吻仔魚的天空

What is Myelin?

Myelin - The white matter surrounding our nerves is made up of phospholipids- proteins of the cell membranes of Schwann cells (parasympathetic nervous system) and Oligodendrocytes (central nervous system) and forms the insulating coating around the nerves. It acts as an electrical insulator and increases the speed of impulse transmissions.

In Layman's terms: Think of the myelin sheath as the insulating cover to an electrical wire, when it is frayed or cut in places; the electrical current of the wire loses its ability to conduct itself to the intended appliance or just shorts out. The same applies to our bodies, if the nerve is not insulated, the message our brain sends to the intended appliance (muscle) loses its path along the way and we lose mobility and capability.

www.myelinproject.co.uk/frameset/core.htm

▲ 主要配方︰三油酸甘油與三芥子酸甘油以4︰1的比例配製而成，是腎上腺腦白質退化症（ALD）的預防性療法。ALD症狀為情緒失控、行為異常、智力與視力減弱、步行困難、耳聾、癲癇發作等，病人的感覺和運動功能會逐步惡化，從痙攣一路退化到癱瘓。

▲價格︰羅倫佐的油頗為昂貴，每月的治療費用約440美元（台幣1萬3415元）。大部分的保險公司以美國食品暨藥物管理局認定其為實驗性療方，而不予給付。

▲運作原理︰這個脂肪酸混合物能降低引發ALD的「極長鏈脂肪酸」（VLCFA）的濃度。它是藉由抑制形成VLCFA的酵素而達此功效。

▲成效︰結合低VLCFA飲食的「羅倫佐的油」使用已顯出有限度的成效，特別是在症狀出現前使用，功效最大。

2005年，該領域名醫摩瑟爾發表在神經學資料庫期刊的研究指出，89名尚未浮現症狀的病童，經使用羅倫佐的油7年後，僅有11%不僅腦斷層掃描（MRI）異常，也出現神經異常現象。摩瑟爾結論指出，羅倫佐的油對於腦斷層掃描正常的ALD未顯患者，有延緩該疾病發作之效。

▲現況︰該療法對出現症狀的ALD患者並無效用，雖然一些病童家屬不以為然。在美國，目前只有參與甘迺迪克瑞格研究中心臨床實驗的病患，才能使用到羅倫佐的油，該實驗由摩瑟爾醫師主持，他雖於去年過世，醫療團隊仍持續進行這項實驗。

羅倫佐的父母

Augusto Odone (born May 4, 1933) and Michaela Teresa Murphy Odone (January 10, 1939 – June 10, 2000) are the parents of Lorenzo Odone (May 29, 1978 – May 30, 2008), a child afflicted with the illness adrenoleukodystrophy (ALD). They became famous for discovering a treatment for their son's "incurable" illness, and this quest was recounted in the film Lorenzo's Oil.

In recognition of the parents' work, Augusto Odone has received an honorary doctorate from the University of Stirling. He continues to raise funds and drive the scientific task force known as The Myelin Project. Michaela Odone battled with cancer for some time and passed away in 2000.

Lorenzo died the day after his 30th birthday. He was almost totally paralyzed but was, according to his father Augusto, "holding his own." He couldn't see, speak, or move on his own. He communicated by wiggling his fingers and blinking his eyes. His mind was intact and he enjoyed music and having people read to him. "Certainly, he has good days and bad days, he is bedridden and he cannot eat more than through a tube… but his mind is still there. He likes that we read to him, that we play music for him and he knows who is around him".^[1] He lived with his father in Virginia and was cared for by nurses and his family friend, Oumouri Hassane.

At the time of Lorenzo's diagnosis, ALD was a recently-identified disease, that had only been named ten years earlier. Very little was known about it at that time, except that the victims were usually young (5-10 years old) boys, who would gradually become mute, deaf, unable to move, and blind, among other symptoms, typically including death within two years.

Augusto and Michaela refused to accept the hopelessness afforded to them, and fought to find a treatment for their son's potentially fatal disease, clashing time after time with doctors, specialists, and support groups, all of whom were skeptical that only two average citizens could produce such a cure. Despite this, they persisted, and through long hours of research and study, the Odones, who had had no previous medical background, came up with a treatment to stop the progression of the disease. This treatment revolved around the consumption of a specially-prepared oil, which became known as "Lorenzo's oil."

The Odones had an important role both in developing Lorenzo's oil and in setting up the Myelin Project, which promotes and carries out research on ALD and other similar disorders. Michaela also insisted on continuing to treat her incapacitated son as a human being and not a 'vegetable', helping him devise a means of communicating with her and others through the blinking of his eyes and the wiggling of his fingers. Eventually, their story was made into the 1992 film Lorenzo's Oil, with Augusto being played by Nick Nolte and Michaela being played by Susan Sarandon, who through her involvement with the movie became the spokesperson of the Myelin Project.

A poem Michaela wrote about Lorenzo was also put to song by Phil Collins. Called "Lorenzo," it was featured on his 1996 album, Dance into the Light.^[2

He began bleeding heavily and died before an ambulance could reach him.

‘He could not see or communicate but he was still with us,’ Mr Odone said. ‘He did not suffer...that’s the important thing.’

Lorenzo was diagnosed at the age of six with adrenoleukodystrophy, or ALD.

His doctors told his parents the disease, which is caused by a genetic mutation that results in a breakdown of the neurological system, meant he would be dead in two years. ALD leads to an accumulation of fatty acids that damage the myelin sheath, which coats the nerve cells.

Nick Nolte and Susan Sarandon starred as Augusto and Michaela, his second wife, in the 1992 film, which depicted the couple’s struggle to formulate an oil that would prolong their son’s life despite their lack of a scientific background.

Sarandon earned an Oscar nomination for her performance. A study published in 2005, based on research involving 88 boys, showed that a treatment made from olive and rapeseed oils – patented by Augusto Odone – can prevent the onset of the symptoms for most boys diagnosed with ALD.

www.brightcove.tv/title.jsp

羅倫佐老爹的錄影談話

他講的很慢
幾乎每句話都很清楚
大家可以慢慢聽
只是裝了假牙有點漏風
從他的臉上可以看出他對小孩的愛
讓他臉上如此疲累
又充滿了包容
簡直有如一個聖徒的臉

羅倫佐爸爸的聯絡方式
請大家多多寫信慰問他
為ALD多作一些事情

Augusto Odone
The Myelin Project Headquarters
2136 Gallows Rd., Suite E
Dunn Loring, VA 22027
USA
Tel. 703 560 5400
Toll Free 800 869 3546
Fax 703 560 0706
E-Mail mp@myelin.org
Internet www.myelin.org