A medical mystery

Editor's note: This is the last in a series of articles from the Dexter Daily Statesman focusing on special needs of special children.

By NOREEN HYSLOP

Dexter Daily Statesman

BLOOMDFIELD, Mo. -- During the fifth month of Sharon Cox's pregnancy, she and her husband, Rodger, were told their baby girl would have problems. An ultrasound revealed the baby had no esophagus. It was a condition known as esophageal atresia, estimated to occur in one of every 4,425 births.

The condition is a congenital birth defect affecting the alimentary tract. It causes the esophagus to end in a blind-ended pouch rather than connecting normally to the stomach. It is not usually related to other complications at birth and can usually be corrected with surgery.

"That was the only thing we were expecting to be wrong," recalls Sharon. "We had no idea of the other complications that would be evident at birth."

Sharon was admitted to Barnes Hospital in St. Louis once she was full term. Her labor was induced and Elizabeth, weighing just four pounds, seven ounces, was born on June 8, 1999. As expected, the esophageal atresia existed, but there were a number of other issues that kept Elizabeth in the hospital for the entire summer of 1999.

Lizzie, as she was quickly dubbed, also was born with a misplaced bowel. Doctors operated on her to reposition the bowel and to remove the appendix in the process, when she was just days old.

Among her many diagnoses was microcephaly, a medical condition in which the circumference of the head is smaller than normal because the brain has not developed properly or has stopped growing. It's a condition that with Lizzie was evident at birth also.

Because of the esophageal atresia, she was immediately placed on a feeding tube to administer her nutrition. Today, that has not changed. Lizzie is 11 years old and has never tasted food or drink through her mouth. There is simply nowhere for it to go. The first attempt to surgically correct the lack of an esophagus took place when she was a year old. That attempt and others that followed were unsuccessful. And so, since birth, she has been fed through a tube in her stomach several times a day.

Without the ability to swallow comes a continual issue of saliva and mucus that the body must expel, so Lizzie wears a bib at all times to help relieve the problem. While she sounds congested, the sound is simply the process of her system expelling what normally would be swallowed.

"To make that a bit worse," says Sharon Cox, "Lizzie for some reason produces extraordinary amounts of saliva and mucus. She did this even as a tiny infant and doctors have no idea why."

There are many medical issues concerning Lizzie for which doctors have no answers; there is no rhyme or reason to define some of her disabilities.

"Lizzie was taken to Children's Hospital in St. Louis as soon as she was born," Sharon explains, "and when we finally got to bring her home, the doctors told us they really didn't know what to expect."

She was three months old when she came home to Bloomfield. She had two holes in her heart, a feeding tube, and a number of issues that made early life at home with the infant a cautious undertaking.

Their daughter might never walk, they were told. She might never talk and it was suspected initially that Lizzie would have both vision and hearing deficits. The family was told they couldn't predict her life expectancy. While Lizzie presented a number of symptoms and disabilities, there is no one syndrome to which doctors can point and attribute her many disorders. She is, by some accounts, a medical mystery.

"One of her doctors once told us that Elizabeth is just Elizabeth. She's got things going on that are hers and hers alone."

One of her early surgeries corrected a vision problem. Other surgeries followed, but as an 11-year-old, it has been a few years since she has had to undergo further surgery. Although she frequently suffers from ear infections, she isn't typically an ill child despite all her areas of concern.

Lizzie Cox has defied the odds and predictions regarding the quality of her life. She is a happy child who smiles and at times shows discontent. Although there is no speech, she produces a low grumble that often indicates her disapproval of a television show or a physical discomfort.

Anyone who knows the Cox family knows what perhaps most contributes to Lizzie's quality of life. Her siblings, 16-year-old Jacob and 8-year-old Madison, are the loves of her life. And it's quickly evident that Jacob and Madison feel the same.

There is a lot of affection in the Cox home with much of it directed toward Lizzie. While she cannot walk, she can "scoot" with her body upright, quicker than most infants can crawl. She uses the method to navigate the household, but is also transported with the support of her family, taking steps as either mother, father, or Jacob carry most of her weight.

"Lizzie loves music," Madison is quick to tell. "She can't talk, but she loves to watch cartoons and she smiles and loves the music that's played on certain cartoon shows."

She also enjoys lying down with an ear placed directly on a lighted device that emits nursery singalongs with the press of a button.

"She knows which button to push," says Jacob, "to make it play again when a song stops."

Jacob and Madison inspire her to smile and favors their attention. The siblings are often found at floor level interacting with Lizzie.

"Sometimes," says Lizzie's grandmother, Pat Robinson of Dexter, "we have to wonder just how much Lizzie is understanding. About the time that we think there's not much comprehension, she'll shoot us a look of disapproval at a comment or motion for something she needs or laugh appropriately."

Lizzie attends the State School in Dexter where she receives therapy and gets considerable one-on-one attention.

"The teachers," her mother explains, "use a lot of different techniques to get Lizzie to respond and it's sometimes amazing what she can do."

Among her recent accomplishments, she has learned to appropriately select her name in print when presented alongside several others. Her feats are baby steps requiring intense direction and therapy, but ones that are celebrated daily.

"Lizzie is different, of course," agree Rodger and Sharon Cox. "And while most people are accepting of that, there are naturally children who step back and don't know what to expect of her."

But Lizzie's mother encourages questions. "Parents sometimes instruct their children not to come over and stare at Lizzie and not to ask questions. But I always tell them, 'Let them ask!' They're never going to feel comfortable around someone who is different if they're not allowed to ask questions."

"She's our very special gift," says her mother, "and we're all blessed to have her with us."