The medical textbooks give a dry, clinical description of the congenital deformity known as Crouzon syndrome: The sutures of the skull fuse prematurely, sometimes at birth, trapping the growing brain. The face is sunken, and breathing may be restricted because the nasal cavity develops improperly. The eye orbits are shallow so the eyes bulge forward, sometimes to the point that the eyelids cannot close. The lower jaw, which grows normally, quickly overtakes the upper jaw, creating an uncomfortable underbite.
But when you’re 10 years old, like Chelsea Mulder, the main thing about having Crouzon is that you might–even just once, and even if he apologized right away–hear some boy yell out to the whole playground that no one should kick the ball to the girl with the “funny” face.
When he recalls the incident, Chelsea’s dad, David Mulder, looks momentarily grief-stricken. “I try to explain to her, ‘That’s the way God made you. There’s always going to be some kids that are going to pick on everybody. They’re not just singling out you all the time,’ ” David begins. And then he pauses. “Oh, I don’t know if I can describe it. You feel sad . . . hurt.”
He, too, was born with Crouzon syndrome, at a time when many children were institutionalized for it.
“From the neck down I’m fine,” jokes David, the charmer of the family, who holds down jobs as a die caster, a part-time fireman and emergency medical technician, as well as a deacon in his church.
He has undergone surgery 23 times in his 36 years to try to correct the effects of a mutation in a bone-growth regulating gene on chromosome 10. Yet his right eye still extends dangerously beyond its orbit, leaving it vulnerable to the slightest brush. A bungled surgery five years ago left him with a four-inch-long bald stripe down the side of his head and “a little upset,” he says. The last three operations were repair jobs on earlier procedures.
For David and his wife, Terri, the thought that Chelsea would have to go down the same path as her father was unbearable. By the time she was 3, she had undergone three operations to expand her cranium, and the surgeon recommended that, for both medical and aesthetic reasons, her brow and face would have to be surgically pulled out later on. For several years, Terri says, she “blocked it out,” but when Chelsea was 8, her husband brought it up again. David had always told his spunky, blond daughter that she was beautiful. Yet, as she approached the teenage years, he feared that his daughter could be ostracized.
In the spring of 2001, the local doctor who had performed Chelsea’s surgeries referred the Mulders to Dr. John Polley, a surgeon at Rush-Presbyterian-St. Luke’s Medical Center in Chicago. He said Polley had developed a procedure that could accomplish in one surgery what frequently took two or three. Chicago is nearly three hours by car from the Mulders’ home in rural Hudsonville, Mich., further than the family usually goes on its summer vacations. But in October 2001, the Mulders drove to the city to meet Polley and his orthodontist partner, Dr. Alvaro Figueroa, who together run the Rush Craniofacial Center.
During their first meeting, Polley, an imposing man with a Clark Kent face and an easy manner, showed the Mulders pictures of patients and described the procedure. In surgery, he would first make an incision across the top of Chelsea’s head, and then cut the bones holding her face to her skull, following her hairline from upper jaw to upper jaw. He would then attach wires at four spots: through eyelets in two pins buried in bone behind both eyebrows and to both sides of a splint anchored around her molars that extended up and around her upper lip. He would fasten the wires to a halo-like titanium frame he designed with Figueroa. The halo would be screwed into either side of her skull.
Twice a day in the weeks following surgery, someone in the family would have to turn a screw to tighten the wires, gently pulling Chelsea’s face forward over the lower jaw by about one millimeter a day. The soft tissues would stretch and the body would detect each new separation and keep trying to repair it. In effect, the technique, known as distraction osteogenesis, would force Chelsea’s body to grow her face forward.
Because their local surgeon had spoken so highly of Polley, David and Terri Mulder were practically sold on the procedure before they walked into his office.
“They said, ‘If you want to think about it, go home and think about it.’ ” recalls Terri, whose bubbly personality and spatter of freckles across her nose make her seem like a happy kid. “But we said, ‘No, we want to do it.’ “
Chelsea, who was then just 9, was quiet during the consultation, but a few weeks later, while watching a Montel Williams show on child makeovers, she made it clear that she understood exactly what was going on.
“That’s gonna be like me, Mommy,” she said. “I’ll have a face makeover.”
Doctors have known for more than a century that pulling slowly on the site of a fracture, a procedure called distraction, would grow new bone, and the technique has been used for decades to repair defects in the long bones, especially in the legs. In the mid-1990s, craniofacial surgeons developed distractors that could be attached to the bones of the face during surgery and operated by tiny cranks coming through the skin. Polley and Figueroa tried these distractors but found it hard to fine-tune the position of the bones, and they didn’t like having the patient undergo a second major surgery to remove them.
Their innovation, which they first used in late 1995 to treat a boy with a cleft lip and palate, was to apply the distraction from outside the body using a modified aluminum halo of the sort long used in neurosurgery. They have since used it to advance the upper jaw of more than 70 patients, and other surgeons have picked up on the technique. In 1997, Figueroa and Polley made a few alterations to the halo and then used it to pull out the entire face and forehead of a boy with Apert syndrome, which is similar to Crouzon.
“As an orthodontist I was comfortable pulling bone and Polley was comfortable cutting it,” says Figueroa, a genial, spirited man who speaks with the slight accent of his native Guatemala.
Polley says his procedure grew out of his dissatisfaction with the traditional operation to advance the face and forehead, which had been used since the 1970s. In the traditional operation, surgeons make the same cuts that Polley does, but the bones of the face are pulled immediately into the desired location and fixed with tiny plates, screws and bone grafts. The surgery typically takes 10 to 12 hours, almost double what it takes Polley, and the recovery time is usually longer.
Most important, Polley says, the infection rate for the traditional procedure is alarmingly high, as much as 40 percent for some surgeons he has spoken with, though he says he never had an infection. Few studies have been done on the problem, but surgeons believe the infections arise from the infiltration of bacteria from the nasal cavity into the temporary space created in the cranium by pulling the face forward all at once.
“People have been looking for an easier way to do this, a safer way,” says Dr. Craig Dufresne, the director of the craniofacial program at Inova Fairfax Hospital in Falls Church, Va. “That’s where the distraction osteogenesis is coming in.”
Some surgeons, however, sharply dispute the high infection rate attributed to the traditional procedure and say that distraction osteogenesis provides no advantages to the patient. Dr. Tim Turvey, a professor of oral maxillofacial surgery at the University of North Carolina in Chapel Hill, says he has done about 25 of the traditional procedures and has an infection rate of less than 10 percent.
“I would much prefer to place the face where I planned it to be, stabilize it, and be done with it,” he says. “With this, you’re struggling with the device for weeks.”
Dr. Harold Slavkin, dean of the school of dentistry at the University of Southern California in Los Angeles, suggests that distraction osteogenesis has not been around long enough for anyone to be able to talk about it with scientific rigor. “It is promising,” he says, but it’s probably not for everyone.
For the Mulders, such discussions were academic. They did not shop around for a surgeon; they simply followed the recommendations of their local doctor. And last Jan. 9, Chelsea became the eighth patient of Polley to undergo the surgery.
The operation went smoothly; she spent six nights in the hospital, her face so swollen Terri could hardly recognize her, and finally got to go home on her 10th birthday. The next week she was back for her first checkup.
Perched on the edge of the exam table, waiting for the doctors, Chelsea lets her head hang practically to her chest. Her scalp is stapled across the top of her head at quarter-inch intervals, forming an odd headband, and her upper lip is cracked and bloodied from the metal wires that arc around it and attach to the splint in her mouth. At home, she is feisty, and a little mischievous, but here, in the fluorescent light of the clinic, she is silent and unsmiling.
“Where’s Chelsea this morning?” coaxes Polley gently, nodding at her mom and the two cheery aunts who’ve accompanied their niece to Chicago on this frigid day. Her nose is dry–a good sign that she has avoided infection–and the swelling is down. Terri answers Polley’s questions in her usual animated way, but when she looks at Chelsea, a worried look creeps into her eyes. She reports that it was “freaky” to turn the screws at first, but she’s used to it now, and no one else in the family will even touch them. They will pull until Chelsea’s eyes look normal, Polley says, even if the jaw isn’t quite lined up right.
“It’s an aesthetic judgment,” he adds.
Figueroa takes the screwdriver to adjust the tension. He tightens the screws on the device one full turn, pulling her forehead imperceptibly forward. Then he fiddles a little with the wires and screws. “Any pain?” he asks, and Chelsea quickly shakes her head “no.”
Crouzon syndrome occurs in about 15 in every million births, but occasionally its presentation is so mild that people aren’t even aware they have it. In 50 percent of the cases, the gene mutation arises spontaneously in the child of two healthy people, and recent research suggests a correlation between older fathers and Crouzon, says Dr. Amy Feldman Lewanda, a medical geneticist at Inova Fairfax Hospital.
But if one parent has the disease, then his or her child has a 50 percent chance of inheriting it. Terri knew the odds before she agreed to marry David, but she didn’t think much about it. “My worry was that we wouldn’t be able to have any kids, and I just wanted to have kids,” she says. “If they have problems, OK, we’ll deal with that as it comes.”
When their first child, Katelyn, now 14, was born healthy, David says he found himself thinking that the syndrome had “run its course.” But when Chelsea was born, they could see right away from her eyes that she had it.
“I was a little upset with myself because I had it, and she got it from me,” David says. “But there wasn’t anything I could do. I didn’t go on any type of depression pills or anything.” Though David and Chelsea combined have been in and out of the hospital 10 times in as many years, Crouzon is not a big topic in their household. The family has not spent time on the Internet investigating Crouzon or thought about getting together with other people who have it.
“We’ve never really sat her down. She just kind of knew it,” David says. “You don’t want to say, ‘You’ve got this, you look different.’ That’s going to scare the kid from the get-go. It’s not like we’re throwing her out to the world, but let’s see how [other people] react. And as far as I know, they reacted OK.”
At the same time, the family is acutely, and sometimes painfully, aware that people are looking at them. “It’s hard when you see adults staring. They can see there’s something different, but they don’t know what, so they try to look to figure it out,” Terri says. “Sometimes people think she’s got Down’s.”
Chelsea herself rarely comments about how she looks, but she once told Katelyn that she actually likes it when people stare at her.
When Polley asked the Mulders if a reporter could follow Chelsea’s treatment, they readily agreed, saying they wanted people to know about Crouzon, though Terri thinks some national media tend to portray people like them as “narrow-minded, right-wing conservatives.” Only the “conservative” part is right, she says. The family’s church, the United Reformed Church, a branch of Calvinism, is staunchly anti-abortion, and that is a stance that Terri reflects on frequently.
“I look at [Chelsea] and say, ‘There is no reason this child should have been aborted,’ ” Terri says adamantly. “Dave always said, ‘No, I don’t understand why I was created like this.’ But we’ve always been taught there’s a reason. God knows all, he’s omnipotent, he knows what’s best for us, and we may not always think that’s best for us, and we may never know why, but we have faith there was a reason.”
But if God has his reasons, should parents try to correct things in their children that they see as problems? Terri’s answer comes quickly and firmly: “We’re given the knowledge to do things, so I’m not going to say no [to] medical treatments.”
To smooth Chelsea’s return to school in the halo, Deb Toering, her fourth-grade teacher at Hudsonville Christian School, decided to have a talk about Chelsea with all the fourth- and fifth-grade classes. Though not all of them know Chelsea’s name, they knew whom the teacher meant.
“They said, ‘Oh, the girl with the eyes that are a little different,’ ” Toering remembers. Terri brought in part of a demonstration halo, and she and Toering cobbled the rest together with pipe cleaners and straws and then Toering held it on her own head so the kids would know what to expect. When Chelsea showed up with the real thing on, she got a lot of looks, but the sensation was short-lived.
Chelsea’s class has prayed aloud for her every day, and presents and cards, some with a few dollars tucked inside, have poured in, even from people the Mulders barely know. Chelsea already has plans for the money. The day the halo comes off, she’s going to American Girl Place in downtown Chicago to buy a doll named Kit who, she says, has “blond hair up to here and she has two eyes and–what—oh, yeah, a pink shirt.”
Three weeks into the treatment, Polley and Figueroa discuss how many more millimeters they have to go to complete the treatment. “About 8 to 10 more,” Figueroa says, as Polley presses along Chelsea’s eyelid, trying to feel the relationship between the rim of the socket and her eye, and then does the same to Terri, whom she’s beginning to resemble. “Oh, no, Al, just four or five,” he says.
Back home, people have begun to comment that they see a change in Chelsea’s face, but her parents cannot yet think of her in categories like “Chelsea before” and “Chelsea after.”
“I do notice there is a difference,” Terri says carefully, “but I don’t see it as dramatic as everyone else.”
The medical benefits of the treatment are starting to appear as well. Before the surgery, Terri and David had never seen their child cry tears, because there was no room for them to pool beneath her eyes. But now, for the first time in Chelsea’s life, a few drops have been seen to tumble down her cheek.
On Feb. 12, Polley decides the advancement is done. Chelsea’s lower eyelids are working for the first time, her bite is good, and she’s starting to learn to breathe through her nose.
“I like right where she’s at,” Polley says, holding her face in his hands. “She looks great. So now it just sits for about three weeks or so.”
“Get the stupid halo off,” Chelsea writes on her calendar as a thing to do on March 11. When the day finally comes, she’s in a talkative mood, telling the residents and technicians at Rush who swarm around her in the pre-op about the doll she plans to buy later that day. But, unexpectedly, there’s a hitch. The nurse anesthesiologist has heedlessly unrolled a towel full of syringes and needles in Chelsea’s plain view, and she suddenly tenses up, realizing that the woman is going to insert an intravenous needle into her arm.
“Have you ever had a mosquito bite?” says the nurse in a kind voice. “That’s all it is.”
David tries to distract her, but Chelsea is starting to panic and moan, and tears are streaming out of her eyes.
“Remember you had the gas last time. That wasn’t good, remember?” David says.
“I liked it better!” she snaps through her tears.
For one long minute, David and the nurse struggle to calm Chelsea, reminding her that if she has to have the gas instead, she may not feel well enough to go to American Girl Place.
“If you have this you’ll be done with it a lot quicker,” he says.
“It hurts! Where are you poking it? It’s gonna hurt!” she cries. “Are you doing it?”
“She’s done already,” David says calmly. Chelsea sniffs back her tears.
An hour later, when her mom and dad approach her bed in the recovery room, Polley is already there. Katelyn comes around the side of the bed, peers into Chelsea’s face and smiles.
“She looks great. She’s done great,” Polley says. “You’re very beautiful.” He’s about to head back into the operating room, when David clears his throat.
“You did such a nice job on her. Would something like that work on me?” David asks. “It’s been bothering me to ask you for weeks, but I just got up the nerve.”
Polley’s answer surprises him. Yes, he says, the procedure can be done even on adults. He has done one so far, on a woman who had undergone even more operations than David had. Before leaving, Polley urges him to make an appointment.
Later, David thinks there’s no coincidence in the fact that he waited until the last minute to ask Polley and then got a good answer. “Maybe that’s a sign that God’s trying to tell me something, that I’ve been going through my life 36 years this way and, ‘I’m allowing you to have a change,’ ” he says.
Less than 90 minutes later, Chelsea skips into American Girl Place. She has a tiny stitch in each eyebrow, and the sides of her head have marks from the screws of the halo. But in her purse is a fuzzy pink wallet carrying $130, mainly in ones.
“How much is it?” she says, pointing at a tiny toy dog.
“Sixteen dollars,” Terri says.
“I want it!” she demands.
Terri gets a certain expression on her face. The kid she calls “strong-willed” is back to normal.
Two days later, Terri pulls her minivan up to the school a few minutes after the last bell has rung. It’s Chelsea’s first day back at school since the halo came off, and the whole school knows it. The front doors swing open and three excited girls–selected by Toering for their ability to remain calm and not bump–run out to welcome Chelsea.
“You should wait to see your locker and your desk!” one girl says.
Iside, a beaming Toering waits next to Chelsea’s locker, which is covered with paper butterflies made by her classmates. “You look beautiful,” she bends down and whispers to her. “Of course we thought you looked beautiful before,” she quickly adds.
When Chelsea enters her classroom, everyone stands and applauds wildly, and she slips into her desk with a wide grin on her face.
“Now you can eat cheeseburgers,” one boy yells out.
Just before recess, seven blond girls gather around Chelsea’s desk. They look at her, and she steals glances at them as she details the contents of her Chicago hotel’s exercise room. She still can’t go out for recess, so one girl volunteers to stay inside for company. The two head over to the computers and load up some games.
“Username” pops up on the screen, and Chelsea unhesitatingly taps in her alias:
P-R-I-N-C-E-S-S.
At the Mulder dinner table these days, there’s talk of Chelsea’s Christmas concert solo, a verse from “Angels We Have Heard on High.” And whether their new baby sister–the one they’re planning to adopt from China this spring–has been born yet. An appointment with Polley and Figueroa is scheduled for January, but except during these occasional follow-up visits in Chicago, the swelling, the staples and the goop that Chelsea had to wear in her hair to ward off infection all seem like ancient history. For the first time in a long while, there are no surgeries on the family’s agenda.
Shortly after Chelsea’s halo was removed, David learned from his surgeon that there was just too little real bone left in his cranium to guarantee a good outcome with Polley’s procedure. Naturally, he was disappointed.
“You know that people are going to accept beautiful people quicker than disfigured people, and I see that in my own life,” he says. “But I’ve done a lot of things in my life, and it’s not really holding me back, except maybe from being a Chippendale dancer or a male model, if that was even in my calling.”
He has observed subtle changes in Chelsea’s public personality. She makes more eye contact with people, and she doesn’t hang back in social settings as much as she used to. “She seems to be a little more sassy or cocky, but in a good way,” he says.
According to Terri, Chelsea’s fourth-grade teacher reports that she’s “still quiet, but she’s right in there listening.”
David has heard Chelsea refer to the period before the surgery as “when I had Crouzon.” He chuckles. “I’m not going to pull her down and say, ‘Really, you still do have it’ ” he says. “She would say, ‘Well, how come I look different then?’ “
Chelsea herself can talk a mile a minute about her penguin collection and her new baby cousin and her camping trips on Lake Michigan last summer, but when the conversation turns to her surgery, she lets her parents do the talking as she listens intently. She giggles, remembering how her cat used to scratch his back on the halo while sitting on her chest.
Did anything ever hurt at all?
“When I got the staples out,” she says.
Does she think people treat her differently now?
“I don’t know.”
Is she glad she had the surgery?
“Yeah.”
Why?
“I just didn’t like it that way,” she says of her earlier face.
Why not?
“I look better now,” she says with finality.
The response from the rest of the world has endorsed her view. In the months after the halo came off, scores of people approached the family with compliments on Chelsea’s new look.
The Mulders also were pleased, but a little surprised, as well.
“Dr. Polley said we would look back and not remember what she looked like before, and that’s true,” Terri says. “You just see her like she is.”
