Weekend America

Second Skeleton

Kerry Grens

JANUARY 10, 2009

Justin Henke

(Courtesy Wendy Henke)

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Basketball season is starting for kids around the country. A team of elementary students in Delaware starts practice this weekend at the Glasgow Reformed Presbyterian Church. Parents can expect their kids to come home with scrapes, bruises, perhaps even the occasional sprained elbow or jammed finger. For one family, the consequences can be much more severe. Even the slightest injury to eight-year-old Justin Henke can inflame a dormant and destructive disease lurking within his genes. Reporter Kerry Grens spent a day with the Henkes to find out how they make it through sporting seasons.

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At a pool in Newark, Del., a dozen kids leap in for swim practice. For the most part, eight-year- old Justin looks just like the other kids, except for a striking crookedness to his big toes.

"At birth, actually, the pediatrician at the hospital had noticed, and we had noticed, too, the peculiar big toes that he had," says Wendy Henke, Justin's mom. "The pediatrician said, typically if there's a symmetric abnormality like that that, it might indicate a genetic condition."

The Henkes were living in Baltimore at the time. Justin was an infant. They took him to Johns Hopkins for an evaluation, but the doctors couldn't diagnose him. Over the next six years, there were other signs something was wrong.

"When he was crawling, he really couldn't lift his neck up to see where he was going," explains Wendy Henke. "With getting dressed, he has a limited range of motion in his elbows. But we just got him some bigger shirts."

"I remember going to amusement parks and water slides and trying to notice people's toes to see if anybody else had the same thing," says Justin's father, Kevin. "But it was always in the back of your mind, gnawing at you, what could it be?"

And then one night a couple of year ago, Wendy was up late.

"I shouldn't have been reading online. I should have been in bed. And I just happened to notice on Yahoo News one of these peculiar news items that said, 'Gene Discovered for Rare Stoneman's Disease.'" When she clicked it, she says it sent chills through her body. "As I read each paragraph, I just knew this was what it was, even though I didn't want it to be."

Stoneman's Disease is also known as Fibrodysplasia ossificans progressiva. Fred Kaplan, a professor at the University of Pennsylvania, was the scientist who found the gene for this disease. He's Justin's doctor and the doctor to all 700 people in the world who have what most people in the field refer to as FOP.

"A child is born with normal muscles, and then one morning they wake up and maybe they bruise themselves or injure themselves," explains Kaplan. "And then the next day or a couple days later, this metamorphosis begins to occur, and within several weeks what was once a muscle now becomes a bone."

Did you catch that? Just a little bruise -- or sometimes being sick from the flu - and muscle turns to bone.

"If you try to remove this extra bone," says Kaplan, "it leads to catastrophic explosions of new bone formation. So surgery is not an answer."

Eventually, that newly formed bone locks the person into a second skeleton. But it's not the type of skeleton you or I have. It's a highly deformed one. And it twists the body into strange and sometimes painful poses. Justin's muscles haven't yet started their transformation. For him, FOP is more a lifestyle restriction than a disease.

"We can't do, like, rough things," explains Justin, "like football and doing headstands or handstands."

Justin's parents say they don't know how much Justin grasps about FOP, just that he knows he has special bones and has to be careful not to get injured. And the full ramifications of FOP are still a mystery.

So far, Justin's FOP has been mild, and the Henkes are hopeful that maybe it will stay that way. But FOP is referred to as a sleeping giant - ready to spring into action when the right trigger comes along. One boy lost all movement in his neck after just one week of painful swellings. The shoulders of another girl, by the time she was a teenager, had twisted nearly perpendicular to her hips. You might think parents of a kid who has this in their future would want to keep them in a bubble. But Justin's parents restrict him very little.

"I like to play basketball, soccer, and swimming," says Justin. "I really want to do tennis."

At swim practice, coach Sandy White says he doesn't go out of his way to treat Justin differently.

"We tend to keep our eye on him, but he dives without any problem," says White. "And he swims all the strokes. He's doing good!"

There's something lurking inside of Justin that could be awakened by an activity like swimming. But his parents have chosen not to watch over his every move. After about 20 minutes, Wendy leaves swim practice. She goes for a jog as the kids do laps, while her husband Kevin is at the office.

"FOP patients, they need to be able to enjoy their childhood," says Kaplan, "because that might be the most freedom they have in their lifetime."

And this weekend, freedom for Justin means shooting hoops. In Philadelphia, I'm Kerry Grens for Weekend America.

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Practice starts for the Justin's basketball team the Lions this weekend, and the coaches and players know about Justin's special bones. His parents also look to him for guidance on his activities.

When Justin's not feeling well or he's in a little pain, he'll serve as the assistant manager instead of being on the court.

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