Preventing future deaths
On November 24, 2017, my sister Audrey G. Hutchison’s great-grandson died from a rare genetic disorder known as ornithine transcarbamylase (OTC) deficiency. Baine Brady Bobka’s death has exposed a trail that this disorder has traveled through—more than seven generations of our family. Our family’s OTC deficiency can be traced to Jane Wright Earl—born in 1822 England, and died in 1913 Salt Lake City, UT. Jane and her husband, Jonathan Earl, were the parents of 13 children; nine who have descendants.
As a 4th generation descendant who is OTC positive, my mission is to contact as many of my relatives to provide knowledge and power that will enable them to prevent the unnecessary deaths from OTC deficiency. Each generation has family members who had undiagnosed flu-like symptoms that resulted in an OTC crisis and subsequent deaths. Often these deaths were attributed to: heart attack, Reye’s syndrome, polio, flu, food allergies, cancer, hepatitis, appendicitis, drug overdose, and more.
Through gene sequencing technology, the gene marker for our family was identified in March/April 2018. As a result, families who test positive for OTC deficiency no longer need to live in fear that their loved ones will die; hopefully Baine will be the last to die from our treatable genetic deficiency.
Ruth G. Jones
The Jane Wright Earl OTC website is dedicated to providing information for descendants who may be affected by ornithine transcarbamylase (OTC) deficiency. This disorder has caused misdiagnoses that has led to early deaths within our family line. Ornithine transcarbamylase deficiency is a genetic condition that is passed down from either your father or mother who are descendants of Jane Wright Earl. With new advancements in DNA sequencing technology, OTC is now treatable. The Jane Wright Earl OTC deficiency has been medically identified as c.-106C>A.