12-year-old eating for 1st time after medical miracle
Published: August 19, 2005
Twelve-year-old Sylvia Craig chopped up a white onion so fast she could have been on a TV cooking show.
As she worked, she kept an eye on her sister, Andrea, stirring a large skillet of Hamburger Helper on the stove.
It was only about 4 p.m., a little early for dinner, but Sylvia was ready to eat.
Not unusual for a growing preteen. But for a child who for nearly 12 years was fed through tubes, her ability to wolf down Hamburger Helper with onions is a “medical miracle,” says her doctor, Mitchell Shub.
Gail Hull, Sylvia’s single mother, is ecstatic.
“I’ve been praying for this for 12 years,” she said.
Sylvia, of west Phoenix, was diagnosed as an infant with microvillus inclusion disease, an extremely rare genetic and often fatal digestive disorder reported in only about 50 people worldwide, including a cluster of cases among Navajos.
Shub, a gastroenterologist at Phoenix Children’s Hospital, believes that Sylvia, a feisty redhead with new braces on her teeth, is only the second MID patient in the world to begin recovering enough to eat real food.
Sylvia and the other patient, whose location was not disclosed, may have a milder but even rarer form of the disease that allowed their intestines to begin working, Shub said.
Still, Sylvia and her family suffered the same clinical horrors of the disease as children with other forms of MID: diarrhea, up to 14 hours a day hooked to intravenous feedings, lengthy hospital stays, infections and likely death in the early to midteens.
Without the intestinal hardware to digest food, MID patients survive on a liquid concoction of protein, carbohydrates, vitamins and minerals sluiced intravenously into the bloodstream to bypass the dysfunctional intestines.
Still the diarrhea persists, draining away 20 percent of the children’s body weight a day, 10 times faster than the normal rate.
Families live with a triple threat: dehydration, infections in the IV line and liver failure caused by the cocktail that substitutes for food.
Given the rarity of the disease, Phoenix Children’s Hospital’s 12 patients since the late 1980s makes “us one of the authorities worldwide,” Shub said.
Half of the 12 have died. Two have had bowel transplants, but it is too early to predict the outcome, Shub said. The remaining patients include Sylvia, who is White, two Hispanics and a Navajo.
In 1996, four of the hospital’s five MID patients were Navajo.
Experts believe that a small band that fled the 1863 Long Walk to New Mexico settled on the western edge of the of reservation, where a cluster of MID cases have been found. Forced to intermarry to survive, they increased the chances that two people with the faulty gene would meet and marry.
In the beginning, Sylvia’s story was much like the other children’s.
She weighed 5 pounds when she was born in Springerville in 1992. Premature, she had jaundice and struggled to breathe. Flown immediately to Phoenix Children’s, she remained for two days. Three weeks later she was back, suffering from severe diarrhea and vomiting.
When she was 3 months old, a biopsy of her intestine confirmed she had MID.
Like other out-of-town MID families, Gail moved to Phoenix to be near Sylvia.
“I never went back to Springerville,” she said. “It was awful watching my little girl suffer and not be the kind of mother who could do everything for her.”
The disease is also hard on siblings.
Andrea Hull, now 20, remembers coming going to the hospital to wait for her mother to leave her sister’s bedside.
“The nurses,” she said, “would help me with my homework.”
MID isolates children.
When Sylvia was well enough to go to school, she watched as other children ate their lunch.
“I got used to it,” she said.
Gail and Andrea tried not to tempt Sylvia by eating in front of her. But she was clearly curious, stirring up concoctions of flour, milk and spices, “and trying to get us to eat it,” Gail said.
A former land surveyor, Gail learned to look for infections and hang IV lines when Sylvia was well enough to come home from the hospital.
In addition to theIV nutrients, Sylvia was fed Tolerex, a predigested formula that flowed into her digestive tract first by tube and then by mouth.
“What made Sylvia unique is that she could tolerate the formula,” Shub said. “In the other kids, it made their diarrhea worse, and they got sicker.”
Encouraged by reports of the other recovering MID patient, Shub slowly weaned Sylvia off the IV concoction, upped the Tolerex, and a few months ago introduced rice.
“I hate rice,” Sylvia said, wrinkling her perky nose.
Also on the list of food she hates: applesauce and cereal.
Lying on a couch in her west Phoenix living room, Sylvia still sips on the Tolerex to help maintain her improving condition. But she also digs into a bag of Funyuns, a favorite, along with cantaloupe, watermelon, honeydew and apples.
A career caregiver for 12 years, Gail now has time to look for a job.
“I’m clueless about what I want to do,” she said. “But I’m grateful for the miracle.”
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