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The Face of Courage
by
Pamela Kramer
Parents, April 2002
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.
The Early Signs
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
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