Sometimes, Sarah Hill gets tired of being strong, sick of being brave and courageous and determined.
"Perky" -- now that would be OK. Ditto with "cute" and "smart." But on certain days, she's just had it up to here with "stalwart."
"Her greatest desire is to be just like every other 10 1/2 -year-old girl. Her biggest frustration is that she's not," said her mother, Hope Barmat Hill. "The amazing thing that she doesn't get about herself at all, is that she's so much more because she has to come so much further each day."
When Sarah was about 18 months old, she awoke from a nap in her Fells Point home with a swollen knee. "No big deal," her mom thought, but she still placed a call to the doctor. It turned out to be a very big deal: juvenile rheumatoid arthritis (JRA), an incurable autoimmune disorder that tricks the body into attacking its own tissue.
In Sarah, the disease has also led to severe eye complications, including an inflammation called uveitis and the better-known fluid buildup called glaucoma.
Sarah's underlying conditions are among the 6,000 or so rare diseases in the United States that don't typically draw much attention from biotechnology and pharmaceutical companies. For business reasons, most prefer to concentrate on cures more likely to be a commercial hit or a safe bet.
So Sarah has had to tackle the issue herself, first going after the scariest of her diseases and the one that affects the most children: uveitis. It's among the leading causes of blindness in the United States, affecting about 115,000 kids under age 16.
This summer, with the help of her doctor and her parents, the about-to-be-fifth-grader launched a foundation at Johns Hopkins Wilmer Eye Institute that she hopes will one day "get people the right treatment."
Called KURE, for Kids Uveitis Research and Education, the foundation has already raised $21,000 in less than two months, in part through the sale of blue bracelets inspired by bicyclist Lance Armstrong's bright yellow "Live Strong" bands.
"Not a lot of people are as lucky as I am," Sarah said last month, fresh from a field hockey practice in Baltimore, her blond hair pulled back into a no-nonsense ponytail. "A lot still go blind."
There are no definitive treatments for Sarah's combination of conditions, though she manages most of the symptoms through a strict medical regimen, half of which is made up of medications developed for other diseases.
During two surgeries a year apart, doctors implanted tiny tubes in her bright hazel eyes to help drain the fluid buildup associated with glaucoma. Four times a day, she takes prednisone drops to suppress inflammation from the uveitis. Twice a day, she takes cyclosporin pills -- originally developed to prevent the rejection of transplanted organs -- to control inflammation from the arthritis.
And every Friday, after her 8-year-old brother, Nicholas, brings her teddy bear so she has a friend to hang onto, she gets an injection of the anti-cancer drug methotrexate, which also happens to help with the inflammation in her joints and eyes.
Writing a book
Those days are especially hard, because the injections rule out sleepovers -- they zap her energy for the next day. But for the most part, Sarah tries not to let her ailments get in her way. In fact, they seem to have inspired her. In addition to the foundation, she's working on a book about her weekly shot called I'm Not Ready Yet, meant to give youngsters with similar problems a story they can relate to.
"My mom got me JRA books, but none really had my type of disease," Sarah explained. "So I just wrote my own."
It's that sort of attitude that sets Sarah apart, said Dr. Douglas A. Jabs, her ophthalmologist and director of the ocular immunology division at Wilmer.
"It's very hard for a child to handle a chronic disease," Jabs said. "I think she's done extremely well. She just accepts that it's part of what is going on and gets on with her life."
In many ways, Sarah is a pretty regular kid -- both cute and perky. On the field, her gear on, she chats with friends, bears down on the ball, looks alternately bored and engrossed with the ebb and flow of the practice. And every now and then, she sneaks peeks at her father on the sidelines and waves.
"She's a girlie girl," said her dad, Dr. Peter M. Hill, director of the emergency medicine department at Johns Hopkins Hospital. She likes painted nails when her mom allows it, ribbons in her hair and the pierced ears she begged for. She likes tennis, pottery, time with her friends, and Katie Couric -- because she's funny and nice.
So far, her medications are mostly doing their job: Sarah's vision is 20 / 15 -- better than the 20 / 20 standard. But she has to be closely monitored for possible toxic reactions, and she still struggles with some of her symptoms.
She needs about 12 hours of sleep per night, and her growth has been slowed. Direct sunlight stings her eyes, and she has to wear a bothersome, "eye cage" when she's playing field hockey to protect the implanted tubes from sticks and hockey balls.
"I go through various periods of frustration being in medicine and essentially being pretty powerless to help her," Hill said. He was able to teach her to ask questions, though -- an asset that she turned into the foundation.
'It's just so touching'
"KURE will directly benefit our research," Jabs said. "There are several unanswered questions about the disease, and we hope that the money will enable us to begin to answer some of these questions. Some may require a fair amount of funding and large-term studies, but you have to start somewhere."
The unanswered questions are what get to Sarah the most. She hates it when friends ask about her diseases and she doesn't know what to tell them. Will she get better? Will she have to be treated forever? Will she ever find a cure?
"Sometimes it's hard," she said. But she has faith she'll have answers one day. And everyone has been impressed by the response to KURE.
"She's overwhelmed, I'm overwhelmed," her mom said. "I have a perpetual headache from crying every day. ... It's just so touching."
Colleagues of Sarah's grandfather have sent donations from as far away as New Zealand. Her 13-year-old cousin sat outside grocery stores, selling dozens of KURE bracelets. And a family friend has arranged a kids' "try-athlon" to be held tomorrow in Baltimore County's Stoneleigh community to raise more money.
"We had hoped that she would see how she could change people's lives [through this]," Sarah's mom said. "I think she's also seeing how individuals are changing her life. ... All these people are making her dreams."
KURE accepts donations at the Wilmer Eye Institute at Johns Hopkins Hospital. Information: http: / / mysite.verizon. net / resqpfbh / kure.