Yet another Prayer Request

The family I personally know who has gone through this has come out stronger, closer, and all the more loving for it! You will all be in my thoughts during the journey now beginning.

One of my favorite quotes of my friend's:

My hardest mom moments are when I know I can't fix things and I have to put my trust in the medical professionals. Years later, there are still memories so raw I'm not sure how we actually lived through them. Another favorite quote: "Laughter through tears is my favorite emotion."

Here are some links (there are many, many more) for you to share with your family - the support network out there for craniosynostosis is amazing!
http://www.craniocarebears.org/ (they have a Facebook page too)
http://www.cranioangelnetwork.com/Crani ... lcome.html (also have a Facebook page )
https://www.facebook.com/groups/181511396904/
https://www.facebook.com/pages/Eagons-C ... 2984643896
http://www.digtriad.com/news/article/18 ... rth-Defect

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Craniosynostosis & Craniofacial Awareness.
You never know how strong you are, until being strong is the only choice you have...

Dear Zachary,

I can’t even begin to tell you how excited I was when I found out I was pregnant with you. We were just starting to think it would never happen when we got the happy news! I was so nervous until I saw you in the first ultrasound and then heard your heart beating. I couldn’t wait to find out if you were a boy or girl, but you had other plans! At the ultrasound, you were breech, and they couldn’t tell me either way. We decorated the nursery so that it would work for a boy or a girl and just resigned ourselves to be surprised.

One night in June 2003, I started having terrible pains, and Dad insisted that I call my doctor. I ended up in the hospital with pre-term contractions, and they had to give me medicine to stop them. After a week of bedrest, I headed back to work, but whenever I walked I would get contractions again, so I had to keep taking the medicine to keep you safe where you belonged. At the beginning of October, I went back to the hospital, dehydrated after a virus and having contractions again. They decided you were still breech, and we scheduled October 30th as the day you would be delivered. You had other plans, and labor started again. This time the doctor said you were ready to come, so on October 23rd, we headed to the hospital to finally meet you.

Dr. Stephens told us it would only be 30 more seconds, but then he kept saying “Oh my goodness, oh my goodness.” Over the top of the sheet, I just remember seeing him twisting and turning. You were stuck under my rib cage and not wanting to budge! Finally I heard him say “He’s a little floppy. Let’s get some oxygen.” But just then I heard you cry, so I knew you were OK! The nurses brought you behind my head, but I could see you with a mirror. I heard the word “molding” for the first time then…it seems your head was not as round as it should be for a c-section baby, but no one thought there was anything to worry about.

The next couple days in the hospital, we again heard a lot of talk about positional molding. We just thought you were cute! We took you home, and at your one-week appointment with Dr Shely, we heard it again, but she said the same thing as the hospital pediatricians – that is was molding and should round out in time. When we returned at one month with no improvement, I heard the word craniosynostosis for the first time. Dr Shely sent a referral to Children’s for an x-ray, and I went home to Google.

As soon as I saw the first picture of a baby with metopic craniosynostosis, there was no doubt in my mind that we had our answer. The keel-shaped forehead and close-set eyes were exactly like yours. In a daze, I took you to your x-ray and then CT-scan and then met with the pediatric neurosurgeon and plastic surgeon for the first time. They matter-of-factly spoke words that no parent should have to hear: invasive surgery, ear-to-ear incision, skull removal, blood transfusion, PICU, eyes swelled shut, 50% chance of learning or developmental delay even with surgery, 5-10% chance of multiple surgeries. Then they delivered the crushing blow – we would have to wait and worry until you were 8-10 months old to have this surgery.

So, we took you home and tried to relax and enjoy your infancy…easier said than done with such a major ordeal looming. I took lots of pictures and settled back into my new assignment at work and tried my best to prepare. I settled for being mentally prepared, because there was no way to be emotionally prepared. I looked at post-op pictures of other babies to try to numb the shock of what it would be like when I saw you. I asked all my questions and tried to accept the answers. I watched as you started to gag on your bottles, wondered if your brain was already running out of room and ICP was starting, and resigned myself that we were doing the right thing. I took you for your pre-op blood work and tried not to cry when you did. I held you and told you how sorry I was.

June 23, 2004 was the hardest day of my life. Jelly came with us to the hospital so we wouldn’t be alone. You were to be the second surgery of the day, but an emergency case bumped us back, so we had an excruciating wait before we got called back. We put you in your gown and answered all their questions. I had to sign a consent form that said I understood the possible complications, including blood loss and death. They gave you Versed, which made you loopy and made us laugh through our fears. Because you were only 8 months old, you would be anesthetized in the OR, so we had to hand you over, wide awake, to a nurse outside the OR door. You smiled at us as we turned and walked away, steeling ourselves for the long hours ahead.

The hours in the waiting room passed slowly. Every few minutes, they would come over the speaker with an update for someone. Every time I heard them call “The Parents of” I would stop and listen for your name, but they never said it. As afternoon turned to evening, the waiting room thinned, until we were the only ones left. I started pacing the room, and they finally called us. Your neurosurgeon told us that his part had gone well. They did nick your dura when removing your forehead, but he assured us that they placed a small stitch and didn’t expect any complications. He expected the plastic surgeon had about 45 minutes of work to do before you were out of surgery. Two hours later, we finally spoke with the plastic surgeon. He rubbed his eyes a lot and told us he had bone dust in them. I cringed – that was my baby’s bone dust in his eyes – my baby who I hadn’t seen in over 6 hours and just wanted to hold more than anything. He also told us that your orbits had been very close together and that he had “spaced them a mile.” He left and sent us up to the PICU waiting room.

When we finally got back to see you, you looked like Lizzie! You had a green turban of bandages to protect the incision and wires everywhere, but morphine kept you comfortable and we all managed to get a little sleep that night. Most of the following week is a big blur to me. The swelling set in quickly and you couldn’t open your eyes. You perked up when all your visitors came. I slept in the bed with you and barely left your side. Your first post-op smile happened when Mimi, Pappy, Jelly, Daddy, & I were all in the room; we all cried happy tears! It was the longest week of my life, but as soon as you could get one eye open, we headed for home and thought we’d never be back.

Before summer was over, we got our first glimpse of the long road ahead. At your ophthalmology follow-up, you were diagnosed with strabismus. Apparently spacing your orbits a mile made your vision worse. We started patching therapy in hopes that we wouldn’t have to go back to the OR. Our hopes were dashed, and you had your first eye muscle surgery that December and your second one the following May. Finally, things were looking up, and we settled down and enjoyed your toddlerhood. You loved Blue’s Clues and would walk around everywhere with your notebook looking for clues. You would pick out what shirt to wear by telling us what color “Joe” you wanted to be that day and would get upset if we couldn’t find the right color Joe in the saved Tivo episodes (or heaven forbid if we only had Steve’s!) You were excited when baby Alex arrived - even though you called him baby Alice.

Our next blow came shortly after Alex was born when you had a follow-up CT scan. I will never forget walking into the hospital for the scan and telling you that the Dr wanted pictures of your head to see how it looked. It was still dark out and you could see your reflection in the windows and said “I like my head. I think it looks good!” Unfortunately, the CT scan showed several large openings in your skull where the bone hadn’t grown in, as well as a return towards the metopic head shape. Your plastic surgeon recommended an endoscopic surgery with cadaver bone paste to fill in the openings and round out your temples. We consented, and sent you back to the OR for your 4th visit in less than 2 years. You came home the following day, but your eyes swelled shut again and you couldn’t keep your pain meds down. You were miserable, and we were scared since you stopped talking and answering our questions. We rushed back to the hospital, and you were admitted for dehydration and pain management. You finally felt better the next day, but we had to stay for observation a couple more days. You ran around the hospital with your IV pole and made everyone smile. I’m not sure I ever laughed so hard. It was hard to believe you’d just had surgery! Finally, we believed your medical journey was over. Unfortunately, it wasn’t even close.

The following year, at 3 ½, you had your third eye muscle surgery. We were getting to be pros at these outpatient surgeries now, and this one was a piece of cake. Just after you turned 4, you had your last CT scan. I stared at it in disbelief. The openings in your skull were still there. Your sagittal suture was also now fused prematurely. I called your plastic surgeon’s office to discuss the results – he wanted to repeat the second surgery, the surgery that had failed. He’d had two chances to get it right, and I couldn’t find it in me to give him another chance. I mailed the scans to your original neurosurgeon who was now in Washington, DC, as well as to a renowned pediatric craniofacial surgeon in Dallas. Both replied quickly saying that you definitely needed more surgery, and both recommended a procedure called a split-skull bone graft. Dr. Fearon in Dallas also suggested that your metopic shape was returning and thought we should consider re-doing the first surgery the right way instead of just fixing the mistakes. We agreed that if we had to do surgery, then we needed to do everything in our power to make sure it was the last one. I worked on getting insurance to cover us to go out of network without going broke, and then got surgery scheduled. The hardest part was still to come: we had to tell you.

The experts recommended waiting until a few weeks ahead of time to tell you, but the experts did not know you. I knew that you needed time to process, time to get all your questions answered. At first the excitement of going on your first plane ride overshadowed the surgery, but gradually you started to have us fill in the details about what was going to happen. You were most scared of the pre-op blood work and the medicine and your eyes swelling shut. I think I was more scared than you were. We headed to Dallas in September 2008 with high hopes that your journey would end there.

We felt instantly at peace with Dr. Fearon and his neurosurgeon colleague Dr. Sacco. We knew we had made the right decision for you this time. Once they had seen you in person, we decided to move forward with a full reconstruction – they would take the bone from the back of your head and make a new forehead out of it. They would take your lumpy, holey forehead and move it back to hide it under your hair, and they would do split-skull bone grafts so that you had no further openings. They would also over-correct your forehead so your brain had enough room to finish growing. It would be your most complex surgery to-date. We made sure to schedule some fun time in Dallas and opted for a place where you could play Laser Tag, putt-putt, and drive go-karts. While driving go-karts the day before surgery, you told us you were the luckiest kid in the world. My heart broke for what I knew the next days would bring.

As usual, once the Versed was on-board on surgery day, you made us giggle! It was the best medicine for all of us in the hour before surgery. I still can’t believe how amazingly well you did with this surgery. Despite the surgeons’ promises, I had seen you through the two previous ones and had expected a similar recovery. You complained that your head felt like a bowling ball and that you wanted to go back to Ohio, but you required no pain medicine. You hated the 24 hours that your eyes were closed, but as soon as you could see again, we left the hospital and enjoyed another day in Dallas before flying home. You healed quickly, and I finally gave myself permission to exhale.

In the years since then, you have brought me so much joy. I sometimes find myself wondering who you would be if you didn’t have craniosynostosis, but in the end, it doesn’t matter: cranio is a part of who you are, but we have tried never to let it define you. I try very hard to remember the good moments and not the bad. You have taught me to find new meaning in ordinary moments. Your accomplishments make me proud, but when I put them in context with all that you have had to go through in your short life, I am even prouder. I know you don't like for your scars to show, but for me, they are an outward sign of your inner strength.

Sometimes it’s hard for me to believe you aren’t years older than you really are. Even as a baby, Jelly remarked that you seemed like you had an old soul. When you started kindergarten and had to tell your class about yourself, I was surprised that you chose to tell them about your trip to Dallas. You said “Don’t worry, Mom. I didn’t tell them about the surgery. They’re just little kids – I didn’t want to freak them out.” I guess it shouldn’t have surprised me when Mrs. Niehaus told me you should skip kindergarten, but in my head, I still heard the surgeons telling me that 50% of kids with cranio have some form of learning or developmental disability. I was glad you decided you wanted to move on so I didn’t have to make the decision for you. I hope you never lose your love of reading and of learning new things.

The past year has been amazing for me. I know the whole family was anxiously awaiting Katie’s arrival, but I was also wondering how it would affect you, Lizzie, and Alex. I worried that Lizzie would shower Katie with attention and leave you and Alex feeling left out. I was shocked when you were the one who didn’t want to leave the hospital the day she was born and that you were the one who had to study her from head to toe for hours. I know everyone says she looks like Alex, but when she smiles, it reminds me of you. The bond you have formed with her melts my heart. The way you both light up when the other one enters the room is something so special that I hope it only grows stronger as the years pass.

It’s been seven years now since the first time we had to put your life in someone else’s hands. Some days it feels like forever ago, and some days it feels like just yesterday. I hope that when you are all grown up, you understand that I always made what I felt was the best choice for you with the information I had at the time, even though looking back now, there are some things I would do differently. One thing I know for certain is that you have wisdom beyond your years and the ability to confront any challenges that stand in your way. You are, and forever will be, my hero.

Love,
Mom