The Hospital Bill!

The bill for Leah's surgery arrived in the mail today, and it's a doozy. Before I give the total amount, let me assure everyone that we have insurance, so we are feeling very fortunate that we won't be responsible for most of this. Still, just looking at this number can cause profuse sweating!

The bill breaks down as follows:

Lab work: $2680

Anesthesia: $5140

Critical Care on the day of surgery: $3800

Critical Care the day after surgery: $1940

Plastic Surgery: $37,600

Neurosurgery: $15,600

Inpatient Hospital Stay: $89,300

Total Bill: $157,300!

Of course, let me also say that the services Leah received at CHOP were worth every penny of this, and besides paying the bill, David and I are donating money to CHOP this year so that they can continue to provide those services to other kids whose families may not be able to pay their bill.

Eight Weeks After Surgery

Leah's surgery was eight weeks ago, and we are still seeing progress on her forehead shape even though we have stopped turning the distractor. Dr. Taylor said we may continue to see changes as her brain "moves in" to the space created in her forehead. In the image below, it's especially noticeable if you look at how much of her right cheek is visible in the before photo (on the left) compared with the one we took this morning (on the right).

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!

Home at last

We made it home on Saturday night without any issues. Leah slept most of the way home, and while she was awake, David sat in the back and entertained her so she never got upset enough to move around much. We picked up a few infant hats at Target on the way home, and the hats managed to create a "shield" around the distractor so it didn't get caught on the car seat at all. The hats also manage to hide the distractor pretty effectively, which is a big deal for my other kids. We talked to both of them a bit before we came home, and assured them that the distractor doesn't hurt Leah at all, but did prepare them a little for how strange it looks. Since we have arrived, Emma has seen Leah without a hat on, and while she said it wasn't as bad as she had expected, I did see her wince a few times when she was holding Leah. John, on the other hand, hasn't seen it, and has started checking with us to make sure Leah is wearing a hat before he enters any room. Since Leah doesn't seem to mind the hats at all (even the ones that are still a little bit too big that fall over her eyes periodically) we are keeping them on her as much as possible. Since the risk of infection at the site of the distractor is pretty great, the hats are also serving as an additional barrier, in addition to the Bacitracin and the hydrogen peroxide.

Since coming home, Leah continues to heal. She is starting to sleep well, and only wakes up one or two times per night. We are still giving her doses of Tylenol and oxycodone, but not as much as we were when we first left the hospital. Tonight, we gave her her first bath since the night before her surgery. It was only a quick bath, and since we can't get her distractor wet, we couldn't wash or wet her head at all. Instead, we are using baby shampoo and water on washcloths on her incision two times per day. I noticed today that two of her stitches have popped open, but since they are disolvable stitches, that should happen with some regularity. Her hair is just now starting to grow back in, and while it's definitely in the stubble stage, the popped stitches are about the same length and so they blend in pretty well.

Otherwise, we are starting to regain some normalcy. There is much more laundry to do since she is still bleeding on anything that her head touches, and since she spits out about half of the Tylenol we give her. We had to swap out all of her usual clothes with either pajamas that button up the front, or onesies that snap kimono-style but that require an undergraduate degree in engineering to figure out. She is very tolerant of all the dressing and undressing, though, and in fact her overall mood is quite good. It's remarkable to that exactly one week ago tonight she was just waking up from surgery and was in rough, rough shape.

Heading home

Though Leah is having a bit of a rough morning, we are going to try to make it back to Hamilton today, which is about four hours away. We aren't sure if Leah is having surgery-related pain or is just being a typical four month old, but she seems especially sleepy and irritable, and is having some stomach and intestine issues...most likely from the pain medication and its side effects. We gave her another dose of Tylenol, but haven't used any more of the oxycodone. I know it's a perfectly safe drug to use for pediatric patients (and in fact Dara, a pharmacist, says that most drug-related visits to the ER for pediatric patients are for Tylenol since parents assume it's totally safe because it's sold over the counter) but I am still interested in using as little oxycodone as possible while still managing Leah's pain. Since she seems to be handling the pain well so far, even when we turned the distractor last night,  we feel comfortable scaling back on the medications.

The drive to Hamilton is daunting to me because I don't know how Leah is going to react. She was in the car seat for about 20 minutes when  we left the hospital, but slept the whole time. This time she will undoubtedly be awake for part of the drive. She is usually fine in the car, but part of that is because she has Emma and John back there with her to sing her songs (John has an especially hilarious rendition of "The Wheels on the Bus" that he does in an Elvis Costello style...) and to entertain her. My fear is that, if she gets upset, she will thrash her head around a bit and will snag the distractor on the fabric and plastic of the seat. We may try to put a hat on her, but if the hat bothers her and causes her to shake her head to get it off, that may make things worse. Since we have no choice, though, and have to get home at some point before my other kids run my parents into the ground, we are going to try, though I have a feeling it is going to be a rough afternoon.

Post-surgery fashion

Working the beret...

First night

Leah made it through her first night out of the hospital with no problems. She is still on pain medication (oxycodone and Tylenol) so she is a little groggy. But she is having a fabulous time playing with her cousin's toys. The swelling in her eyes is down dramatically and so she is starting to look more recognizable. The site of the distractor is still bleeding so we are keeping clean  cloth diapers under her at all times. We have managed to snag her distractor a few times because it is almost impossible to avoid. But we are learning to manage it, and the good news is that she still seems unfazed by it.

"Home" from the hospital

We made it out of the hospital and over to David's sister, Dara's, house, which is just about 25 minutes from the hospital. Before we left, Dr. Taylor gave us another lesson in turning the distractor, and had both of us do it from beginning to end so we feel completely comfortable with the responsibility. I'm not sure either of us are going to ever feel completely comfortable with it, because it's incredibly strange to attach a wrench to your child's head and turn a screw, knowing that you are actually moving the bones of her skull apart while you turn. But since we have no option, we want to make sure we understand EXACTLY what we are doing. We have decided to follow the lead of the nurses in the hospital who double-check absolutely everything they do, and we will make sure that both of us are present every time we turn the distractor and that we are verifying that we are turning in the right direction.

I knew going into this that I wasn't going to love having the distractor to deal with, but I underestimated how nervous it would make me. It's basically a hollow metal tube that protrudes from Leah's skin about two inches. It's straight, and is attached to her skull with six sets of screws. Because of where it is placed, it does touch whatever Leah is laying on when her head is straight, and when she turns her head to her right, it digs into whatever she is on if it's a soft material, like a mattress. If she laying on a hard surface, like the floor or in a car seat, the distractor prevents her from continuing to turn her head. When I notice this, I try to move to her left side or move a toy towards her left, so she will turn her head in that direction, but I know that I won't be able to catch her every time she does this. Dr. Taylor has assured us that it won't hurt her, and if she turns her head far enough to the right so that it does hurt her, she will just turn her head the other way. I'm unsure that I buy that completely, and so I'm still worried about it. Every time I look at the distractor, I'm surprised that there's no better design for the device...something with some kind of protective material around it, etc. The reason there is not, though, is that because it is embedded in her skull, there's essentially an open wound on her skin that is reopened every time we turn the device. It does bleed a bit all day, and bleeds a fair bit when we turn it, so we are keeping pre-folded cloth diapers under it at all times. If it were covered, or padded, that material would create a dark, wet environment that would undoubtedly attract bacteria and would quickly get infected. The way that the distractor exits her skin allows us to clean it out with hydrogen perixode and to keep it dry, which minimizes the possibility of infection.

Dr. Taylor did say, though, that we have to be careful not to snag the distractor on something. We also have to make sure that nothing catches on it and pulls it away from her head. We have just started to hold her upright today, and already I have caught it on my shirt, and my mother-in-law caught it on her shirt as well when we got to Dara's house. It's actually pretty easy to catch it on things, and when I am holding Leah upright and she turns her head, it just seems like it's in the way, or right in my face. There have been many, many times today in which I have thought, "Two days down and 28 days to go..."

The other thing that is more difficult about the distractor than I had imagined is that it is very hard to look at. Walking out of the hospital carrying Leah was actually a very difficult thing to do today. I know that her appearance is pretty startling, but it's hard to see the shock in people's eyes when they first see her. She does look like a child who is undergoing chemotherapy because of her shaved head AND who has undergone brain surgery because of her swelling and zig-zag shaped scar. On top of that, the distractor resembles a nail coming out of her head. I understand that it can be a lot to take in, and I feel bad when I see people notice her and look away quickly. On our walk out of the hospital, I noticed that children tend to look at her for a long time, while adults will take very quick glances, but will keep looking away to be polite. I also know that when we take Leah out in public this will be even harder, because today we were in a hospital where almost all of other people around us were dealing with, and accustomed to seeing, children with medical conditions of their own. In public, that won't be the case.

On the other hand, I felt very lucky to be walking out of CHOP today, since I know that there are so many children there who will not be leaving anytime soon. Leah's condition can be corrected, while many of theirs can only be managed. Some of their stories are just incredibly hard, and the things they are facing are infinitely more difficult that what we have faced in the last few days. When we moved onto the general surgical floor yesterday, we were moved into a shared room. At CHOP, the hospital "rules" for a shared room are different than they are for private rooms, and while a parent can stay overnight in a shared room, they are not supposed to use the room's bathroom. The room to which we were moved was also occupied by a boy who looked to be about ten who clearly had craniofacial anomalies and severe developmental delays. He was playing in a hospital crib with a toy that was meant for a toddler. I think he was in the hospital for the placement of a tracheal tube, but he clearly had other medical conditions as well. When we arrived, he was being attended by a nurse. I asked her if I could use the bathroom to express breastmilk, and she told me to go ahead. "There's no parent for this patient," she said, which seemed to me both shocking and incredibly sad at the same time. I knew the little boy's name was Calep from the sign on the door (like Caleb, but with a "p" at the end) and that's such an unusual name that I couldn't stop thinking about who named him, what happened to that person, and why he was here alone. As we put Leah's things in the room and put her in the crib, and as all four of us (me, David, his sister and his mom) leaned over her and sang to her and exclaimed about what a great baby she was and how much better she was going to feel soon, I couldn't help but think about Calep and how he had no one standing over him. It really made me wonder why the world is arranged the way it is, and why some kids have so much while others have so little.

Discharged

We are leaving the hospital! Nervous about giving up the resource of the surgeons and nurses, but looking forward to getting Leah unhooked, cleaned up, and out of this hospital room!

Two eyes opened...

...and the only IV left (which she still hates with the fiery passion of a thousand burning suns...)

Nurses' favorite!

Mastering the bottle, and working the dimples!

Much improved!

The good news is that Leah is doing very well today. She we moved out of intensive care and on to a general surgical floor. While we miss the fabulous nurses on the seventh floor, we like the progress the move signifies. Her heart monitor was removed, which is another good sign, and they are now just taking her vitals every four hours. Best of all, her swelling is down, and her left eye is especially looking great.

More good news: David convinced me to leave the hospital last night and sleep at Dara's house while he stayed here in an armchair with Leah. As a result I got almost eight hours of solid sleep, so an feeling like a completely new person. This is all much easier to face when one has a fabulous husband!

Today we are hoping to talk in depth with Dr. Taylor about how we are going to manage care of Leah's distractor and how we can help her to manage it.

A little bit of daylight

Leah can finally open her left eye a little bit. This afternoon she has been awake and looking at us out of the opened eye, and even playing a bit. One of the nurses gave her a bath because she is still covered in dried blood and surgical tape residue. She is still uncomfortable from all the swelling, so she didn't love the bath. But once she was cleaned up we held her for a while and she fell asleep again. The nurse took out the arterial line going into her neck, so that makes it much easier for us to hold her. Still, we are looking forward to the swelling going down some more in the next days.

Distractor Turning

A gentle heads-up: the text and photos in this post may not be appropriate for the squeamish...

Dr. Taylor came by our room this morning after the bandages were off to turn Leah's distractor for the first time, and to teach us how to do it. He explained that the device, which is like a wrench, will attach to the end of the distractor like a sleeve. The distractor and the wrench are hexagon shaped on the inside, so they have to be fitted together. Once they are fitted together, we turn one full turn in the direction in which the arrow printed on the wrench is pointing. This is obviously very important, since if we turn the other direction, we will reverse any progress, and this entire surgery will have to be repeated. Not a good scenario...

Each time we turn the wrench, it will expand the distractor by .5mm. Since we will turn it twice per day, we are expanding the space in her skull by 1mm per day, and since we are looking for a total expansion of about 20-30 mm, this will be in place for about 20-30 days. At that time, we will come back to Philadelphia to have the external part of the distractor removed, while the internal part will remain in place until Leah's second surgery, which will probably happen sometime in January.

All of the doctors who have seen her incision say it looks great, and that she is doing better than expected. She continues to drink bottles well, and has wet diapers, so we know her usual systems are working. The nurses took out her arterial line this morning as well, which she did not love. It bled pretty substantially, but they managed to clean her up a bit and give her some more morphine, so she is now sleeping comfortably.

Some incision and distractor photos are below:

Bandages off

The plastic surgery team was in this morning to check on Leah. She had been awake and upset for a good while before they came in, and I had just managed to calm her down, so I was a little dismayed that they decided to remove the bandages at that exact time. But they pressed on and cut away the bonnet, then started to unwrap. There was a lot of gauze, then several layers of antibiotic padding which they took off a little more carefully because it was up against the incision. They checked her incision with a flashlight and said it all looks great. Then they stepped back and let me have a look.

I was seeing quite a bit all at once, so it was hard to take: her shaved head, more swelling of her whole head and neck, the ear-to-ear incision, and the distractor. Frankly, it is awful. I was trying to prepare myself  for it, but I have apparently failed. It will take me a few hours to become comfortable with it before I can post photos.

Awake and playing

She may not be able to see but she can still play!

Modest progress

Leah is taking a bottle from David now, which is a good sign since she hasn't had much to eat since the surgery. At this point, she has had about 5 ounces of breastmilk, which is quite a bit. There's a high possibility that she is going to projectile vomit most of it back at us, but since there's a small chance she will keep most of it down, we are proceeding. Mostly, it's a good sign that she is able to get some liquids besides what is going in through the IV. The nurses have scaled back the amount going into the IV as a result, but if she changes course overnight and stops taking bottles, they will switch it back to its original amount. 

At this point, her eyes are completely swollen shut, but she doesn't seem to be too bothered by it since she's still only semi-conscious. We can see her eyes moving behind the lids a bit when she starts to get fussy, so it must be confusing to her not to be able to see. The nurses who have worked with "cranio" babies in the past say that they will start to tip their heads back because it allows them to see out of the inside corners of their eyes once the swelling starts to recede even a little bit. She said we should expect to see that tomorrow or Thursday.

Dr. Taylor was in to see Leah this morning, and said everything looked great. It's strange for someone to look at her in this state and say that she looks perfect, of course, but since he's the expert, we are going to trust him! We have also had an amazing nurse today, Peggy, who has really been great about getting me some extra help and information about how to nurse Leah with all those IV lines and monitor wires coming out of every limb. She has also been really great about advocating for us. This morning when a host of doctors and med students came by to do "rounds" outside the door, she grabbed me and took me out there too, introducing me to the doctors as "Mom" and then nodded her head so they would proceed with rounds. They seemed a little surprised, but went on and launched into a whole laundry list of points to discuss about Leah's case. I didn't understand all of it since some of it is in medicalese (the point of which I still don't understand--if edema means swelling, why not just say swelling?...), but overall it was really informative. This is a teaching hospital, so the attending was asking questions of the group like, "Given her increased sodium and potassium levels, what might be a natural concern?" and using words like "extradural exposure" which made about 75% sense to me. But it did help me to see how the doctors negotiate, and agree upon, a plan of action for Leah for the day, and now I can be watching for things on that plan to make sure they are carried out. It has made this day much easier for me in the end.

I have also learned that, with the constant changing of the nurse and doctor shifts in the hospital, I can stay on top of information by always answering the question, "Did they explain to you..." with a "no." Even if the last doctor or nurse did tell me in great detail what was going to happen when, for what reason, it's highly likely that the doctor or nurse coming on will either have a different understanding of that who-what-when, or will add some information that will be helpful for me to know in the end. It does sometimes make me forget which person told me which bit of information, but it's a worthwhile tradeoff.

Still swelling

They weren't kidding when they said her eyes would swell...

Swelling

Leah's swelling is getting worse as the morning goes on. For some reason the left side is worse than the right, which is the side where the distractor was placed and more of the tissue, skull and dura was exposed. The nurses guess it's because we have her head turned to the side and the fluid is draining to the left. Her heart rate is also up but that's most likely from the pain, so they have given her more morphine in the hopes that will slow it down.

When I was nursing her she passed some gas, but it didn't smell like a dirty diaper. I figured there was no way she was dirty so soon after surgery, but I stuck my finger in the side of her diaper just to check. What a rookie mistake...!

Overnight

We made it through her first night after surgery, and she continued to sleep well. She woke up about every two hours and cried a bit, but it seems like she is crying from discomfort more than real pain. They stopped giving her morphine yesterday evening, but plan to give her another dose this morning after the surgeons are in to see her.

I was also able to nurse her early this morning, and while she didn't take a lot, the little she did take made me very happy, since it has been over 24 hours since she had any milk at all. She is getting IV fluids, but her mouth looks really dry when she cries, and combined with her very hoarse and raw-sounding voice from the breathing tube, she seemed to be in rough shape. Now we just have to watch that she doesn't throw up, which is pretty common after anesthesia.

Our room is full of all kinds of high tech pieces of equipment, but my favorite is a big flatscreen over the nurses' desk that shows all of her numbers at the same time...her pulse and blood oxygen, but also all kinds of lab results which are constantly being updated. She has a central line in her neck, so I suppose that something to which she is hooked is taking readings of her blood. That means she doesn't have to be stuck with a needle anytime they need information, which is a relief. On the other hand, about half of the numbers are written in red, meaning they are outside the normal range. When I asked about this, they reassure me that they aren't worried at all since she just went through major surgery and it is going to be a while before her body adjusts. Still, I am hoping that by getting some milk in her, the numbers will go back to white today.

We are still in the intensive care unit of the hospital (where there is absolutely no difference between night and day, I noticed) but I am hoping that when the surgeons come to see her this morning they decide she is stable enough to be moved to a regular room. Today we are also going to be watching her swelling, which has gotten much worse overnight. Unfortunately there is nothing they can do about it other than to give it time and keep her comfortable.

All is still well

Leah is still getting morphine so she is still very quiet and relaxed. She is sleeping a lot, but when she is awake her eyes are open and looking around. She even managed a weak smile for one of the nurses. They remove her catheter, and having even one fewer line running into her tiny body makes us feel better.

Leah noticed the IV line going into her arm, which is held on by a stiff board, and she isn't thrilled with it. I know that when she feels well enough to grab her toes and finds another board and IV there, she's going to be really upset. Without being able to suck on her toes, I don't know what she will do.

For now, we are just standing by her crib, talking and singing to her when she opens her eyes, and holding her hand when he eyes are closed. We know the swelling in her head and around her eyes will get worse, and once the morphine wars off she will start to feel much more uncomfortable, but for now, we are happy that she is calm and stable. More than anything, we are very thankful that the day has gone as well as it did.

Awake

Leah is slowly coming out of anesthesia. She is alternating between crying, and laying very still and looking around with a bit of a dazed look. She took her "bobby" for the first time, which seems to be a good sign. And we are playing some of her favorite songs on David's phone, which is keeping her calm. The nurses are still administering morphine to control her pain, but because she is awake but not inconsolable, we feel like she is handling this better than we had anticipated. She still seems very sleepy, though, so as that wears off, her response may change. So far, though, so good.

Starting to wake up

Leah is still very groggy, and when she cries her voice is very hoarse from the breathing tube. But she's hanging in there...

First pictures

Still asleep...

Distractor benefits

We are learning that there are some added benefits to having chosen the distractor surgery as the day goes on. We anticipated that they would install a drain in Leah's head to deal with the excess fluid, and that the drain is pretty noticeable and uncomfortable for babies. Since the distractor placement requires less time in surgery and can be considered less invasive than cranial vault reconstruction, the surgeons don't use a drain during distraction surgery. We were relieved to hear that, as it was part of the recovery we were most dreading. As a result she is leaking some blood and fluid from the back of her head since the drain isn't taking it away, and it's causing a bloodstain on the sheet behind her head. The nurses asked the plastic surgeons and neurosurgeons to come up and look at her bandages because of the bleeding, but they did and said it was of no concern at all.

We know that many children with coronal craniosynostosis have related issues with their optic nerve and eye muscles, especially on the side of the fused suture. While Leah was given a clean bill of health from the eye doctor last week, we will need to monitor her closely in the coming months for eye issues as the space around her eye changes and the pressure levels in her skull change along with it. When he was meeting with us after the surgery, Dr. Taylor said that he is working with Leah's ophthalmologist, Dr. Forbes, to study "cranio" kids with distractors to see if they have less need for later eye surgeries. While the usual rate of additional surgery is about 50%, at this point they have had NO distractor patients who have needed eye surgery at a later point. It's obviously very early to tell if that week be the case long-term, as they have been doing this surgery for only a few years, but he did say it's another reason why they are increasingly turning to distractors to treat coronal craniosynostosis.

All is well

Dr. Taylor came to talk to us, and said everything went very well...he said it was 100% as it should be. The bone was solid and in good shape, which means there was good surface on which the distractor can be affixed. In his opinion, her blood loss was minimal for this kind of surgery, and the anesthesiologists gave her 150 ml during the transfusion.

They are finishing surgery now, taking out her breathing tube, and will begin to wake her up. Her head is going to be bandaged, and that bandage will stay on until Wednesday.

We are now in the NICU, in another small waiting room, and may be here up to an hour while the surgeons finish and bring her up.

Finishing surgery

We just moved to a new, private waiting room because they are finishing the surgery. Dr. Taylor will come to talk to us, then we will go to the NICU. They will remove Leah's breathing tube and when she is breathing on her own completely, they will take her up to the NICU as well.

Transfusion

The doctors told us they would start a blood transfusion for Leah when the neurosurgeon started to work at the front of her skull. The head is full of blood-rich vessels, so they told us to expect a substantial amount of bleeding. Dr. Taylor told us it's likely that Leah will lose her entire volume of blood during the surgery. To keep her heart, lungs and kidneys from knowing that her body is undergoing surgery they start to give her blood right away. Because neither of us match Leah's blood type and because they prefer to use whole blood, the blood is coming from the Red Cross blood bank (which makes me thankful that I have been a blood donor for years...).

Neurosurgery

Our nurse just came out again with another update. The anesthesia team has finished and is stepping back to let the other surgeons start their work. Dr. Taylor goes first and makes the incision, which goes across her head from one ear to the other. He did that part at 9:35 a.m., and now the neurosurgeon, Dr. Heuer, is taking over.

When Dr. Taylor spoke with us earlier this morning, he asked if we wanted him to shave a strip of her hair where the incision will be, or if we prefer him to shave her whole head. We opted for the second, which seems like it will be easier to care for, and will mean her hair won't get caught in her distractor when it is turned. Dr. Taylor said that he supposed this was her first haircut so he would save her hair for us, which I appreciated very much. The last thing he said to us was that, when he got into the operating room, he would treat her as if she were his own daughter, which was incredibly comforting.

Anesthesia update

Our nurse just came out with an update: Leah's central line and arterial line are now in, and anesthesia is still doing their part. The plastic surgery team is in the room with her but they haven't started their work.

Just before surgery...

One more picture of Leah, taken right before the nurses took her into the operating room...

In surgery

We arrived at 6:00 am at the hospital, and everything is fine so far. We changed her into a gown and played with her while a series of doctors and nurses explained to us what will happen. She seemed pretty interested in everything that was going on around her, and when she finally lost interest she fell asleep. David had to hand her over to the nurses because I would not have been able to do it...

They took her into surgery at 7:30, and at 8:00 am our nurse came out and said Leah was already asleep and her breathing tube was in, and they we going to start her central line but she wouldn't feel a thing.

Pre-Op Appointments

We are just back from a day at the hospital to go through the pre-op checklist with anesthesia. The hospital sent us a booklet a few weeks ago with instructions about what to feed Leah and when (and what not to feed her and when) on the day of surgery, and most of our appointments today were a reiteration of those instructions. We started in a small exam room where they took Leah's vitals, which were all good. Then we saw a surgical nurse who explained how the day would go: we will arrive at our appointment time (which we had not yet been assigned), park our car and enter the hospital in the surgical waiting area. A nurse will bring us to a small exam room where we will change her into a hospital gown and wait to speak with anesthesia again to review whether we had followed the pre-op instructions (apparently, if we have not, the surgery is cancelled, so it's a big deal). Our two surgeons will come into the room to review the procedures with us at that point. Our surgeons are Dr. Taylor, a pediatric plastic surgeon, and Dr. Heuer, a pediatric neurosurgeon.Then a nurse will take Leah from us, and they will give her general anesthesia through a mask. The nurse said that takes usually only a few seconds, and after she is asleep they will place her IV and finish surgical preparations.

The nurse said the surgeons had reserved the operating room for 4 hours, but she said that they always underestimate how long surgery will take because they only count the time they will need, rather than the pre-op and anesthesia time, so we are preparing for a bit more. A nurse will come to our waiting room every hour to provide us with updates, and when the surgery is over, our surgeons will come out to review the procedure with us again and let us know what to expect. Leah will go right to the pediatric intensive care unit, where she will stay for about 24 hours if all goes well. We will meet her there, and one of  us will be able to stay in the PICU with her overnight (though they warned us that we shouldn't expect to get any sleep while there).

Because we have done a good deal of research, we know to prepare for substantial swelling, especially around Leah's eyes. We also know that the swelling will get worse over the next 48 hours, as excess fluid around her skull tries to drain. The surgeons will install a drain, but it won't be able to clear all the fluid, so her eyes will likely swell shut. That's going to be hard for her, because she won't understand why she can't see. We are preparing for that by taking a few of her softest stuffed animals and blankets (many of whom have been given to us by wonderful friends in the last days) and sleeping with them now so they will smell like us when she holds them in the hospital. I'm not sure how comforting that will be, given the amount of pain she is going to be in, but at this point we are willing to try whatever we can to make her feel more comfortable.

The nurses are hesitant to give us much information about what will happen after that first 24-48 hours because it depends so much on how the surgery goes and on how Leah responds to it. We expect to be in the main part of the hospital after the first 24 hours, though, and to stay there for an additional three to five days. Some of that is dependent on how quickly the swelling around her skull goes down, which will allow them to remove her drain, and some of it depends on whether or not she shows signs of infection and needs to be given antibiotics.

We also met with an anesthesiologist who wants to enter Leah into a clinical trial for FDA approval of IV Tylenol in babies younger than 6 months of age. She explained that usually babies are administered morphine when they indicate they are in pain after surgery, but in the clinical trial they would be administered Tylenol instead. I had to ask how they determine if babies are in pain at this age, and she explained their 10 point scale that assesses facial expression, vocalization, arm and leg movement, etc. She assured us that with or without the clinical trial, Leah would be given pain medication sufficient to alleviate any of her pain, so we opted to participate in the trial since it seems not to have any ill effects, and would only mean that she would possibly have less exposure to morphine.

Finally, we went to the lab to have Leah's blood drawn for type during the transfusions. We know she is A-, but the hospital obviously can't take our word for it, so they collect samples. They will use the same samples to do the genetic screening as well, so she only had one pin prick. Only one parent was allowed in the lab, and David volunteered, but I heard every scream from the waiting room, so I know she wasn't a big fan of the process.

We were then instructed to call the hospital between 3:30 pm and 6:00 pm to be assigned an arrival time, and to be instructed AGAIN about the feeding instructions. The nurse over the phone told us to arrive at 6:00 am on Monday, so we are going to take advantage of this weekend to get some extra rest.

Opthalmology and Genetics

Leah had two pre-op appointments today at CHOP. Since we are driving quite a distance to the hospital, they have been very understanding about bunching appointments together for us. We arrived at the hospital at 12:30 and went right to opthalmology. This appointment was the most important of the day because kids with coronal craniosynostosis often have related eye issues, either because of the  facial asymetry that it causes or because of increased pressure on the optic nerve. The doctor spent just few minutes checking the focus of her pupils (he used his iPhone to play a video through a PBS app to keep her attention) then dilated her pupils to check her optic nerve. Leah didn't really love the process, but the doctor said at this point everything looks fine, and he sent us on our way with directions to come back six weeks after the surgery to check her pressure.

We then went to the Plastic Surgery department, where we usually go to see Dr. Taylor. Leah discovered a Geronimo Stilton book in the waiting room that struck her fancy:

 

This appointment was quick as well, and the geneticist asked us several questions about our family history. Since no one in either of our families has had craniosynostosis, or any related craniofacial, hand or foot abnormalities, she also said that everything looks fine. She explained to us the causes of craniosynostosis, but we already have a more advanced understanding of the defect than she provided so that wasn't terribly helpful. I noticed she spent a lot of time looking at Leah's hands, and since Leah does have notably long fingers, that made me nervous. But at the end, she said she didn't see anything for concern. Since we will have blood drawn at Leah's pre-op appointment with anesthesiology tomorrow, she ordered specific genetic screening of the fourth and seventh chromosome on her blood just to make sure Leah doesn't have any cranio-related abnormalities. The results will take about a month to get to us, but we anticipate that they will show that everything is normal, which is very good news.

Two Weeks to go

Two weeks until the surgery. Leah is trying on hats to prepare for that distractor...

CT Scan

We took Leah to CHOP yesterday for a CT scan. We expected the appointment to take several hours but because Leah fell asleep in the waiting room, they took us right in, put her in the scanner and turned it on, and she was done in about 20 minutes. The scan confirms the unilateral fusion on Leah’s right side. It also shows that there are several “dimples” at the back of Leah’s skull, which are caused by increased intracranial pressure (ICP). Dr. Taylor said that these mean her brain is trying to grow but the skull isn’t expanding to allow for that growth. That image makes us thankful that we chose to have the surgery performed earlier. Because we see Leah every day, we don’t notice the asymmetry of her face, and so are surprised at how asymmetrical her facial structure is on the scan. The scan also shows that her soft spot is pulled far to the left, and is also asymetrical.

While we were waiting to see Dr. Taylor that afternoon, I saw a baby boy in the waiting room as he was leaving who looked like he had recent surgery. His head was shaved, and he had a z-shaped scar from ear to ear across the top of his skull. He also had what looked like a very long tube implanted in his skull, on the inside of the skin. Before I saw this child, I assumed that this distraction surgery used springs, so I was surprised to see such a visible device. When we met with Dr. Taylor, I asked if he could show me a picture of the distractor. He pulled up a CT scan of a child who had just had the surgery. The distractor wasn't at all what I had imagined: it was the size of a pencil and it was screwed into the bone on either side of a cut that was made in the skull along the suture line. A second screw sticks out of the skin at the back of the head.

When Leah has the distractor installed, we will attach a kind of Allen wrench to that screw and turn it two times per day, which will spread the two parts of the bone 1 mm apart each day. The bones need to be separated by 20-30 mm in all, so we will turn the screw for about three or four weeks, then will return to have the external screw taken out. The rest of the distractor (the parts that are screwed to her skull) will stay in for two more months, to give the new bone that is formed time to harden. A second surgery will be done in late January to remove the entire device.

Had we seen that baby while we were choosing the type of surgery, we may not have chosen the distraction osteogenesis because it seems quite drastic, and frankly terrible. On the other hand, after seeing the CT scan, we may not have been comfortable with waiting past October 21 to have the surgery.