Cornelia de Lange Syndrome: Andrew’s Story

There’s a mischievous sparkle in Andrew Patitucci’s eye. He’s pretending to ignore his mother’s conversation, but despite being born deaf, this 11-year-old misses nothing.

When the conversation turns to whether Andrew, who has Cornelia de Lange syndrome (CdLS) and is mostly nonverbal, knows the alphabet, he instantly switches his iPad to a language app and starts to hit the letters: A, B, C. And he grins. So there!

andrew It’s a not-so-subtle message to never underestimate him, despite the challenges he faces. He had been working a word program, Proloquo2go, on the iPad, and kept pointing to bowling balls and bowling pins — obviously angling for a recreation stop on the way home from his appointments at The Children’s Hospital of Philadelphia.

Before that, he was whirling through hundreds of photos on an iPhone, stopping the flow now and then when a picture aligned with the discussion.

“You can see how smart he is,” says his mother, Beth. “It’s amazing what he can do with the iPad. Everyone here says he inspires them to look for more ways to help children with CdLS.”

“Here” is CHOP and its Center for Cornelia de Lange Syndrome and Related Diagnoses. When Andrew was born in 2001, there was no formal CdLS center, but there was Ian Krantz, MD, now the center’s director.

A center for children with CdLS

When Andrew was 3 months old, Beth and her husband, Paul, brought him to CHOP. Even then, no other facility had the same level of expertise in this rare disorder, which affects one in 10,000 children.

Before the center opened three years ago, Beth participated in a focus group to give the parent perspective on what it should provide. She wanted it to reflect what she considers an unmatched CHOP asset: coordination of care so children can see specialists who are familiar with CdLS and its complications (such as limb differences, developmental delays, autistic-like behaviors, small stature, cardiac problems and GI issues). The center holds a monthly multispecialty clinic where children can been seen by a variety of specialists on the same day.

Beth has also been an asset for the center.

“Sometimes, when a family is coming to the clinic for the first time, I’ll get a call: ‘They’d love to talk to someone who’s been here.’ I’m always happy to talk about my experiences,” she says.

The Patituccis have hosted events for families affected by Cornelia de Lange syndrome and related conditions — giving parents a place to connect and share their stories. If there’s a research study, they volunteer.

“If we can help, we want to help,” Beth says.

Reaching his potential

In his 11 years as a CHOP patient, Andrew has been hospitalized several times, mostly for GI issues. He also sees specialists from ENT, genetics, neurology, nutrition, orthopedics, ophthalmology and developmental pediatrics.

“He looks forward to coming to CHOP,” Beth says. “We always make it fun. He loves to ride the elevator, visit people and go to the atrium to see the ball sculpture. For him, it’s a fun place.”

For Beth, CHOP has been more than a clinical resource; Drs. Krantz and Matthew A. Deardorff, MD, PhD, have been supportive as she tried innovative ways to help Andrew with the iPad, for example, or by giving him real food, blended into a liquid, through his feeding tube.

“We respect each other and collaborate in the truest sense of the word,” says Beth. “We have the same goal: Help Andrew reach his fullest potential.”