Hopes rest on screw turn

Healing halo:  Ira Berryman, 5, with sister Edith, 8, and parents Benj and Maria. Ira has Apert syndrome, a disorder that causes deformation of the skull, and has undergone more than a dozen operations.
Healing halo: Ira Berryman, 5, with sister Edith, 8, and parents Benj and Maria. Ira has Apert syndrome, a disorder that causes deformation of the skull, and has undergone more than a dozen operations.

Every day the bones in Ira Berryman's face are screwed a little bit further forward, all in the hope that he can live the life of any other young boy.

The first five years of the Wellington child's life have been marked by at least 16 operations to correct a rare genetic disorder, called Apert syndrome. Ira was born was a deformed skull, a sunken midface, and fused fingers and toes.

"It's when the bones grow different and they can't do as many things as other people," sister Edith, 8, explained.

One of Ira's earliest operations was to widen his nasal cavities to allow him to breathe. He has since had his skull cut open to relieve pressure on his brain, springs loaded into the back of his head, and his fingers surgically separated.

He couldn't use his hands or eat properly until he was 1, and the disproportionate size of his head meant he was slow to walk and still a little clumsy.

"Those first two years were pretty full on," father Benj said. "I quit my job for a year because we really needed someone fulltime, he was in and out of hospital so much."

The latest operation, done about 10 days ago by Wellington surgeon Charles Davis, is the one his family hope will allow him to start living a more normal life.

It involved peeling back Ira's facial skin, cutting away the middle of his face, and fitting his head with a metal frame, or halo, screwed into the top of his skull.

Every day, twice a day, parents Benj and Maria take out a small purpose-built screwdriver and move Ira's frame 0.5 millimetres forward, widening the gap between the bottom and top of his face. After about a month, Ira's face will have shifted more than 2.5cm, giving it more normal proportions, but also helping him breathe more easily.

Maria said helping him breathe would have the biggest impact on his development, allowing him to sleep for more than an hour or two at a time without choking himself awake.

"He is just knackered a lot, and his development at school is getting a bit behind.

"We just want him to be able to have a normal life."

In many ways, he is already like many 5-year-old boys: he loves cars, and thinks his purple halo is "girly". His parents hope that, with this latest surgery, he can catch up with his mates at Ngaio School.

"There is no reason why he can't go to university and get a good job," his father said.

Davis, one of only a handful or surgeons performing this sort craniofacial surgery in New Zealand, said he did about four procedures a year, in which the face of a child is essentially cut out and dragged forward.

He usually waited until children were older for the surgery, but Ira had been having particular trouble breathing, with his sunken midface squashing his airways.

"The operation is really about giving his brain more oxygen."

The Dominion Post