A Uniquely Beautiful Life

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This is Claire. Our beautiful daughter. Our second born, planned for, always wanted and incredibly special little miracle. When people look at her they often don't even notice anything different about her because of her bright smile and fierce determination. The thing is though, she will always be a little different because she was born with spina bifida and hydrocephalus.

Our journey began on the day of our 18 week ultrasound. I had already done the screening for spina bifida and down syndrome at 12 weeks and the results had come back perfect. So I had little to worry about - or so I thought. During the ultrasound the tech was very quiet and it seemed to be taking a long time, but I accounted that to me just being anxious to see my new little baby on that tiny screen. Finally she got up and left the room, saying that she needed to check to make sure she had all the images she needed. It took her a long time to come back and when she did she said, “My next patient is here. I don't have time to show you the baby.” We were given no pictures, only the excuse that my bladder wasn't full enough to clearly see the baby.

Later that afternoon my mom came upstairs and asked how it all went. I said, "It wasn't my favourite ultrasound experience." And I told her why. Mom was angry. Livid, is probably a better word. She phoned the department head and explained what had happened and demanded a CD be made and left for pick-up that night. The department head said, "Oh. I heard about this case. There's something her doctor needs to tell her."

Mom got off the phone and said, "There's something wrong with the baby." I was instantly in shock. I grabbed my head and stared at her and said, "This isn't happening." as if I could will it away. I collapsed on the floor sobbing. Our first child, Nathan, was so confused. He didn’t understand what had just happened to his mommy.

My mom called my family physician's wife at home and got his cell phone number. She called it and asked what he knew. He said he was still waiting for the radiology report. Nathan and I went downstairs to the kitchen to wait for the call. About half an hour went by then the phone rang...

"There are problems." My doctor stated.

"There are problems?" I repeated.

"Yes. The baby has hydrocephalus. This is water on the brain. And there is a defect in the low spine called a myleomeningocele. You have probably heard of this. It's called spina bifida."

I wrote it down as he told me. My Mom read it and gasped. I cried.

"What does this mean for my baby?" I asked between sobs.

"It means that surgery will be done after birth and your baby will likely not walk."

"Where do we go from here?"

"We refer you to a specialist."

 

My mom wanted to talk to him at this point. She asked the same questions. The call ended. We cried. I shook uncontrollably. Mom almost fainted so she laid flat on the floor. Then she called in sick for her night shift.

I called my husband Josh and told him that he needed to get home. When he arrived he sat down with me at my mom and dad's kitchen table and I told him the news. "Our baby has water on the brain and spina bifida. We are going to have a very disabled baby." His eyes got red and he started to cry. Through his tears he said, "If that's what God wants, that's what He wants."

We got an appointment to see a fetal medicine specialist in Toronto. Though anxious and excited, we were still filled with hope. Staff performed an ultrasound and the obstetric fellow told us everything that he was seeing as he went. They confirmed that the baby did in fact have spina bifida. My heart sank a little at this news because I had such hope that they would find nothing wrong.

The doctor scanned me himself and then took us to another room to talk about the diagnosis. He told us that the condition would get worse toward birth, the baby would not walk and likely have bladder and bowel issues and may have mental delays. In the next breath he stated that we should seriously consider terminating the pregnancy. I was 20 weeks pregnant at this point! My husband told him without hesitation that we weren't going to abort our baby.

Then we brought up the option of in-utero surgery to correct her lesion before birth. The doctor  told us a little bit about it, saying they had never gone this route with a patient before. He was very off-putting, cold, and unsupportive. I was panicked and heart-broken. Hoping to get some sincere advice I asked him, "If this was you, if I was your wife, what would you do?" He sat back in his chair and laughed at me.

If I hadn't been so sure of my beliefs and so committed to preserving the life of my unborn child, in my devastation I would have terminated Claire's life.

The next month was a blur of doctor's appointments and phone calls. We were going to pursue in-utero myelomeningocele repair surgery in Philadelphia. My husband and I were determined to do anything we could to help Claire. If that meant a risky and expensive surgery in another country, we were going to do it!

A few days before we left for the Children's Hospital of Philadelphia, we decided to cancel the surgery. From Claire's fetal MRI, they could see that her condition was already too severe for the surgery to be of much benefit. This was saddening, but also a great relief. Now I could continue to be a mom to Nathan, my 19 month old baby boy, and spend the remainder of my pregnancy planning for the arrival of our life-changing gift.

Time went quickly and Claire was growing quickly too. When I was 35 weeks pregnant Claire was examined by ultrasound once again and she weighed in at 7lbs 11oz. It was comforting knowing that even though she would be pulled out of me earlier than she wanted to be she would at least be a good size.

After the ultrasound, the fetal medicine specialist met with us and reviewed the images of her latest photo session. I got really nervous when he frowned. He looked again at the pictures of her little brain. I knew what he was looking at her ventricles, the fluid-filled spaces in her brain. They had been carefully monitoring their size since day one. He measured them while saying "Hmmmm." My hands started to sweat. He told us they had gotten big, really big. They were now calling it severe ventriculomegaly. There was no doubt she would need a shunt and they may even place it while closing up her back. Panic welled up inside of me as I let myself think about her brain being irreversibly damaged.

We talked more about delivery and the doctor mentioned that her head size was still fine to deliver vaginally but if it got too big there was something they could do. It's called a cephalocentesis. They would insert a long needle into the stem into her brain and drain off fluid before delivery so that her head would fit through the birth canal. At the end of his description of the procedure he said that unfortunately the side effect of this most often is fetal death! I was so taken aback. I stammered, "Well, we don't want to do anything that would make the baby die!" He said "Okay. I'll make a note of that." I was so confused. Why after everything we had done, and gone through, and fought for, why would we do that to Claire?

That day after we left Mount Sinai we headed over to Sunnybrook Hospital and met Dr. Paige Church and her nurse Julia from the Spina Bifida Clinic at Holland Bloorview Kids Rehabilitation Hospital. I immediately felt comfortable. I started to cry when she was speaking with us because it was the first time that I felt like a doctor truly cared and valued our little girl's life like we do. From the beginning, she referred to Claire by her name telling us over and over how she will be beautiful and amazing. This hospital was an incredible place filled with amazing doctors and therapists and families going through the same thing as us. I had never left an appointment before feeling so loved and supported.

On June 13, 2012 we rose early to travel the 3 hours to Toronto for our final obstetric appointment. Claire's induction date was scheduled for June 18th, but God had other plans. Even though I was only 37 weeks and 5 days pregnant, when I was checked at our appointment they found me 3-4 centimetres dilated and ready to give birth at any moment. It's a good thing that we brought our hospital bags because I spontaneously went into labour that afternoon.

At 7:24 pm on June 13, 2012, Claire made her grand entrance into this world! The doctor said, "Open your eyes and look at your baby!" I looked down at the screaming baby I had just delivered. I finally got to see the little person I already knew so much about.

I said to Josh, "Take a picture, for goodness sakes, take a picture!" As soon as the picture was taken she was whisked off to the "resus" room next door, out of our sight. After they had gotten me cleaned up they wheeled me into the recovery room, where they fed me a plate of dinner. When I was halfway through it a nurse came in and asked if we wanted to see our little girl. Oh, I was so excited! They wheeled my bed into the resus room where my little Claire was laying, bright-eyed and content.

She had a head of dark brown hair and dark blue eyes. Her hands were so tiny and delicate. Around her tummy and back was what looked like saran wrap to cover and protect her open lesion. My eyes trailed off to her legs. They were twisted around each other and her feet were bent unnaturally but I didn't care. I was so in love! She was SO beautiful!!

I reached out and let her tiny fingers wrap tightly around mine. I stroked her hand with my thumb and told her how beautiful she was. The nurses told us that she was doing well. She had a little trouble breathing at first. Josh and I were given about 10 minutes with our daughter before it was time for them to take her across the street to Sick Kids Hospital. We said goodbye and they wheeled me out of the room and back into recovery. Josh left to make some phone calls.

I sat in my bed by the door and watched as women were being wheeled in with their brand new babies all wrapped up tight in their arms. I kept a smile on my face but inside I was so sad. My arms ached to hold my baby and to feel her warmth on my chest. That would not be the last heart-wrenching moment I would experience.

At one o'clock the next afternoon (16 hours after being delivered) they wheeled our precious baby down the hall to the OR. Josh pushed me in the wheelchair behind her incubator. When we got there I eased myself out of my chair and gave Claire a kiss on her warm chubby cheek. Tears flowed down my face as I whispered my goodbyes. It took a team of neurosurgeons and plastic surgeons 4 hours put her spinal cord back where it belonged and sew her up.

When Claire was 12 days old she underwent surgery once again to have a shunt installed into her brain because her head circumference was now 2.5cm larger than it was at birth. It was so heart-breaking to have to hand her over once again to a bunch of strangers but we knew she desperately needed this little device. Her brain, and her life, depended on it.

Claire's was discharged on her 2 week birthday and we finally got to bring our little dolly home! Life with a baby with hydrocephalus has not been easy. We were given signs of shunt trouble to watch for; fever, lethargy, vomiting, irritability... the list goes on. Every time Claire has one of the things on the list we ask ourselves, “Is it the shunt, or is she just teething?”, “Is it the shunt, or does she just have the flu?”, “Is it the shunt, or is it a side-effect of the vaccination she just had?” It has been exhausting but as she's getting older we are getting more skilled at deciphering the signs.

Sometimes though, it is the shunt. When Claire was just about 6 months old her choroid plexus grew into her shunt and blocked it. This meant another operation. When the neurosurgeons took out the blocked portion of her shunt, her brain began to bleed. She spent the next day with an EVD (external ventricular drain) to drain off the bloody CSF before going back into the OR to put in the new shunt part.

We are so thankful that 2013 has been a healthy year for Claire so far. She is such a little fighter and so full of joy! Our little girl army crawls around our house getting into all sorts of trouble and she loves to play with her big brother. She can sit up nice and straight, get up onto her hands and knees and has recently started crawling a few steps on her hands and knees!! She has been working really hard at her physiotherapy sessions and they predict that she will one day be able to walk with a walker!

Life for us now is so much different than we had ever dreamt it would be, but this is our “new normal”. Our life with Claire has made us appreciate the little things, made us passionate advocates, and has forced us to re-evaluate what is really important. Most of all it has made me loosen the death-grip I thought I had on my life and give it all to God who we believe is ultimately in control. Claire is beautifully made and made for a purpose. She had already touched so many lives. We know her future is bright and we feel so blessed to have a front row seat as we watch it all unfold.