Feb 9, 2011

Mishpacha: The Gene Marker's Promise

The Gene Marker's Promise

Rabbi Yosef Ekstein of Dor Yeshorim Vowed that No Couple Would Know His Pain
When Rabbi Yosef Ekstein's fourth Tay-Sachs baby was born, he knew he had two options - to fall into crushing despair, or take action. “The Ribono Shel Olam knew I would bury four children before I could take my self-pity and turn it outward,” Rabbi Ekstein says. But he knew nothing about genetics or biology, couldn't speak English, and didn't even have a high school diploma. How did this Satmar chassid, a shochet and kashrus supervisor from Argentina, evolve into a leading expert in the field of preventative genetic research, creating an international screening program used by most people in shidduchim today?


Rachel Ginsberg

He spoke little English, knew nothing about genetics, and never set foot in a college classroom. So how did Rabbi Yosef Ekstein, a shochet from Argentina , manage to create an international system that has virtually eradicated the occurrence of Tay-Sachs and other recessive genetic illnesses common to the Ashkenazic Jewish community?

Today, Dor Yeshorim is a household word, at least among families with shidduch-aged children. Who in the Torah world looks into a prospective match without first ascertaining genetic compatibility by calling in their numbers to the Dor Yeshorim hotline? But in 1983, when Rabbi Ekstein first set out to prevent others from experiencing what he was so painfully living through himself, people thought he was a dreamer at best -- a digger of skeletons at worst.

Maybe it was because he was a war baby -- born while the Budapest building where his mother had taken shelter was being bombed -- that Yosef Ekstein became such a stubborn fighter, forging ahead with his program in the face of raised eyebrows and skeptical smirks. Yet at the beginning of his personal journey, his only fight was to get through his own pain.

The year was 1965, and Rabbi Ekstein, a Satmar chassid, shochet, and head of kashrus in Argentina, had just become a father to a newborn son. How simple it seemed in this generation, he thought. It was nothing like the situation his parents faced when he had been born.

His father, Rabbi Kalman Eliezer Ekstein, used to tell him, “You survived by a miracle. I don't know why, but it must be for a purpose.”

After his mother, Pessel, crawled out of the rubble of the bombed-out building with her hours-old infant, she hid him for several months until they could plan their escape. Meanwhile, Rabbi Kalman Ekstein was hiding in a pit. Having contacted smugglers who promised to get them over the border to Romania, Pessel and her sister -- who looked like a non-Jew and donned a cross around her neck -- dressed little Yosef up as a girl (they had given him a bris and didn't want anyone checking) and took a train to the border. At the border station, however, the little group realized they were being followed by nilosh -- Hungarian bounty hunters who whispered to each other, “They're ours.” Then, when one of them excused himself to the bathroom, the other one fell asleep waiting, and the Ekstein trio, hearing the snoring, tore out of the station and hid in the high grass until they could make contact with the smugglers. Rabbi Ekstein, who was to arrive the following night, got word that the smugglers had suddenly gone out of business, and so remained hiding in his pit until the end of the war.

The family was finally reunited and made their way to Israel, where they lived for nine years before moving to South America, where Rabbi Ekstein senior established the kehillah in Buenos Aires.

However, for the younger Rabbi Ekstein, twenty years removed from war-torn Europe, becoming a father proved to be far from simple.

“For the first six months, my son seemed to be thriving,” Rabbi Ekstein recounts, describing the shock of his first Tay-Sachs baby, the heart-wrenching onset of the disease and its devastating progression, as the infant seemed perfect for the first half-year of his life. “But soon I noticed he began to regress. There is nothing as horrible as watching your child deteriorate in front of you. It began with floppy muscle tone, then he couldn't swallow, and soon it progressed to seizures, paralysis, mental retardation, and blindness.”

In 1965, there was no testing or diagnostic method available for Tay-Sachs, other than seeing a red patch that eventually develops on the retina. Finally, at age two, the now severely disabled child was diagnosed with Tay-Sachs, an incurable genetic disorder that always results in a very young death.

“We looked on in horror as he turned into a vegetable and died at age four.”

The next child, a girl, was also afflicted with the disease. “With every child born the suspense was horrible, waiting to see if the child would be healthy or not.”

The Eksteins had buried two children, but forged ahead faithfully. The third child was healthy. But the next two …

In 1983, the Eksteins' fourth Tay-Sachs baby was born.

“The Ribono shel Olam knew it would take four children for me to get out of my own self-pity and turn my pain outward,” Rabbi Ekstein says. “I had two choices: despair or action. It took me nearly twenty years to get the message. But He paid me back with interest. Since starting Dor Yeshorim, we've been zocheh to five more healthy children.

“You have to understand the culture then. Everyone would hide their sick children, especially if we were talking about a genetic disease. It would put a black mark on the family. Who would want to do a shidduch with a genetically diseased family? At the time, we were living in Monroe. I was probably the first individual in our community who came out and said, 'I have a problem. I'm bleeding to death. Let's make sure no one else has to go through this.'”

We're All Carriers
Without genetic screening, the Eksteins, and thousands of couples like them, could never have known in advance that they were carriers for Tay-Sachs, a genetic mutation noted for its frequency among Ashkenazic Jews of Eastern European descent. This is because it is biologically impossible for a carrier to actually have the disease himself. However, if two carriers marry, each child born will have a 25 percent chance of falling ill.

Truth be told, everyone is a carrier of various genetic mutations and recessive lethal diseases that we'll never be aware of -- sickle-cell anemia, cystic fibrosis, Tay-Sachs, spinal muscle atrophy, familial dysautonomia, and Bloom syndrome, to name a few. But because our parents bestow upon us two “copies” of every gene, as long as there is only one genetic mutation between the two, the carrier will not be affected by the mutation.

That genetic mutation is still transmitted, however, and makes the person who receives it a “carrier.” As long as both parents are not carriers, there is no danger of their child being affected by the mutation carried by only one parent. However, if both parents possess the same genetic mutation, their children are at risk of inheriting a matched set of mutant genes. These genetic mutations can pass quietly for generations until two carriers marry; then their children have a one-in-four chance of being afflicted with the disease.

To purchase the full edition, go to Mishpacha.com

1 comment:

  1. This is mind-blowing. What a story.

    ReplyDelete

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