Big Shot

Denny Sim is years beyond the “little kid” some acquaintances mistake him to be.

Although he is 4 feet tall and weighs 42 pounds--roughly the size of a 6-year-old boy--he is far from youthful.

A year ago, he had coronary bypass surgery, which doctors hope will prolong his life. He is hairless, his skin is wrinkled and he lives in a fragile body experts say is aging 10 times faster than average. His face resembles that of a man in his 80s, but Sim recently celebrated his 17th birthday.

Sim has progeria, a rare, incurable disease that accelerates aging and has a reported incidence of about one in 8 million newborns.

Sim knows all about progeria and its degenerative effects, that only 100 cases of the disease have been identified since it was first described in 1886 and that he is among 27 known current cases worldwide.

And he also knows he is an exception to the disease’s grim realities.

“Most kids with progeria don’t live past 13 years old,” Sim said.

And they don’t play tennis for their high school team.

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Medical experts say it is rare for a teenager with progeria to be as active as Sim. Most are in too much pain or have been rendered immobile by then. But Sim insists the problems he encounters on the tennis court have nothing to do with physical pain.

“The hardest part is losing,” he said.

Matt Oliphant, the tennis coach at Monroe High School in North Hills, said Sim, who plays on the junior varsity, has never complained of pain during workouts. Still, Oliphant keeps a watchful eye.

In a preseason workout in February, Oliphant instructed his players to run laps around the tennis court complex. When the players took off, Oliphant realized Sim was in the middle of the pack and called him out.

Told that he wasn’t required to run with the team, Sim asked to run smaller laps inside the courts. Somewhat reluctant, Oliphant obliged.

“That day really kind of turned me around to start treating him like the rest of the kids,” Oliphant said. “I try not to make him a special kid. And I think he likes it that way.”

The coach said Sim’s limitations haven’t kept him from becoming a formidable high school doubles player--or leader on the court.

“Whoever he [plays] with, he seems to take the leadership role,” Oliphant said. “All the kids love playing with him. They all want to be his partner because he’s good and he doesn’t give up a lot of points on mistakes.”

To compensate for his lack of size and strength, Sim, a junior, has an unorthodox playing style. He uses two hands on both his backhand and forehand. He stands well inside the baseline, in tennis’ “no man’s land,” taking balls on the short hop to save himself some running.

“He’s able to take whatever comes at him and reverse the power right back,” Oliphant said. “His technique is tremendous.”

For overhead shots, Sim draws upon the skills he mastered several years ago as a baseball player in the Mission Hills Little League Challenger program for disabled children. Instead of reaching up for the ball, Sim hits it on the fly with a waist-high baseball swing.

“It’s a very hard shot because the ball is coming straight down and if your timing’s not right, you’re not going to hit the ball,” Oliphant said.

Even so, Sim rarely misses or mis-hits.

“He’s a little more advanced than some people on the JV team,” teammate David Nguyen said. “He’s more consistent and hits the ball more direct.”

As many of Sim’s opponents have learned, appearances can be deceiving. Recently Sim and his teammate defeated a team from Sun Valley’s Polytechnic High School, causing the losing players to be ridiculed by their teammates.

“Obviously they hadn’t taken a real close look at how good he was,” Oliphant said. “They just looked at his size and expected to win. It didn’t happen, so when they came off the court, all of their teammates were saying, ‘You lost to that little kid.’ ”

Thomas Ji, a sophomore on the Monroe varsity, said size is the last thing he sees when he looks at Sim.

“I don’t even [think] of him as a small guy anymore,” Ji said. “It’s like when I see him, he’s just one of the guys to me. I don’t treat him any different.”

Surprising opponents with his athletic skills is Sim’s greatest joy.

“Since I’m little, they take advantage of me,” Sim said. “But then when I’m on the court with them, they’re really amazed at how I play.”

A Change of Countries

Denny Sim is the youngest of three children born to James and Anna Sim, who emigrated from Korea when Denny was 2 years old.

Sister Jane, 22, is a premed student at UCLA. Helen, 20, is a student at Wheaton College in Massachusetts.

“In a way, I feel I’ve raised him, too,” Jane Sim said. “My parents always worked, so I baby-sat him ever since I was in first grade.”

When Sim was an infant, Korean doctors diagnosed his affliction as a skin disorder.

“When I was little, I had hair and everything was normal,” Sim said. “Then, after a while, my parents noticed something about me was different. So they came [to America] to seek treatment.”

Doctors appointments were a family outing, Jane Sim said, mainly because the parents do not speak English and the children made fine interpreters.

But when doctors could not pinpoint their son’s disease, James and Anna--without health insurance at the time--decided more doctors appointments were useless, especially because Denny felt fine.

“They went initially because they wanted to find out what it was, not because he was sick or anything,” said Jane Sim, translating for her parents.

“[The doctors] never came to anything conclusive. My parents thought it was pretty hard on Denny, so they just stopped going.”

The diagnosis of progeria--which is called Hutchinson-Gilford syndrome when it affects children--came years later, when Denny Sim was 7. Doctors showed the Sims pictures of others afflicted with it.

“That’s when doctors told us he would only live to age 12,” Jane Sim said.

Denny was told of his life expectancy while in junior high. But even after receiving that devastating information, he never seemed overly affected by it, his sister said.

“The only moods I’ve ever seen him in is either happy or mad. I’ve never seen him depressed,” Jane Sim said.

Sim, who said he is constantly busy with homework and tennis, is as upbeat and active as ever. When he sets his mind to something, he is a bulldog, his sister said.

His parents, who tried to discourage their son from trying out for the tennis team, have learned it is easier to give in to his wishes, even if it causes them sleepless nights and heavy hearts.

“My brother is very stubborn, so whatever he says he’s going to do, he’ll eventually do,” Jane Sim said.

“He wants to do everything his friends are doing, so sometimes I feel like he’s being selfish. He’s not really thinking about his health. But he knows his limits. He doesn’t do things that he knows he can’t handle.”

The Sims say they do not dwell upon Denny’s disease. With few exceptions, they go about their lives like any other family with two parents working to maintain a comfortable lifestyle in a modest Granada Hills apartment.

As for Sim, his mortality is something he says he never thinks about.

“There’s no reason for it,” he said.

16 Years Without Incident

Most progeria victims suffer from dwarfism, baldness, a pinched nose, large head with a small face, stiff joints and cardiovascular problems. Their cause of death is usually a heart attack or stroke.

Sim, who said he went nearly 16 years without a serious medical problem, first experienced chest pains about 18 months ago. “Helen would sit up with him once in a while,” Jane Sim said. “He would get up in the middle of the night because it felt like somebody was pressing on his chest. He couldn’t sleep.”

Yet, with the exception of the pains that led to coronary bypass surgery a year ago, Sim said he rarely suffers from the ailments that plague others with progeria.

The difference may be Sim’s lifelong love affair with sports.

“Denny was always active since he was young,” Jane Sim said. “He didn’t like staying home. He always played outside.”

Doctors have told the Sim family that Denny’s physical activity has played a large part in maintaining his health. As long as sports don’t cause him pain or discomfort, Sim has been encouraged to continue.

“[The heart specialist] said his heart was very strong and the reason was because he has been active all his life,” Jane Sim said.

Dr. W. Ted Brown, the nation’s leading progeria researcher and world-renowned human geneticist, expressed surprise when told of Sim’s tennis exploits.

“Usually by age 17 they have had enough medical complications that they are not running around so much because they are subject to heart disease and degenerative problems with their hips,” said Brown, of Staten Island.

The cause of progeria is unknown, and its diagnosis is based on the physical traits associated with it.

It affects both sexes equally and all races, yet the victims have a remarkably similar appearance despite their racial background. Medical experts speculate the disease is caused by a single abnormal gene, mutated at the time of conception.

The oldest known survivor of progeria died at 29 in 1985, about two years after the Sim family came to the U.S.

Sim ignores such statistics. He has a driver’s license and loves to fraternize with his friends, play tennis at the park, go to at least one movie a week and stay out late.

He is a typical teenager, his sister said.

“Sometimes I ask my parents, ‘You know, you never let Helen and I stay out that late [in high school],’ ” Jane Sim said. “And they said, ‘Well, you guys are girls.’ ”

Indeed, even though the Sims are less restrictive with their only son, they are twice as concerned.

James Sim, a pool cleaner, fears he will receive a call from school officials telling him his son has had a heart attack.

“It’s on their minds every single day,” Jane Sim said.

In the living room of the Sims’ three-bedroom apartment, photographs tell a story of a happy, involved family. There is a volleyball picture that includes sister Helen standing among her teammates. On an adjacent wall is an 11-by-16 glossy of Jane, posing on a runway for the 1996 Miss Korea of Southern California competition.

Anna Sim, an assembler for a computer company, leaves for work at 5:30 a.m. Each workday, before she leaves the apartment, she stops to watch her son sleep.

“She’ll pray while he’s sleeping,” Jane Sim said, “and hope that everything goes well for the day.”

(BEGIN TEXT OF INFOBOX / INFOGRAPHIC)

Progeria

Premature old age. There are two distinct forms of the condition, both of which are extremely rare.

In Hutchinson-Gilford syndrome (Denny Slim’s type), aging starts around the age of 4, and by 10 or 12 the child has all the external features of old age, including gray hair, baldness and loss of fat, resulting in thin limbs and sagging skin on the trunk and face. There are also internal degenerative changes, such as widespread atheroscierosis (fatty deposits lining the artery walls). Death usually occurs at puberty.

Werner’s syndrome, or adult progeria, starts in early adult life and follows the same rapid progression as the juvenile form.

The cause of progeria is unknown, although cells taken from affected people show only a few generations off cell division before they stop reproducing, instead of the 50 or so generations that occur in cells taken from healthy young people.

--The American Medical Assn. Encyclopedia of Medicine.