GW Winter 2015-16
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Volume XXVII , Number I Winter 2015-16 From Our Readers THE SEED OF JOSEPH: A STORY OF GENETICS, FAITH, AND ENDOGAMY IN THE MOUNTAIN WEST (AN ETHICAL DILEMMA) Doctors Mark Hueftle, Colleen By Reuel Jake Measom (Winner of the 2014 Student History of A Disease Essay) Morris, and Arrah Curry acknowl- And cursed shall be the seed of him that mixeth with their seed; for they shall be cursed even with edged the same cursing. And the Lord spake it, and it was done. The Book of Mormon appreciation of our efforts to record Nevada history of In 1990 pediatric neurologist Dr. only 13 cases had been docu- medicine. Theodore Tarby at Arizona State’s mented—a deficiency of an Patricia D. Cafferata, Esq. Children’s Rehabilitative Services, enzyme in the metabolic process writes, “Thanks for dedicating your saw patients from the polygamist of the Krebs Cycle. article about Vietnam to Treat and settlement of Colorado City, Ari- The boy lacked fumarase, an the others who served there.” zona. Despite being well seasoned essential enzyme for production It was brought to the editors’ in practice, Dr. Tarby was reminded of energy. The cause was un- attention by Dr. Tom Brady that of the vastness of disease when a known Dr. Tarby took a family Dr. Jack Pershing (Carson City) woman from Colorado City carried history and discovered a daughter was a Vietnam veteran. We regret in her young son. Dr. Tarby quickly diagnosed with “cerebral palsy.” that we were unable to find all of saw something was amiss and He saw that she had the same the names of Vietnam doctors mental retardation was obvious. condition as her brother, disprov- from southern and rural Nevada. If Even the untrained eye could see ing that fumarase deficiency was a you have such information please the physical abnormalities, particu- random chance mutation. A let us know so that we can ac- larly the boy’s misshapen skull. Dr. reality was soon recognized—the knowledge them in a future edition Tarby was positive that this was not genes were inherited through of Greasewood Tablettes. a case of cerebral palsy or Down selective breeding. These genes syndrome and sent a urine specimen were located in the fundamentalist to Dr. Steve Goodman at the Mormon settlement of Colorado University of Colorado. Dr. City, a place where blood lines Goodman discovered a highly were braided by arranged marriage unexpected condition so rare that and inbreeding. John Yeates Barlow founded propagate the problem, the hence both parents possessing the Colorado City, originally called community did not take heed. gene increased the likelihood of Short Creek, in the 1920s. The In 1937 Hans Adolf Krebs its occurrence. Aleck and the settlement was the result of a established and formalized the others describe the effects of the schism within the Mormon Church concept of the citric acid cycle– disease in detail. The disease following the 1890 Manifesto by also known as the tricarboxylic causes seizures, hypotonia, and Church President Wilford Woo- acid (TCA) cycle or the Krebs early onset encephalopathy, but druff that disbanded polygamy as Cycle—as a cornerstone in the key to diagnosis is excessive a felony. The practice, originally human’s metabolic processes. Its urinary fumaric acid. taught by Mormon founder Joseph role in creating useful coenzymes The researchers traced the Smith, was considered an essential from carbon-based molecules is defective gene to Joseph S. Jessop step in attaining the highest degree crucial for life. A paper in the and wife Martha. They discov- of heavenly glory, a place in the The Mitochondrion states, “This ered the extent of the gene’s Celestial Kingdom. Government pathway is so crucial to the distribution and demonstrated pressure and exclusion from metabolism of living cells that that most of the residents are statehood moved Mormon leaders any significant defect is incom- related to the patriarch. They to abolish the practice, but many patible with life.” A few years discussed the situation with considered polygamy to be man- before Dr. Tarby stumbled on church leaders and explained that dated by God and felt that Presi- fumarase deficiency, a defect intermarriage produced children dent Woodruff had fallen into sin. existing in the TCA cycle was with disabling abnormalities. Thus, accordingly they sought believed to be an impossibility Their warnings fell on deaf ears solitude in the wilderness. and incompatible with life. and town members were con- Joseph Smith Jessop followed Donald Whelan described the vinced that it was in the water or John Y. Barlow as the community condition in 1983 when he and a result of older women having leader. Jessop, a deeply religious his colleagues reported on 2 babies. Historian Bistline stated, man and a patriarch within the adult siblings with fumaric “It is something that is simply not Mormon priesthood, married his aciduria and mental retardation. talked about.” It is a taboo first wife, Martha Moore Yeates in They discounted an inborn error extending from the belief that 1889. They had 14 children and of the fumarase enzyme, citing physical maladies are a manifesta- did not know that mixing their two that the only place fumarase has tion of God’s indignation towards bloodlines would cause fumarase a function is in the TCA cycle, sin. Despite the polygamists’ deficiency. This became obvious and any error would be disas- indignation, Doctors Tarby and when their 12th daughter married trous. The enzyme defect was Aleck convened a meeting and her second cousin, John Y. Barlow. not recognized until 1986 when laid out what it would take to In the Fundamentalist Latter Arthur Zinn reported an infant eradicate the condition. These Day Saints (FLDS) a prophet male with severe early onset measures would require the governs the community, and encephalopathy. Zinn became genetic screening of members important decisions go through convinced that the boy’s condi- and the prohibition of marriage him, including marriages. To tion was fumarase deficiency. between carriers of the gene. It maintain “pure bloodlines” ap- Doctors Tarby, Kirk Aleck, also meant that families showing proximately 80% of the commu- and a team of physicians from the condition should cease having nity is related to Joseph S. Jessop St. Joseph’s Children Hospital in more children. The FLDS did not or John Y. Barlow. Benjamin Phoenix went to Colorado City. accept these conditions. Bistline, a historian and former The result was a landmark study The community was not member of the Short Creek po- on fumarase deficiency and the willing to make changes because lygamist community, labels the largest documented cluster in the the condition is uncommon. issue “religious totalitarianism.” world. They reported 8 cases and Bistline, who married into a mix Despite the discovery of fumarase thought there might be more. of the Barlow and Jessop line, deficiency and repeated warnings The disease is inherited in an described two of his nieces with that the practice of endogamy will autosomal recessive fashion; the disorder. He stated that he was lucky that none of his children community resulting in a no signs of altering their religious possessed the malady. He felt his higher likelihood of inbreed- practices, an ethical question is progeny were able to “over power” ing. This could result in an raised. What is the role of the the Jessop line. He alluded to the increased opportunity for scientific community and govern- notion that there is a negative conno- propagation of the defective ment to protect the unborn and tation with the Jessop line in con- gene. prevent practices known to cause junction with the illness. Whether the As there is no cure for the genetic diseases? polygamist community believes the condition, and the FLDS show condition to be due to past transgres- sion is unknown, but some believe that the seed of Joseph S. Jessop is Dr. Hugh Collett Family Donates Leonardo cursed. What is known in the scien- da Vinci Anatomical tific community is that as long as intermarrying continues the condi- Etchings To The School Of Medicine tion will grow. The Collett family donated in When Dr. Tarby conducted the seven years, as did Dr. George A. June 2015 anatomical etchings by Collett, who came to Elko 11 study, FLDS prophet Warren Jeffs, Leonardo da Vinci to the December 1946 after practicing in who had not been seen since 2003 University of Nevada School of Crawfordsville, Indiana. He died and is now incarcerated, was part of Medicine. They are now suddenly at 63 years of age after the community. To prevent the displayed in Savitt Library at the practicing in Elko for 7 years. His children from being photographed entrance to the History of dreams for a future medical center the doctors conducted the clinic in Medicine Library. Elko’s Dr. were promoted under his dynamic Arizona, but they feared the distance George A. Collett collected the leadership. He was also involved in also would inhibit genetic counseling. etchings over 80 years ago. civic affairs and was named president Colorado City’s religious influence Leonardo da Vinci made the of the Elko Chamber of effects the medical community and muscle-skeletal drawings around Commerce. 1510, and in 1796, John government officials in protecting Elko’s medical heritage was Chamberlaine published his primarily due to the standard of future progeny. People have religious Imitations of Original Designs by medical and surgical care of the freedom rights that can cause propa- Leonardo da Vinci, with etchings by earlier partnership of Dr. A. J. Hood, gation of a terrible illness. Sadly, Francesco Bartolozzi. The original Dr. Charles Secor, and Dr. R. P. obedience to their beliefs, such as etchings are on display at the Roantree.