Kenneth Dansie - Questions and Answers: Living With OTC Deficiency

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1990 - Kenneth Dansie

4th Generation
Descendant of Andy Earl Wright

Birth: 1953
OTC symptoms confirmed 1990 age 37
Living with OTC

Questions and Answers: Living With OTC Deficiency

Starting in grade school, Kenneth lived with flu-like symptoms, throwing up, and going in and out of consciousness.

1983 - Kenneth was hospitalized and diagnosed with Reye’s Syndrome. 

1984 - Kenneth’s cousin, Lowell Kamerath, introduced him to the idea that his symptoms were related to a family genetic defect. Because of Kenneth’s earlier diagnosis of Reye’s Syndrome, his connection to an OTC deficiency was not identified at that time. The critical need to identify and develop a standard means of testing and treating OTC deficiency continued.

1990 - Dr. Leonard, geneticist at the University of Utah, confirmed Kenneth’s OTC symptoms following the death of his brother, Lynn Dansie, from OTC complications. 

1992 - Kenneth and his cousin, Lowell Kamerath, were referred to John Hopkins Clinic by Dr. Leonard to participate in drug research and treatment of OTC deficiency. Kenneth’s and Lowell’s OTC deficiency was contrary to what was accepted at the time. Young babies who lived for two months were considered long term OTC survivors.

The medications to treat their OTC were based on the urea cycle disorders—not for OTC deficiency. However, some of these medications helped treat the OTC deficiency. These medications required a “trial and error” approach. As they consumed more than 40 pills per day, they encountered sides effects such as illness and boils on their backs. 

They dissolved the drugs in Tang drink powder so they could more easily consume the medications. Later, the drugs were made into a capsule.  

1993 - Kenneth participated in a yearly “question and answer” clinic at the University of Utah where there was a low protein lunch served. Family members who attended shared stories, traded genealogy connections, and attempted to understand the OTC gene that was affecting their lives.

Family members who attended shared stories, traded genealogy connections, and attempted to understand the treatable OTC gene that was affecting their life’s.

Kenneth and Lynn had an older brother Dwain Leslie Dansie who died in 1942, at age 4, from misdiagnosed appendicitis.

Because Kenneth reached out to family members, it resulted in widespread awareness of a treatable OTC deficiency and the importance of being tested for high ammonia levels.

Note from Kenneth Dansie:

When I found out that I had OTC deficiency, I felt my days were numbered—what was I going to do, how was going to care for my family? It seemed as though there was not much of a future for me. Today, my future has hope; I have a regular life. We are fortunate because we have the technology available and if we have OTC deficiency it can be treated—we can live our life without fear.