Conquering Crouzon Syndrome

Whitefish 7-year-old battles rare congenital defect

Shandra Bell's goals in life are simple.

She'd like to master the monkey bars. She'd like to eat an orange without choking on it. And she'd like for her ears to line up in the same spot on both sides of her head.

All of those wishes may come true, but it will take time.

The 7-year-old Whitefish girl was born with Crouzon Syndrome, a rare congenital defect that affects the skull and facial bones.

Shandra was born a month early during a late spring blizzard in Sidney. Doctors at the small community hospital knew something was drastically wrong, but the weather prevented her from being flown to a larger hospital where specialists could fully determine the problem.

"She looked like an alien baby," Ronda said. "The doctors told me she may not make it through the night. But after an hour on a respirator, she pulled it out. I knew then she was a fighter."

At 8 months, doctors removed the top half of her twisted skull, reshaped it, created a soft spot and reattached it with dissolvable screws. They also reconstructed facial bones to reinforce her eye sockets.

Since then, her progress has been a marvel to the team of doctors in Great Falls that has followed her growth.

In 2000, the blonde-haired, blue-eyed girl also caught the attention of the Children's Miracle Network. She represented Montana as one of 50 "Champions Across America" who traveled with their families to the White House to share the message that their lives are better because of the exercise offered through Network that raises money for children's hospitals.

Life for Shandra has been a revolving door of surgeries to reshape her face as it grows. Her team of doctors includes a plastic surgeon, neurosurgeon, otolarynologist, audiologist, eye specialist, dentist and geneticist.

The next surgery she faces is orbital socket surgery to reshape her forehead and eye sockets.

"When she turns 12 they'll reconstruct her jaw," her mother, Ronda Bell said. "We chew up and down, but she chews criss-cross. We still have problems with her choking. She came home from school one day and said she had choked on an orange."

" 'It's OK, mom, I know what to do,' she said,'" related her mother.

Another effect of Crouzon is a narrow esophagus, which compounds the tendency to choke on certain foods.

Shandra's ability to take in stride the malady that has trailed her since birth is nothing short of inspirational. Another surgery? She shrugs, and asks what kind of Beanie Baby she can have when it's over.

Through physical therapy, some of Shandra's problems are being corrected. She's undergoing pool therapy this summer to strengthen her neck and upper body muscles. Doctors suspect those muscles are especially weak in Shandra because she wasn't able to develop them as a baby.

No one knew how bad the neck muscles were until her teacher at Bissell School called and asked her mother if Shandra was getting enough sleep, because she was literally holding her head up with her hands in class.

In addition to the swimming therapy, Shandra is undergoing horseback therapy and speech and sign therapy. She also undergoes some therapy at home, which has the family looking for a larger home than their current small mobile home, to accommodate the therapy activities.

Small victories are cause for celebration in the Bell family.

"She's now able to button her clothes," Ronda noted. "One of the goals was to be able to dress herself. For her, to zip a coat was a hard thing. She just learned how to tie her shoes, too."

Shandra has hearing loss and poor eyesight (she's legally blind in one eye) because of the Crouzon Syndrome, and will likely deal with those losses throughout her life. She can pass a hearing test, her mother said, but in the central auditory processing, sounds get lost between the ear canal and the brain. The result is she hears words differently than others. The word "die" becomes "buy" for Shandra on oral spelling tests, for example, a frustrating thing when she gets the word wrong and is sure she's heard the right word.

But those frustrations pale in comparison to what doctors initially told the family.

"We were told she'd never hear or talk," Ronda recalled. "When she was a year old they wanted to put her in the deaf and blind school, and I said 'you're not taking my baby.' And look at her now. She's proved everyone wrong."

Once Shandra's bones quit growing, the surgeries will likewise come to an end. "The doctors say they'll give her any surgery she wants once she gets to that point," Ronda said. "She wants her ears lined up."