Girl Beats Life-Threatening Odds in Craniofacial Transformation

Claire Kubacki is a sweet, outgoing, good-humored 7-year-old who was born with a rare, malformed craniofacial structure. During the prenatal development process, the seams between the skull bones fused prematurely and her orbital (eye socket) and nasal bones formed abnormally, forcing Claire’s eyes to be asymmetrical and widely set apart, a condition called craniofrontonasal dysplasia.

Claire, 6 months

Otherwise, Claire was a normal, happy baby. Due to Claire’s rare medical condition, and the danger of surgery to transform her craniofacial structure, the family met with top surgeon teams from across the nation. "Many of them were top of their field in terms of talent and reputation," says her father Ryan.

When the family decided to move back to Chicago from Seattle, Daniel Kwan, MD, a resident in plastic surgery at the University of Chicago Medical Center and a personal family friend, as well as Joseph S. Gruss, MD, their surgeon from the University of Washington Children’s Hospital, highly recommended Medical Center plastic surgeon Russell Reid, MD, PhD.

The couple was immediately impressed. "When we met with Dr. Reid to discuss the surgery, he said 'I can tell you as much or as little as you would like.' When we opted for as much information as possible, Dr. Reid spent two hours with us going through every detail," says Ryan. 

Summer 2009: After Claire's first surgery

The surgery took place in January 2009. It involved cutting the bones of the orbit and resetting them into a new, more anatomically correct position. Chief of Neurosurgery David Frim, MD, PhD, provided critical assistance by removing the frontal (forehead) bone, offering access to the base of the skull. The surgery is technically demanding because vital structures such as the frontal lobes of the brain and optic nerve are situated nearby. "In a surgery fraught with potential life-threatening complications, Claire’s went extremely well," says Dr. Reid.

Demonstrating her playful sense of humor, the first words out of Claire’s mouth once the breathing tube was removed the next day, was "Nice job guys," when the surgeons came to check on her.

Comfortable reading was among the many things we take for granted that Claire struggled with since early childhood. Previously, Claire tilted her head to the side and used one eye to read until becoming fatigued or too frustrated.  "'Silent reading' was Claire's favorite subject in first grade because she could rest her eyes and not actually read," her mom, Jana, explains. Now she loves reading. "Claire was enjoying reading books within just three weeks."

The family’s positive assessment of the Medical Center goes beyond the surgical team. "When it comes to bedside manner, accessing information, and making Claire feel comfortable, everyone was great," says Ryan. "The nursing team, especially in the pediatric ICU, was on top of its game. They worked hard to keep Claire's body comfortable and her spirits up."

"U of C has great systems to be able to pull up everything on a desktop -- scans, test results, medical records. Everything is convenient and comprehensive. We really appreciated that," adds Jana.

While Claire was in the PICU following surgery, her parents noticed a plaque commemorating a gift by Terence and Susan Graunke to support the PICU. Ryan and Terence knew each other from various business and community meetings. Familiar with the couple’s leadership and integrity, the Graunkes support of Comer Children's Hospital at the University of Chicago was "One more assurance that we were in the right place," says Ryan.

Medical staff treated the Kubackis like valued customers more than patients, according to Jana. "Dr. Reid took personal ownership of Claire's entire experience, not just her surgery.  When we wanted to speak with him, he made himself available," she says. For instance, when the couple had an insurance question, they emailed Dr. Reid and were shocked that he emailed back within a few minutes having already called the insurance company himself to clarify the question. 

Russell Reid, MD, PhD

"I couldn’t ask for a better surgeon than Dr. Reid; not just technically, but as a person," says Jana. "His surgical work is miraculous, his bedside manner is priceless. He and Dr. Kwan are the type of guys who personify why doctors become doctors."

"During her eight-day stay, Dr. Reid came to see Claire nearly every day and each visit put a smile on her face.  Claire had full confidence in Dr. Reid and knew that he was there to help her," says Jana.

The Child Life staff also made Claire’s inpatient stay more comfortable and fun. "They made it clear to her that they’re only there for fun. They wouldn’t be administering any meds, no needle pokes, no changing bandages. Just play," says Jana. Adds Ryan: "Their visits for arts and crafts really took Claire's mind off where she was. Claire looked forward to seeing the Child Life staff."

"We waited a long time for everything to be right, and everything just fell into place."

Claire is awaiting an eye muscle balancing surgery tentatively set for late July. The orbits have to be set and healed, and the swelling resolved first. Much less time-consuming than her first procedure, the muscle balancing surgery will require a very brief recovery time.

Claire and a new baby cousin in June 2009. Claire is scheduled to have eye muscle balancing surgery at Comer Children's Hospital.

Despite her facial difference, Claire has always had a good sense of self, according to Jana. "Unfortunately, society has a different perspective," she says. While the physical and functional benefits are the most important reasons for the surgery, Jana says the psycho-social effects will become critical as Claire gets older. Months after her surgery, Jana says she can bring Claire into a store without people staring at her. "We go up and down the aisles without those long second looks. You don't realize how you anticipate those stares until they are thankfully gone."