Jesse's Intent is the story of Jesse Geslinger. Jesse was born June 18, 1981, the second son of Paul and Pattie Geslinger. Jesse was diagnosed with ornithine transcarbamylase deficiency in March 1984. Ornithine transcarbamylase deficiency is an x-linked disease which causes ammonia to build up in the blood as proteins are broken down. OTC deficiency is caused by a mutation in the ornithine carbamoyltransferase (OTC) gene, and is a urea cycle disorder. According to the Genetics Home Reference web page, “The urea cycle is a sequence of reactions that occur in liver cells. It processes excess nitrogen generated when protein is used by the body, to make a compound called urea, that is excreted by the kidneys (2006 ).” OTC deficiency occurs when the enzyme that starts a reaction in the urea cycle is damaged or missing. Since the cycle is unable to continue normally, nitrogen builds up in the blood as ammonia.
Jesse grew up a normal kid, living with a rare disease. He went to school, he held a part time job, he had friends and a family that loved him. He was sick, but it was manageable with diet and medication. At the age of 18, Jesse entered a clinical trial that, although it wouldn't help him, might help those most in need, the infants that are born with this, or one of 25 other liver disorders. Jesse and his family were given assurances of safety and promising results from other participants. Jesse fell into a coma on September 13, 1999, shortly after being injected with a viral vector, and died the next day. It was later determined that serious concerns were ignored which cost Jesse his life.
After his death, it was discovered that monkeys had died in clinical studies, but the vector had been changed to one that was “much safer”. An web based data base (The Gene Therapy Information Network), that was meant to protect the participants of such studies, was disbanded. When questioned, the FDA representative response was “my superiors answer to industry”. Shering-Plough had claimed the adverse affects in comparable gene transfer studies as proprietary, and was able to cover up the adverse information. The research team had violated protocol by not reporting serious adverse reactions in other patients, and withholding adverse reaction information in animals to the FDA.
Jesse's father eventually...