What is going on in the wonderful world of Sarah?
Sarah's surgery in early May went well, and has mostly healed. She had follow-up visits with both surgeons involved in the surgery.
The bone that they had to work with was "paper thin." That made the reconstruction work difficult. It is thin because it the bone has been pressed from the inside. I discussed it a bit when she had her first surgery in September. Normally the plates in your skull are free floating at first. The brain grows, the plates come apart, the bone grows and around again until you are done growing. If the plates fuse to early, the brain still grows, and it grows where it can. The growth can cause dangerous pressure concerns. The pressure pushes the bone which sort of molds around the brain. The bones can be thinner than normal and not as smooth.
So, that is what the surgeons are up against. They have to make sure that Sarah's brain is out of danger. They want to minimize surgeries. They want growth to be as normal as possible, both cosmetically and developmentally.
This surgery was a big one. And it seems to have gone very well. About a week after the surgery we noticed something pointy pressing from underneath the skin on the top of her head. We had already scheduled our follow up visits, and we were glad. We showed both surgeons and neither seemed particularly worried that one (or a few) of the screws would have come loose.
Side note: Before I was a mom I was a preschool teacher. Once, a four year old boy climbed into my lap with a tool box and a look of consternation. He picked through his tools very carefully, and chose a bright orange screwdriver.
"You have a few loose screws," and he set to work fixing them, just above my temple. Maybe loose screws are genetic.
In any event, we were told to be sure to call if the screws actually poked through the skin, in which case it would be a simple matter to remove them. Otherwise, we were not to worry.
In the follow-up visits, we also discussed the next step for Sarah. Both surgeons have worked on many children with Sarah's syndrome, and other craniofacial issues as well. Sarah's case is, apparently, unique and difficult. One suspects that all cases are unique and difficult.
The next step for Sarah is not typical for children with Apert syndrome. The next step is a helmet.
The helmet is custom made. You have seen the kind of helmet she will get. When children sleep only on one side and they develop flat spots, this is the helmet that is used to help correct the problem. The helmet does not push and shove the head into shape, as say braces on teeth work. Instead, it is molded very precisely to the shape of the head. It is form fitting, without pressure. Then they carve out the interior of the helmet where they want the bones to grow. The head grows into the helmet. Then they carve out more. The idea is to train the growth of the bone, not to remold what has already grown. So it only works when the kids are babies, because that is when the most significant growth happens.
For Sarah, there is hope that this helmet will help to reduce the necessity of future surgeries. Also, if it works well, it will help her head to grow in a more normal, round shape. So, win-win. Cosmetically useful, medically a good choice.
There is risk though. And I think this is why it is not typical for kids with Apert syndrome. These kids have larger than average brains and typically more fluid in the brain. Pressure is already a concern. Add a helmet, and you can add pressure. That is obviously not insignificant. Pressure can damage her brain, as it did before she was born.
Furthermore, the actual molding is more art than science. This might be true for most of Sarah's interventions.
For the next few months, after she gets her helmet, we will follow-up once a month with the orthopedist who is making and reshaping the helmet. We will follow-up once a month with the plastic surgeon, who wants to keep a very close eye on the work, because it is atypical, and typical is already not simple. We will follow up once a month, with a CT and a visit with he neurosurgeon to make sure we are monitoring intracranial (in the head) pressure very closely. We will watch closely at home for any neurological symptoms. It is a little intimidating, the number of doctor visits that will be required over the next few months.
We are excited, and confident that it is the right thing for Sarah.