“How’s kindergarten?” he asked, looking up.
Above him, sitting in a chair, was a dark-haired 5-year-old from Bridgewater, New Jersey, named Joey Hollywood. His parents, Suzanne and Joe, sat in the corner of the exam room. Joey liked towering over his doctor. He smiled down at Kaplan as he kicked his legs under one arm of the chair and then slipped them under the other. “I ride the bus,” he said.
“Joey,” Kaplan said, “let’s play Simon Says.” Kaplan stood up and slapped his hands to his sides. Joey swung out of his chair and stood as well. Kaplan twisted his head to the left to look at Joey’s parents. Joey did not turn his neck. Instead, he pivoted on his feet to turn his entire body. Kaplan turned back to Joey and raised his arms to the ceiling. Joey tipped up his hands at his sides.
“He’s quite adaptive,” Joe said. “At school they were horrified to find he was using his face to turn on light switches. So they gave him a stick.”
“Can we slip that nice shirt off?,” Kaplan asked. “I’m just going to check your back.”
Joey let Suzanne draw his shirt over his head, revealing two tangerine-size mounds on his back, each faintly filigreed with veins.
Joey was born with malformed big toes, like Peeper and most other people with FOP. A few months later, a lump appeared on his back. “When I saw it,” Suzanne told me, “I said, ‘That can’t be normal.’ ”
Joey’s symptoms came and went, but not until the fall of 2011, when he was 4, did it become clear that something was seriously wrong. Bones had grown in his neck, freezing it hard as stone. The Hollywoods were referred to Kaplan, who has replaced Zasloff as the world’s leading FOP expert. A few months later, Joey’s right arm fused to his ribs, and more swellings appeared on his back.
As Joey munched on pretzels, his parents asked Kaplan about the risks of hearing loss (in young patients, ear bones sometimes fuse together), and about what had happened to Kaplan’s other patients.
“I’ve seen 700 patients with FOP around the world, and it’s clear that there’s a lot of different ways to divide patients,” Kaplan said. One identical twin might be only mildly affected, while the other would be trapped in a wheelchair. Some patients developed a frenzy of bones as children, and then inexplicably stopped. “I’ve seen it go quiet for years and years.”
“So it’s very unpredictable,” Joe said, hopefully.
Suzanne looked over at Joey. “This is my son every day,” she said. “I don’t want to have him look back at his childhood and say, ‘My parents were always sad.’ ”
“When you’re here, we focus on FOP,” Kaplan told her. “Remember the things that are important and helpful for Joey to live as safe a life as he can.” He shrugged his shoulders. “And then forget the FOP.”
When Kaplan started out as an orthopedic surgeon in the late 1970s, he treated patients with a wide range of common bone diseases, such as osteoporosis and rickets. In the mid-1980s, however, he became interested in genetics. He suspected that for many of his patients’ treatments, a pipette of DNA would become more useful than a bone saw.