Disorder forces teen to travel a rare path

Windsor girl doing her best to cope while coming of age

Joe Garner, Rocky Mountain News

Saturday, June 11, 2005

WINDSOR - Kelley Sperry packed heavily so she could shine this weekend:

Seven outfits for four days, including a new pink-and-white dress to wear with her first heels, which lift her only a nudge but still require practice so she won't stumble. There's also makeup to match every mood, along with three irons to curl her hair and another to straighten the waves, just in case.

Thirteen can seem like a bad-hair year.

"You never want to wear something twice on the same weekend," Kelley advised, enjoying the advantages of traveling with, at the very least, a Junior Denver Broncos Cheerleaders duffel bag, a Nike bag and a purse to a much anticipated weekend retreat.

As Kelley comes of age, she is coping with a variation on the quintessential teenage contradiction: She wants to be noticed, but she does not want to stand out, despite a disease identified as Parry-Romberg syndrome that is causing the right half of her face to waste away.

"I don't talk about it," she said. "I tell people, 'Don't be so nosy.' "

Almost four years ago, the Rocky Mountain News introduced Kelley Sperry, then a shy little girl, one year after her diagnosis.

The special section titled "Beyond the Mirror" chronicled her family's struggle to come to terms with the rare disease, which has no known cause and no known cure.

The disease is more common among women than among men, and usually it affects the left side, according to the National Institute of Neurological Disorders and Stroke.

Some who suffer from it lead full lives, but the lives of others are shortened by stroke or other health problems.

Since the original story was published, Kelley, parents Jay and Donna Sperry, and brothers Dillon and Jesse have moved north along the Front Range from Westminster to Windsor.

They have made a fresh start as a family, but they still are coping with the stress of Kelley's baffling, disfiguring disease.

Meeting kindred spirits

The Sperry family is evolving, and Kelley is growing up in a blur of cell phone calls, endless hours of hanging out with friends and dreams of stardom as a country singer. Or maybe she'll be a lawyer. Or, for sure, a country singer.

"Sometimes, she's 13 going on 21," Jay Sperry said. "But sometimes, she's 13 going on 5."

Reader's Digest is scheduled to publish a story about the family's challenges in its July issue.

Now more a young woman than a schoolgirl, Kelley Sperry also is taking center stage at a gathering this weekend in Lake Geneva, Wis., for people afflicted by the disease.

They include young people like her, as well as men and women who have made careers and raised children of their own. The support group, called Romberg's Connection, links people who had been enduring the disease alone.

Kelley met Ashton Modlin, another teenage girl with the disease, at last summer's gathering. They exchange e-mail messages brimming with chatter and confidences. "She's been one of my close friends ever since I met her," Kelley said.

Beneath Kelley's luminous, smooth complexion, the soft tissue, bone and muscles of her face continue to be attacked by Parry-Romberg syndrome, agonizing her parents even as she practices nonchalance.

Kelley's right eye is drooping, her right nostril is atrophying and her right lip flares up, transforming her mouth into a diagonal gash across her face - until she laughs.

When she laughs, both sides of her face express joy in unison, her blue eyes gleam and she is full of herself. She becomes the persona known as "Kellbell," brimming with sarcasm and spunk, agog over cute boys and ready to enter Windsor High School.

"We both have older brothers, so we know what high school is going to be like - kinda," said Matti Sperry, 13, Kelley's cousin, as they burst into giggles together. "We're both smart, but we need to apply ourselves more."

If only Kelley's headaches didn't hurt so much when she reads.

Growing wary of doctors

"We found that Kelley had been hiding Tylenol bottles in her room," said Jay Sperry, 45.

Donna Sperry, 46, lectured her daughter on the dangers of relying on ibuprofen to make it through middle school.

Based on living with Kelley day to day, her parents say their daughter's disease still is "actively progressing," but surgery is not a choice until Kelley has matured, and then only if Kelley and her parents find a doctor in whom they have confidence.

After a childhood of being poked and prodded, Kelley acknowledges that doctors "aren't my best friends."

Neither will her parents tolerate doctors who treat their daughter as a medical curiosity.

"Our goal is to bring attention to the disease to educate the public and, especially, the medical community," Donna Sperry said.

"The medical community does not know what they are dealing with. Some of them even have to ask how to spell the name of the disease. Wanting to cut on people while the disease is still active is really doing more harm than good," she said.

Kelley's parents "are concerned about Kelley's face because it's so important to a young girl," said Donna Sperry, who is writing a book about her daughter's disease. "But we are more concerned about the strokes.

"We don't know what's happening in her brain. We've lost a lot (of people afflicted with the disease) through strokes."

Kelley has suffered at least seven strokes, her parents said, which have affected her vision. She has told them it's as if she's seeing through a spyglass, with black blotches obscuring her limited field of vision.

Kelley takes an 81 milligram aspirin daily to reduce the risk of another stroke, but she takes no prescription medicines. She sees a doctor only as needed.

"We have not held her out of any activities," Donna Sperry said. "At one point, she wanted to play volleyball. It was a secret worry because of the pain if she got hit in the face, but we didn't say no."

In the course of getting settled in Windsor, Jay and Donna Sperry asked for an appointment with officers at the Windsor-Severance Fire Protection District to alert emergency medical teams to their daughter's disease and its ramifications.

Such small-town medical attention has made the family feel at home among a widening circle of friends in Windsor, a booming bedroom community between Fort Collins and Greeley.

"Kids up here have been raised to have more respect," said Kelley, who endured a barrage of taunts and stares when the family lived in metro Denver.

"Outwardly, she does appear to be bubbly, confident and perky," Donna Sperry said. "But at home, behind closed doors, she has these very sad moments when she cries and cries."

Thoughtful helpers

Kelley's hair was maroon colored after a fling to turn her from a brunette to a redhead veered off the color chart.

"You can read the instructions and still get some crazy colors," counseled Katy Deckard, the hairstylist at the Cost Cutters on Harmony Road in Fort Collins who rescued Kelley from maroon.

Since that episode in the spring, Deckard, 23, has streaked Kelley's long brown hair with blond.

The blond strands are stylish, but they also mask patches of frizzy gray hair that first appeared when she was about 3 - one of the first signs of the disease that has set her apart from other children.

"Because I'm 23, I think I can relate to her," Deckard said.

"I know how hard it is to be 13 and how cruel kids can be. They pick up on every little imperfection."

Similarly, Angie Donovan, 32, a cosmetics clerk at the Walgreen's in Windsor, helped Kelley on a particularly low day this spring, choosing makeup to cover a bluish hue that Donna Sperry attributes to wasting from the disease.

"People made fun of me all through high school because my face started breaking out when I was in sixth grade," Donovan said.

She waited on Kelley and Donna Sperry for about 45 minutes, letting them sample the full palette of shades and tones, without asking about Kelley's face.

"I didn't think it was necessary for me to ask," Donovan said. "I didn't want to make her feel uncomfortable."

Donna Sperry said she saw her daughter "lighting back up again."

A typical teen after all

Kelley Sperry drinks regular Pepsi - forget the diet variety. She orders pizza topped with cheese only. She considers Jessica Simpson classier than Britney Spears. She swoons over Hollywood heartthrobs Ashton Kutcher and Chad Michael Murray.

She argues with her two redheaded brothers, Dillon, 19, and Jesse, 14.

Because of the six-year age difference with Dillon, Kelley is closer to Jesse, a popular football player who will be a sophomore.

"We talk about a lot of things," he said. "A lot of her friends are my friends."

He's his sister's shield and protector.

"I notice when people are noticing her, and so you explain she's your sister and she has a disease," Jesse said. "There will be kids in high school who will ask questions about her disease. I think she just doesn't want to believe that will happen."

Still, Jesse said, "Although Kelley has a disease, she's one of the happiest, most energetic people I've ever seen."

Beyond the mirror

Kelley Sperry has matured from a shy schoolgirl to a nonstop high school freshman since the Rocky Mountain News first introduced her and her family in 2001. While she's still reluctant to reveal much of her feelings about the rare illness, Parry- Romberg syndrome, that is disfiguring her face, her family has grown into new understanding of her disease and her pain.

• For more information on Parry-Romberg syndrome, visit www.geocities.com/rombergs.

Parry-Romberg syndrome

• What is it? A rare disease characterized by the slow wasting of facial tissue on half of the face. Some doctors refer to the disease as Romberg syndrome, Romberg's, PRS, progressive facial hemiatrophy or progressive hemifacial atrophy.

• When was it discovered? An English doctor, Caleb Hillier Parry, first reported the disease in the early 19th century. Heinrich Moritz Romberg of Germany wrote about it in 1846.

• Is there any treatment? There is no cure and there are no treatments that can stop the progression of the disease. Reconstructive surgery may be needed to repair wasted tissue. Surgery is generally put off until after the disease has run its course and the facial bones are fully grown.

Progression and symptoms

• Facial changes above the upper jaw or between the nose and corner of the lip, progressing to disfigure the angle of the mouth, ear, eyebrow or neck. In rare cases, facial bone, cartilage, muscle and even limbs are affected.

• Severe headaches that last for weeks, nausea, migraines, facial pain and seizures.

• Darkly pigmented skin or white patches, called vitiligo. White and bald spots on the scalp, eyebrows or eyelashes.

• Abnormal exposure, delayed eruption or wasting of the roots of the teeth on the affected side.

By the numbers

355 Number of members worldwide of an Internet support group called The Romberg's Connection

32 Number of countries represented in the membership directory

40 Number of U.S. states represented in the list

199 Number of members in the U.S.

6 Number of members in Colorado, including Kelley Sperry

garnerj@RockyMountainNews.com or 303-892-5421