn't concerned, but because she was due to go on vacation and it was Christmas time). But the wait was awful. We couldn't let him "cry it out" in his crib like we had been doing because every time he cried, more of his eye would scab. Parts of it would start to fall off, but then once he cried for whatever reason, it would start to form that nastiness again. It was just no fun at all for any of us and Mike and I have been sleep deprived and grumpy (more so than usual, anyway).

Anyway. This Monday finally came and went and his doctor wanted to go ahead and schedule him for surgery the following morning. So yesterday morning, she cleaned all the funkiness from his eye and discovered that the expander was basically spitting itself out! It was pushing through his skin rather than through the incision! How awful, huh? My poor little guy has gone through so much crap and this was just the worst. So, Dr. Sruggs removed the implant and luckily his eye wasn't infected at all. There was a bit of skin from his "lid" that wasn't viable but it wasn't enough to require a skin graft (which was a concern). Whew! Instead, she put in a tiny conformer and sewed his eye back up and he looks like his old self again.

The part that sucks though is that it looks like we're going to have to start all over with his left eye and the expanders. All this time that the expander was rejecting itself, all the tissue that had been expanded over the last six months moved back into place and so all that space and progress that was made is pretty much gone. His right eye is fine, so I guess that's something. He should be able to start with a conformer in that eye soon.

Despite all of this, we did manage to have a good Christmas. Maile was sick during the entire Christmas break, but that didn't keep her from enjoying all of her gifts from family and from Santa. Kale has been loving all his toys--lots of noisy ones, haha. But it's all good. We're a super strong family and we can pretty much take on anything. It may not be easy, but it's possible.

I've got more to share, but I'll cut it here for now.

Here's hoping 2010 will get better...