Elephant Man

His illness afflicts thousands

Quasimodo probably had it. Digger Barnes of the television soap opera Dallas has developed the symptoms. In real life the most famous victim was John Merrick, the grotesquely deformed "Elephant Man," who became a sought-after celebrity in Victorian England.

The affliction is neurofibromatosis, a terrifying and, so far, incurable neurological disorder usually accompanied by varying degrees of deformity. Though most Americans had not heard of it before the Dallas episodes, or before The Elephant Man, a play about Merrick, opened in New York City this year, the ailment is surprisingly common; in the U.S., it affects some 100,000 people.

The first signs of neurofibromatosis usually appear in childhood: small, brown skin discolorations known as cafe au lait spots. Later, neurofibromas--ugly but benign skin tumors that can grow to look like brown cauliflower--may form anywhere on the body, particularly on the back, chest and abdomen. In severe cases, the body is eventually covered by thousands of these tumors. Some may develop internally, attaching to the brain's acoustic or optic nerves and other vital tissues. Another, rarer manifestation of the disease is "elephant skin," large hanging folds of epidermis.

Merrick, who had both external and internal signs of the disease, was so shockingly deformed that producers of The Elephant Man wisely decided against using any grotesque makeup for the actor in the title role; Merrick's appearance is merely suggested by the actor's body language and the reactions of others in the cast. Lame and in constant pain, the real Merrick was covered with lesions and pendulous folds of skin. His right hand, nose and feet were unrecognizable.

One eye was nearly occluded by an outgrowth of bone. He could barely speak, was unable to smile, and his face, according to the attending doctor, looked like "a block of gnarled wood." The only way he could sleep was to rest his head on his knees. In 1890, longing to sleep "like other people," he lay down on his back, dislocated his neck and died of suffocation.

In milder forms of the disease than Merrick's, physicians are now able to deal with much of the deformity of neurofibromatosis by surgery. Some of these operations are for purely cosmetic reasons. In one recent case, for example, plastic surgery was used to treat a girl of eleven who had a fold of fibrotic skin hanging from her genital area. Said Dr. P. Bela Fodor, who performed the operation at St. Luke's Hospital in Manhattan:"There's a good chance she will never have a recurrence and that she will go on to live a normal life."

Sometimes the surgery is vital. One man had the disorder all his life with no serious complications until his 50s, when he developed a tumor on his brain stem that caused vertigo, deafness and numbness of the face. The tumor was successfully removed.

Plastic surgery often has to be repeated over and over. Lynne Ann Courtemanche, 35, of Manhattan, has spent 18 years undergoing surgery on her face and body; the tumors are removed as soon as they appear. For Courtemanche, president of the National Neurofibromatosis Foundation, the continuing ordeal is preferable to no treatment at all. She recalls seeing, at 17. a picture of John Merrick. Says she: "I thought, 'This is what I'll look like in a couple of years.' I didn't really know if I wanted to live that long."

Though neurofibromatosis is still poorly understood, medical researchers have recently been focusing on a blood protein that is unusually high in some victims of the disease. But a cure seems far off, and Courtemanche and the foundation are concentrating their efforts on showing victims how to live with the disorder. Says Courtemanche: "Merrick bore up with tremendous dignity and grace. You have to develop an inner peace with yourself -- otherwise you're just condemned to a living hell."

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