Fumarase Deficiency and the FLDS - A Tragic Secret
Two weeks have passed since the raid on the polygamist compound in Texas, giving the nation a look at some of the beliefs and practices of the secular FLDS church. Allegations of physical and sexual abuse, forced marriages of girls as young as 13 and plural marriage are being reported on news broadcasts around the world.

Church members have allowed a few members of the media limited access into their compound to speak out against both the manner in which the raid was handled and the way they and their children have been treated. This has also provided us with rare glimpse into the environment beyond the normally locked gates that have served as a barrier between two very different worlds. Video depicting soft spoken women dressed in handmade, full length dresses, all with similarly styled braided hair. Combined with the finely built, log buildings and the isolation of the surrounding area, one gets a feeling of stepping back in time, back to when life wasn't so complicated.

The FLDS has always been a secretive society, but perhaps their most guarded secret is an extremely rare and crippling disease affecting an increasing number of children back in Hildale and Colorado City, which is where the Texas members originated.

Fumarase Deficiency is an enzyme irregularity that interferes with the cells ability to transform food into energy. It causes a wide variety of devastating symptoms such as severe mental retardation, epileptic type seizures, physical deformities and can leave the patient unable to care for themselves.

"The disease itself is very rare in the rest of the world," according to Dr. Vinodh Narayanan of Arizona's St. Joseph's Hospital & Medical Center and Barrow Neurological Institute. Until recently, only about 13 cases were known worldwide. A few years ago, a pediatric neurologist named Dr. Theodore Tarby began treating some of the FLDS children in Colorado City and was shocked to discover several children were suffering from this genetic defect. By the early 1990's, about 20 cases had been documented. "Arizona has about half the world's population of known fumarase deficiency patients," according to Dr. Tarby.

Unexpected Consequences:
The genetic defect has been traced back to one of the community's founding patriarchs, the late Joseph Smith Jessop and his first wife, Martha Moore Yeates. One of their daughters married another of the community's founding patriarchs and religious leaders, John Yeates Barlow. By the time Joseph Smith Jessop died in September 1953, he already had 112 grandchildren, the majority of them directly descended from him and Yeates.

According to community historian and former FLDS member Benjamin Bistline, more than half of the 8,000 people now living in Colorado City and Hildale are blood descendants of the Barlows and the Jessops. It's believed that more than half the residents carry the recessive gene. If both parents carry the gene it is likely that their children may be affected or become carriers, passing the defect on to future generations.

"This problem is going to get worse and worse and worse," predicts 40-year-old Isaac Wyler, another lifelong Colorado City resident who was born and raised in the FLDS before he was booted out in January 2004. Wyler's ex-wife's sister has had two babies afflicted with fumarase deficiency. "Right now, we are just looking at the tip of the iceberg."

A Horrific Disease:
Most children born with Fumarase Deficiency usually do not survive infancy. Those that do survive often suffer debilitating conditions, requiring constant care, often unable to walk or even sit up.

"The kids that I have seen have terrible seizure disorders and developmental delays," says Dr. Aleck. "They are functioning way below their chronological age. "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 its way behind their peers."

Dr. 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. 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.

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. "They are totally helpless," he says.

Cost of Faith:
The only long-term solution to the health crisis is for Barlows and Jessops to have children with spouses from outside the polygamist community. "They have to outbreed," says Dr. Aleck, director of the Pediatric Neurogenetics Center at St. Joseph's Hospital.

But according to Bistline, that's not likely to happen. "They are discouraging any new blood," says the former member. "They claim to be the chosen people, the chosen few," he explains. "And their claim is they marry closely to preserve the royal bloodline, so to speak."

For those who practice a religious doctrine that requires men to be strictly obedient to religious leaders and requires women to give birth to as many children as possible to increase the sect's numbers, change is an unlikely. Rather than take steps to avoid the problem, the FLDS loyalists may believe it is their duty to accept their fate. "They think it is a test from God," says Wyler.

But if they refuse to stop and rethink the path they are on, the biggest issue facing the FLDS in the future is one of continuity. If preventative measures are not adopted by the group, further intermarriage between the Jessop and Barlow families will surely result in more afflicted children, perhaps hundreds of new cases, for future generations to suffer the consequences.
Originally published April 25, 2008