Youngest patient to have new surgery has improving vision

PHOTOGRAPHER: Sara Foss

Corey Haas’ schoolwork had always been printed on 11-by-17 inch sheets of paper, in large, easy-to-read type. His books were in extra-large print.

But after Corey underwent experimental surgery to improve his vision, the school began printing his classwork on regular-sized pieces of paper. He began using regular-sized books.

His vision is still far from perfect, and improvement is expected to be gradual. But he and his parents have already noticed big changes.

In September, the 8-year-old from Saratoga County became the youngest person in the world, and the second American, to undergo the groundbreaking surgical procedure, which uses gene therapy to treat a rare, degenerative eye disease called Leber’s Congenital Amaurosis. So far, 10 people have received the surgery, seven at the Children’s Hospital of Philadelphia, where Corey went, and three at Moorfields Eye Hospital in London.

Corey suffers from night blindness — the inability to see in dim light. His vision is so poor that he is considered legally blind, and the scope of what he sees — his visual field — is restricted. While people with normal eyesight have a circular field of vision, Corey’s field of vision is shaped more like a potato. He is also extremely nearsighted.

“It will be several months before we notice dramatic changes,” said his father, Ethan Haas, during an interview at the family’s Hadley home. “But he’s already moved down a line on the eye chart.”

At school, Corey still uses a special “clarity” machine, a 12-inch computer monitor hooked up to a camera, to magnify and brighten the writing on the chalkboard. He reads worksheets with the aid of a lamp with a 100-watt bulb and a magnifying glass. His equipment occupies a second desk. But it’s getting easier for him to see faces. The pupils of his eyes, in the past always fully dilated, began changing shape after the surgery, becoming smaller in bright sunlight.

“That was the most blue I’d seen in his eyes in years,” recalled Ethan Haas of the first time he noticed the change, on a trip to the Philadephia Zoo. “We told the doctors that the sun was bothering his eyes, and they were like, ‘Good.’ ”

The Haases found out about the surgery in April, through their doctor at the Children’s Hospital in Boston. The surgery is still considered experimental, which means it has yet to be approved by the Food and Drug Administration, but Corey’s parents didn’t hesitate. Because Leber’s is degenerative, Corey’s vision was expected to worsen over time, with or without the surgery.

“In his mid-20s, he would probably be completely blind,” said Ethan Haas, 36, who works in the maintenance department at International Paper in Ticonderoga. “We figured, ‘What’s the harm?’ … It’s kind of a no-brainer. Even if it doesn’t correct [his vision], it’s supposed to stop it from getting worse.”

rare diagnosis

From an early age, it was clear that something wasn’t quite right with Corey. As an infant, he didn’t make eye contact while feeding, and he was always staring at lights. By 10 months old, he was wearing glasses. But it wasn’t until 2006 that Corey was finally diagnosed with Leber’s, which affects approximately 3,000 people in the United States.

“He used to bump into a lot of stuff,” Ethan said. “He was using a cane. He was learning Braille.”

The disease makes things more difficult, he said.

“It’s pretty frustrating when he can’t see something or can’t pick up something because he doesn’t see it,” he said. “If he goes to a friend’s house and it gets dark, you have to go get him.”

Leber’s, which prevents the retina from processing light, is caused by recessive genes, which means both parents carry the genes and pass them on to their child.

The experimental surgery Corey received was developed by a husband-and-wife medical team, eye surgeon Dr. Al Maguire and gene therapy expert Dr. Jean Bennett, at the Children’s Hospital of Philadelphia. The couple figured out how to insert a virus carrying a healthy copy of the defective gene into the retina using a tiny needle, about the size of two eyelashes. During surgery, billions of genetically modified viruses were delivered to Corey’s retina; these viruses were able to produce an enzyme Corey’s eye was incapable of making on its own.

In interviews, researchers have said they hope the gene therapy technique could be used to treat a wider variety of visual disorders, such as macular degeneration.

So far, so good

Because the Haases agreed to participate in a long-term study, the surgery was paid for by the Children’s Hospital of Philadelphia and the University of Pennsylvania. Corey traveled to Philadelphia last week for a check-up; such visits will become less frequent over time.

“I can see better,” said Corey, a soft-spoken boy who wore a Batman cape during the interview. He said he’s glad he had the surgery, “rather than just going totally blind in the future.” There is still a bright red spot on his left eye from where the needle was inserted during surgery.

Even though Leber’s makes life more challenging for Corey, it doesn’t prevent him from doing what he wants to do.

Bright lamps are set up on tables in the living room and dining room to make it easier for him to see when he is playing with toys or doing his homework. He likes to draw and showed off detailed sketches of robots. He likes to build things with Legos. When he plays video games, he sits close to the screen. He can ride a bicycle. He plays tennis using special balls that beep.

Corey, a third-grader at Stuart M. Townsend Middle School, has an excellent memory and a very good sense of smell and hearing, his parents said.

“You can read him a book, and if you miss a word, he’ll tell you,” said his mother, Nancy Haas, 41, who works at Lowe’s in Queensbury. “He can memorize the eye charts.” (Doctors are aware of this and adjust the eye charts accordingly). “If a guy across the street is burning a fire, he can smell it before I can smell it,” she said.