Augusto Odone was a charismatic Italian economist whose job with the World Bank afforded a cosmopolitan lifestyle. But the important work he performed for impoverished countries paled by comparison to the job that began to consume him in 1984: saving the life of his gravely ill son.
That year, Odone's 6-year-old son Lorenzo was diagnosed with a fatal genetic disorder that doctors said caused the loss of voluntary movement and death within a few years.
Rejecting the grim prognosis, Odone and his linguist wife, Michaela, immersed themselves in medical journals to learn everything they could about the disease, adrenoleukodystrophy, or ALD.
Odone ultimately helped develop a treatment that defied medical orthodoxy — a concoction based on two common cooking oils — and slowed Lorenzo's decline.
"Lorenzo's oil," as the treatment was named, provoked cries of quackery from many doctors and researchers, but a study published in 2005 showed it was effective in preventing the onset of symptoms in boys who had been diagnosed early.
Odone, who also promoted research that has led to a screening test and other therapies for ALD, died Thursday in the city of Acqui Terme in the Piedmont region of Italy. He was 80 and had heart problems and other ailments, his daughter Cristina Odone said.
Portrayed by Nick Nolte in the 1992 movie "Lorenzo's Oil," Odone outlived his wife, who died of cancer in 2000, as well as his son, whose death in 2008 at age 30 was a remarkable testament to the Odones' devotion and ingenuity.
"In the end, his heart just gave way," his daughter said Thursday. "He had an extraordinary life force. He didn't accept a death sentence with his son and he didn't accept a death sentence with himself."
The first signs of trouble emerged when Lorenzo was in kindergarten in Washington, D.C. He was falling, having problems hearing, slurring his speech and throwing tantrums. A precocious child who loved Greek myths and opera — he once chided his father for not being able to tell Placido Domingo from Luciano Pavarotti — he clearly was not himself.
At first his parents thought he might have contracted a mysterious disease in the Comoros Islands off Mozambique, where Odone had been sent on a World Bank assignment.
After extensive testing, doctors delivered the devastating diagnosis of ALD, a hereditary disease carried by females and afflicting males, mainly young boys. According to the Stop ALD Foundation it strikes about 1 in 18,000 people. Within a few years of his diagnosis, Lorenzo was virtually paralyzed.
Doctors scoffed at the Odones' determination to find a treatment for the disorder, which destroys the myelin, the protective sheath surrounding nerve cells in the central nervous system that control bodily functions. They were told that scientists were working on treatments but that a solution would not come in time to save Lorenzo.
Slogging through hundreds of academic articles, Odone learned about the link between ALD and abnormal levels of "very long chain" fatty acids. Gradually, he gleaned from numerous accounts that animals fed olive oil had lower levels of very long chain fatty acids and that the key component producing the effect was oleic acid. He also learned that erucic acid, a component of rapeseed oil, might also help counteract the harmful fatty acids. He began to form the idea that a mixture of rapeseed and olive oils might save Lorenzo.
It was a battle to convince experts that the oils might help. One of the first he consulted was Hugo Moser, a neurologist at the Kennedy Krieger Institute in Baltimore, who told Odone that erucic acid was harmful in mice and could not be tested on humans.
Odone pursued other experts, finally finding a Toronto biochemist who told him that erucic acid was safe for people.
By the end of 1986, a British biochemist produced an edible extract of the two oils for Odone. After testing it on a family member, he tried it on Lorenzo, with dramatic results: Within three weeks, the boy's levels of the harmful long-chain fatty acids began to drop to normal levels. He credited Lorenzo's oil with extending his son's life many years beyond what doctors expected.
"One problem with medical research," Odone told the Washington Post in 2008, "is that doctors think they know everything. In fact, they know very little."
In 2002, Moser published results of a 10-year trial that followed 104 boys with the ALD gene who were under 6 years of age and symptom-free when the experiment began. He found that boys who did not strictly follow the oil treatment regimen were almost three times as likely to develop symptoms as those who took the oil without fail.
"Given early enough, it has benefits," said Dr. Ian Duncan, a neurologist at the University of Wisconsin-Madison who is chairman of the scientific advisory board of the Myelin Project, a nonprofit group the Odones founded in 1989 to promote research into ALD and other diseases, such as multiple sclerosis, that destroy myelin.
Although experts have shifted their attention to other remedies, including bone marrow transplants and gene therapies, Duncan gives Odone huge credit for pushing medical science to focus on solutions.
"He could see the big picture beyond Lorenzo and understood that what he was doing was very important for many other children," Duncan said.
Odone, who was born in Rome on March 6, 1933, moved to Washington in 1969 to work for the World Bank. He had two children — Cristina and Francesco — with his first wife, who survive him along with one grandchild.
This year, he published a book about his family's odyssey, "Lorenzo and His Parents."
"Over the years, people have said to me, 'August, you are crazy with this obsession. The kid was going to die. Why make him suffer?' But to say that implies that Lorenzo had gone, that what was left was a shell, a void, a living, breathing void," he told London's Daily Telegraph in 2008. "But Lorenzo was still with us. He enjoyed being read to, listening to music. It is so complex, but I do believe that."Copyright © 2014, Los Angeles Times