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A Tragic Legacy : Why is Tay-Sachs, a rare genetic disorder, killing so many children in a tiny Cajun town? The answer seems to lie in the region's melting-pot heritage.

November 06, 1990|J. MICHAEL KENNEDY | TIMES STAFF WRITER

IOTA, La. — The mystery begins in this small farming community deep in the heart of Acadia.

The sweet singsong of Cajun French is still spoken here, where the Woodmen of the World Lodge and Ritter's Bar and a few other storefronts anchor a town of 1,200 surrounded by soybean and rice fields. It is where the makings of tragedy have been passed down, unbeknown to anyone who lived here, from generation to generation.

The killer in this story is Tay-Sachs disease, a genetic affliction that attacks the nervous system of children just out of infancy and kills them by the age of 5. It is untreatable and marches unchecked through the body's functions, causing loss of motor skills, seizures and, eventually, blindness, deafness and paralysis.

The mystery is why it appeared here, seemingly with such suddenness. Tay-Sachs is associated with East European Ashkenazi Jews and their descendants. It was rarely even heard of in this staunchly Catholic slice of the country settled by French Canadians.

Then Renee Abshire turned the key and found the first clues of Iota's secret. She did so when she found out her baby was going to die.

Abshire's daughter, Maigon, began behaving strangely at the age of 7 months. The baby was hesitant to do such things as hold her bottle. Her mannerisms had kind of a lazy way to them. It seemed to the parents that Maigon should have more strength to hold herself up.

"It was like a newborn, you had to give her so much support," said Abshire.

The doctor didn't think at first there was much cause for alarm. But two months later, the Abshires enrolled Maigon in a special program for the mentally handicapped, convinced their daughter needed special attention. And then came the seizures.

One Baton Rouge neurologist thought the baby had cerebral palsy. But then another specialist in New Orleans examined Maigon. The doctor peered into Maigon's eyes and saw the cherry-red spot that is the sure indicator of Tay-Sachs, the disease that strikes only one child in 400,000 in the United States.

"I was numb and in shock," Abshire said. "I had never even heard of Tay-Sachs, didn't even know it was a Jewish disease. I was born and raised in Texas. My husband was from Louisiana. I thought, 'How in the world can this happen to us?' I started searching for someone else who was dealing with what I was dealing with."

She began calling local doctors. Over time, and after many phone calls, Abshire found three other families in which there was a Tay-Sachs baby. No outsiders, these. In three of the four cases, at least one parent had attended Iota High School.

For such a small town, it was an appallingly high number. How could this have happened?

Soon, Abshire had more pressing worries. Maigon died in February, 1989, at age 3. The costs of caring for the child had left the family bankrupt.

Still, the mystery of Iota was unsolved. It took a doctor from New Orleans to open the door to the mystery just a little wider. His name was Dr. Emmanuel Shapira, a geneticist from the Tulane Medical Center.

In his role as a genetic counselor, Shapira already had met both the Abshires and another family, the McClellands, from nearby Crowley. He had told the two families what to expect as Tay-Sachs took hold. And he had explained how the disease spread, of how both the man and woman had to be carriers, but that even then the chance of having a Tay-Sachs baby was only one in four.

Shapira was not surprised at Abshire's findings in Iota. Louisiana, particularly in the southern part of the state, was known as a place where isolation and inbreeding over the centuries had produced clusters of rare diseases.

He decided to take some blood samples in Iota to see how they stacked up against the norm. Last July, Shapira brought 230 test kits to the Knights of Columbus Hall. He ran out because so many people wanted to know if they were carriers of the disease. The results, completed last month, were astonishing: Of the 230 people tested in Iota, 17 were carriers. It meant that the carrier rate in Iota was roughly twice that of Jews and 20 times the population as a whole.

The other part of the test was answering a questionnaire in which the townspeople were asked if they had any of the surnames of the three families with Tay-Sachs babies. Of the 230 people, 83 had at least one of the surnames and 14% were carriers. But even of those 147 people who did not have the surnames, five still tested as carriers.

The number of carriers in Iota is so great that Shapira believes parents should be tested before the decision is made to have children. And he said he will begin looking at surrounding communities to see if there are other clusters of people carrying the Tay-Sachs gene.

"In this area, people have continued to intermarry since the 1700s," said Shapira, who speculated that other children from past generations may have died of Tay-Sachs but had their deaths attributed to something else.

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