Lynn Dell Kraamia - 20 Years Later: Suspected Genetic Link To OTC Deficiency

Lynn Kraaima_SM.jpg

2010 - Lynn Dell Kraamia

4th Generation
Descendant of Edwin Wright Earl

Birth: 1958
OTC symptoms diagnosed 2010
Death: 2010 age 52

20 Years Later: Suspected Genetic Link To OTC Deficiency

According to his family, his diet consisted of salads, Pepsi, and Snickers candy bars; he preferred sweets over meat. The doctors suggested he may have instinctively self-medicated himself with the carbs in the Snickers and Pepsi as well as the Phenylalanine in the Pepsi.

2010 - Lynn was given a high doses of steroids to treat his cancer. Soon after he started vomiting and continued vomiting for two days with flu-like symptoms. He had “weird” conversations, with strange and bizarre behavior. He was taken to the ER where he became very uncooperative. He required help to get in and out of the car. By the time he arrived at the hospital, he did not recognize his wife. He was fighting and kept trying to climb out of the bed and had to be held down.

He was in a self-induced coma with ammonia levels over 400 and was given dialysis and nitrogen scavenger drugs to treat his symptoms. The liver specialist referred him to the pediatric metabolic specialist, Dr. Longo, at Primary Children’s Hospital. It was suspected that Lynn had a very rare metabolic illness that was possibly connected to OTC deficiency, and that it was probably triggered by the steroid he had been given to treat his cancer.

While in the hospital, a family connection was formed between Lynn Kraamia’s family and Kenneth Dansie. Consequently, they discovered that they were both descendants of Jane Wright Earl through two of her children. Lynn’s family, who are potential carries, was introduced to the ongoing study of drugs to treat OTC deficiency.

Lynn died Tuesday May 25, 2010 from OTC complications. His symptoms were similar to those of his younger brother, Keith Kraaima who died in 1989 at the age of 24.

Because of Lynn, the family presentation of a metabolic disorder with flu-like symptoms was medically exposed again after 20 years, however, the genetic markers of the treatable OTC deficiency were still not identified.

Note from Diane Kraamia, Lynn’s Mother:
As I think about my two sons, Keith and Lynn, who died of OTC symptoms, I look forward to someday meeting them again. I am also comforted and confident with the strides being made to prevent anymore deaths from this illness. It feels good knowing there is help on the way. There are so many people working on this and I appreciate all the work that is being done.