From Deseret News archives:
Birth defect is plaguing children in FLDS towns
Fumarase Deficiency afflicts 20, is linked to marriages of close kin
An especially severe form of birth defect is on the rise and may mushroom in coming generations.
According to experts and former Jeffs followers, the cause of the birth defect is clear: Intermarriage among close relatives is producing children who have two copies of a recessive gene for a debilitating condition called Fumarase Deficiency.
They predict the scale of the problem will increase dramatically in the future. Wyler, who has lived in the polygamist community most of his life, said he expects residents to continue marrying close relatives.
"Around here," Wyler said, "you're pretty much related to everybody."
Fumarase Deficiency is an enzyme irregularity that causes severe mental retardation, epileptic seizures and other cruel effects that leave children nearly helpless and unable to take care of themselves.
Until a few years ago, scientists knew of only 13 cases of Fumarase Deficiency in the entire world. Tarby said he's now aware of 20 more victims, all within a few blocks of each other on the Utah-Arizona border.
The children live in the polygamist community once known as Short Creek that is now incorporated as the twin towns of Hildale, Utah, and Colorado City, Ariz. Tarby believes the recessive gene for Fumarase Deficiency was introduced to the community by one of its early polygamist founders.
According to community historian Ben Bistline, most of the community's 8,000 residents are in two major families descended from a handful of founders who settled there in the 1930s to live a polygamist lifestyle.
"Ninety percent of the community is related to one side or the other," Bistline said.
For many years, Bistline was a member of the Fundamentalist Church of Jesus Christ of Latter-Day Saints (FLDS), which today reveres fugitive polygamist Warren Jeffs as a prophet.
"They claim to be the chosen people, the chosen few," Bistline said. "And their claim is they marry closely to preserve the royal bloodline, so to speak."
Wyler, who says Jeffs kicked him out of the FLDS group two years ago, has observed some of the "Fumarase children" in their home environment.
Recent comments
There have been many women and men run away from/come out of the LDS...
Sad | Sept. 16, 2008 at 7:29 p.m.
AMEN Ankhorite!!
Nancy | Aug. 8, 2008 at 3:09 p.m.
Religion has nothing to do with this. This is a matter between right...
Nancy | Aug. 7, 2008 at 5:30 p.m.
- Aggies beat Spartans in snowy Logan 4:31 a.m.
- TCU 55, Utah 28 4:24 a.m.
- BYU 24, New Mexico 19 4:21 a.m.
- Jazz game at a glance 3:00 a.m.
- Real Salt Lake: Game at a glance 3:00 a.m.
- Stanford ends Y's soccer season 2:20 a.m.
- Jazz hope D-Will returns soon 2:19 a.m.
- Snow, SUU lose 2:18 a.m.
- Toone saves day for Wildcats 2:15 a.m.
- Win in New Mexico good for Y? 2:15 a.m.
- Apostle's wife felt comfort in attack
- Can BYU root for (ick) Utah Utes?
- Short-handed Jazz fly past Sixers
- D-Will home for daughter
- Utes excited for 'dream' game
- Bench proves fruitful for Y.
- Crash on snowy road kills woman
- BYU happy to escape with victory
- GameDay in Fort Worth
- Born of water and the spirit
- SLC council OKs gay rights policies
358 - Editorial: Mormons and gay rights
200 - BYU happy to escape with victory
194 - Will state consider gay rights law?
148 - TCU creams U.
134 - Can BYU root for (ick) Utah Utes?
130 - Letters: Strange breed in Utah
129 - Utes remain silent about BCS
120 - Pratt pleads not guilty to sex charges
106 - Celtics crush Jazz
104
If you are looking for a bird on the cheap, the following specials from...
How do you handle kids and contests? Our oldest daughter, 7, is of the...
So Great!!! So Proud - Love RSL - Bring Home The CUP!!!!!
mr cannon's bold assertation that the purpose of the first ammendemnt as...
Great great great game!!!! Nicky Rimando is a god! We're the most complete...
I had the game on DVR and just watched it. That was the most exciting game...
financially cannot this year, but I will watch loyally, how great to hear...
This is hardly surprising. Bennett has a remarkable arrogance which is also...
I guess that is why "they play the game" as Herman Edwards would say.. ...
What was the score of the LSU vs LA tech game? Alot closer than you'd like to...
Has Fedor not said that THIS IS OUR YEAR all year long? Go back and...
This is just a small glimpse of the future with Obamacare: corruption, waste...

