GENETIC DISASTER
Generations of inbreeding in a polygamous US sect has led to terrible mental and physical disabilities in the community
THE cemetery is a mess of overgrown weeds. Sticking out of the cracked, red earth are tiny tombstones – hundreds of them, each one marking the grave of an infant or toddler. It’s a heartbreaking sight. How is it possible that in this small, secluded spot in rural America so many children have died at such a young age?
After examining the residents of the isolated community who live in the twin towns of Hildale, Utah and Colorado City, Arizona, it didn’t take scientists long to solve the mystery. The outside world first got a hint of what it was in 2007 when Warren Jeffs went on trial for sexually assaulting children and it came to light that he’d been forcing girls as young as 14 to marry their first cousins.
And now, six years after Jeffs started serving a life sentence for his crimes, the consequences of all the inbreeding the eccentric prophet – as head of the Fundamentalist Church of Jesus Christ of Latter-Day Saints (FLDS) – encouraged is plain for all to see. Children of the church are being born with horrific disfigurements and scientists are warning that the secretive religious sect is facing a “genetic disaster”.
The community of the nearby towns, an area collectively known as Short Creek, holds the dubious distinction of having the world’s highest known prevalence of fumarase deficiency. This is an inherited metabolic irregularity which is incredibly rare and causes children to be born with severe physical and mental disabilities.
There are kids in the community who are missing parts of their brains, who can’t sit or stand without help. They’re incapable of speech, have IQs of around 25 and often suffer from seizures.
The condition is so rare that most children have only a one in 400 million chance of being born with it – because in order to get it both parents need to carry the faulty gene. But as Short Creek has such a shallow gene pool, it’s estimated that between 75 and 80% of the community of roughly 8 000 people are related in some way. It’s thought that thousands of residents may be carrying the gene.
Every time a couple here brings a child into the world it’s like a game of genetic roulette. It’s suspected that many of the graves in the cemetery – some of which date as far back as the ’50s – may belong to children who’ve died as a result of this condition, which is commonly referred to as Polygamist Down’s. And tragically, even though it’s been almost two decades since the first official diagnosis of the disorder in the community, kids continue to be born with the deformity.
This is because the conservative polygamous sect point-blank refuse to believe that the debilitating condition is a direct result of their sexual practices.
“They have their mythology,” says Dr Theodore Tarby, a Utah-based paediatric neurologist. “They think it’s something in the water or something in the air.”
HE FIRST encountered a case of fumarase deficiency 17 years ago when he met the son of a couple who belonged to the church. The boy had unusual facial features including a prominent forehead, low-set ears, widely spaced eyes and a small jaw. He was also severely physically and mentally disabled.
Tests pinpointed that he had the condition but because it’s so rare, Tarby initially put it down to bad luck. But he
soon discovered that the boy’s sister, whom the parents believed suffered from cerebral palsy, had it too.
Tarby and a team of doctors diagnosed a total of eight new cases in children ranging from 20 months to 12 years old who lived in Short Creek.
“Arizona has about half the world’s population of known fumarase deficiency patients,” he says.
“It exists in a certain percentage of the broader population but once you get a tendency to inbreed, you’re inbreeding people who have the gene, so you markedly increase the risk of developing the condition.”
Although polygamy has been illegal in the US since the late 1800s the FLDS, a Mormon splinter group, has continued to practise it. After the Mormon Church decided to stop the practise of polygamy more than 70 years ago the sect broke away. In order to secretly continue practicing polygamy they settled in secluded Short Creek.
The church is so conservative that members are forbidden to watch movies, read newspapers or use the internet. Although Jeffs has been behind bars since 2007 for sexually assaulting two girls – one of whom was a 12-year-old he considered to be one of his 78 wives – it’s thought that he still controls the church.
One of the sect’s core beliefs is that a man needs to have at least three wives in order to get into heaven. This, together with the fact that it’s such a closed-off community, has resulted in rampant inbreeding. Fumarase deficiency isn’t the only congenital disorder that’s a problem in the community – hare lips, cleft palates and club feet are also common. Experts believe this may be down to the fact that 80% of people in the area are blood relatives of the two men who formed the sect in the remote desert region in the early 1930s.
The fumarase deficiency gene has been traced to Short Creek founder Joseph Jessop and his first wife, Martha Yeates. The couple had 14 children, including a daughter who went on to marry co-founder John Barlow.
Faith Bistline, who escaped the community in 2011, recently revealed she had five cousins suffering from the condition whom she used to help look after.
“They’re completely physically and mentally disabled,” says Faith, whose father had three wives and 28 offspring.
Out of the five of them only one cousin can walk and communicate.
“She can make some vocalisations and sometimes you can understand a little bit of what she’s saying, but I wouldn’t call it speaking,” Faith says.
All have feeding tubes and require 24-hour care.
Before he retired a decade ago Tarby tried to educate the community about the disorder. He told them it could easily be prevented if marriages between people with the recessive gene were barred, or if those couples avoided having children. But Tarby says all his suggestions were rejected.
Since the 1990s there have been 20 confirmed cases but experts reckon there may be plenty more children with the disorder who’ve gone undiagnosed.
Former members of the group say that instead of being horrified by the disfigurement, adherents see affected children as special “angels” sent from God to “test their faith”.
They also believe that when a leader of the church arranges a marriage for them they have a duty to go through with it – even if the spouse who’s been picked out for them is a blood relative.
“They’ll tell you if that’s what God wants for you, then that’s what you’ll get,” says Gary Engels, an investigator with the Mohave County Attorney’s Office. “They don’t think too much about marrying cousins and things like that.” SOURCES: BBC.COM, REUTERS.COM, HEALTH24.COM, DAILYMAIL.CO.UK, TIME.COM, MIRROR.CO.UK, BROADLY.VICE.COM
‘They think it’s something in the water or something in the air’