In the winter of 1988, I was born with Pfeiffer Syndrome,
a condition that is part of the
family of craniofacial syndromes which includes Apert Syndrome and Crouzon
Syndrome. These syndromes are characterized by premature fusing of the
skull bones, which causes severe breathing issues, vision/hearing issues, and
cosmetic differences that make constant staring, teasing, and rude questions
inevitable. The rare nature of the syndromes (approximately 1 in 100,000)
makes information in the medical community inconsistent. One doctor told
my parents that I was severely mentally retarded. However, in the 22 years
since I was born, information sharing has improved immensely and there are now
more resources available online for doctors and families (see the
Links page for some examples!).
The hospital I was born in didn’t think any surgery was necessary and refused to pay for anything, and of course, if the hospital says something’s unnecessary, who’s going to question it? In what I can only credit to God’s amazing divine providence, one of my mom’s cousins was a medical student at the nearby UCSF medical school at the time. She had just done a stint under Dr. Michael Edwards, one of the best pediatric neurosurgeons in the country. His schedule was completely overbooked, but she was able to convince him to meet me and my parents during one of his lunch breaks. He said I definitely wasn’t mentally retarded, but I definitely needed surgery right away.
The hospital and the insurance company continued to refuse to pay for anything, but money took a back seat to my health and I had the sagittal synostectomy. According to Dr. Edwards, when he made the cut and opened up my skull, my brain just expanded as if it were a sponge that had been bunched up together until then. I shudder to think what I would be like mentally today had I not had that surgery when I did.
The hospital bill without help from insurance was enormous. Donations flooded in from relatives and our church; they even had a garage sale for me. After lawsuits were threatened and angry letters were sent from my surgeons to the lawyers, the insurance company finally settled and paid for the surgery. It was, of course, only the first of many many surgeries to come. But when I look back on my life, and as I continue to live, I'm always amazed at how things always work out – it’s like every medical episode became an opportunity to witness God’s miracles.
When I turned six, my family moved from the bustling Bay Area to a small town in Oregon. As much as I love the Bay, I am so grateful to have grown up in a small town where virtually everyone knew about my condition. Though I still got teased and had to put up the unrelenting stares of strangers in public places, I was incredibly blessed to always have friends who were protective of me. Whenever I had surgery, they would send me cards and emails; one of my middle school teachers even stopped her class to hold a few minutes of silence to “send good energy” to me during particularly major surgery. I’m still amazed to realize how much support I always had.
My parents always supported me too, whether that meant taking big chunks of time off work to be with me after surgeries and medical appointments, or listening to me cry and whine about how much I hated the kids who made fun of me. I know a lot of you on this site are parents and I have to say, major kudos to you for all that you do. Kudos to siblings too; my younger sisters have always been patient when they’ve had to stay with relatives when both our parents needed to fly out of state with me for surgery or when they are asked nosy questions about why their sister looks so weird.
Anyways, I had a lot of surgeries growing up (20 and counting!), but I won’t bore you with my entire medical history. Instead, I'll jump to the year 2001 when I was 13 and went through the RED procedure, which pretty much all craniofacial kids have to go through at some point, which is the focus of this website.
At the time, the idea of distraction -- that is, separating the midface from the rest of the skull and moving it forward gradually with a complex system of hardware installed during the surgery, which allowed for new bone to grow into the gaps gradually as the midface was advanced -- was relatively new. Many craniofacial surgeons were in transition from using the traditional LaFort II procedure, which involved separating the midface and advancing it all at once, using bone samples from another area of the body to hold it in place. The all-in-one procedure allowed for much less control and carried more risks for complications later in life.
There are two versions of distraction that are used: internal and external (also known as Rigid External Distraction, or RED). My surgeon felt that I would do well with the internal distractors, so in May of 2001, I went into surgery and began the distraction process. After recovering from the initial surgery, we noticed that nothing was happening, despite the fact that the screws were being turned regularly. One of the screws had "slipped out" internally because it had not been stitched in securely. I had another surgery to fix what had happened. However, after recovering from that surgery, it became apparent that my skull had already healed itself and my midface was no longer isolated. So, a week later, it was decided that I needed RED. I went in for my third surgery that month to have my midface re-separated and have the RED put on.
For the next month and a half, I went through the external distraction process and began seeing results almost immediately. I could breathe through my nose for the first time in my life and my eyes finally had some protection. The results proved that the entire ordeal had been worth it. It would be lying to say that the entire process was simple and easy in any way, but, then again, that's why this website was created.It's been about nine years since I went through RED, and since then, I've had several more surgeries, and still get the occasional stare, but I'm finally living a pretty normal life. I'm a student at USC on a Presidential Scholarship double-majoring in cinema production and political science.
Although this RED stuff is all behind me now, I still remember how incredibly unfun all of this medical stuff can be, so I hope that you can trust that I understand what you are going through. Please email anytime! I love hearing from you guys!
©2010 Lia Burton. All rights reserved.