CINCINNATI – Valeka Riegel and her “miracle” baby Zakary are celebrating his first birthday Friday thanks to the team of surgeons and caregivers at Cincinnati Children’s.
Zakary was born with an encephalocele – a rare, dangerous birth defect that caused tissue to protrude in a sac from his skull and cover most of his face. He needed surgery to reconstruct his brain and skull as well as his face - and to determine if the tumor was cancerous.
“You could see it on the ultrasound," Riegel says in a video produced by Children’s. “So, of course, I did what we should never do – I got on the web and googled it - and that’s when my panic began.
“Basically, the growth covered his face from his eyes down to mouth. When you looked at him as a baby, all you saw was a little mouth.”
Zakary’s mom recounted the whole grueling experience in “A Letter to My 1-Year-Old Son, Who Had an Encephalocele,” on the Children’s blog. Here’s an excerpt:
"From the fifth month of pregnancy, I learned of your encephalocele and the choices I needed to make for both of us. In shock, I stared at the fetal MRI and ultrasounds because they were not consistent with the vibrancy I felt with every twist, turn, punch and jab. I knew then you were and still are a fighter.
"For five months, I cried, became angered, fearful and resentful of God. Why would he bring you into my life, at the age of 45? Only to become a statistical probability of surviving 1 in 5,000 live births? I was confused. As a nurse, I had medicine to believe in. But as a mom, I chose to surrender to my faith.
"From the moment of your birth, we were separated into two hospitals. I saw you only for a second, because I hemorrhaged and was immediately intubated by the ICU team. They saved my life, while you were down the street fighting for yours. I could not see your face, only a large protrusion and tiny little lips – no eyes, no nose, no eyelashes. Yet, you were still beautiful to me."
Zakary was treated in the Newborn ICU for four-and-a-half months while he grew strong enough for surgery. Dr. Charles Stevenson, pediatric neurosurgeon who led the team involved in Zakary’s care, explained how complicated it was.
“There were several steps involved in terms of exposing the forehead, exposing the defect, amputating all of that tissue, preserving the normal brain, and then reconstructing all of the normal layers or barriers which had formed,” Stevenson said.
Stevenson said he called on a friend, Dr. Brian Pan, to handle the reconstruction of Zakary’s face.
“I knew we would need a complex reconstruction of his face and his nasal bridge in order to make this work,” Stevenson said.
When the nine-hour surgery was finished, there was good news all around. Zakary's tumor was not cancerous, and Zakary’s mom couldn’t believe what she saw.
“I walked into that room and I cried because I just couldn’t imagine seeing a face – seeing his eyes, seeing his nose. I kept thinking, ‘There’s really a baby in there!’" she said.
“The first thing he did after extubation was smile, and 23 hours later he was out of the pediatric ICU,” Riegel said.
When Zakary was born, his mom lived in Toledo with her two other sons and commuted to visit Zakary in the hospital. She has since moved her family here to be closer to his caregivers.
Although Zakary is behind in his verbal development, he began to crawl this month and stood up for the first time. His surgeons said they were thrilled with his progress and prognosis.
"He's just going to do it on his own timeline," Pan assured his mother.
Stevenson agreed and said no further surgeries should be required.
“He seems to be attaining milestones in a time frame we would expect,” Stevenson said. “From here on out, we’ll just be following him in order to maximize the aspects of cosmetic reconstruction.”
Pan said he's looking forward to watching Zakary grow up.
“Watching these kids grow up, watching them thrive and really return to a normal life … that’s what I appreciate most about working in pediatrics,” said Pan.
At the end of Riegel’s emotional letter to her son, she thanks the Children’s staff, says the experience confirmed her faith and expresses her unconditional love to her third son.
"I am forever grateful to all the doctors and care providers at Cincinnati Children’s. In particular, I am so thankful for Dr. Stevenson who said “we can” when other providers said “we can’t.” And for Dr. Pan who helped unveil your truly hidden beauty.
"I don’t know what lies ahead for our journey, but I do know we will cherish every moment we are granted together. I will never allow you (or myself) to say “I can’t.” We can endure all things if we are willing to accept life as it truly is – perfectly imperfect. We will love one another as we were designed to do —unconditionally.
"Happy Birthday, Zak! I Love You, Mom"