Rarities: Declan and Dandy-Walker Syndrome

Declan: Living with Dandy-Walker Syndrome

Declan was born with Dandy-Walker syndrome, a rare congenital condition shared by fewer than 50,000 people in the U.S. His care journey began in the womb when an ultrasound scan revealed two worrisome conditions: a small hole in his heart, known as a ventricular septal defect, and an underdeveloped brain. “He was our first pregnancy, our first child, and to get that news is tough,” said his mother, Krystal, “because they say something’s wrong, but they can’t say what’s wrong or how his life will look. And that’s hard. It was kind of just a wait and see.”

He was delivered via C-section at 37 weeks, suffering from hypoxia (a lack of oxygen to the brain). “It wasn’t quite the picture perfect experience you hope for,” said Krystal. Declan’s color wasn’t right and he wasn’t crying. “I knew right then, okay, something’s off,” she said. Declan’s Apgar score—which measures a newborn’s breathing, heart rate, muscle tone, reflexes and skin color—was very low. He was bundled off to the Neonatal Intensive Care Unit (NICU) at Danbury Hospital, part of Nuvance Health, a Connecticut Children’s Pediatric Care Alliance partner. It was there that Connecticut Children’s pediatric neurologist William Yorns, Jr., DO, suggested that Declan had Dandy-Walker syndrome, a diagnosis that would be confirmed by MRI a few months later.

A rare brain abnormality

Dandy-Walker syndrome causes underdevelopment of the cerebellum, the part of the brain that controls movement and balance. After two weeks in Danbury Hospital’s NICU, Declan was transferred to Connecticut Children’s NICU in Hartford—one of only two Level IV NICUs in the state—where there were more resources and pediatric specialists available to handle his complex care. After five weeks at Connecticut Children’s, Declan went home with a gastrostomy tube (G-tube) in his stomach for feeding and on oxygen to help him breathe.

“It was definitely a bit overwhelming, because he had a lot of equipment and there were some health concerns,” said Krystal. “We had a home nurse who checked in with us once a week, but it was kind of just us. We were the ones caring for him. I learned how to use medical equipment that never in my life I thought I would.”

Hitting new milestones

Today, Declan is six years old, in kindergarten and a big brother to two younger siblings. Like many children with Dandy-Walker syndrome, he has both physical and visual impairments. He still uses a G-tube for feeding, but he is making progress. “He’s doing things that I think we never expected him to do, which is really exciting,” said Krystal.

He remains nonverbal, but nonverbal doesn’t mean non-communicative. “He is a quick-witted kid,” said Krystal. “He actually knows and understands a lot more than people give him credit for.” Declan uses an iPad as a communication device and is working on making the “h” sound as his way of saying ‘hi’ to his friends. He also has a very distinct giggle.

“People love his laugh. It’s very contagious,” said Krystal. “He has a perfectly timed laugh sometimes where I’m like ‘Wow! He understood what we’re saying or he got the joke.’”

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Ongoing care in Westport

To keep those milestones coming, Declan receives ongoing care in a variety of specialties at Connecticut Children’s. He has frequent speech and occupational therapy appointments. He sees Dr. Yorns and a gastroenterologist every few months and visits pediatric cardiologist James Enos, MD, once a year to monitor his heart due to the ventricular septal defect. Connecticut Children’s new Specialty Care Center in Westport means Declan’s parents don’t have to drive far from their home in Fairfield to get their son the therapy and care he needs.