Saturday’s edition (11 February 2006) of the Casper Wyoming StarTribune.net carried an interesting Associated Press article about Fumarase Deficiency, a rare birth defect. At least it’s rare around the world, with only 50 cases documented world-wide.
Unfortunately, Fumarase Deficiency is becoming common among members of the Fundamentalist Church of Jesus Christ of Latter Day Saints (FLDS) residing in the twin towns of Hildale, Utah, and Colorado City, Arizona. Six years ago Dr. Theodore Tarby co-authored a medical journal article in which he described eight cases of the condition in the FLDS community. Today that number has grown to 20 known cases.
The AP article, under the headline “Doctor: Birth defects increase in inbred polygamy community” described the problem:
“Intermarriage among close relatives is producing children who have two copies of a recessive gene for a debilitating condition called fumarase deficiency. The enzyme irregularity causes severe mental retardation, epileptic seizures and other effects that often leaves children unable to take care of themselves.”
Ben Bistline, described as a community historian and former FLDS member, was quoted:
“Ninety percent of the [FLDS] community is related to one side or the other. They claim to be the chosen people, the chosen few. And their claim is they marry closely to preserve the royal bloodline, so to speak.”
A related article in the Salt Lake Tribune (“Rare gene disorder common in FLDS”) reported that Dr. Tarby met with 150 members from the FLDS community in November to explain Fumarase Deficiency and how it could be prevented. He apparently came away from the meeting without much hope for change. He said,
“They [FLDS members] consider these children to be their responsibility from God and their duty is to produce as many children as possible. There isn’t any reason in their view to slow down the having of children.”
Another related article, this one from Deseret News, pointed out that the afflicted children have severe mental retardation with IQs around 25. They require constant care, and receive it lovingly from parents and close relatives. However, Dr. Tarby said, medical care for the children is frequently borne by taxpayers.
