More visits to CHOP

We spent the morning at CHOP again yesterday for an appointment with the ophthalmologist. At this visit, Leah was presented with a clean bill of health again. The doctor dilated her eyes and checked her optic nerve for signs of increased pressure. He said all looked good, as did her ability to track objects and focus. So, we were sent away with instructions to come back in about four months.

We were also able to make an appointment for the next surgery with Dr. Taylor's scheduler. While the eight-week period is over in late January, his schedule was a bit full, so we ended up securing February 3, 2014. That's another Monday, and while we won't know what time the surgery is until the Friday before, we assume that it will be another 6:00 am surgery. The benefit of that is that we are Dr. Taylor's first case of the day, but of course the downside is that we need to leave the house just after 5:00 am. But, since this will hopefully be her last surgery, we won't mind the early wake-up call in the least!

Done!

We are just back from meeting with Dr. Taylor, and the appointment could not have gone better. He took an x-ray, which told him that Leah was done with the "activation phase," meaning that we don't have to turn the distractor any more. We haven't made any turns since last Thursday night, when we were unable to connect the device to what was left of the distractor. In all, we turned it 30.5 mm, or 1.2". During the office visit, Dr. Taylor used a similar device to pull a pin at the end of the distractor. When the pin was pulled, the joint holding the two parts of the distractor together released, and the part that was external to her skull came out. Leah didn't enjoy that part, obviously, because a good part of it was beneath the skin, but she recovered pretty quickly. Dr. Taylor asked us if we were ok as well, since it's always hard to see your child in pain, but at this point, we are pretty resilient, and this procedure was nothing compared to what we dealt with a month ago!

So, now we are left with a little hole in her skull, which he says should close over in about 48 hours or so. Of course, the main part of the distractor is still in place, and will be until the next surgery, which will take place in about 8 weeks. We are hoping to get a call back from the doctor's scheduler today so we know when that will be, but it will most likely be the end of January or the beginning of February. That surgery will be similar to the first, but less invasive, as he will just be removing the distractor rather than installing it. He told us to expect that surgery to last no more than an hour (plus anesthesia and prep time), and that she will probably be in the hospital for only one night. Plus, while the incision will be along the same line as this one, and will be the same length, we probably won't need to shave her head, which is good because her hair is growing back pretty quickly!

That main distractor is still pretty pronounced, and we will need to be careful that she doesn't bump it on something, but now that the external part is out, it's going to be much easier to take care...no more turning, cleaning, worrying! That also means she can go back to day care, and back out into the world...good news for the whole family!

Here's a picture of the part of the distractor that he removed in the office today:

We did ask about the shape of her forehead, which is still not perfectly symmetrical. He said that with the additional space in her skull, her brain will now start to grow in the right direction, and will likely fill in that part of her forehead on its own. He said we may not see those results for six months or so, and if it turns out that there's still a flat spot in the front, we can discuss helmet therapy to shape her skull. We are obviously hoping that won't be necessary, but are just going to put that worry aside for now, and see how the next several months go before we start to think about that!

Small milestone!

There's an upside to a very, very short distractor: over-the-head clothes! Leah is wearing her first non-pajama outfit in over a month this morning!

A pause, or an end, to the turning

Tonight, Leah's distractor was finally too short to turn. David tried to attach the wrench but there was so little of the distractor left that he was pinching her skin when he tried to attach it. She was crying, and though he kept trying to make it connect so we could get at least one turn out of it, we ended up giving up before we could turn it at all. Leah was pretty upset by the whole thing...it wasn't fun for any of us! We emailed Dr. Taylor, and he suggested we stop turning and come to see him on Monday of next week. He said he would do an xray before he decided if he would try to turn it anymore. I assume that if he did want to continue to turn it further, it may need to be done with a different type of device, and hopefully with at least a topical anesthetic, if not something more. We have turned it 30.5 mm so far, and we were going for 20-30 mm in all, so it's reasonable to think we may be done. If only her forehead on the right side had come forward further, I would feel much better at this point.

We need more distractor!

We sent more photos to Dr. Taylor this morning. From the photos below, you can see that we are starting to make some progress!

Here's a longer-range comparison, where the gradual change is more noticeable.

And just for fun, here's a comparison of Leah's face. The change in the symmetry of her face is kind of unbelievable in this collage. I admit that in the "before" photo, her head is a little bit tilted forward, so perhaps the asymmetry of her face is a little over-emphasized, but still...that's pretty remarkable! I have to admit that this photo alone is making me feel better!

So, the issue is that we are running out of distractor. See photo below for illustration:

When we emailed Dr. Taylor today, he said that Leah's forehead still isn't as advanced as he would like it, so we will keep turning. He did say that we will get as much out of the distractor as we can, and then perhaps we will deal with the rest of the forehead "down the road," which is obviously not what I want to hear. That could mean either a positioning helmet, or CVR (cranial remodeling) surgery later. We are hoping to avoid either of those, so we will just continue to hope that the forehead comes forward very, very quickly. There is no extension that we can add to the distractor, so when we run out, that's it. I am guessing that we will run out in a day or two, but David thinks we have more length than that, and Dr. Taylor seems to think so as well. He did say that we shouldn't dive under the skin in order to turn the distractor, which is a great relief. If we get to the point where it is going under the skin, he may ask us to get an extra mm or so out of that last turn, but I would email another round of photos to him before we get to that point, just to make sure we are still turning correctly.

On the bright side, he did say that her orbital morphology is fantastic, and that it's much better than he would have anticipated had we chosen the traditional treatment (CVR). That's fabulous news, of course, but we can see that a little bit each day, as her eyes align and her nose straightens. It's interesting to go to her crib each morning when she wakes up, since she looks just a little bit different each day. Of course, babies grow quickly, and that's true of all babies, but especially striking when the changes are happening with the help of a distractor!

Cappskids

Since we first found out that Leah had craniosynostosis, we have used several online resources to help us understand the condition, and the treatment that she would be facing. These include the wonderful Cranio Care Bears, which is a not for profit started by two moms of "cranio" kids who solicit donations of items that may be helpful for kids and their families during cranio surgery. We received a care package from Cranio Care Bears in the days before Leah's surgery, and the package included a prayer chain that we hung over her crib at CHOP, which appears in many of the hospital photos.

We also used the Cappskids.org website, which provides lots of easy-to-understand articles and videos about the condition, and its various forms. They publish stories of "cranio kids", which we found really helpful in knowing what to expect during surgery, and in the long term. They also publish a calendar each year which features photos of "cranio kids" on their "cranioversary." The calendar just came out today. Recognize anyone on the cover?

Three Weeks Post-Op Progress

We sent another round of photos to the surgeon this morning. His response was that we should keep turning, and that she looks good. He said that he thinks her "activation phase" will soon be over, which is a great relief. I have to admit that I am still not seeing the progress that I hoped to see, especially from this perspective. If anything, her skull looks wider than it looks "reshaped" and the front still does not look balanced to me. The surgeon said we were going to overcorrect the right side, so I expected at this point to see her forehead looking more even from the top. Of course, this is not really a big deal if her skull is expanding in some spot at least enough to allow her brain the space it needs to grow. In the grand scheme of things, this from-the-top perspective is one that no one usually has for other people, and unless she is going to sell shoes or work in sewers, no one is really going to be looking at her from this angle when she is an adult!

My lingering fear, though, is that we are running out of distractor! We have moved it 22.5 mm so far, which is .88 inches. The part sticking out of her head is getting shorter, and we are just about to reach the point on the distractor where the first flared area is going to go under her skin. I am hoping that this won't cause additional pain for her when we turn the distractor because the hole in her skin will have to get bigger, but the surgeon assured us that we should continue to turn beyond this flared area. He also assured us that we will probably stop turning it before the whole thing is sub-cutaneous. Here's a photo of what I mean:

Of course, we are just going to keep doing what the surgeon tells us to do, but I can't help but worry at the same time. Weeks ago, I was counting the days until we could go back to Philadelphia to have this external part taken out, but now it has gotten so short that it's really not in the way, and when she is wearing her famous beret it's not even noticeable at all. I suppose we are looking forward to having the entry point to her head removed and the external part removed so that the hole will heal. That will mean that we can take her back to day care and out in the world again, and I'm sure she will appreciate a return to normal life as well. But, as evidenced by the photo below, she is doing very well, and still has no idea that any of this is going on inside her head!

Leah, following her first taste of solid food...

More Questions for our Surgeon

We were in touch with Leah's surgeon again today with some questions about the distractor. As we are turning, we aren't exactly sure what is happening under her skin to the distractor. We know the size of the distractor is not changing, but we are starting to guess that the amount in which it sticks out the back of her head is getting smaller. If that's the case, that also means that the amount by which it is protruding near her forehead is getting larger. Here's an illustration of what I mean:

I have noticed in the last few days that the distractor really seems to be noticeable from the front of her face, under her skin, and it of course looks painful to me. She will periodically have a reddish spot right at the end of the distractor on her skin, as if she has rubbed on something and the skin is irritated there. As we continue to turn, I imagine that is going to get worse. So, I emailed our surgeon again today to see what he thinks. To be respectful of his time, I basically sent the photo and asked him to confirm if this is what he expects to see at this point. He said it is, and that the 1mm per day change is "well within the skin's inherent elasticity." Having just gone through a pregnancy, I trust him on that one!

Here's another set of photos that shows the change at her forehead:

Since the internal part of the distractor will be in place until late January, or possibly a little later if we cannot schedule the second surgery right away, I hope that the placement of the front part doesn't become an issue for her in the coming months.

Two Weeks Post-Op

This morning we sent our first set of photos to Dr. Taylor for his review, to see how Leah is progressing and how much the distractor has changed the shape of her skull. We have turned the distractor 13.5mm, which is about a half of an inch. In the photos below, you can see the change. It's a bit difficult to really see much progress, especially because the distractor is still pulling her skin away from her head. But you can see that her eye is no longer visible on the right hand side when looking at her face from this perspective, so there is a bit of a difference.

We are really pleased with the way her incision is healing. The stitches are all still in place, and thought they are dissolvable, we know it may take several weeks for them not to be noticeable. We are still taking care of her incision by cleaning it and by applying bacitracin to cut down on the risk of infection, but we will soon transition to "scar care"and will focus on massaging her incision rather than treating it with topical antibiotics. The site of the distractor is a bit more difficult to care for, as it is essentially a fresh wound every time we turn it. It still bleeds, so we are coping with that by placing a clean cloth diaper under her head whenever we put her down.

We are also managing the distractor by putting hats on her as much as possible. Not only does that reduce the risk of exposing it to germs from something like a cough or a sneeze, but it is also helping our other kids to keep from seeing it. They have each seen it at least once, but neither of them like it, and still ask if she is wearing a hat before they enter the room. Ironically, the only person in the family who is not bothered by the distractor is Leah!

Healing well

What surgery? With a hat on, you almost can't tell that Leah had surgery at all!

All continues to go well with Leah's recovery from her first surgery. We are almost two weeks in, and have turned the distractor 11.5 mm so far, which is just shy of a half of an inch. The swelling has gone down considerably, but it's still difficult to see the progress we are making because the shape of the distractor is changing the shape of her face. In the photo below, however, you can see the cut on the right side of her skull, which shows the area that is being advanced every time the distractor is turning.

Her pain seems to be diminishing substantially, though her sleep patterns are still off. Before the surgery, she was sleeping through the night, but those days are over! At this point, she wakes up two or three times per night, though most times she can easily get back to sleep. Hopefully we will see some improvement in that area in a few more days.

On Monday, we will send her first set of photos to Dr. Taylor. He will have a look at them and determine whether or not we need to keep turning the distractor. Most likely he will say that we should continue, since we are probably half-way, or even a third of the way, done. We do have a few questions for him as well. I am a little concerned that the front part of the distractor is poking through the front part of her skin, near her face, and while I'm sure he will assure me that it doesn't hurt her, I can't imagine that she doesn't notice it. I am also going to ask him about the shape of her skull, because while the front part is coming forward, it still seems too flat on one side, and I can't imagine how the distractor will address that. Dr. Taylor has mentioned the possibility of putting Leah in a helmet after the second surgery, and as I see her skull move forward but not change shape, I am guessing that we have a good chance of needing that in the end. 

Otherwise, Leah is healing very well. Her incision looks great, and even the site of the distractor is looking good. It continues to bleed, but not nearly as much as it was in the first week post-surgery. We have started to keep hats on Leah all the time, and she is getting quite used to them. It also helps Emma and John deal with the distractor, which they still find difficult to see. We haven't taken Leah out of the house yet because of the substantial risk of infection, but when people come over, the hat helps them as well. And of course, we had her wear a pumpkin costume with a hat for Halloween, to keep trick or treaters who came to our door from thinking we were using the baby as a seriously disturbed practical joke!