Augusto Odone
doctor Without being created Lorenzo's oil to combat the incurable disease of their child and inspired a movie. Now continues his fight to help others. He writes the book he promised before his death Lorenzo.
" I am a father. And my involvement in the disease called A drenoleucodistrofia (ALD) is not the love of science but of love for my son Lorenzo and my desire to help "Augusto Odone said the magazine New Scientist a few years ago. Odone, with his wife, Michelle, became world famous for finding a treatment for incurable disease of their son Lorenzo. While in 1992, Nick Nolte and Susan Sarandon took their story to the movies in the movie "Lorenzo's Oil."
History
In the fall of 1983 had just returned to live in Washington, DC, with Lorenzo, his son five years, having spent three Comoros islands, facing the east coast of Africa. Augusto, who worked as an economist at the World Bank, had gone to that land to prepare a development plan for the fledgling republic.
Michaela and Augusto were married in 1977. Lorenzo, his only son, was born the following year (May 29, 1978), when Michael was 39, Augustus 45.
the African sun, the boy had made strong climbing trees and swimming like a native. In his household spoke English, French and Italian Lorenzo dominated the three languages. He also had a good ear for music.
" Our son was a godsend " Michaela account. " had come into our mature years, and I really adored him ."
Then, for no apparent reason, the nightmare began. Slowly at first, so that only his parents noticed the child began to drag the words. Then came the tantrums. When I went to the bathroom at school, wandered.
Concerned, his mother took him to practice some studies, but the neurologists found him completely healthy. Felt that it was likely that Lorenzo was hyperactive, and nothing else.
One day in late 1983, the child was on the phone with his grandmother, and unexpectedly handed the headset to Michaela:
- not know what you're talking my grandmother "he said, exasperated.
performed Hearing tests revealed that Lawrence had suffered a loss of 50 decibels of hearing acuity.
prognosis. Looking for another opinion, Odone took their son to Children's National Medical Center in Washington, where Dr. Donald Fishman, a pediatric neurologist, called for blood tests and CT. The Odone never forget that day in April 1984, when Fishman cited them in his office.
- I have very bad news "he said. Lorenzo suffers a rare disease called adrenoleukodystrophy (ALD), that progressively destroys the brain's white matter. There is no cure for ALD. Your child will miss the speech, vision, motor function and, finally, die.
parents listened in disbelief. - much time is left of life ? Augusto asked at last.
- The average among these patients is two years "replied the doctor.
When they left, dazed with shock, the same idea occurred to them both: that Dr. Fishman had made a mistake.
Disease
disease portrayed in the movie is called adrenoleukodystrophy (ALD). This disease is due to loss of lipid from the myelin sheath surrounding neurons (thin cells of the brain). Neurons, of course, allow us to think, that we move and we do all voluntary and involuntary functions of our body (eg, control our muscles).
"Leuco" is white (referring to the myelin covering neurons), while "dystrophy" refers to "abnormal development." Although there are many different symptoms of ALD is shown in the film begin at age 5 to 12 and death occurs within a couple of years (Moser, 1997). Symptoms (dementia, vision loss, hearing loss, loss of speech and the ability to walk) are attributed to the solubility and loss of myelin sheath around nerve cells by an accumulation of saturated fatty acids of very long chain (VLCSFAs) in the body.
In the same way as for the transmission of impulses in an electrical cord are essential appropriate insulating layer (more when we're talking, for example, a cable that transmits information, such as the telephone or a TV antenna), the neurons are coated with myelin to allow electrochemical signals that travel through the nerve cell is not lost on the road. But not only makes insulating paper, but functions as an intensifier of signals along axons.
Miracle
A Augusto doctors told her that her son lost speech, vision, motor function and, finally, die in a period not exceeding two years. Lorenzo was six years old. He knew that the disease affects 1 in every 17 thousand people. And that is characterized by the accumulation of fatty acids long-chain saturated brain cells that cause the loss of myelin, or fatty sheath that covers nerve fibers and transmits impulses properly. "We thought: love our son and do not want to lose . " What is the enemy in this situation ? fatty acids Wicked! Then, find the way to destroy ", Augusto said.
He, who worked as an economist the World Bank, and Michelle, who was a music teacher, were far from the medicine. But they managed the "miracle": the disease was stopped due to an oil obtained from the combination of two fats extracted from olive and canola oil. In recognition of such an effort, Augustus received the honorary doctorate from the University of Stirling (UK). Since then started looking for ways to cover the damaged nerves with myelin. In 1989, the Myelin Project was born.
Augusto spent 24 years with 75 - dedicated to the care of Lorenzo and search for new therapies to restore damaged myelin, not only because of this disease but also other neurodegenerative diseases such as Multiple Sclerosis. In 2005, a study published in Archives of Neurology, and which worked the same Odone, found that of 89 children diagnosed with AML who started taking the oil immediately Lorenzo, only 24% of cases were completed building anomalies detected by MRI and in 11% of neuroimaging and neurological abnormalities. You never get 100% success with any drug, medicine or treatment.
The efficacy of Lorenzo's oil depends on starting therapy before the onset of neurological symptoms, and results clinical bone marrow transplantation are better if the procedure is performed when the damage to myelin is minimal. So one of the most important advances in the Myelin Project, which is developing a test (screening test) at the Kennedy Krieger Institute, is that from a drop of blood from newborn heel identify children who are at risk in order to initiate appropriate therapy before the onset of symptoms.
The method promises to be an accurate and reliable tool for early detection of this condition, but you must first minimize the risk of false positives, ie misdiagnosis. In addition, the Myelin Project is supporting two research lines: on the one hand, cell transplantation and on the other, the possibility to create drugs that replace the lost myelin. "When a scientist asks money for important medical research can take two years to get it. If the Myelin Project OKs get the money within weeks" Odone stands. He adds: "When they ask for money to conduct experiments in mice, rats, cats, dogs and monkeys, they often respond. But we say no, they should begin to experiment with people. At least 15 other organizations have adopted this formula" .
An example of this is the transplantation of stem cells. In 2001 supported the work of researchers at Yale University that marked a milestone by transplanting myelin-forming cells in the human central nervous system. The patient was a 53 year old woman with multiple sclerosis. Then followed other cases and are still evaluating the results, although not submitted until a final conclusion.
Meanwhile, Augusto Odone continues revalidating what a professional once said about the case of Lorenzo: " This story should be taught that no one can condemn a patient with a final diagnosis and urgent without even sought alternatives, inside or outside the established knowledge .
Goodbye Lorenzo
" Micaela went up to 16 hours with Lorenzo. I think that caused excessive stress on the immune system and helped to develop lung cancer "he said about his wife, who died in 2000.
founded Odone Myelin Project with the dream of returning to Lorenzo lost many of the features. Today you do to help others, though, for health reasons in January last year had to leave the presidency of the project.
three weeks ago (May 30, 2008), Lorenzo died of pneumonia, a day after his 30th birthday, Virginia, USA. However, Augusto continues to lead the Myelin Project to fight against adrenoleukodystrophy (ADL), the degenerative neurological disease of genetic origin that condemned his son's life.
now also holds the time writing the book he had promised his son to when he turned 30, not knowing what would be the last to tell how were all these years. For this return to his native Italy after his son's remains be cremated and laid alongside those of his wife Micaela .
Sources:
Myelin Project Presentation
Myelin Project: http://www.myelin.org
"Nisebe Mariana." ClarÃn.com Editors,
"Bradley J. Stith, Ph.D. Professor Department of Biology, University of Colorado at Denver,
"Reader's Digest " June-96
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