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Ahhh… there are not enough hours in the day! I have been meaning to write ever since the surgery… thank you to Meg for reminding me during a time when I have a few minutes!

Violet did GREAT during her surgery! Here is an update in pictures:

She was the happiest girl in the world just before getting wheeled off to the operating room:

Her surgery lasted SEVEN hours! The surgeons were oh-so-careful with her because of the bone disease and the fact that kids with OI tend to bleed more, so they certainly took their time! Here she was just after surgery, in the PICU:

She had IVs and tubes everywhere… IVs on every limb, a Broviak catheter (also known as a central line) in her groin, a catheter, blood pressure cuff, monitors for her vitals, etc. She was hysterical because she was disoriented from anesthesia and because her pain meds hadn’t caught up to her pain level yet. The next few hours were among the worst of my life.

The day after was the worst day of swelling:

Her left eye swelled shut and she started to bruise. However, her spirits improved dramatically when her catheter was removed and we could pick her up. Within a couple of hours, she was clapping and smiling again. It was incredible.

Two days after surgery, her bandages came off. Check out this major incision:

We estimate that she has 60+ stitches in her head. They are dissolvable, thankfully! It sure wouldn’t be fun to hold her as all those stitches were removed!

Later that day, Violet was in a great mood and really showed us that she is an incredibly tough little girl!

Her surgery was on Thursday morning (Aug. 9), and she was released on Monday, August 13th–their 11-month birthday! We were so glad to come home, and she has been doing so great with recovery ever since. It is amazing to see how quickly she is healing.

She was fitted for a helmet while in the hospital and received one a few days later. Her surgeon felt that because the bone disease causes bone to be lesser quality than normal, she should wear it when out of our care for protective reasons. So if she goes to daycare, she wears it, or if she is being particularly rambunctious. We don’t want her to hit her head and break one of those healing bones or damage a plate or screw that is holding everything together.

The weekend after Violet came home, we dressed them up to attend a family function. We usually don’t dress them alike, but these dresses were a gift from my mom and were too cute!

Layla’s on the left, and Violet’s on the right.

Both babies are starting to enjoy some solid food, finally! They are very picky about flavors and textures, and more often than not refuse what we feed them. I started out feeding them only made-at-home purees, but found that all they were eating consistently were bananas and sweet potatoes. We branched out and started in with the Gerber products–Puffs, Cheeto-like snacks, yogurt melts. They do like those, of course, because they are pretty much junk. They liked Cheerios briefly but now spit them out–must be too bland. They’ve refused all of our table food for a few months, but are finally starting to turn around. They like pizza and macaroni and cheese, so far!

Certainly not nutritious, but we are just happy that they are eating anything off our plates! We’ve offered them healthier options–blueberries, pieces of grape, corn, etc.–but they have refused it all. Work in progress, I guess.

They are crawling like crazy. Layla still only army-crawls, but she’s still getting wherever she needs to go, so we guess that’s okay. Violet crawls, and is quick! They are both also standing. Layla pulls up effortlessly on everything and has “cruised” a step or two. Violet can stand, but only if you stand her up. She’s a little behind, which I have read is normal with clubfoot treatment. She is also really scared of falling, and that is something we’re going to work on.

Now we are planning for their first birthday! They turn ONE on September 13th–only 15 days away! I can’t believe it. This has been an incredible year on so many levels. I just look so forward to watching their personalities emerge even more and for them to accomplish milestones and all of their goals. We are so lucky!

Surgery Plan

Violet’s surgery is scheduled for Thursday, August 9th, first thing in the morning. Upon closer analysis of her CT scan, she was diagnosed with both unilateral coronal craniosynostosis AND sagittal craniosynostosis. This means that the suture on the left side of her head and the suture at the back of her head have fused prematurely. In addition, her right orbital rim (the big bony ridge just behind the eyebrow) sits back 4mm further than the left, so that will be reconstructed during surgery as well. I took her to see the neurosurgeon on the 13th, so that he could look at her head and take some notes before surgery.

Here’s how the operation will work: we’ll arrive at the hospital at 5:30 in the morning, and she’ll be prepped and then go to sleep under anesthesia at 7:30. Then, she’ll receive a central line. This PICC line will be threaded through her groin up to her superior vena cava. Having the PICC allows the staff to give her blood more easily, since she will need blood transfusions during surgery. Next, the craniofacial surgeon will expose the skull and mark the bones that need to be taken off. The neurosurgeon steps in and removes those bones, with all precautions to avoid touching the brain in any way. Then the craniofacial surgeon molds the bone as necessary to reinstate a proper skull shape. The craniofacial surgeon will also be the doctor who reconstructs her orbital rim.

The surgery will last approximately four hours. She’ll be in the PICU for most of her stay, where she can be closely monitored. The scar will be big, but it’s done in a zigzag fashion so as to be less noticeable. Doctors used to cut horizontally from ear to ear, and that was obviously much more noticeable. Her head is going to swell tremendously afterwards–her eyes will swell completely shut, and some babies even get blisters all over their forehead because of the swelling. She’ll wear a little cap on her head afterwards for a few days to protect the skull, but after that she won’t need a helmet or anything. Some doctors choose to employ the use of a helmet for further reshaping, but Violet’s surgeon aims to have her skull shape corrected before she leaves the OR. She’ll be feeling much better within a few weeks, though–it’s just the few days immediately following surgery that are going to be rough for her (and for us).

I am pretty much numb when I think about her surgery, which is pretty much all the time. I believe that’s my body’s way of protecting myself from the panic and anger and sadness that are boiling in my heart. I have no doubt that those four hours that she’s in the operating room are going to be the worst of my life. We’ve already met the nurse liaison who has the duty of relaying information from the surgeons to us, and she was very nice.

The last step before surgery is to attend a craniofacial clinic on August 3rd. It sounds similar to the OI clinic that we attended in April, during which specialists performed some evaluations. Because there is some risk of additional surgeries needed (she may develop soft spots the size of pennies that would require bone to be placed over them), she’ll attend this clinic annually for evaluation until she’s five. At that time, she’ll “graduate.”

Her head has become noticeably more misshapen during the last few weeks. Her brain is growing, and because it doesn’t have enough room, it’s pushing her bones out to compensate. So the left side of her forehead looks very misshapen, and her forehead in general looks asymmetrical.

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See the bulge protruding from the left side?

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The top of her skull looks like a bunny slope. There is a “valley” in the middle of her head that is becoming increasingly more prominent.

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We’ve noticed over the last few weeks that her neck appears to be very stiff, so that is a new concern. I actually took her to the ER last Tuesday night for neck and spine x-rays, because I was worried about a compression fracture. The x-rays were negative, but something is going on. Sometimes craniosynostosis causes vision problems, and babies can develop torticollis because they are trying to overcompensate for their lack of vision. However, I had thought this usually happened with very young babies, so I’m not sure. She doesn’t seem to be able to turn her head completely to the right, and she gets VERY angry when I try to stretch her neck in that direction. She also can’t seem to look down at me when I lift her up in the air (she looks straight ahead instead), and I feel that she arches her back when I lift her and that she hunches her shoulders when she tries to turn her head to the right. I addressed all of these concerns with the ER doctor, who didn’t seem terribly concerned. But I think this is one of those circumstances where it takes her mama to know that something is wrong. I have a call in to the craniofacial surgeon’s nurse to inquire about whether this is common for a 10-month-old with craniosynostosis, so hopefully I’ll hear from her tomorrow. The babies are scheduled for physical therapy on Thursday, but I’m going to see if I can move it up so that the PT can evaluate Violet. We certainly want to be proactive so the problem doesn’t worsen.

My next post will contain some milestone updates (they’re crawling!) and pictures of something other than Violet’s head. : )

Diagnosis

Violet was diagnosed yesterday with unilateral coronal craniosynostosis. A CT scan showed that the bones on the left side of her skull have fused prematurely. Left untreated, her brain won’t have room to grow, and developmental issues will occur. Surgery is the next step and will occur in August. A cranio-facial surgeon and neurosurgeon will separate the fused bones and remove a chunk of skull to give her brain room to continue growing. She’ll spend 3-5 days in the hospital. Obviously we are very upset and reeling from this news. It’s unbearable to think about sending my child off to an operating room to undergo a complex surgery. We have read that her head will swell tremendously and that the 48 hours after surgery can be difficult. I am just devastated.