My Craniofacial Reconstruction Journey

 

Sitting in a large conference room, we are surrounded by what feels like two dozen men and women in crisp, white coats that stand out against the dark paneling on the walls. It is standing room only as more and more of them pile in. There are three chairs in the center of one wall for Mom, Dad and me. Everyone else is crowded around us. They all want a view of the unusual specimen. They all want a view of me. Dr Mulikin is right in front of me, a bit too close for comfort. He has me remove my sneakers and socks and is examining my feet. I don’t really understand why. Isn’t it my face that needs to be fixed? It tickles as he examines each of my toes. He asks me to count them in Korean. He loves that I can count in Korean and has me do it every time. I’m just happy that I only have ten toes, because I don’t know how to count any higher.

  As he continues to examine me, he turns to the people behind him explaining and pointing out things in some kind of medical jargon. The only words I can make out are “see this?” and “see how her face is blah blah blah.” I just want to get out of there. I look over to Mom. Her eyes are half closed, but she is trying to look like she knows what they are talking about. It has been a long day of appointments and tests. I can’t imagine that entertaining an eight-year-old, who has spent the day being poked and prodded, is any walk in the park either. To my left,  Dad is wide-eyed on the edge of his seat. I’m not sure if it is his daughter being studied as some kind of rare animal, or Doctor Padwa’s short, tight skirt under her impressive looking lab coat that is pleasing him more.

****

 As years pass, my parents and I continue to make our annual pilgrimage to Boston Children’s Hospital. Once we stepped out of the parking garage and proceeded towards the hospital entrance, we were greeted with the same not-so-gentle city smells that force their way into our nostrils. Heavy exhaust, stale cigarette smoke and the scent of the rushing storm drain. It always seemed to be raining. We pushed our way through the revolving glass door and made our way to registration.

Here we received the itinerary for the day: the orthodontist, the radiologist, the audiologist, and the neurologist. I am sure that there were plenty of other “ists” that we visited over the years, but there were too many to remember. At the end of the list, Doctor Padwa and Doctor Mulikin were always present, followed by the dreaded “Craniofacial Clinic,” when everyone from the course of the day would come together to “ooh” and “ah” over my disfiguration, one that only one in every 15,000 children are lucky enough to deal with.

****

I was born with Pfieffer Syndrome, a rare genetic disorder that keeps the bones from growing properly. The sutures in my skull that wouldn’t normally be sealed until I reached my early twenties, closed long before my tenth birthday, causing my face to develop abnormally. My lower jaw now sits far beyond my top teeth, making it impossible to eat anything harder than pasta or steamed vegetables. Steak and apples are out of the question. I did not know it yet, but there was a series of monumental procedures in my future that would reconstruct my face and allow it to function “normally.” Doctors were going to peel off my face like a banana peel, cut apart the bones, and mount a titanium “halo” to my face for a couple months while the bones grew back together.

 After years of planning, years of measuring, poking, pulling teeth and drawing blood, the day has finally arrived. I was now 13. Today I would get a new face.  I remember waking up in the hotel room that morning on August 1st, 2002. It was just before 5:30am. When  Mom woke me up, Dad was already packing a bag. M*A*S*H was playing in the background. It was going to be a long day for my parents. My surgery was scheduled to last about 12 hours.

 Doctors Mulikin and Padwa found me in Pre Op. Gone were their pristine white coats. Doctor Padwa’s finely sculpted hair was now covered with a surgeon’s cap. They stood by the side of the bed and asked my parents if they had any questions. “I have a question,” I chimed in. “Are you sure we have to do this?” My parents’ eyes quickly darted in my direction. The doctors just smiled. Dr. Padwa picked up my black belt from the side of my bed. I had brought it as one of my “comfort objects.” “Wow!” she exclaimed. “If you can earn your black belt, you can get through this.”.She stopped to think for a moment. “Can I wear it?” she asked. “What?” “Can I wear it in the OR?” she repeated. She had me show her how to tie it around her tiny waist. Everything got kind of foggy after that. They forced this orange liquid that tasted like lighter fluid down my throat. It burned on the way down.

****

 

 When I close my eyes I see the spots from the lights that were just blasting into my pupils. There are no clear images, just foggy swirls as the anxiety is just about at the breaking point. Every few seconds I get flashes of the anesthesia mask. I proceed to punch at it with martial arts fists, only to miss and swat at the air. They are standing over me now. I keep fighting. It is hopeless as my strength is dropping faster and faster. I lose control completely and lie there. I’ve been defeated. My body is limp and all the tension is released like air from a balloon. I hear something. Ten, nine, eight the voice never did get to whatever number comes next.

****

 

I wake up feeling like my body is covered with a pile of bricks. I can open my eyes only enough to see that damn blinding light. I have no sensation in my extremities, and I am unable to talk or breathe. There is a man’s voice to my left, and a woman’s voice to my right. They have no idea that I have woken up as they are trying to replace my breathing tube, and I am unaware that my nightmare has just begun.

  Within a matter of moments, my eyes must have closed again. I woke up for a second time. This time, my eyes do not open at all. I panic. I have no idea where I am or why my eyes won’t open. All I can hear are some beeping sounds, and “Full House” playing somewhere nearby. I try to scream for my mom, but my voice is not working very well, and I can only make a small squeak that sounds like one of my brother’s hamsters. I sense that there is someone in the room. I let out another squeak. “You are in the hospital,” someone is whispering to me. “Your parents have gone out for the evening.” There is someone else in the room now as I thrash my torso around on the bed. The man spoke. “Just relax.” It was then I learned that my eyes had been sutured shut.

****

 

Four days had passed in darkness. I was beginning to identify the nurses by their voices, and the time of day by whatever was playing on the television on the other side of the room. I had started to feel the weight of this foreign mass that had just been drilled into my skull as it curved out around my forehead and down in front of my nose like some sort of robot getup you would see on bad sci-fi movie.

 I had made some strides though. I was now able to sit up in a big chair that was only a few inches from the bed, the one that folded into a cot and had become my mom’s sleeping, sitting, reading, comforting and keeping-it-all-together place. It was about lunch time. Time for more meds, and another attempt at drinking that fortified milkshake mixture through a long plastic syringe which I could barely fit between the teeth in my swollen mouth..

 As I brought the syringe to my lips, I felt a funny sensation in my left eye. It was as though a block of ice was slowly cracking and breaking away from a glacier. Gradually, my eyelid released itself and light came flooding in. Immediately after I adjusted to the light, I saw my mom standing over me. In that moment, I felt like I regressed in age about ten years. “Mommy!” I squealed, “I can see you!” As though she did not believe me, she leaned in closer to examine my eye. I saw the giraffes prancing across the wallpaper border. I saw the day nurse who looked nothing like what I had been picturing. She had long hair that was died a very unnatural red, and twice as many wrinkles than I had pictured. Finally, my eye settled on the large metal frame for the first time. After studying my face for a moment, a familiar grin stretched across mom’s clearly exhausted face. She sent a nurse to find my dad who was out in the hall giving the daily update to whoever was on the other end of the phone line.

Out half-an-inch from the tip of my nose, I saw it. There was now a vertical titanium rod right down the center of my field of vision. At the base of the rod, long wires and metal pieces extended into my mouth and were screwed up into my gums. My face and eyes were so puffy that I had to look up and over the apples of my cheeks to see that there was anything in front of me.

There was a drainage tube protruding from the side of my head that emptied into a small plastic reservoir. The rest of my body was entangled in so many tubes and wires that I could not so much as lift a finger without running the risk of setting off one of those ear piercing beeping machines which would send doctors running through the doorway as if they were participating in Boston Children’s Hospital edition of Running with the Bulls.

The days in the hospital seemed to all blend together. It was like I had suddenly returned to my first year of life, and every little accomplishment was celebrated. The first time I was able to sit up for more than a few minutes at a time, the day that I was able to stand. Then there was the day that I was able to walk, all the while balancing my new heavy metal exoskeleton that seemed like it weighed about ten pounds.

The first time I saw myself in the mirror is a moment that will be forever tattooed in the recesses of my brain. I was finally strong enough to get up and use the restroom. Mom followed close behind me as I pushed my I.V. pole through the wide wheelchair-sized doorway. As I finished, and made my way toward the sink, she stepped in front of me, purposely blocking the mirror. “I want to see.” “Are you sure?” she asked in a final attempt to protect me. “Yes, I’m ready.” I could tell by mom’s reluctance to move that I needed to brace myself. She slowly stepped aside, stood behind me, and placed her hands gingerly on my shoulders. There I was in all my swollen glory. I gasped. The flesh of my face was as purple as the frame that was now mounted to it. I could feel my knees lose their rigidity. The face in the mirror had absolutely no resemblance to the one that I had always known. Seconds seemed like an eternity, but something kept me from turning away. I moved my arms and watched the mirror, just to make sure that it was really me staring me in the face.      

As the numbness and shock began to fade, anger and fear began rushing through my body. Suddenly the innocence and dependence that I had felt being coddled and waited on hand and foot turned to a ball of overwhelming fear and rage which settled in the pit of my stomach. I had been happy with my face before. I did not know any different. Why were these people trying to change me?

****

 

 By the time I finally returned to Vermont and passed through the kitchen door, the swirling emotions of rage, loss and defeat had taken over. I took a bottle of hand soap from the bathroom sink, and using my finger as a writing implement, I wrote “Helen is Ugly!” across every mirror and glass surface I passed. The orange-scented ooze dripped down the surface, stretching my traced letters, and making the words appear all the more terrifying.

 It was only a short time before I exhausted myself and was not able to continue my rampage. I slumped down on the couch in the living room. To the right of the couch, my cello stood in the corner. It was glistening under a sconce and appeared to be the brightest object in the room as the sun was setting on this incredibly emotional and stressful day, the day of my homecoming. Something about the cello drew me in. If only for a moment, I forgot about the anger and hatred that I had now been feeling for days in between the cranking sessions, when my mom used a small arsenal of tools to crank the screws, slowly pulling apart the bones in my head.

 With whatever energy I had left, I slowly pushed myself off the couch into some sort of hunched over standing position and made the few steps toward the cello. The chair and the music stand were right where I had left them two weeks ago. I grabbed a hold of the cello, pulled out the end pin and slowly dragged my bow across the strings. Did I still remember how to use this thing? A book of my favorite Celtic tunes was open in front of me and I began to play.

 It was like nothing had happened. It was just me and my instrument. My fingers floated up and down the cello as if I had not missed a day of practice. For a moment I felt no pain, no anger. I was free and I was myself again. I played Celtic tunes until my fingers turned cold and it was time to eat whatever Mom had liquefied for me in the blender by way of syringe.

Meeting Olivia

I was born with Pfeiffer Syndrome, a rare condition which affects only 1 in every hundred thousand births. I had 13 surgeries in my first 14 years of life, the biggest one being the reconstruction of my face when I was in 8th grade. I had lived my entire life not ever meeting anyone else who shared the same condition. Someone else who had their bones cut apart and screwed back together. Someone else who sat on the side of the bed while their mother cranked the bones of their skull apart, trying to offer comforting words through her own tears of pain and fear. I had found a community on Facebook a number of years ago and chatted with people occasionally, but nothing had really come of it.

Sometimes, even on an ordinary day, moments happen that change your perspective. It was a typical Friday night: four of us sprawled out on the couch with sodas and pizza watching TV. We all seemed to be reaching the food coma state of the evening where everyone is staring blankly at their electronic device of choice while the large screen of the television glows and murmurs in the background. It was the end of the semester and no one had much energy or brainpower left.

Scrolling through my Facebook feed was nothing new. I could do it in my sleep; in fact I may have been, given the state of things at that particular moment. The screen scrolls up; I’m not really reading, just skimming, as if on some sort of college student half-asleep Friday night autopilot. Then something finally catches my attention: the Sanborns are on their way to Maine! I make a quick comment out of curiosity which would end up changing my life forever. The Sanborns were one of the first people I connected with who understood. They have two young daughters. Olivia is in the 5^th grade. She loves animals, ballroom dancing and softball. And she has Pfeiffer Syndrome.

 Where in Maine? I ask. Within a few minutes I have a response. Wells. What I wanted to say was “Wow, come visit; I would love to meet you!”, but I did not want to sound crazy. They probably had plans anyway. I set my excitement aside, and simply hit the like button. Another comment appeared: “Where are you again?”…“Old Orchard Beach”. Long pause.

Ah! She finally replies, “Is that like an hour away?”…“About 30 minutes” I reply. At this point I was picturing Laurel at the other end, considering a meet-up to be a possibility, but still I did not want to get too excited. Then finally after what seemed like forever, the moment I had been waiting for appears on the screen: “We are eating at Mike’s Clam Shack tomorrow night if you want to join us.” “Yes” I reply, without as much as a thought. We work out some of the details. It was then that she expresses great enthusiasm, and I know that the overwhelming excitement was mutual.

I close out of Facebook and look around the room again. No one had moved. It felt like a lifetime since I had surveyed the half-eaten pizza and the friends mesmerized by their phones and their tablets, and the hum of the large screen TV that no one seemed to be interested in. Nothing had changed. But to me, the room seems brighter, more vibrant, less “exhausted college student” and more “adult who had amazing opportunity in life”. It is incredible how quickly things can turn around simply by changing perspective.

That morning, I saw each of the Sanborns’ activities as their posts appeared on my news feed. They took several pictures at each stop as the time of our meet-up drew closer. As my excitement continued to build, so did my nerves. How was I going to act? What questions did I want to ask? Did I want to ask questions? What if I do something stupid? I was driving myself crazy. What if they don’t like me? What if they have trouble with my weight, or the fact that I’m gay? Would I bring Amy with me? She is my wife after all! How could I hide something, or someone, which defines so much of who I am? It was strange how these thoughts seemed to overwhelm the main connection, the main reason for the meeting in the first place. I share something with them that for my entire life has made me feel alone in this world. We already shared a common bond that made all the other stuff, all the other shit that everyone worries about when meeting people and fitting in, seem trivial.

Finally the moment had arrived. I took in one last deep breath and straightened my hair in the visor mirror as Amy and I pulled into the dusty parking lot. She looked at me and smiled. “This is going to be good” she said, picking up my hand and squeezing it tight. I could see that she too was nervous. She knew what this meant for me. She walked behind me up the long stone pathway. Olivia and her sister Amelia were watching for us through the window. Before I was close enough to open the door, they grinned at us through the fingerprint-covered glass. I grinned back, and my fear melted away.

Once inside, the sisters greeted us each with big hugs. It was as if I had known these girls forever. They lead us to the table where their parents were waiting. More hugs. The vibe was less like an awkward first meeting and more like a family reunion. As we all squeezed into the booth, all I could do was smile.

Our next meeting would be in Vermont, when I had to go home after suddenly losing my father. I found solace in another meal with them. I kept forgetting we had only met once before. We laughed together and talked. Olivia had the amazing ability to make me laugh and forget for a moment the real reason we had traveled the 211 miles on a weekday. They told me that they wanted to attend the service. I was overwhelmed by their desire to support me in a way that was so personal and profound. They never had the opportunity to know him, and yet our connection was so strong that they made the service a priority.

The next morning my family and I pulled up in front of the church. We were greeted by the priest and the funeral director. I saw my brother, his girlfriend on his arm, talking to people as they entered the chapel. Amy was already playing the organ and the music carried out through the wide open doors down State Street. After instructions from the funeral director, I took my mother’s hand. We lined up as if in procession; behind us the rest of the family followed suit, and we started up the steps and down the aisle.

It was overwhelming. I blocked out the sound of the music and the colors of the glass which bounced off the walls. I blocked out the 400 faces that were all turned in our direction. The aisle seemed to go on forever, and at the end of it was my father in a small granite box, and his choirmaster robe draped over the altar like some reference to Jesus or something. It was all too much! As we made the long walk toward the front of the church, I saw someone out of the corner of my eye. It was Olivia smiling at me! I couldn’t help but smile back at her. I wasn’t so scared anymore after that.

Sometimes, even on an ordinary day, moments happen that change your perspective.  The day I met Olivia and her family at Mike’s Clam Shack on an ordinary Saturday, I went from being the only person I knew with Pfeiffer to a part of something greater. Never before had I been able to explain the condition in “we’s”; I always used a very isolating “I”. It was an instant bond between two people, two families, which has changed my perspective forever.