Shielding Sarah
BY BETSIE FREEMAN
OMAHA WORLD-HERALD STAFFWRITER

Sarah Inks may never know the pride of earning a college diploma. She may never know the joy of that first bridal kiss or a newborn child. And she may never know she's dying. That's the way her parents want it. Sarah has a degenerative genetic disorder that destroys brain cells until its victims can't walk, talk or eat. They usually die before they turn 21. Since she was diagnosed with Juvenile Batten Disease in the spring, the 9-year-old has gone blind. Sarah's parents, John and Sue Inks, aren't giving up on a miracle. But without one, they want her too-short life to be happy. So they're keeping details of the disease from Sarah and sharing only limited information with their three other children. While that might shock some people initially, John and Sue knew from the beginning they would handle it that way. Sue says nothing anyone could say would change their minds. "There's no point in sharing mortality with a 9-year-old child. Why on earth would I tell her?" Sharing Sarah's story may complicate that, but the Bellevue family says the benefits outweigh the risks. They want to raise awareness of the disease to help generate money for research and the family. Sarah trusts her parents to protect her, Sue says. "I'm not in the habit of lying to my children. But if I have to lie to her to make her more comfortable with her life, I will." About a year ago, Sarah couldn't see the chalkboard and books at school anymore. At first her parents thought they were dealing with a common problem. But glasses didn't help. Doctors ordered more tests and found Sarah had retinal dystrophy, a symptom of Batten disease, a malady so rare that only one other child in Nebraska is known to have it. By March, they diagnosed the disease. By May, Sarah could see lights and shapes but little else. The disease will give Sarah seizures. Eventually, she'll forget all she has learned in school and need a feeding tube. "I read about it in small doses because it gets pretty upsetting," Sue says. This summer Sarah began to use a cane and study Braille. Her parents met with staff at Bertha Barber Elementary School to plan for their daughter's future. They want her to be in school as long as she's comfortable there. And that might be a long time. Sarah loves school.

On a recent Thursday morning, the first day of fourth grade, Sarah climbed up and down the stairs of the family's split-level house. She still wore the oversized T-shirt she slept in as she nervously waited for Mom to help her dress. She did the chicken dance, then played with the light switch - off, on, off, on. Her exasperated dad gave her a timeout in her room, but she didn't stay there long. Timeouts, Sue says, may not work much longer. After she's been left to reflect on her misdeed, Sarah sometimes can't remember what earned her the punishment in the first place. That short-term memory loss is showing up in Sarah's schoolwork. She earned A's and was the top math student in second grade. John and Sue also worry about Sarah's schoolmates. While some have been wonderful, they say, others teased Sarah last year and put objects in her way to trip her. Some were put off when Sarah invaded their personal space trying to see them. "Some kids would quietly set her off on purpose, going behind her and saying 'Sarah, you're a loser, you're a loser,'" Sue says. The disease and the rapid loss of sight make Sarah overreact. "It's not super-extreme, but it's enough to make her seem different," Sue says. "She seems less mature than other kids." She and her husband worked with school staff to educate students about Sarah's lack of sight. Vision specialist Betty Widoe explained Sarah's condition to the fourth-grade class, enlisting their help as Sarah's guides. She taught them to identify themselves to Sarah when they approach her. The training is paying off. So far, fourth grade is going well. "I have a new backpack," Sarah announced loudly when she arrived for her first day. The backpack contained a journal for teachers to communicate with John and Sue. Sarah says she likes the playground and the library the best. That's not surprising: She loves to read and be read to. Teachers report that she's doing most of her work at a fourth-grade level, Sue says, although she's not sure how long that will continue because the first days are review. Their goal to keep Sarah in school looks less lofty. But, John says, if Sarah becomes self-conscious and it's too difficult to watch others make progress, they'll bring her home. One thing they won't do, he says, is put her in an institution. "She will be staying home all the way through to the end."

John and Sue, together 10 years, lean on each other. They're proud of their "strong, really good family," including two sets of grandparents who live nearby and people they consider more than friends. They have more than their share of stress. John, recovering from his second surgery for Crohn's Disease, recently got a viral infection that affected his balance, causing him to tumble down a flight of stairs and fracture some vertebrae. Both parents work with disabled adults in jobs that are rewarding emotionally but not financially. "Stuff isn't important, but human services jobs don't pay well. Medical bills wipe us out," Sue says. Still, she says, "I think we're more fortunate than a lot of people." Sarah's former principal, Arlana Whitney, finds their outlook remarkable. "They look at each day as a gift," she says. Whitney says she feels a special connection to Sarah. "She just kind of grabs your attention," Whitney said. "Sarah always makes me smile." Sarah's siblings are extra considerate of her, although they know her condition doesn't mean she can get away with things. It's hardest on Savannah, the closest to Sarah. She worries about her sister, yet doesn't really understand what's happening. John and Sue did a lot of soul-searching before they decided to share Sarah's story. Sheltering the family is the most important thing. To them, that means carefully parceling out information and keeping Sarah from being scared and depressed. Their approach is understandable, says the Rev. Jim Clifton, an ethicist with Creighton University Medical Center. "For parents, the ethical obligation is to give as much information at the right time as the child wants to know and can process," says Clifton. In the end, parents should be able to say they gave a dying child appropriate information for her age, answered questions and presented the true story over time. "That's a wonderful thing to look back on and to have accomplished," he says. The Inkses took the first step toward that when they told Sarah about her eyes. "We said her eyesight would get worse, it would never get better," Sue says. "There was a very good possibility she'd go blind, but we'd be here to help her."

Unlike adults, most children don't need to resolve complicated spiritual issues to die in peace. Sarah believes in God, heaven and angels and doesn't seem to fear death, Sue says. "I think she would if we told her, 'You're going to die young.'" The girl loves to sit up front at church on Sundays, listening to the homily and adding her own comments. "I love Jesus," she once told the congregation. Sue says she learns from her daughter. "She doesn't feel sorry for herself. She's a happy, upbeat person." The family will battle to keep her that way and hope for a cure. You never know what science can do, Sue says. In the meantime, they'll spread the word about Batten disease and nurture Sarah. Says Sue: "We've weighed the benefits. If there is hope for our daughter to have a cure, people have to know about it. They have to have a passion to want to help."

PHOTO: RUDY SMITH/THE WORLD-HERALD
CAPTION: Bertha Barber Elementary schoolmates are learning to be sighted guides. Longtime friend Trevor McManigal helps Sarah down the hall.