小魚的異想世界

Filed at 12:26 a.m. ET

WASHINGTON (AP) -- The man whose parents' battle to save him from a nerve disease was depicted in the movie ''Lorenzo's Oil'' died Friday at his home in Virginia, having lived more than 20 years longer than doctors had predicted.

Lorenzo Odone, who doctors had predicted would die in childhood, died one day after his 30th birthday, said his father, Augusto Odone.

Lorenzo Odone had come down with aspiration pneumonia recently after getting food stuck in his lungs, his father said. He began bleeding heavily, and before an ambulance reached their home his son was dead, Odone said.

''He could not see or communicate, but he was still with us,'' Odone said Friday. ''He did not suffer. ... That's the important thing.''

Odone was found at age 6 to have adrenoleukodystrophy, or ALD. His doctors told his parents the disease -- caused by a genetic mutation that causes the neurological system to break down -- would lead to death in two years.

The disease leads to the accumulation of substances called very long chain fatty acids in cells, which damages the material that coats nerve fibers in the brain.

Susan Sarandon and Nick Nolte starred as Michaela and Augusto Odone in 1992's ''Lorenzo's Oil,'' which recounted their efforts to formulate the oil they said helped their son fight the neurological disease, despite lacking scientific backgrounds.

Sarandon earned an Academy Award nomination for her performance.

A study published in 2005, based on research with 84 boys, showed that a treatment made from olive and rapeseed oils -- patented by Augusto Odone -- can prevent onset of the disease's symptoms for most boys who receive an ALD diagnosis.

Odone plans to take his son's ashes to New York to mix them with those of his wife, who died in 2000. Then, Odone said, he will sell his home in Fairfax, Va., and move back to his native Italy.

Odone also plans to write a book memorializing his son, ''to tell the story of Lorenzo as a way to make him live on.''

蘋果日報

【李寧怡╱綜合外電報導】由真人實事改編的電影《羅倫佐的油》（Lorenzo’s Oil），描述男童羅倫佐6歲時罹患罕見疾病腎上腺腦白質退化症（ALD）後，父母竭盡心力對抗命運，研發出特殊油脂，有效延緩羅倫佐的病情。故事的主人翁羅倫佐，前天在美國維吉尼亞州家中因吸入性肺炎逝世，得年30歲，較醫師預估的壽命多活22年。

 

為羅倫佐歐東（Lorenzo Odone）奔走奉獻一生的父親奧古斯都（Augusto Odone）表示，羅倫佐因將食物吸入肺中，引起吸入性肺炎而大量出血死亡。他強調兒子的死「不是ALD造成」。

醫估活不過8歲
羅倫佐過世前一天，剛度過30歲生日。ALD是一種遺傳性基因缺陷疾病，患者因腦部神經傳導受阻，會逐漸喪失行動自主能力及語言能力，進入癱瘓狀態。羅倫佐6歲被診斷出罹患ALD後，醫師宣告他最多只能再活兩年，沒有科學背景的奧古斯都與妻子米凱拉（Michaela Odone）不願接受醫師說法，大量閱讀艱深的醫學論文，廣邀全球專家召開研討會，終於自橄欖油與菜籽油提煉出一種特殊油脂，有效延緩羅倫佐的病情惡化。
奧古斯都與米凱拉為子奮鬥的故事拍成電影後，賺盡觀眾熱淚，飾演米凱拉的女星蘇珊莎蘭登（Susan Sarandon）因此片獲提名奧斯卡最佳女主角，還堅持邀請米凱拉一同參加頒獎典禮。但這部電影也引來醫界批評，指奧古斯都已取得專利的「羅倫佐的油」效果有待檢驗，電影的戲劇性情節會帶給ALD病童家屬錯誤期待。
直到2005年，一項醫學研究證實，「羅倫佐的油」在患者產生腦部病變之前服用，確可延緩病情惡化。對奧古斯都而言，這才終於還他一個公道。奧古斯都說，羅倫佐能長期存活，全靠「羅倫佐的油」。羅倫佐發病後不久即癱瘓在床，需要24小時看護。他已失明，但聽力尚存，能感知周遭環境的變化。他可用眨眼或發出特定聲音的方式，進行有限的溝通。他喜歡「聽」小說，也很享受泡在游泳池裡。

羅倫佐奧登（Lorenzo Odone）生平
．生卒：1978/05/29-2008/05/30，得年30歲
．5歲前：是個能說3種語言的好動男孩，隨在世界銀行工作的父親奧古斯都與母親米凱拉，在東非科摩羅群島居住3年。
．6歲：被診斷罹患腎上腺腦白質退化症，壽命僅剩2年。父母竭盡心力尋找療法，終於研發出「羅倫佐的油」。
．14歲：故事被拍成電影《羅倫佐的油》，由喬治米勒編導，影星尼克諾特與蘇珊莎蘭登飾演其父母，莎蘭登因此片獲提名奧斯卡最佳女主角。
．24歲：母親米凱拉因肺癌逝世。
．30歲：失明、無法言語、癱瘓在床需24小時看護的羅倫佐，在30歲生日隔天因吸入性肺炎病逝。
資料來源：羅倫佐之父創辦的「髓鞘質」組織網站（www.myelin.org）

腎上腺腦白質退化症小檔案
．病名：Adrenoleukodystrophy，簡稱ALD
．病因：因X染色體基因缺陷，體內缺乏某種酵素，導致超長鏈飽和性脂肪酸無法正常代謝，沈積在大腦白質和腎上腺皮質，進而侵蝕腦神經的髓鞘質，妨礙神經傳導。
．遺傳模式：由母親遺傳給兒子，發生率約為兩萬至五萬分之一。
．病程發展：多在3至5歲發病，患者聽力退化，進而視力不佳、走路不穩、語言能力逐漸喪失，進入癱瘓狀態，一般在10歲前死亡。
．療法：在發病前服用「羅倫佐的油」（一種經特殊提煉的橄欖油與菜籽油綜合油脂）；較可能根治的方法是骨髓移植，成功率約6成。去年10月法國研究人員首度以基因療法成功治療ALD。
資料來源：衛生署遺傳疾病服務窗口、《蘋果》資料室