Saturday, May 31, 2008

Lorenzo Odone Ends His Long Battle With Adrenoleukodystophy (Lorenzo Disease) Update: Dalton's Journey Continues ~by Legal Pub


If you are looking for a funny story this Saturday, please look else where. Today's subject deals with a genetic mutation known as Lorenzo's disease. The disease became better known in a movie called "Lorenzo's Oil" which starred Nick Nolte and Suzan Sarandon. The man who was the subject of the movie died Friday at his home in Virginia. Lorenzo Odone lived more than 20 years longer than doctors predicted. Lorenzo Odone was 30 years old.

Lorenzo's father, Augusto Odone, said that Lorenzo had come down with aspiration pneumonia. He began bleeding heavily, and Lorenzo died before an ambulance arrived. According to his dad, Lorenzo, " could not see or communicate, but he was still with us," Odone said Friday. Lorenzo Odone at age 6 was diagnosed to have adrenoleukodystrophy, or ALD. This is caused by a genetic mutation that causes the neurological system to break down. Lorenzo was originally given two years to live. The disease leads to chain fatty acids in cells damaging the membranes that coat nerve fibers in the brain. Augusto developed an oil that helped his son fight the neurological disease. A 2005 study, showed that a treatment made from olive and rapeseed oils (patented by Augusto Odone) may prevent or delay the disease's symptoms for most boys with ALD.

Augusto plans to author a book about his son's battle with the disease.
Update 6-2-08 For those with this genetic mutation, U.S. researchers believe Lorenzo's Oil can prevent the onset of the devastating neurological disease in young boys. Researchers used validated scientific methods to prove the formula works. Researchers found that subsequently, 74 percent of the boys showed no signs of disease progression.
The most dangerous form is a childhood cerebral form. With this form, brain cells are destroyed. Symptoms are devastating and include a loss of the ability to speak, reduced strength and coordination and, eventually, death. At present there is no cure, but potential treatments include cholesterol lowering drugs such as lovastatin and bone marrow transplants.
Dr. Hugo Moser of the Kennedy Krieger Institute in Baltimore was an expert in the field. While Dr. Moser is deceased, his work lives on at the Kennedy Krieger Institute.
Update 3-20-10: Legal Pub was contact on behalf of Dalton, a young boy with this dreaded disease. For anyone interested in reading about Dalton's journey, follow the LINK

Update 11-6-13:  Augusto Odone, the Italian economist who ignored conventional wisdom to invent an experimental medicine that extended the life of his terminally ill son and inspired the film," Lorezo's Oil," died on Friday November 1, 2013 at the age of 80.
Universal Pictures
The 1992 film based on his family story starred Nick Nolte and Susan Sarandon.  While the movie was a hit, Lorenzo’s Oil did not cure Lorenzo Odone, his son, who died in 2008 at age 30.  Adrenoleukodystrophy remains an incurable neurological disease known as ALD. But the moviedid generate financing for research that has confirmed the benefits of Lorenzo’s Oil in some patients.
 
Mr. Odone wrote an essay in 2011 chronicling his journey with the disease and propounding the need to find a cure. A compound using extract of acids in olive and rapeseed oils, became known as Lorenzo’s Oil. It apparently breaks down long-chain fatty acids that are considered a cause of damage to nerve cells in people with ALD.  A 2005 study suggested that Lorenzo’s Oil helped children with ALD if used before they started showing symptoms.  Unfortunately, it was found to be less effective once the degenerative process had begun. The Food and Drug Administration considers Lorenzo Oil "experimental."

Augusto Daniel Odone was born in Rome on March 6, 1933.  He grew up in Gamalero, Italy and received a law degree from the University of Rome.  The world was a better place because he shared his passion with humanity.


46 comments:

Anonymous said...

A. Odone's formula has benefitted so many little boys by giving them the chance to grow up into men.

Secrets said...

I remember the movie. Thats very sad.

Ms Calabaza said...

My thoughts and prayers are with the family. I hope Lorenzo is in a better place.

Legal Pub said...

I know a single mom whose son has these genetic defects. It can be real depressing. The boy looks and acts normal at age three, but it is only a matter of time. The Oil helps slow the deterioration.The mom sometimes posts here, so perhaps she will share some insights.

Anonymous said...

It is an awful disease!

harry p. said...

The disease portrayed in the movie is called adrenoleukodystrophy (ALD). The disease involves the removal of white, lipid sheaths (myelin) that surround long thin cells called neurons. Neurons, of course, allow us to think and to move (e..g, control our muscles).

"Leuko" is white (referring to the myelin that covers neurons and makes neurons look white) whereas "dystrophy" refers to "abnormal development." Although there are different forms, the symptoms of the form of ALD shown in the movie begin at age 5 to 12. Death usually occurs within a couple of years (Moser, 1997). The symptoms (dementia, loss of sight, hearing, speech, and ability to walk-ambulation) are believed to be due to solubilization and removal of the myelin sheath around neurons by a build up of very long chain, saturated fatty acids (VLCSFAs) in the body. Without a myelin sheath, nerve cells do not conduct action potentials; in essence, the neurons stop telling muscles to contract and the patient cannot move.

Multiple sclerosis, using a different mechanism, will also remove myelin.

Harry P.

Anonymous said...

Lorenzo's Oil may reduce but not prevent the onset of the disease if the oil is given well before the onset of symptoms. This is not proven and it is difficult to prove. For ethical considerations, ongoing clinical trials are without control groups. However, Moser estimates that about 30-40% of kids in the study should have developed symptoms, and the prophylactic use of the oil may have reduced this to about 10%.

In September of 2002, Dr. Moser summarized a ten year study involving 69 boys in US and 36 boys in Europe. Data shows that Lorenzo's Oil reduced the development of ALD symptoms by two-thirds! The use of Lorenzo's Oil was best if started before the onset of symptoms. See: www.kennedykrieger.org for more information.

Thanks A. Odone!

Anonymous said...

Augusto you are a hero!

Anonymous said...

Wonderful discussion of this horrible disease!

Anonymous said...

one can only hope prognosiss will get even better with future research.

Anonymous said...

Because of this disease I wake up up and have to wonder if my son is going to be able to see today, speak, hear, walk to me, put his arms around me, ask me to play with him. I continually ask myself if today is the day when the disease will make him symptomatic. He takes his treatments twice daily of Lorenzo's oil (41ml), walnut oil (10ml)and steroids (10mg). He is on a low fat diet of no more than 18 grams of fat per day with no more than 5 of those fat grams being saturated and 1500 calories. He is very grown up about taking his medicine and knows that he will be sick without it even though sometimes the oil makes him sick anyway and he can't keep it down. More research is being done and we continue to wait for the day when the cure is at hand. Until then, I don't take a second with him for granted. The latest research shows the HIV virus being used to correct the degenerative cells(Declan Butler)

Anonymous said...

Hang in there! Your son is statistically likely to never develop severe symptoms as long as he keeps on the diet and takes the Lorenzo Oil as instructed!

Keep a smile on your face, because it will likely work for your son!

Anonymous said...

Thank you Legal Pub for publishing this story. No matter what the disease or illness that any of our children face, it's good to acknowledge its presence, and in doing so increase our chances for more research to be done, and maybe even a cure to be found.

Anonymous said...

My thoughts and prayers go out to Loronezo's family. My prayers also go out to the families posting on this that have children afflicted with this disease. My heart goes out to you and I could never even begin to understand what you are going through.

Legal Pub said...

This forum will be open to post comments and information to be shared among those interested.

Anonymous said...

Thanks Pub. Our family has been dealing with this for years...

Anonymous said...

Kennedy KRieger institute is the place to be if your kid gets this diagnosis.

Kate said...

I would appreciate anyone who suffers from this disease or knows of someone who does to contact me for support. Thanks

Anonymous said...

Hot photo! Is that really you?

Anonymous said...

Brains and beauty with bad DNA?

Kate said...

Correct;)

Anonymous said...

and modesty I see...

Anonymous said...

The disease is really scary at first. But if your son is one of the lucky ones, severe symptoms may never show up!

Kate said...

And I continue to pray that he is one of the "lucky" ones. His treatment started before any symptoms showed so I'm hopeful that he will never have the effects of the disease. My Dad has the adult form of it and is in a wheelchair. He's looking into a study where they use cobra venom to stop the deterioration.

Viper said...

Cobra venum? If a snake such as I were to bite you, would it help your son? I might be willing to help if it would bring hope. LOL

Anonymous said...

lucky is as lucky does...

Anonymous said...

Dr. Moser is now dead. But the Docs at Kennedy Krieger are all top notch state of the art health care providers.

Anonymous said...

The oil and a good diet is the secret, in my opinion.

Kate said...

I take my son out to Kennedy Krieger once a year and he's in the research study at this point. I took him this morning to get his monthly blood draw taken care of. He's four and has to explain to me how to sit still and not worry because he's a big boy and can handle it.

Anonymous said...

What a brave lad.

Anonymous said...

Keep strong Kate, it will be worth it in the end!

Anonymous said...

The end game is what it is all about

Legal Eagle 3 said...

Skin care adds have nothing to do with this serious disease.

Consequently, the DELETE button!

Legal Pub said...

Update 3-20-10: Legal Pub was contact on behalf of Dalton, a young boy with this dreaded disease. For anyone interested in reading about Dalton's journey a link is on the front page.

Anonymous said...

His passing is bittersweet. He was cremated and buried his ashes in New York.

Anonymous said...

thanks for the update!

Anonymous said...

This is a heartbreaking disease.

Anonymous said...

Debilitating...

Anonymous said...

i am a free illustrator, is there a chance you'd like to use some of my pics? i guess it would be fun for your cms :-D
really enjoy your site structure! send me a email please in case you want to colaborate

Just One Mom of Many said...

Illustrations for this disease might bring folks to tears. Children die all the time but not with this much suffering.

Anonymous said...

Just popping in to say nice site.

Anonymous said...

nice site, but horrible disease. Let's pray for a cure.

yossi@paradisefriends.com said...

Incredible, I just finished watching the movie and wanted to find out what was happening 20 years later. Very sad!

Anonymous said...

You did a great job on this subject. Lorenzo was a hero. Shame more cant be done for these poor kids.

curious said...

So sad for these children.

Legal Pub said...

Update 11-6-13: Augusto Odone, the Italian economist who ignored conventional wisdom to invent an experimental medicine that extended the life of his terminally ill son and inspired the film," Lorezo's Oil," died on Friday November 1, 2013 at the age of 80.