A Melbourne baby given no chance of survival has amazed doctors after being saved with one of the biggest long shots in medical history.

澳洲墨爾本一個原已判定沒有任何存活機會的寶寶，歷經醫學史上最大的風險賭注後被救回一命，連醫生都嘖嘖稱奇。

"Baby Z's" brain started virtually dissolving soon after she was born 18 months ago because she had too much toxic sulphite in her system. Baby Z suffered from a rare metabolic brain disease called molybdenum cofactor deficiency type A which had no known cure and her condition was worsening by the hour.

「Z寶寶」18個月前出生後沒多久，因體內有太多有毒亞硫酸鹽，腦部幾乎就開始溶解。Z寶寶罹患一種罕見的腦部新陳代謝疾病叫做「A型鉬輔脢缺乏症」，目前還無藥可醫，而她的病情幾乎每小時在惡化。

The disease stops the body expelling sulphite and causes babies to suffer increasingly severe seizures and brain damage until they die.

這種病因身體無法排除亞硫酸鹽，而使寶寶抽搐痙攣，腦部傷害日益嚴重，直到死亡。

Baby Z was born on May 1, 2008, with the metabolic disorder which affects one in 500,000 babies and started having seizures 60 hours after her birth. She was deteriorating fast as doctors desperately searched medical literature in the hope that somewhere in the world there was help.

Z寶寶於2008年5月1日出生，每50萬個寶寶只有一個會得這種新陳代謝疾病，她在出生後60個小時就開始抽搐痙攣。正當醫生拚命翻尋醫療文獻，希望世上某個角落可以找到解藥之際，她的病況急轉直下。

But her parents and doctors refused to give in to the genetic condition and stumbled on a highly experimental drug.

但她父母和醫生都拒絕向這種遺傳病投降，這時他們碰巧找到一種高度實驗藥。

They found a medical paper written by German plant biologist Professor Guenter Schwartz of Cologne University who had been working on an experimental drug called cPMP for 15 years, but had tested it only on lab mice.

他們找到德國科隆大學植物生物學教授史瓦茲所撰寫的醫學論文，他已經研究一種實驗藥物cPMP達15年之久，但只有在白老鼠身上實驗過。

It normally takes years for new drugs to get approval but Baby Z's parents refused to let their baby die without a fight. They urged doctors to break normal medical procedures and let their baby be the human guinea pig for the unapproved drug.

新藥通常要等好幾年才能核准上市，但Z寶寶的父母拒絕不戰而降，坐視寶寶死亡。他們催促醫生打破正常的醫藥核准程序，讓他們的寶寶當這種未經核准藥物的實驗鼠。

The doctors contacted Schwartz who immediately sent all of his available compound to Melbourne. The parents argued before the hospital's ethics board that their baby was about to die an agonizing death so there was nothing to lose in giving the drug.

這些醫生立刻和史瓦茲聯絡，他馬上把手邊所有的cPMP寄到澳洲墨爾本，這對父母在醫院倫理委員會前據理力爭，表示他們的寶寶快被痛苦折磨至死，因此投藥沒有任何損失。

But the Monash Medical Centre insisted they also get approval from a court. A court hearing was hastily arranged and after a tense day of arguing, the judge allowed use of the unapproved drug.

但莫納希醫學中心堅持他們要先得到法院許可，法院立刻著手安排一次聽證會，經過激烈辯論後，法官同意使用這種未經核准的藥。

The doctors led by neo-natal specialist Dr Alex Veldman raced back to the hospital as the baby was fast deteriorating."The hospital pharmacist sprinted up the stairs with the substance in his hand and we started to treat the baby on that afternoon, actually about 40 minutes after we got the court order," Dr Veldman said.

由於寶寶病情急速惡化，以新生兒科專家維德曼醫生為首的醫療團隊立刻趕回醫院。維德曼醫生說：「醫院藥劑師手持藥方衝上樓梯，當天下午，事實上是在法院許可後40分鐘，我們就開始醫治寶寶。」

"What then happened was really amazing, because the baby was waking up, she was starting to move around, starting to look around, she was starting to drink milk from a bottle, she just improved massively." he said.

他說：「接下來發生的事十分神奇，因為寶寶醒了，她身體開始動了，還四處張望，她開始從奶瓶喝奶，她病情大幅改善。」

Within hours of receiving her first daily dose of cPMP, tests showed Baby Z's sulphite levels immediately dropped from near 300 to below 100. Within three days they fell to the normal level of about 10. Baby Z's neurological development is delayed due to some brain damage in the weeks it took to find the cure, but she is now improving.

在施打完第一天的劑量數小時後，Z寶寶體內的亞硫酸鹽濃度立刻從將近300降到100以下，三天內，濃度再降到「正常水平」的10。Z寶寶的神經功能發展較遲緩，主要因為找解藥那幾周腦部有點受損，但現在已經改善。

Dr Veldman said the girl is now 18 months old and is doing well although she has some problems and has to be injected with Schwartz's compound every day. "She is such a delightful little lady and the parents love her," he said. “The amazing thing is that I spoke to her mum and she said that she never believed that her baby would die, she always knew that she would fight for it until we find something."

維德曼醫師說，小女娃現在已經一歲半，情況良好，雖然她還有些問題，每天仍得施打史瓦茲醫生的藥方。他說：「她是個快樂的小娃娃，父母很愛她。最令人驚喜的是我和她媽媽談過，她說，她從來不相信她的寶寶會死，她一直知道，她會奮戰到底，直到我們找到療方為止。」

"It was the most challenging and traumatic time of our lives," Baby Z's overjoyed mother said. She never doubted her baby should be the human guinea pig for the untried German compound. "There was courage and there was death - we opted for courage. The procedure was a tiny bit of hope but, when you have nothing, that is a lot of hope. She might have one bad gene but she has a lot of other good and strong genes."

Z寶寶大喜過望的媽媽說：「這是我們一生中最具挑戰性、也最痛苦難忘的時光。」她從不懷疑她的貝比應該當德國未經人體實驗藥物的人類白老鼠。「要嘛拿出勇氣，要嘛面對死亡，我們選擇勇敢面對。手術只有一點點希望，但當你一無所有時，那就是很大的希望，她可能有個壞基因，但她有很多其他又好又強壯的基因。」

Baby Z's mother said she had grown into a happy and determined little girl. "Every day just gets better and better. We look at her every day and just think, 'Wow'," she said.

Z寶寶的媽媽說，她已經長成一個快樂固執的小女孩。「情況一天比一天好，我們每天看著她，不禁想『哇，不可思議』！」她說。

This handout photo taken on May 5, 2009 and received on November 5, 2009 shows 'Baby Z' in Melbourne. The first person to be cured of molybdenum cofactor deficiency, Baby Z has made world medical and legal history for Monash Medical Center. The child and her parents cannot be named for legal reasons and to protect their privacy.

圖為墨爾本的Z寶寶攝於今年5月5日，並於11月5日發布的照片。Z寶寶是第一個治癒「鉬輔脢缺乏症」的患者，她已經為莫納希醫學中心改寫醫療和法律史。孩子及父母因法律原因及為了保護其隱私辜隱其名。（法新社）