Lorenzo’s Oil : Westside Boy’s Family Hopes Treatment Will Prevent Deadly Illness

Fate dealt Patti Benton a double blow five years ago. At the same time she learned that her brother had developed a rare, deadly illness, she discovered that she might have passed on the gene for the same disease to her three sons.

Doctors predicted that tests would show that at least one of her sons had inherited the gene. The boys were immediately tested for adrenoleukodystrophy, ALD for short. The genetic disorder, which is passed to sons from symptom-free mothers, relentlessly attacks the brain and spinal cord. ALD robs its victims of their ability to talk, walk or eat as it brings on paralysis and almost certain death.

Benton’s worst fears were realized when her eldest son, Michael, who was 8 at the time, tested positive for the gene. Although he showed no symptoms of the illness, the presence of the gene meant that he had a 50% chance of coming down with it.

Even before the genetic results were known, the Bentons learned about an organization of families of ALD patients. Patti Benton, her husband, Bob, and brother Richard Louiselle flew to Baltimore for a national conference.

“It was a horrifying experience because we were with families whose boys had died,” recalled Patti, who lives in Pacific Palisades.

In Baltimore, the Bentons learned of a treatment other families were using that seemed to be working: Lorenzo’s Oil, which is featured in the recent Universal film of the same name.

Although it had not been given FDA approval, the Bentons decided to put Michael on the oil, telling him only that he was allergic to fat and needed to take medicine. When clinical trials began in 1988, Michael immediately joined. Michael, now 13, and his uncle, Richard Louiselle, 36, are among 208 people in the United States and Canada who are participating in the five-year trial for Lorenzo’s Oil.

Every day, Michael swallows four tablespoons of what looks like cooking oil, disguising the taste with soda, milk or juice. “It tastes bad,” Michael says with a grimace.

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But even with its foul taste, the oil has been a “miraculous gift,” Patti Benton said. So far, Michael has remained symptom-free.

He could still develop adrenomyeloneuropathy, or AMN, the adult form of the disease that affects his uncle. AMN progresses more slowly than ALD but shortens its victim’s life expectancy.

“As long as I keep taking the oil and stay on my diet, which is eating no fat, then I think I’ll stay well,” Michael said. “By my staying well so long, I think they’ll come up with a cure and I won’t have to take it any more.”

ALD causes long-chain fatty acids to accumulate to toxic levels in the bloodstream, causing the disintegration of myelin, the fatty sheath that protects nerves in the brain and spinal cord like a coating of insulation around a telephone wire.

Since Richard Louiselle’s diagnosis, he and his sister have learned that a sibling who died as a child, Bobby, also had ALD. Louiselle, who lost the ability to walk normally, said the oil he takes as part of the clinical trials has stabilized his myelin loss and has prevented further deterioration.

The blend of refined olive and rapeseed oils exists because of the efforts of Michaela and Augusto Odone, whose son is an ALD patient and whose story is told in “Lorenzo’s Oil.”

In 1984, 5-year-old Lorenzo Odone rapidly lost his ability to walk, talk and eat. Doctors gave him only two years to live.

The Odones learned that because so few people are affected by ALD--only one in about 25,000 people--the “orphan disease” had received little attention by the research community. When they were told that there was no cure, they refused to give up.

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The couple, neither of whom has a medical background, began an exhaustive search for something that would help their son. Eventually, they found that daily doses of oleic and erucic acids lowered the toxic levels of fatty acids and stopped the progression of ALD. At 14, Lorenzo has regained his ability to sit and to communicate, slowly blinking for no and wiggling his hands to signal “yes” and “perhaps,” Michaela Odone said.

Although the Odones and Bentons are sure that Lorenzo’s Oil is a miracle, medical experts are not convinced.

Dr. Hugo Moser, who is directing the clinical trials on Lorenzo’s Oil at the Kennedy Krieger Institute and the Johns Hopkins Medical Institutions in Baltimore, Md., said he thinks that the movie overstates the certainty of the oil’s benefits.

Preliminary results of the trial show that boys who are already very sick aren’t greatly helped by the oil, Moser said.

And although Lorenzo’s Oil appears to have helped Michael and others who began taking it before they developed symptoms, about 50% of those with the genetic defect would escape severe ALD even if they did not take the oil, Moser said.

“We are giving the oil in the hope it will act as a preventative measure,” Moser said. “Whether it does or not is the major question.”

Augusto Odone, who has received an honorary medical degree from the University of Sterling in Scotland, understands Moser’s caution but remains a believer.

“The scientists, you can hound them and hound them, but they will not come out and say something when they are not 100% sure because they have a reputation at stake,” he said. “But common sense tells you if there is something that kicks out the bad guy, you go for it.”

The Bentons need no convincing. They are counting on Lorenzo’s Oil and a diet low in fatty acids, to keep Michael healthy. “With Lorenzo’s Oil and the no-fat diet, we’re hoping since his levels are in the normal range, that he’ll never develop either the childhood form or the adult form,” Patti said.

Since they began taking the oil, Michael and Louiselle have visited Lorenzo and his parents at their Fairfax, Va., home. The Bentons have grown fond of the Odones and say they are indebted to them for their tenacity and perseverance. “That’s why their next cause is our next cause,” Bob Benton said, referring to the Myelin Project, the Odones’ latest plan of attack.

The nonprofit partnership between lay people and doctors is looking for ways to restore the lost myelin for the nearly 1,000,000 people who suffer from myelin diseases, which include multiple sclerosis.

The Bentons are now separated, but they continue to work together on their cause and have raised more than $9,000 for the Myelin Project from friends and relatives who attended a benefit screening of “Lorenzo’s Oil” in Brentwood, Patti Benton said.

Throughout the experience, Michael has become something of an expert on genetics and neurology. An honor student who thinks about becoming a brain surgeon, Michael throws around such phrases as magnetic resonance imaging as if he were talking about baseball. He has appeared on the “Maury Povich Show” and CNN.

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His illness aside, Michael is like any other 13-year-old. He wears baggy jeans and scuffed-up Nikes, likes girls, goes to school, plays baseball and pals around with his little brothers. But the daily dose of oil reminds him of just how lucky he is. In prayers, Michael said, he often asks God to bless the Odones and their son Lorenzo.

Information on the Myelin Project is available by calling (800) 8-MYELIN.

Experimental Remedy

More than 200 people are taking Lorenzo’s Oil in a five-year clinical trial. The blend of refined olive and rapeseed oils may prove helpful in reducing the accumulation of fatty acids that lead to the degeneration of nerve-cell coatings in people who have a genetic disorder called adrenoleukodystrophy.