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Yet, Aleck says, some children are more seriously affected by the disorder than others. "Some are very debilitated and some aren't," he says.
Some fumarase deficiency children, he says, develop a small degree of motor skills over time: "They don't remain infantile their entire life. They do develop to some degree, but it's way behind their peers."
Dr. Tarby, who routinely treats fumarase deficiency children at a state-funded clinic in Flagstaff, says, "They are funny-looking kids [with] biggish heads and coarse, thick features."
Their brains, he says, "are strangely shaped" and are frequently missing large areas of brain matter that has been replaced by water. An MRI of the brain of one fumarase deficiency child showed that more than half the brain was missing.
Tarby says most of the children "can say at least a word or two," but that all of them "have severe mental retardation" with IQs of less than 25.
Some of the kids can walk, but others have a difficult time even sitting. The children who can't walk, the medical experts say, have most likely suffered strokes during severe seizures.
Despite the secrecy in the community over fumarase deficiency children, Wyler says he has observed his ex-wife's sister's children and others on several occasions.
"People don't like to talk about their fumarase babies for obvious reasons," Wyler says. "I don't know how many who die within the first two or three years that we don't even ever know about."
Wyler says he has seen some fumarase deficiency children who can walk, but others can barely move and spend their entire lives prone.
Children of the latter variety, he says, "can't crawl. They can't sit up. They are lucky if they can even move their head and eyes a little bit."
All of the fumarase deficiency children Wyler has seen remain dependent on the parents or caregivers.
"They are totally helpless," he says.
Frequent and powerful seizures are among the most disturbing characteristics of the disease. Wyler says he once saw a fumarase deficiency child suffer a seizure while she was sitting with her mother and two other children also suffering from the disorder.
"All of a sudden [with] this one little baby, everything tightened up and she arched her back so hard her head was almost touching her toes," Wyler says.
"The mother," he says, "was just sitting there rubbing her hands on [the child's] back trying to get her to relax."
Families with fumarase children receive in-home help from the Division of Developmental Disabilities, a unit of the state Department of Economic Security. Much of the state care is simply helping parents with hygiene, feeding and mobility of the child.
"One lady I know, she just cannot physically pick [her son] up anymore to get him into the bathtub," Wyler says. "A lady comes in and helps her. And it takes two of them to get him into the bathtub just to wash him down and clean him up."
One advantage of polygamous families, Wyler says, is that the mother of a fumarase child will likely have other women in the household to lend a hand.
"A sister wife would be a godsend just to be able to help out," he says. "Not only to help physically, but to be somebody to talk to."
Arizona used to send doctors from Children's Rehabilitative Services, which is a division of the state health department, to Colorado City on a regular basis to examine fumarase deficiency children.
But doctors stopped going to Colorado City after the state and press stepped up scrutiny of the community in 2004. Doctors feared that the media would photograph fumarase deficiency children as they were entering a medical clinic in Colorado City.
"We had no desire to encounter ABC News at the clinic entrance," Tarby says.
The doctors only agreed to talk to New Times after Tarby was approached with a copy of the fumarase deficiency study.
Families now must drive fumarase children to Flagstaff for regular evaluations. Despite the frustrations doctors have with dealing with a community that refuses their recommendations on how to prevent the condition in the future, there is no question that treatment will continue.
"We do not deny medical care to people because of religious beliefs," Tarby says.
In fact, the state's willingness to provide medical assistance to afflicted children may be allowing Utah families to receive treatment paid for by Arizona taxpayers. "I don't know if all the patients I treat are technically eligible for my services [because they may live out of state]," Tarby says.
Researchers have identified a gene on the first chromosome that causes fumarase deficiency, but no test has been developed that could be used to identify individuals carrying the malady. If such a test were developed, a community-wide screening program could be instituted that would identify those carrying the fumarase gene.
Dr. Vinodh Narayanan, a pediatric neurologist at St. Joseph's Hospital, says he is seeking funding to develop a test that would allow public health officials to collect voluntary blood samples from as many FLDS members as possible. The samples could be tested for the gene at the Translational Genomics Research Institute in Phoenix.
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