Tay Sachs almost eradicated

This is great news and how often do we hear about great news in the Jewish genetic disorder sphere?

From Haaretz Daily English Edition

Tay-Sachs, the `Jewish disease,' almost eradicated
By Tamara Traubman, Haaretz Correspondent

The genetic mutation disease Tay-Sachs, a fatal inherited disease of the central nervous system that mostly affects Ashkenazi Jews, has been almost completely eradicated, experts say, who claim that a genetic illness has become extinct for the first time.

"Last year not a single Jewish baby throughout North America was born with Tay-Sachs," says Prof. Robert Desnick of the Department of Human Genetics at New York's Mount Sinai Hospital. Prof. Desnick is in Israel as the guest of Jerusalem's Hadassah hospitals. He said Monday that of the 10 babies born in North America in 2003 with Tay-Sachs, not a single one was Jewish.

Figures from Israel paint a similar picture. According to Prof. Joel Zlotogora, who heads the Health Ministry's Department of Community Genetics, just one baby was born with Tay-Sachs in Israel in 2003. Insofar as is known, not a single baby in Israel was born with Tay-Sachs last year, but as the disease takes some six months to manifest itself, the figures for 2004 are not final.

Desnick says that the data for the past two years may stem from a coincidental fluctuation in the incidence of the disease, and that isolated cases may appear this year or the next. He stresses, nevertheless, that whatever the case may be, the disease appears to have disappeared almost completely from among the Jewish nation.

Prof. Gideon Bach, who heads the Department of Genetics at Hadassah University Hospital, Ein Karem, says the eradication of Tay-Sachs can be attributed primarily to the fact that the general public in Israel is advised to carry out, at the expense of the state, genetic tests to diagnose the disease before the birth of the baby. In the event an unborn baby is diagnosed with Tay-Sachs, the pregnancy is usually terminated.

Another reason for the eradication of the disease, Bach says, is the work of the ultra-Orthodox association, Dor Yesharim. The association carries out tests on young individuals to check whether they are genetically "suitable." The results of these tests are passed on to the matchmaker. If there is a risk that a designated couple may give birth to children affected with Tay-Sachs, the matchmaker will report that the match is unsuitable.

Bach, who works with Dor Yesharim, says that numerous intended couples have been split up in the wake of genetic testing.

Some 1,000 years ago a Jew developed the genetic mutation which, it turned out, causes the fatal inherited disease. It has since been passed on among the Jewish people through the generations.

Tay-Sachs is a fatal genetic disorder mostly found in children that causes progressive destruction of the central nervous system. In general, children affected by the disease do not live beyond the age of 4.