Healing Zade: Baby undergoes eight-hour surgery to fix rare genetic abnormality

From s.e.Parent magazine

By the time he was four months old, Zade Darin Hickey had his own craniofacial plastic surgeon, pediatric neurosurgeon, hematologist and dermatologist. He'd been subjected to IVs, morphine, blood transfusions, an array of healing creams and ointments, and well over 150 stitches in his head.

Three months later, his fuzzy newborn hair barely covers the scar that zigzags across his skull, ear to ear -- the only physical reminder of the eight-hour surgery Zade underwent to fix a rare genetic abnormality.

Zade was born June 29, 2008 -- just one day before a scheduled c-section. His mother, Sarah Hickey, recalls that he was breech hrough most of her pregnancy, a position that sometimes causes the baby's head to be elongated or misshapen. When the doctor started asking questions, though, Sarah and her husband Darin knew the inquiries had nothing to do with the breech position.

Sarah and Darin recognized almost immediately that their newborn had craniosynostosis, a rare genetic condition that causes the sutures of the skull to fuse prematurely and severely misshape the head. About 30 years ago, Darin's head had looked much like Zade's at birth -- bulging at the front and back -- and he had undergone corrective skull surgery at only seven weeks old. Like his father, baby Zade was in for a difficult few months.

KIT DOYLE ~ kdoyle@semissourian.comZade Hickey's craniosynostosis is a rare genetic condition that he inherited from his father Darin, who had a similar surgery when he was a child.

In addition to a misshapen head, craniosynostosis may cause sunken eye sockets, retardation, a cleft palate, kidney or liver problems, at least 10 other major syndromes, or even death. Doctors know little about the condition, except that it is genetic, and only within the last year did they find the gene tied to it. Zade hasn't had any of the related problems, and thanks to Darin's history with the condition, the Hickeys and their doctors caught the problem sooner than most would.

"He didn't have any (symptoms), and that was enough. We're very blessed," says Darin, noting that it is very rare to have a stand-alone condition, or the skull abnormality without any other symptoms. When Darin was a baby, he was delayed in learning to walk and losing his first tooth, and he says his mother still wonders if it was because of the craniosynostosis. No one else in Darin's family has had the condition. "We're just happy they said everything was all right with Zade," he says.

At nine days old, Zade had x-rays and doctors officially diagnosed him. Two days later, the family traveled to St. Louis Children's Hospital to meet with a pediatric neurosurgeon who gave them two options for surgery: endoscopic, in which doctors make two incisions, remove sections of the skull and mold it back together; or open surgery, in which doctors cut around the entire scalp, cut out slivers of bone, and put the skull back together. Though the first type would take only two or three hours and be an overall lower risk, future surgeries would be inevitable, and Zade would have to wear a protective helmet for about a year. The Hickeys opted for the open surgery, which would last up to 10 hours and involve more blood loss, but would solve the problem in only one procedure -- a "one shot deal," says Darin. They set the date for Oct. 21, 2008.

In the weeks leading up to the surgery, Zade cried constantly and hysterically. Doctors explained that he was in pain because his brain was putting so much pressure on his skull, which was only two to three centimeters thick in some areas. However, they said that as soon as the skull was cut during surgery, Zade's brain would pop into position. "When I heard that, I thought, 'How could your head not hurt?' Every parent knows that when your kid hurts, you hurt right along with him," says Darin. There was no way to comfort Zade, says Sarah, and it was scary because she never knew if he was crying over a bellyache or a headache. "It was more stressful to me not to have the surgery," she says.

The entire family traveled to St. Louis for the surgery, including Zade's two-year-old sister, Ashlynn, and both Sarah and Darin's parents. They stayed in the attached hotel, but Darin says he spent every second he could with his son during his five days in the hospital.

"To see your four-month-old go behind those doors and know he'll be out for eight hours, that's kind of nerve-wracking," says Darin. That morning, he was so nervous that he got sick to his stomach and found it impossible to sit still for the rest of the day. Medical staffers updated the family every hour of the surgery, but nothing could keep Sarah from crying and "losing all control" when Zade was taken for surgery.

"The surgery was the worst part," she says. "It was the longest time ever. There is nothing to occupy your time ... I just stared into oblivion. I didn't eat or drink ... there was no way I was walking away from that phone."

The family saw Zade almost immediately post-surgery, but he was far from better. "His head was round and beautiful, but he was very pale," Sarah recalls. He had had one transfusion during surgery, and needed another one 45 minutes into recovery. Although both Darin and Sarah had already volunteered to give blood, doctors had advised against it. If Zade ever needed blood or an organ in the future, his body would already be resistant to his parents' blood. Instead, both of Zade's grandmothers donated blood for his two transfusions.

Post-surgery, Zade's eyes were swollen closed. He had an IV in each leg, one in his arm, a drainage tube behind his ear, and boards fastened to his arms and legs. By day four, the Hickeys had permission to pick him up, but he screamed as soon as they touched him. His entire back was red and blistered -- an allergic reaction to either the pad used to ground the cauterizing and cutting instruments during surgery, or to the adhesive used to place the pad on the skin. "It looked like a third degree burn," says Darin. A dermatologist gave Zade various ointments and creams to heal the burn as well as the incision on his head. Because doctors were worried about the possibility of a staph infection, Zade stayed two additional nights in the hospital.

Today, seven-month-old Zade has made a complete turnaround. In the first week home, Sarah and Darin noticed an immediate difference in their baby boy, who now smiles constantly. His incisions and back burn healed within two weeks, but doctors say he will have headaches for six months after the surgery. For the next few years, Zade will also have annual checkups to make sure he is developing and recovering as he should. And while Darin still has 25 to 30 staples, several divits, and a scar running from the front to the back of his head -- his own surgery was largely "experimental" in 1977 -- Zade is left with only a scar, cut in a zigzag pattern so that his hair will someday grow over and completely cover the marks.

"I don't have anything bad to say about the experience, other than it had to happen," says Darin of his son's surgery. "It was the worst day ever, but in the same token it was the best day ever because they fixed Zade."

The experience has also made the Hickeys appreciate how blessed they have been. The kids who go to St. Louis Children's Hospital have major issues, and many stay there so long that all their parents know each other.

"Sitting in that waiting room was humbling experience," says Sarah. "The other kids have major anomalies that made our little skull surgery look like nothing."

They say they couldn't have made it through the experience without support from each other, and especially Ashlynn, because it's impossible to be sad around her. "We've been each other's lifeline," says Darin. God, friends, family, their churches, and even people they'd never met offered support and encouragement.

"We had faith in Jesus and the Lord. We couldn't get through this without prayer," says Darin. "I have no doubt that someone else had a hand in this."

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