One interesting story about fats and fatty acids is the story of Lorenzo's oil. Lorenzo Odone was a little boy diagnosed with adrenoleukodystrophy (ADL) and given two years to live. ADL is associated with the build up of very long chain fatty acids which appear to have a damaging effect on the myelin sheath of nerve fibres. This damage leads to loss of function and an irreversible loss of faculties in those afflicted. Paralysis, blindness and death were the normal outcomes of all boys diagnosed with ADL. Most unhappily, cognitive function of the brain seems to be unimpaired so the sufferer finds himself trapped within an increasingly non-functional body.
At the time of Lorenzo Odone's diagnosis, little was known about the disease, except for the elevated levels of long chain fatty acids in the blood. Conventional therapy used nutrition and diet control to eliminate all sources of these long chain fatty acids in the diet.
Counterintuitively, this regime seemed to increase the levels of the dangerous long chain fatty acids in Lorenzo's blood. So, his father, Augusto Odone, and mother Michaela Odone, started to teach themselves some biochemistry and got together a symposium of many scientists doing research into ADL and anything that might be related to it.
As a result the Odones decided to load Lorenzo's diet with a specific oil, derived from olive oil, but modified in a particular way. This oil, Lorenzo's Oil, seemed effective in halting the progress of the disease in Lorenzo. It has since been used, with somewhat limited success in other boys having been diagnosed with ADL. It is a standard treatment now in pre-symptomatic boys who carry the ADL gene.
Lorenzo died in 2008 at the age of 30 following a bout of pneumonia caused by the aspiration of food into the lungs, a common cause of death in patients suffering severe neurological dammage.
The Myelin Project was created by Augusto Odone to coordinate attempts to restore damaged myelin in patients suffering from ADL and related disorders such as multiple sclerosis.
Lorenzo's mother died in 2002, from lung cancer. Augusto continues to support the Myelin Project which he created. Lorenzo outlived his mother by 6 years. Doctors gave him 2 years to live in 1984.
Fats are so interesting!