Parry-Romberg Syndrome: The Face of Courage
Ten-year-old Kelley Sperry tries to ignore the kids who tease her -- and bravely waits for the disease that is gradually destroying her face to go away.
The Early Signs
On good days, Kelley Sperry feels confident and happy, likes playing with glittery makeup, and says she wants to be a cheerleader when she's older. On bad days, Kelley refuses to look at herself in the mirror. Her right nostril is twisted, her right eye is slowly sinking into her skull, and her lip is pulled up into what seems like a sneer. The teeth on one side of her mouth are starting to jut forward and may eventually fall out because the roots are so weak.
Kelley often says that she's ugly. She hates it when kids in her hometown of Westminster, Colorado, call her "Crooked Nose." Strangers stare and give her mean looks because they think she's scowling at them. "I try to ignore it, but I can't," Kelley says. "It's too hard."
What those strangers don't know is that Kelley suffers from Parry-Romberg syndrome, a rare and mysterious disorder characterized by a slow deterioration of tissue, muscle, and bone on one half of the face. The disease isn't fatal -- but there's no treatment or cure, and the physical changes can be totally unpredictable. "The average pediatrician has never dealt with this disorder, and the symptoms are subtle when they first appear," says Joseph McCarthy, M.D., director of reconstructive plastic surgery for the Cranio-Facial Anomalies Unit at New York University Medical Center, in New York City.
When Kelley was 3, her parents noticed that their adorable little girl was developing some curious physical symptoms: She had a few strands of gray hair on the right side of her head, no lashes on her lower right lid, and a white spot on the right side of her neck. A dermatologist they consulted wasn't alarmed and said the spot was probably vitiligo, a common skin condition that causes a loss of pigmentation. "We thought it was odd that all of these problems were on her right side," recalls her father, Jay, a paramedic and firefighter.
The Sperrys, who also have two sons, Dillon, 15, and Jesse, 11, didn't start worrying again until Kelley was 5. Her right nostril seemed to be shrinking, and she was making funny noises in her sleep. An ear, nose, and throat specialist said that her breathing was slightly obstructed but dismissed their concerns. "He told us, 'Come back when she's grown and have her nose fixed if it's still important to you,' as if all we cared about was having a perfect-looking child," says Kelley's mother, Donna, a fourth-grade teacher. "That made us really mad -- and discouraged us from getting help for a while."
Over the next few years, however, Kelley's nose grew more crooked and her lip began to pull upward. She developed a dent on the right side of her forehead, milky blotches covered the right side of her neck, and she had terrible migraines and facial pain. Donna and Jay knew that something was seriously wrong. They took Kelley to doctor after doctor, desperate to find someone who could tell them what was happening to their daughter. Finally, when Kelley was 9, a geneticist pieced together the puzzle and diagnosed her with Parry-Romberg syndrome, a disease named for the two physicians who identified it in the early 19th century. The doctor reassured the Sperrys that the condition wasn't life-threatening but told them that there was no way to control the bizarre array of symptoms (see "Facts About Parry-Romberg Syndrome," below). The only good news: Although the disease usually gets worse for anywhere from 2 to 10 years, it then stops, or "burns out," on its own.
The Sperrys were devastated. "We felt frightened, helpless, and alone," says Donna, who was so upset that she took a brief leave of absence from teaching. "Finding out that Kelley had such a rare disease and that there was nothing we could do about it was almost more than we could bear." Jay was in the middle of remodeling their home but brought the project to a screeching halt. "We had no desire to do anything," he says. "All we wanted to do was comfort each other and care for Kelley."
Searching for Answers
Donna spent countless hours combing the Internet for information sites about the disorder. She eventually found the Romberg's Connection, an online support group for afflicted children and their parents. There, the Sperrys saw pictures of people whose faces were sunken and twisted and read their personal stories. "I couldn't stop crying because I realized Kelley might look like that in a few years," Donna says.
She also read an article about a renowned craniofacial surgeon in Colorado and prayed that he might be able to help her daughter. The doctor was very kind but said the only procedure that could realign Kelley's face would require breaking many of the bones and then resetting them. "Afterward, she would have had several screws protruding through her skin, which we'd have to crank every day," Donna recalls. "We were horrified but almost went ahead with the operation because the doctor told us that this is what he would do if she were his daughter."
Fortunately, they decided to seek out other opinions, and nearly every other doctor consulted said it was important not to begin reconstructive surgery until after Kelley's skull was fully grown and the changes had stopped. "One doctor refused to let us leave his office until we promised not to let Kelley have surgery at that time," Donna says.
Although the Sperrys have found a medical team they trust, even these experts don't have all the answers. They don't know how long the deterioration will continue, for example, but it seems to be getting progressively worse. Kelley can no longer breathe through the right side of her nose, and she's started to have severe nosebleeds that last for hours. What troubles the Sperrys most, however, are the mild strokes that Kelley has suffered over the past year. As a result of these, she has lost some of the vision in her right eye. Her neurologist attributed the strokes to atrophy of the tiny blood vessels behind her eye and prescribed small doses of aspirin to thin her blood and prevent clots. "We are terribly worried that Kelley might completely lose the vision in her right eye or have a more severe stroke," Jay says.
Helping Kelly Cope
The Sperrys are equally concerned about the emotional toll that the disorder is taking on their daughter. Although she still dances around the house with her headphones on and practices her cheerleading moves, she's spending more time alone in her room, writing in her journal. "Kelley puts a lot of effort into being upbeat and brave, but she's holding in a lot of her grief and sadness," Donna says.
Kelley's frequent headaches often frustrate her. "She's always been a great student, but now she hates reading because it makes her head hurt," Donna says. During class, her teacher sometimes has another student read to her. At home, Dillon and Jesse turn down their music and let her watch her favorite shows whenever she's not feeling well. Sometimes, when a headache hits late at night, Kelley crawls into her parents' bed and cries.
Although she spends time with her school friends, she's cut herself off from most of the kids in the neighborhood because they started teasing her behind her back. Donna, who role-plays with Kelley about how to respond when a child makes a cruel comment, encourages her to explain that she has a rare facial disorder. But Kelley usually just walks away instead. "I don't like to waste my time on the mean kids," she says.
Kelley does have a special new friend, Molly, an 11-year-old who read an article about Kelley in the Rocky Mountain News and invited her to a cheerleading clinic. The girls get together often now, talk on the telephone for hours, and recently cheered together in a local parade. "We've told Kelley that it's not how many friends you have
that counts -- it's the quality of your friendships," Donna says.
Dealing with the uncertainty of her disease is also less scary now that Kelley has met Megan Bean, a 20-year-old from nearby Aurora, Colorado, who had the disorder when she was younger. Even though Megan underwent multiple surgeries to correct her face during high school, she was voted prom queen in her senior year. "Megan has been a wonderful inspiration to Kelley, and she has helped all of us see that having Parry-Romberg syndrome does not mean having an unhappy life," Donna says.
For now, the Sperrys live one day at a time. They attend their sons' football games on weekends, and Kelley brings along her pom-poms and cheers on the sidelines. Even though Donna thinks her daughter is too young to wear makeup, she lets Kelley put on sparkly lip gloss to help her feel better about how she looks, and she helps style her daughter's hair so that the gray patches don't show. Kelley likes helping take care of the babies and toddlers in her church nursery on Sundays because they treat her just like any other kid. She also enjoys spending time with her grandmother, who lives nearby.
Recently, one of the parents at the school where Donna teaches decided to make Kelley's dream of being a cheerleader come true. She threw a party for her at a local restaurant and, as a surprise, arranged for a Denver Broncos cheerleader to attend. To Kelley's delight, six cheerleaders showed up and presented her with her very own white chaps, blue pants, and orange shirt. They even invited her to cheer with them at an upcoming game. Dressed in her brand-new outfit, Kelley was all smiles in front of the 76,000 fans. "I didn't feel nervous or think about my disease," Kelley says. "Having Parry-Romberg syndrome stinks, but some people are extra nice to me, and I've met new friends who like me just the way I am."
Facts About Parry-Romberg Syndrome
Also called Progressive Facial Hemiatrophy, Parry-Romberg syndrome afflicts fewer than 1,000 people nationwide. Unfortunately, because little research has been done on the disease, doctors still don't know what causes it. It seems to occur randomly, although some experts believe there may be a genetic component.
The first symptoms usually appear by age 3: a bald spot or white patch of hair, or a light or dark mark on the face or neck. One cheek or one side of the nose or chin may appear slightly smaller than the other, and children usually develop a dent on one side of the forehead. The severity of deterioration varies. "I've seen people who literally lose the entire half of their face, and others who just have subtle changes to their skin and hair," says reconstructive plastic surgeon Joseph McCarthy, M.D. Once the disease runs its course, surgeons can repair some of the damage.
For more information, log on to the Romberg's Connection Website at www.geocities.com/hotsprings/1018