Rare few know her pain

Six-year-old Emily Koesters rose at 5:30 a.m. Tuesday, like she does every day, to take her first dose of medicine.

Every hour or so, she takes meds.

Medicine for her kidney infection. Medicine for her cancer. Medicine to boost her immune system.

She gets a three-hour break at 2 a.m. until she wakes again to start her day.

Apart from all those doses, Tuesday was an especially difficult day for Emily. She lost her friend Mitchell.

He was probably the only person in Emily's world who truly understood her pain.

Emily suffers from Schimke Immuno-Osseous Dysplasia (SIOD), a complicated medical term for one of the rarest forms of dwarfism.

Unlike many dwarfs, the Gretna girl is perfectly proportioned but stands only 38 inches tall and weighs about 38 pounds. She is only one of four in the United States who have been diagnosed with Schimke, and one of only 45 in the world.

Dr. Richard Lutz of the University of Nebraska Medical Center said he's seen only one other case of Schimke in his career.

“One in every million children in North America will get it,” Lutz said.

Mitchell had Schimke, too. The kids met last year. Well, technically, their parents did.

For nearly two years, the Koesters lived at an Omaha hospital. Emily's medical treatment was relentless. The family needed to believe she'd get better. So mom Erin made it her mission to research Emily's disease.

She came across “Little Giants,” a Schimke Web site dedicated to Mitchell Cupps, a 5½-year-old Oklahoma boy who shared the same doll-like size traits. Mitchell's family organized the Little Giants Foundation to generate more awareness.

The families connected last March. They e-mailed and phoned one another.

They eventually met when Mitchell's kidneys began failing. He came to Omaha for treatment.

Mitchell's family wrote about his journey to diagnosis, which can take years. Most children die from kidney failure or stroke before they've been accurately diagnosed with Schimke. Children with the condition typically don't live past age 15.

“We're in a fight against time,” Erin said. “Right now there is no cure.”

A musical benefit Friday at Slowdown will help pay Emily's medical bills and fund more research.

Emily has had 39 major surgeries — brain surgery, a kidney transplant and stomach surgery to wash out an infection — in two years.

“We've started with her head and worked down to her toes,” her mom joked. She tries to stay upbeat.

Emily's battle with Schimke started in July 2007, when her face swelled. Within hours, her entire body was swollen.

“She looked as if she gained five pounds overnight,” Erin said. “We thought it was allergies.”

Then Emily had kidney failure. Her father, Joe, gave Emily a kidney in a May 2008 transplant, but her health problems continued to snowball.

Emily was diagnosed with Schimke last January. She experienced seizures in September, losing much of her cognitive and mobile skills. Doctors recently discovered a fungal infection in her donated kidney. A side effect of the anti-rejection drugs is lymphoma. Emily has stage 3 cancer.

She requires around-the-clock medical attention. Her parents have drastically cut their work hours. Mom is a part-time English composition teacher in the Ashland-Greenwood Public Schools. When she gets home, she immediately changes her clothes and showers.

“I can't take the risk of getting her sick,” the 38-year-old said.

Dad is a 43-year-old self-employed construction worker who does roofing and dirt removal. He hasn't been to work in months. Shortly after the transplant, doctors found an incisional hernia. Six months later, he was back in surgery for a second hernia that ruptured.

Emily is their only child. She's vivacious and an animal lover. She's raised money for the Henry Doorly Zoo and the Cheetah Conservation Fund in Africa.

“She wanted to buy the penguins and polar bears ice because of global warming,” Erin said, chuckling.

Emily's cozy yellow bedroom is covered in cheetah photos that Dr. Lee Simmons, former director of the zoo, shot for her. One wall is a sun-moon mural of trees, birds and Mother Earth. Mom designed it, and a friend painted it.

Dad made her tiny crutches. Emily can't walk more than 100 feet before her legs give out. Getting up and sitting down are hard. Climbing steps is out of the question. Since the seizures and brain surgery, she's had to relearn the basics.

“She was really good at writing her name,” Erin said. “Now she scribbles.”

“I know I'm writing my name,” she'll tell her mother. “But all I see are scribbles.”

At first, she was frustrated. But she's coming around. Emily recently started to trace letters again. “We try not to get too excited,” Erin said. “We try to keep it as normal as possible for her, whatever that means.”

On Tuesday, Erin and grandmother Jeanette Roy tried to keep Emily occupied. They were missing Mitchell.

Emily tried walking with her homemade crutches and bumped her leg.

“You're not feeling good,” cried Grandma J. “What happened?”

Emily told Grandma J. about the boo-boo on her leg. A minute later, she bounced right back up. She baked a Jell-O cake in her little oven. She played “Taste the Animals,” a game she invented. She told Grandma J. stories of wild adventures in the jungle. Sometimes she's a veterinarian cop or a cheetah having babies.

Just then, the room went silent. Emily crept near her grandmother and shouted, “Boo.”

“Oh, my,” said Grandma J., pretending to be startled. “Why, hello, Emily.”

Emily doesn't get many visitors. It's Mom, Dad and Grandma J., day after day. This may explain why she seems more mature than many 6-year-olds.

Friends say Emily has an old soul. She's witty and can carry on a conversation with adults better than children.

During one of her hospital stays, for example, Emily tried to greet another child who was in a bad mood.

“Hmm,” Emily considered. “She must be on Ativan and morphine.”

Drugs that help calm you and take pain away.

“She knows her meds,” Mom laughed. “She knows her body.”

She knows her limitations. She can't attend Friday's benefit.

A cold can quickly become pneumonia. The family asks friends to communicate by phone or e-mail so Emily's not exposed to germs.

But she also knows hope. Grandma J. says they concentrated on a word for December — believe, taken from one of Emily's favorite movies, “Polar Express,” in which the characters have to believe they will make it home.

It worked for Emily. After two long years, she has been out of the hospital for a month.

“Knock on wood,” Mom said. “That's the longest time.”

Contact the writer:

444-1075, j.loza@owh.com

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