Lethal Legacy

No one is yet sure how much the still developing art of "genetic counseling" will help mankind. Kenneth Swier, 39, is among those who can see some possible benefits--though not for himself. A tall, rawboned man who yearns to work, Swier has spent eight years in depressing idleness in Colton, S. Dak. (pop. 601). He suffers from spinal cerebellar degeneration, a hereditary nerve disease that will probably kill him before his 45th birthday. But it now appears that a remarkable piece of genetic sleuthing may save many of Swier's relatives from sharing his fate --and provide a technique for controlling other hereditary diseases as well.

For six generations, members of Ken Swier's family have discovered in their mid-20s that they were doomed by a lethal legacy. Spinal cerebellar degeneration attacks the central nervous system, first affecting balance and coordination, then impairing speech and finally breathing. The process lasts about 15 years, usually ending in death with pneumonia. The best doctors can do is to prolong the victim's life. They cannot even diagnose the disease before it appears--generally at an age when the patient has already had children. The disease can be passed on only by those actively afflicted; half of their children are likely to suffer from it.

Fascinating Questions. Ken Swier's huge family includes 61 relatives living in or near the town of Colton. Until recently, most of them simply tried not to think about their susceptibility to the disease, which is now also killing Ken's brother Vernon, 35. But last summer, encouraged by a local chiropractor who had been treating them, some of the Swiers sought help from the National Genetics Foundation, which was formed in 1953, and now traces and helps treat about 2,000 known hereditary illnesses. The N.G.F. tackled some fascinating questions: How did the Swier family pick up the disease? Were any unknown relatives alive and vulnerable? And if so, what should they do?

One member of the family had succeeded in tracing the disease as far back as Ken Swier's great-great-grandfather, Gerrit John Vandenberg, some of whose children came to the U.S. from Holland. Of Vandenberg's eight children, four inherited his ailment, including one daughter who passed it on to seven of her nine children. Last month the N.G.F. staged a reunion in South Dakota for 95 of Vandenberg's descendants, who came from five states and Argentina. On hand were Drs. William Nyhan and Roger Rosenberg of the University of California School of Medicine at San Diego, plus Dr. Lawrence Schut, a Minneapolis neurologist who is one of the unaffected members of the Swier family. With the family's permission, a crew from WCCO-TV of Minneapolis, Minn., flew in to film the gathering for a documentary.

Last Generation. The reunion enabled the doctors to trace 600 people of Vandenberg ancestry and compile the most complete genetic record in med ical history. But tragedy dominated the gathering. In a daylong series of examinations, the doctors found possible early signs of the disease in twelve family members under the age of twelve, and definite symptoms in eight adults, including Joyce Eggen, 21. the pretty mother of a 14-month-old son.

Researchers have developed prenatal tests for several hereditary diseases that enable them to recommend therapeutic abortion if an unborn infant is seriously affected. But no such test exists for spinal cerebellar degeneration, and until one does, genetic counseling offers the only help. As a result, doctors have advised Swier's relatives who are in line for the disease not to have children or to delay their families until they can be certain that they have escaped their inheritance.

Most of Vandenberg's descendants are stoical about their situation, and one 22-year-old man whose father has the disease is frankly unwilling to forgo fatherhood himself. "Aren't I entitled to the same kind of life as anyone else?" he asked when a doctor suggested that he not have children. But others are determined that theirs will be the last generation to suffer from spinal cerebellar degeneration. Joyce Eggen has decided that her son. who has a 50% chance of inheriting her disease, will be her only child. A 21-year-old nephew of Swier's. who does not yet know if he will have the disease, has gone even farther. To make sure he will have no children, he has undergone a vasectomy.

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