Arthrogryposis multiplex congentia is a Greek term for “curved joint,” a condition that limits the range of joint motion. The condition began in her mother’s womb, where Emily was immobile and unable to move her joints. When joints aren’t moved within the uterus, extra connective tissue grows to fix them in position. Tendons connecting the joints fail to stretch to their normal length, making normal joint movement difficult.

“What’s a tiger say?”

“Grrr!”

“What’s the cat say?”

“Woo-Woo.”

Emily Allen stops and turns her tiny head to look out the sliding glass door into the gray Buckner afternoon. There’s a cat as gray as the sky stalking up the driveway, brushing up against a hubcap.

Everyone stops and waits for what will happen next.

“What does the dog say?”

Emily pauses and lifts her head, which appears as loose as a half-finished sucker on its stick. But she shows surprising strength as  she looks over at the cat, glancing around and up at the people in the room.

“Ruff-ruff!” she said with a strong voice, looking at the cat.

When someone asks her if she wants to resume her exercises, or rather therapy sessions meant to treat her arthrogryposis, her face wrinkles like a plastic bag.

“No!”

Emily’s refusal to exercise is about the only anticipated behavior on her part. Diagnosed with classic arthrogryposis, or “curved joint,” before she was born Jan. 12, 2008, Emily is the embodiment of the word unanticipated.

Upon first sight, she appears smaller than most 22-month-old children. When she eats, she needs help from either Mary, her grandmother, or her father, Jesse, either of whom sit in front of her, guiding her arms that are held suspended by bands attached to a plastic pole above her.

Arthrogryposis multiplex congentia is a Greek term for “curved joint,” a condition that limits the range of joint motion. The condition began in her mother’s womb, where Emily was immobile and unable to move her joints. When joints aren’t moved within the uterus, extra connective tissue grows to fix them in position. Tendons connecting the joints fail to stretch to their normal length, making normal joint movement difficult.

Kristen Allen, her mother, said the condition remains a mystery.

“I was told that it could have happened because the blood didn’t go through her spinal cord correctly,” Kristen said. “Doctors started to notice something was wrong when she wasn’t moving inside me.”

Kristen was 16 when she had Emily, who was born weighing 4 lbs, 5 ounces. The condition has affected Emily’s hands, wrists, elbows, shoulders, hip, feet and knees. She had club feet when she was born, which required surgery on her achillies tendon, but that’s been the extent of any surgeries so far.

But they’re coming.

Jesse, her father, knows they are, but everyone expresses positive feelings concerning their daughter’s future.

“She’ll never be 100 percent cured,” Jesse said, “and she can digress as she’s growing, a little less as she’s older. She may need to use a power bike or crutches, but we know she’ll walk. We do.”

The severity of the condition varies, according to medical Web sites, and her physical therapist, Paul Chang, said he has seen about five or six cases during the past 30 years.
Chang, who has a clinic/office in Grain Valley, works with Emily on both land and in water.

 On Friday, Emily takes water therapy in Sedalia.

When he comes to her grandmother’s home in Buckner, he works with her on the floor, straightening and flexing her limbs, keeping her active.

“It’s always about playing, not therapy,” Chang said.

On this day, Emily is fussy when Chang takes control. Sitting before her, Chang balances firm therapy technique with humor, making high-pitch  noises, letting her run a toy into his fingers. While she’s quick to cry, she’s also quick to smile and laugh.

“Do you want to do your activites?” Chang said, scooting forward.

“No!”

Everyone laughs.

“She’s smart,” Mary, her grandmother, said. “I think one of the reasons why she is so smart and aware is that she’s with adults most of the day. And she notices the littlest things.”
Like the cat in the driveway.

“She’s as sharp as a razor blade,” Chang said.

Unlike a typical 2-year-old, Emily is under constant supervision. There is little she can do by herself. Recently she started learning to self-assist in her own eating, utilizing adaptive equipment that will most likely become a constant presence in her life as she gets older.

Jill Gonterwitz, an occupational therapist for Children’s Mercy Hospital, works with Emily and her family. Aside from the equipment that helps her eat on her own, she also uses leg braces and a specialty table that allows her to straighten her legs as if standing freely. At the table she sometimes fingerpaints and plays with playdough.

“We work with a lot of different materials,” Gonterwitz said. “Anything to strengthen her joints, keep them moving. The ultimate goal now is to help her be more independent and successful in her ‘occupations’ as a toddler.”

Adaptive equipment will become more important as she gets older, Gonterwitz said, and such equipment will help her do things like brush her hair and get dressed independently – even if she doesn’t have full range of motion. Other kinds of adaptive equipment include a speech-to-text program on a computer, or headphones if she isn’t able to hold a phone to her ear.

Chang didn’t wait, however.

Last summer, Chang drew up plans to convert a large toy truck into an adaptive equipment prototype, one that Emily can sit in and steer with her chin. Friends of his in Michigan, an engineer and automobile mechanic, built the machine – and delievered it the 14-hour drive from Detroit.

Come summer, look out pedestrians!

“She loves to use it,” Jesse said. “She loves running into me, too.”

But it’s had its mechanical problems, Chang said, steering issues that Goodyear Tire and Rubber Company in Blue Springs have agreed to fix.

Providing for Emily takes time and resources, her mother said. And the challenges are compounded because Emily has a brother, Gabriel, who was born a little over eight months ago. He has not shown any symptoms of arthrogryposis before or after his birth.

“I was worried about Gabriel before he was born,” Kristen said, “but he’s fine.”

Jesse expressed concern about when Emily goes to school. He knows how kids can be, how they can tease other kids who are different, but he also knows how brothers can be. As he sat on the floor near his sister, Gabriel kept an almost protective eye on her.

“I worry about her in school, but I’m sure he’ll be there for her,” he said.

Still, challenges persist. Struggles with transportation and the need to get places.

“We don’t have a car right now, so that makes it really hard, because there are appointments she has to go to,” Kristen said.

Trips include the Shriner’s Hospital in St. Louis, where there are five to six active cases of arthrogryposis, and where doctors provide information and advice about Emily; to Sedalia for water therapy, and basic errands around Buckner and the county. The family would like to attend a conference in Seattle, where doctors who specialize in the condition meet a few times during the year.

“It’s awfully stressful,” Jesse said, “but I haven’t given myself any other option. I just want her to walk, brush her hair. I’ve never doubted in my mind that she’ll walk and do normal things.”
Kristen agrees.

“She has a lot of people who love her,” she said. “She has a lot of people who give her a lot of support.”