Spina Bifida: Ciarlo’s Story

It was a hectic day when Kira learned she was pregnant with her second son. Her first son, Dominic, had accidentally locked himself in the bathroom of their new home. The roller-coaster ride would continue when the family learned their baby had a serious birth defect.

Ciarlo at CHOP A 19-week ultrasound revealed that Ciarlo had spina bifida, a birth defect in which the spinal column doesn’t close completely during development, leaving the spinal cord and surrounding nerves exposed through a hole in the back. The exposure to amniotic fluid in the womb causes progressive damage to the spine which can lead to a variety of issues, including bladder and bowel problems, club feet, paralysis, and weakness or loss of feeling below the location of the opening.

Kira and her husband Ken were shocked. Their obstetrician at Abington Hospital told them right away to go to The Children’s Hospital of Philadelphia (CHOP), which has extensive experience with prenatal diagnosis and treatment of spina bifida, including fetal surgery, as well as a specialized Spina Bifida Program.

“He didn’t sugar coat it — he said it’s going to be difficult, but he was positive and gave us hope,” says Kira.

Treatment options

Kira and Ken traveled from their home about an hour away for a day of evaluation and advanced diagnostic testing at CHOP’s Center for Fetal Diagnosis and Treatment. There, the multidisciplinary team, including fetal imaging specialists, a fetal surgeon, a neurosurgeon, a maternal-fetal medicine specialist and a nurse coordinator, gathered additional detail about Ciarlo’s diagnosis.

Julie Moldenhauer, MD, sat down with the family to explain the diagnosis and treatment options. She walked through Ciarlo’s test results in detail and explained what everything likely meant for his future.

Ciarlo’s spine was exposed at the L1 level. The location of the lesion determines the extent of damage and problems expected in the future.

“She gave us a lot of hope, but not false hope, which is so important,” recalls Kira. “She was honest and to the point. She made us feel this was not a death sentence, and that our son could have a great life — just a different life than what we might have expected.”

The team planned for Ciarlo to undergo traditional postnatal surgery shortly after birth. Prenatal repair is another treatment option for babies diagnosed with the most severe form of spina bifida.

A cesarean delivery was planned in CHOP’s Garbose Family Special Delivery Unit (SDU). The SDU is the first birthing unit located within a children’s hospital designed for healthy mothers carrying babies with known birth defects who will require immediate care after delivery.

Preparing for the unknown

In addition to preparing for the delivery, Ken and Kira had to plan for the hospital stay, Ciarlo’s surgery, the travel back and forth to CHOP, and caring for 2-year-old Dominic.

While so much was unknown, the team at CHOP did everything they could to prepare the family for what to expect and answer all of their questions.

“We knew the treatment plan down to the smallest detail,” says Ken. “It was comforting to know they were both in the best possible hands.”

On Aug. 31, 2010, Ciarlo was born in the SDU.

“He came out screaming bloody murder, and I figured well, that’s a good healthy cry, he’s a fighter already,” says Kira.

Ciarlo was taken right next door to an infant stabilization room. The SDU allows mother and baby to stay close while having immediate access to highly specialized care. After being stabilized, the team brought Ciarlo to Kira’s bedside before taking him to the Harriet and Ronald Lassin Newborn/Infant Intensive Care Unit (N/IICU), just a short walk away.

Ongoing care

Less than 24 hours after Ciarlo was born, pediatric neurosurgeons performed the delicate surgery to close the hole in his spine. The procedure went well and while he recovered in the N/IICU, Ciarlo began the next chapter of his journey to manage the complications the condition had caused in utero.

Like many patients with spina bifida, Ciarlo developed hindbrain herniation, where the hindbrain descends into the upper portion of the spinal canal in the neck, blocking the circulation of cerebrospinal fluid. This blockage causes hydrocephalus, an accumulation of fluid in the brain. Ciarlo has a shunt to relieve the pressure on his brain caused by the buildup of fluid, and walks with braces.

He is followed by CHOP’s specialized Spina Bifida Program, where a multidisciplinary team, including pediatric urologists, neurosurgeons and orthopedic specialists, work together to provide coordinated care.

“The doctors and nurses are amazing. All his different specialists, they work as a team and care for him in a way that is above and beyond. They know his diagnosis and truly understand spina bifida and everything going on with him,” says Ken.

Ciarlo has won the hearts of his medical team, but they’ve won his as well. He has his favorite nurses who he talks about all the time at home, and he loves his doctors. According to his mom, he actually gets excited to go to the hospital. “He’ll have a cough and say, ‘Oh, I think we have to go to the hospital Mommy!’” says Kira.

“CHOP has become our home away from home. Everyone there has become part of our family.”

“The Mayor”

Now 5 years old, Ciarlo started pre-kindergarten this fall. Energetic, intelligent and chatty, he has been nicknamed “the Mayor” because he loves to talk to everybody and make friends.

When his parents think about his future, they imagine an independent boy who conquers anything that holds him back and does what makes him happy. They see him achieving big things, maybe just going about it in a different way than others.

Every time the Liples family comes back to CHOP they make sure to go up and say “hello” at the CFDT and the N/IICU to thank the people working so hard to help babies like Ciarlo.

During those visits, they’ve also made several deliveries of slippers with a special note attached to let other moms in the SDU know that they’re not alone in their journey, and to offer some comfort when they walk down the hall to visit their babies in the N/IICU.

"Almost every time we visit, we meet a spina bifida family or two who are pregnant and coming in for that first consultation," says Kira. "They get to see him, and I think he has given so many people hope when they didn’t have any.”

Watch the video to meet Ciarlo and his family.

 

Originally posted: September 2015
 


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