The Joseph Illness

Antone Joseph liked California better than the Azores, and so he jumped ship in San Francisco. That was back in 1845. Joseph brought with him a rare form of degenerative nerve disease. He died of it in his 40s, but before he died he fathered six children. His legacy to them has proved lethal. In the 130 years since Joseph entered the U.S., at least 48 of his 300 or so descendants have died of this fatal family malady.

The phenomenon of genetic disease is not new. Researchers have identified some 2,000 hereditary ailments that can be passed from parent to child along with the genes for brown hair or blue eyes. They have even learned how to manage a few, like hemophilia and diabetes, and to detect Tay-Sachs disease.

The "Joseph illness" is peculiarly unpleasant. It usually starts with a loss of coordination, a drunken, staggering gait, and gradual slurring of speech. It ends, generally 20 years after its onset, in death, usually from pneumonia brought on by a gradual paralysis of the muscles involved in breathing.

Doctors have yet to determine the genetic defect responsible for Joseph illness. But they have been able to trace its transmission. The disease can be passed on only by those who are actually afflicted; each of their offspring has a fifty-fifty chance of inheriting the ailment. Unfortunately, it is almost impossible to detect before it begins--sometimes in the victims' teens, more usually in the mid-20s. By then, they have often started having children of their own and have passed the family disease along to them.

"I can remember my father with the disease," recalls Mrs. Rose Marie Silva of Livermore, Calif. "When I saw my brother stagger for the first time, I just knew he had it." For years, family members, some of whom believed erroneously that the problem was congenital syphilis, kept the disease a secret in their clan. Finally, Mrs. Silva broke the silence. After reading about a family afflicted with a similar hereditary illness (TIME, Jan. 25, 1971) and carrying the clippings in her purse for three years, she finally wrote the National Genetics Foundation last February and asked for help. The result of her call was a massive effort to trace all of Joseph's descendants and a gathering of the clan last week at Oakland's Children's Hospital Medical Center.

In many ways, the meeting was a typical family reunion. Cousins who had not seen each other for years renewed acquaintance; youngsters met relatives they had not even known existed. But the occasion was hardly happy. Drs.

William Nyhan of the University of California at San Diego and Roger Rosenberg of the University of Texas Southwestern Medical School in Dallas met with nearly 100 of Joseph's surviving relatives. They were able to assure those whose parents had escaped the disease that they ran no risk of developing--or passing on--the family ailment. But ten were found to have the disease, and 26 others--children of parents who have or had Joseph illness--may develop it.

Changed Outlook. Ricky Donahue. 20, whose mother died of Joseph disease and whose brother Kerry, 21, already has it, walked out of the hospital when he learned that he too had the disease. "I couldn't come back here; I had to leave and just walk," said he. "It was heavy, it really was."

But most victims have accepted the inevitable. Mrs. Violet Weldon, 41, whose mother died of the disease that she herself has had for several years, has adjusted to her affliction. "By the time I was told I had the disease, I had already come to the realization that the disease was a part of me," she explains.

Other family members have begun to rethink their plans for the future as a result of their new understanding of their heritage. "Time becomes important," says Ricky's brother Dennis, 23, who so far shows no signs of the malady. "Everyone wants to get rich and famous, but when you don't have a lot of time to live your life, those things are not so important any more."

The two doctors, who have studied the family closely, still do not know either cause or cure; all they can do is attempt to relieve the symptoms with drugs--and suggest that victims who have not yet had children consider forgoing having families of their own.

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