【聯合報╱By NATALIE ANGIER╱李京倫譯】
Doctors Can Cure Leprosy, but Stigma and Shame Linger
Throughout his adolescence, José Ramirez Jr. , now a clinical social worker in Houston, had a shifting array of bewildering symptoms.
Sometimes, he was feverish; other times, nauseated. He’d find swellings on his hands and his feet, and sores that wouldn’t heal. He’d grow hypersensitive to touch, unable to bear even the rustle of a bedsheet. His forearms would turn numb, a complication he gamely sought to exploit.
“I’d insert safety pins into my skin,” he said, “to try to impress girls.”
Dermatologists were baffled.
Spiritualists spoke of possession. Finally two doctors at the local hospital sent biopsied tissue to federal researchers in Atlanta.
“Within 24 hours, the director of the Texas Health Department came to see me,” Mr. Ramirez said. “He told me I had leprosy.” It was 1968, Mr. Ramirez had just turned 20, and he would spend the next seven years at the National Leprosarium in Carville, Louisiana.
Today, Mr. Ramirez, 66, is considered cured of the disorder, and he has no visible signs of it — no facial scarring or disfigurement, no loss of digits or clawing in of the hands and feet.
Emotional scarring is another matter. The “stigma, guilt and shame” that dog the disorder defy belief, Mr. Ramirez said, and ignorance about it remains profound. That is why he has given talks globally, with the message that everything you think you know about Hansen’s disease, about leprosy, is probably wrong. That message resonates with researchers as well, who say that leprosy continues to surprise and confound them. The illness can now be cured through a sustained course of antibiotics, yet the basic nature of Mycobacterium leprae is still being sketched out.
One research group proposed that leprosy may be the oldest infectious disease to go specifically for human beings, with origins dating back millions of years .
But scientists have also found that the leprosy bacillus is poor at migrating between human hosts. It dies quickly outside the body — a couple of hours on a lab slide, and that’s it — and about 95 percent of people appear immune .
“I refer to it as a wimp of a pathogen,” said Richard Truman, of the National Hansen’s Disease Program in Louisiana .
Recent studies have found that nearly half the DNA in M. leprae consists of pseudogenes, inert genetic sequences . That proportion of defunct code helps explain why the leprosy pathogen has one of the slowest generation times of its kind, dividing just once every 12 to 14 days, compared with 20 minutes for the ubiquitous E. coli.
Slow-growing though the bacteria may be, if left untreated, they will multiply into the many trillions, forming thick, scaly nodules on the face and extremities — “lepra” is the Greek word for scaly — and destroying the Schwann cells that protect the peripheral nervous system.
Today, Hansen’s is classified as a rare disease, yet it still strikes some 200,000 people a year, most of them in Brazil, India and other developing nations.
Dr. Erwin Schurr of McGill University in Montreal and other investigators have identified a handful of leprosy susceptibility genes, variants of human genes that for still mysterious reasons appear to enhance the likelihood that exposure to the pathogen will result in the disease.
Medical historians likewise are questioning the assumption that people with leprosy have always been shunned and reviled.
Scholars long cited the frenzied construction of leprosariums across Europe from the 11th through the 14th centuries as evidence that the leprous were considered so dangerous they had to be isolated. But recent historians have argued that the era’s leprosy mania was as much about fund-raising as anything else, a way for villages to attract wealthy donors.
“If you look at where leprosaria were located, they were often at major crossroads, where it was easy to conduct business,” said Monica Green, a medical historian at Arizona State University .
The new generation of leprosariums in the 19th and early 20th centuries were indeed isolation colonies . The fear of contagion arose even though Gerhard Armauer Hansen, the Norwegian doctor who discovered M. leprae in 1874 , failed to offer proof of its pathogenicity. Nothing Hansen injected with samples, including himself, ended up with leprosy.
Researchers remain hampered by its refusal to grow in cell culture or nearly any nonhuman animal.
The one puzzling exception is the nine-banded armadillo . Researchers say they believe wild armadillos — native to the Western Hemisphere — first contracted the infection from European settlers a couple of centuries ago .
In coastal marsh es where population densities of armadillos can be high, 20 percent or more are thought to be infected and capable of passing it on to people.
Mr. Ramirez suspected he contracted Hansen’s during childhood, with his migrant worker grandfather in the field, where armadillos were a common sight.
Dr. Truman advises people who spot a wild armadillo to steer clear . He said, “I can’t think of any way of exposing yourself to greater risk than trying to shoot armadillos .”
中譯
拉米雷茲現在是休士頓的一位臨床社工師，他整個青春期卻經歷了多變且令人不知所措的症狀。
時而發燒，時而惡心。手腳出現腫塊，還有不會癒合的瘡。他變得對碰觸異常敏感，連床單的窸窣聲都無法忍受。他的前臂麻木，而他還興致勃勃地利用這個併發症。他說：「我會把安全別針插進皮膚裡，讓女生印象深刻。」
皮膚科醫師大惑不解。通靈論者提到邪魔附身。最後，當地醫院的兩位醫師把他的活體組織寄給亞特蘭大的聯邦研究人員。
拉米雷茲說：「不出24小時，德州衛生處長就來看我，說我得了漢生病（俗稱麻風）。」
那是1968年，他剛滿20歲，此後七年他都待在路易斯安納州卡維爾的國家漢生病院。
如今他66歲，醫師認為他痊癒了，外觀沒有明顯徵象：臉部沒有疤痕或變形，手指和腳趾既未缺損，亦未卷曲。
情緒上的疤痕可就是另一回事了。他說，圍繞漢生病的「汙名、罪惡感和羞愧」令人難以置信，人們對它仍然非常無知。因此他到世界各地演講，告訴人們他們自以為對漢生病、對麻風的了解，恐怕全是錯的。
這訊息與研究人員的說法若合符節。他們說，漢生病不斷讓他們驚訝與困惑。漢生病已可用持續的抗生素療程治癒，漢生桿菌的本質卻仍有待勾勒。
一個研究團體認為，漢生病可能是源自數百萬年前、只有人類會得的最古老傳染病。
不過，科學家也發現，漢生桿菌很難在人類宿主之間移動，在人體外死得很快，在實驗室載玻片只能存活幾小時，就這樣了，而且約95%的人似乎免疫。
路易斯安納州國家漢生病計畫中心的杜魯門說：「我管它叫病原體中的窩囊廢。」
最近的研究顯示，漢生桿菌近半數去氧核糖核酸包含偽基因，也就是不活動的基因序列。這種比例的無效遺傳密碼可解釋漢生病原體的倍數生殖期何以在同類中殿後，每12到14天才分裂一次，相較之下，無所不在的大腸桿菌每20分鐘就分裂一次。
雖然這種細菌繁殖很慢，但如果不治療，會繁殖成幾兆個，在臉上和手腳形成粗大乾燥的結節（漢生病的希臘文lepra即鱗片狀之意），並摧毀保護末梢神經系統的許旺細胞。
現在漢生病被歸類為罕見疾病，每年卻仍侵襲約20萬人，多數在巴西、印度和其他開發中國家。
加拿大蒙特婁麥基爾大學的舒爾博士和其他研究者已辨識出少數漢生易感基因，這是人類基因的變項，似乎會使「接觸漢生病原體後染上漢生病」的可能性提高，但原因仍然不明。
醫學歷史學家同樣也在質疑漢生病患始終遭受排斥與辱罵的假設。學者一向舉11到14世紀全歐洲建立漢生病院的狂潮為例，證明人們認為漢生病患非常危險，必須隔離。不過最近歷史學家主張，當時對漢生病大張旗鼓，募款是個重要目標，這是各村莊吸引富人捐款的一個方法。
美國亞利桑納州州立大學醫學歷史學家莫妮卡‧格林說：「當時漢生病院往往位在方便做生意的重要路口。」
19世紀與20世紀初的新一代漢生病院的確是與世隔絕的聚落。雖然1874年發現漢生桿菌的挪威醫師漢生無法證明漢生桿菌的致病性，人們仍害怕漢生病蔓延。漢生用漢生桿菌樣本注射的對象都沒有染上漢生病，包括他自己在內。
漢生桿菌無法進行細胞培養，也幾乎無法在人類以外的任何動物體內繁殖，研究人員仍受制於此。
九帶犰狳是個令人困惑的例外。研究人員說，他們相信幾世紀前歐洲來的移民首先把漢生病傳給在西半球土生土長的野生犰狳。
在犰狳密度高的海岸濕地，20%或更多的犰狳可能感染漢生病且能傳給人。
拉米雷茲猜測，他是在童年與移工祖父一起下田時感染漢生病，田裡犰狳很常見。
杜魯門博士建議，看到野生犰狳應閃避。他說：「我想不出有什麼事比試圖射殺犰狳更危險。」
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