Jul 31, 2008

When there is miracle,there is hope.

Lorenzo Odone, whose parents’ battle to save him from a rare nerve disorder was dramatised in the 1992 film ‘Lorenzo’s Oil’, died recently (30 May 2008) one day past his 30th birthday, from aspiration pneumonia, caused by food getting stuck in his lungs.

At the age of six he was diagnosed with adrenoleucodystrophy, a genetic disease that progressively destroys the myelin sheath around the nerves, and then the brain of young boys. The condition is extremely rare. It was also considered incurable, and ultimately fatal.

The disease leads to a build-up of very long chain fatty acids (VLCFAs) in the blood, and within a year children are paralysed blind, and unable to speak. Lorenzo was given less than two years to live, but his parents refused to accept the doctors’ prognosis. Augusto and Michaela Odone consulted every doctor and specialist they could, but the response, although sympathetic, was basically that nothing could be done, and so they decided to take matters into their own hands.

Neither parent had any training in medical science, but they contacted experts in the field and examined all the available, if limited, research. They discovered that oleic acid, found in olive oil, helped suppress production of VLCFAs, but the addition of erucic acid, a derivative of rapeseed oil, in a ratio of one part erucic acid to four parts oleic acid, dramatically increased its effectiveness.

The oil, known as Lorenzo’s oil, works by inhibiting the enzymes required to synthesise the VLCFAs, but how this prevents the devastating symptoms is uncertain.

The change in Lorenzo was remarkable. After two years of treatment he had developed ways of communicating with his parents, blinking his eyes for “yes” and wiggling his fingers for “no”. He could also distinguish left from right, colours and shapes, swallow small amounts of soft food, and flex certain muscles.

A 10-year study ending in 1999 concluded that Lorenzo’s oil did delay the progression of adrenoleukodystrophy, being more effective if used before the onset of symptoms.
But it is no magic cure-all. The 1992 film ended on a suitably upbeat Hollywood note, but children continue to die, even when treated with the oil.

In the case of Lorenzo, however, his father is convinced that the oil gave him an extra 20 years of life, despite the conventional medical wisdom of the time that said it was impossible.

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