As you all know, September is Craniofacial Awareness and Acceptance Month. I wanted to take this opportunity to share stories from other families that have gone through a cranio journey that feature other types of craniosynostosis. There are different kinds of craniosynostosis, and there isn’t always a syndrome associated with it.
Today’s feature is from Grace, whose daughter Rosalie was born with Metopic(Trigonocephaly) craniosynostosis. Metopic craniosynostosis involves the sutures that runs down the forehead, so when it’s fused prematurely it causes the forehead to become narrow and pointed, creating a triangular shape, and making the eyes appear close together. The frequency of Metopic(Trigonocephaly) is between 1 in 2500 and 1 in 3500.
Rosalie -Our Journey with Metopic Craniosynostosis
Submitted by: Grace Favatella
After three beautiful and crazy boys, we were elated that our surprise baby was a girl. Please don’t misunderstand, I adore my boys. They have given me more joy and gray hairs than I could ever have imagined. But having a daughter filled my heart with a whole new type of love. She completed us. I have always had high risk pregnancies, and so I was monitored very closely from day one. I had monthly appointments with my OB, appointments with geneticist, with a nutritionist and dietitian for Gestational Diabetes, ultra sounds, endless blood work and kick counts and stress monitoring during my last 6 weeks due to having a preemie years before. No one noticed. No one said anything. How could they?
As ready as I was to see her and hold her, she made me wait the full 40 weeks to the date. Her birth was fast and scary. I had my amniotic fluid measured that morning and all looked well. I did my chores and around mid-day contractions started. They were inconsistent at best. The only thing that alarmed me was an occasional stabbing pain. I kept sitting to see if I was leaking fluid. I didn’t notice anything.
Finally that next morning around 1am I knew it was time. I labored for a couple hours in the quiet before I woke my husband and we were off. It was a total of 3 hours and she was finally here. However, she had no fluid, zero, and had been stuck in the birth canal for some time and was acting like a corked bottle. She was drowning. In a matter of 30 minutes, with no signs of stress on the monitor to the time she came out she had turned blue and was making a horrific gurgling sound. It was a blur. My mother immediately started snapping pictures and they stopped her. The respiratory technicians and nurses came running; my husband’s face was white. It felt like a dream, but everything was just so fast. Within a few minutes I head her cry, a few more passed and she was pink, soon thereafter she was in my arms. It was lightning fast. And we were completely in love. And no one noticed anything.
The next day the pediatrician came to release us. I had begun to notice her head shape. Every baby comes out a bit coned shaped. But her head never full returned to a “normal” shape. Her forehead was pointed and jutted out. Her temples sunk deeply into the sides of her head. The pediatrician took just a few minutes and ordered X-Rays before we could be released, as well as a “specialist”. And so we waited. We heard the word Craniosynostosis. And I googled and cried and cried. We came home, and I cried for her, I cried because it just wasn’t fair, I cried because someone had already made a comment about the way she looked. I cried bitter angry tears for her, because I knew how cruel the world could be. I was then suddenly hit by my nemesis, kidney stones. It was a post-partum nightmare.
But despite it all, I was completely smitten with her. The whole family was. We were referred to Children’s Hospital Los Angeles and saw Neurologist Dr. Maccomb. Diagnosis: Moderate to Severe Metopic Craniosynostosis with Trigonocephaly. And the whirl wind began. We were then seen by Plastic Surgeon Dr. Urata, we saw the Ophthalmologist and underwent a sleep study. She had pre op CT scans and saw both the developmental Psychologist and the Geneticist. We waited. Waiting for the day we would receive the call with our surgery date. I cried in the quiet. I cried in the shower so no one would hear me or see me. My heart was broken. I loved her face. Kissing her in her sunken little temples seemed natural and the perfect spot. It was during this time that it hit me; her face was going to change. I searched what felt like thousands of before and after pictures. I laid it all out, and surrendered it over. I didn’t have it in me. I had nothing left. My faith would have to carry me. With that new resolve, we continued to wait.
The phone rang that afternoon. And before I even saw the caller ID, I knew it was the hospital. I wrote it down, contacted my husband and kissed her and kissed her. We prayed and prayed. We prepared. And then she got sick. A simple cold turned into a respiratory infection and surgery was cancelled. I cried harder than I ever have before. People didn’t understand why. We would simply just be rescheduled. No big deal. But I had prepared myself to say goodbye. I was ready to let go of her face and fall in love with the new one. I was ready. I had waited long enough with a giant gorilla on my back and a frog in my throat everywhere I went. And then I was told to wait more.
To ensure she wouldn’t get sick again, we quarantined ourselves best we could for almost 30 days. It was hard, but necessary. I treasured that month. I held her more and more and snuggled every time I could. The house was a mess, I didn’t care. We tried to prepare our boys best we could. A change in routine is always difficult for children under the Autism Spectrum. And before we knew it I was packing for the hospital.
The day before, my sweet friend offered to come and take “before pictures” of her so that I would always have them. It took me weeks to look back at them. But I truly am so thankful for them. That night we bathed her, and put her in the jammies she would just wear to the hospital, I set out my clothes. My husband watched Netflix in bed till he fell asleep. I turned and turned and turned. We woke up and I nursed her right away. He served coffee; I loaded her into the car. He prayed over us. That was it. Almost complete silence. We didn’t speak on the dive there, just held hands. We checked in and they sent us for a blood draw for her. Needless to say I was less than pleased with how they did it. I left hot and angry with her in my arms. They took us back, checked her lungs to be sure they sounded clear, gave us her gown and led us to the back. We took turns holding her. My heart was so loud in my ears, they throbbed and my eyes burned and my stomach was in knots. We spoke with the Anesthesiologist and the doctor. She was given her “happy juice” and we giggled and laughed with her. And then it was time. We walked her to the door handed her over and watched them turn the corner. She was gone.
I walked straight and deliberately to the food court. I stuffed myself full of French toast and bacon. Then we went and sat and began the longest wait of my life. I colored, I tried to read, I paced, I went to the bathroom, I redecorated my house with Pinterest and planned my sisters imaginary wedding. My sister came and my mother and later my father in law. I kept everyone up to date every 30 min or so on Facebook. The clocks ticked loudly, the murmur in the room seemed far away. But I didn’t cry. Not once. I expected to. I was waiting for it. But I didn’t, perhaps the tears had run out. I became single minded. I needed to see her, and hear every single thing that happened, I wanted to see numbers, and wanted to know facts. Dr. Maccomb came out and said he had finished with his part, and that she was doing well. I needed more. I watched the numbers go higher and higher on the screen where each patients number was, telling me she was still in the OR. At 400 minutes I thought I was going to lose my mind.
Right at 7 hours, they were done and we could go see her. I ran. Literally, and my sister slowed me down. So I walked fast. I walked into her room and my heart melted. There she was. Tiny, covered in wires, bandage across the top of her head but asleep. She was clearly very round, her temples no longer sunk in. but her eyes were the same. I could tell, even with them closed. My hand shook as I placed it gently on her to feel her breathing. I looked at the hanging Stats and sheets, and went into information overload. Once I felt I understood it all, we sat down, family left and we were left with the beeping of machines. That first night was the hardest. I was running on literally minutes of sleep. She would wake up briefly and cry. It was the most heart wrenching sound I had ever heard, she was in pain and scared. I couldn’t reach her to hold her over the damn railing. She kept trying to sit up, but her head was so heavy and full of fluid that she would just simply start to tip over. And so I would just try on my tip toes to hold her up right until she would lay down again. I finally woke my husband and I got some sleep after they gave her morphine to help settle her and manage her pain. The next day I signed a waiver and they brought in a bed for me to lie in with her, so she wouldn’t feel alone. We held her in a modified upright position 24 hours a day until we left.
That second day the swelling began and by mid-day her eyes were completely swollen shut. It was difficult but because we had expected it, we felt prepared. We held her and sang and read out loud and watched movies, I nursed her whenever she wanted and she would just camp on the breast for hours at a time, being sure to switch sides to help with the swelling. Our nurses both day and night were spectacular. Aaron was able to go home and see our boys, and our boys were able to come to the hospital and face time with me and their sister from down stairs. By Friday we were getting anxious to leave. Her pain was being managed and she was reaching for toys and smiling with her eyes completely swollen shut. And then one opened. She was so happy to see our faces. They doctors wanted both eyes open before they felt comfortable releasing us. And so we waited. By this time I was becoming grumpy with my hubby. She opened her other eye early Sunday morning and I’ll never forget that smile, the wires were coming off a few at a time until she was finally free. We were given the green light and sent home.
Coming home was another animal. We were able to manage her pain, but her appetite took almost a week to return after we got home, sleep was difficult. She wanted to sleep in arms and was terrified of being laid down. The questions however I welcomed. I was amazed at how interested and genuinely concerned people were when they saw her, they asked good questions and little ones did as well. We welcomed questions and answered them happily. Her hair has begun to grow in and her scar has faded and undetectable to those who don’t know her story. It seems like so long ago, and yet still so fresh that reliving the details bring tears to my eyes. It has been a long road, even now as we are almost 5 months post op. Her personality and beautiful smile seems to not have been diminished. She is happy, spunky and amazing. She is loved by all who meet her, and I am truly honored to be her mama.
Grace is currently donating bottles of lavender to Cranio Care Bears for their care packages. If you are interested in donating a bottle contact Grace on her Facebook Group Love, Lavender, and Cranio Strong.