Orthodox Jews use gene science to protect family and tradition

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Yossi Dworcan with his son, Eli. (Image courtesy of Sarah Dworcan)

Yossi Dworcan with his son, Eli. (Image courtesy of Sarah Dworcan)

Before many ultra conservative Orthodox Jews tie the knot, they call a hotline: Dor Yeshorim.

Dor Yeshorim means Upright Generation, or Righteous Generation. It’s a genetic screening service used by hundreds of thousands of young Orthodox Jews.

You get a blood test in high school, and a card with your identification number on it. Later, when you’re older and about to get married, you and your significant other call up Dor Yeshorim. The service checks your DNA, and compares your ID numbers to make sure you aren’t both carriers for one of the genetic diseases more common in some Jewish populations.

But why do these diseases occur more often among Jews — specifically Ashkenazi Jews?

Bottlenecks

Harry Ostrer is a medical geneticist at the Albert Einstein College of Medicine and author of Legacy: A Genetic History of the Jewish People.

In ancient Rome — about 2,100 years ago — Ostrer said, it’s estimated that one in 10 Romans was Jewish. For comparison, today about 1 in 71 Americans are Jewish.

But then came some bottlenecks — drastic reductions in the Jewish population. These were following major world events — the rise of Christianity, the fall of the Roman Empire; and the start of Rabbinical Judaism — which made the communities more closed off.

“A group that may have been as small as 350 people led to the formation of contemporary Jews as we know them,” he said.  “ That’s really quite an extraordinary number when you consider today that there may be as many as 10 to 11 million Ashkenazi Jews.”

And when a population grows from a smaller offshoot, you lose genetic diversity. Geneticists call it the founder’s effect. Here’s how it works: Say in a given population 1 in 100 Jews is a carrier for an inherited neurological disorder called Tay-Sachs disease. But then 25 of them move away, and one is a carrier for Tay-Sachs disease, or as Ostrer puts it, they have the mutation — or gene variant — for Tay-Sachs.

“They will bring the mutation with them to their new locale. Whereas the prevalence of the mutation may have been relatively rare in the progenitor population, its frequency then got increased as a result of there being relatively few individuals in the founder population who then went on to have significant number of offspring,” Ostrer said.

In other words — one in a hundred — just became one in 25.

In the 70s, a basic blood test was developed to screen for Tay-Sachs disease. The disease damages the nervous system, and Ostrer saw its effects firsthand at a Brooklyn Tay-Sachs ward that once cared for up to 50 very young patients.

“It was really quite a tragic scene because the Tay-Sachs ward had a lot of babies and young children who had this profound neurodegenerative disease and so they were unaware of their surroundings,” he said. “They were blind, they had these pronounced startle responses because of how their nervous systems had been developed. They couldn’t swallow, so they had to be tube fed.”

As a public health student at Columbia University, Ostrer and other students set up Tay-Sachs screening, but the program only managed to screen a few hundred people.

There was carrier stigma among the conservative Jewish Orthodox community — people were worried about carrier status affecting their marriage prospects.

200 weddings

To understand more about the role marriage and family plays in these communities, I sat with my friend, Jeremy Berg, who grew up Yeshivish Orthodox — what he calls “black hat” Orthodox — in Queens, New York.

Between the ages of 20 and 25, Berg said, he went to more than 200 weddings.

Berg left the community later in life, but his family still practices.  And, his siblings all went through an exhaustive matchmaker process to find their partners.

“Essentially you fill out a resume of sorts which is like who you are. Girls put in their measurements. And there are some really out-there questions,” he said. “Some matchmakers want to know what type of tablecloth you use on your table for Sabbath meals, do you use china versus plastic? But things like mostly pedigrees, who you are, who your parents are, where you went to school, what you do for work.”

No one wants Tay-Sachs carrier on their matchmaker resume.

As we spoke Berg brought up a ‘bottleneck” that Harry Ostrer, the geneticist, hadn’t. It was another event that greatly reduced the Jewish population: the Holocaust

“If you factor in human inflation for these roughly 7 million Jews that were killed throughout the Holocaust, we could in theory be two-thirds larger than we are right now,” Berg said. “There’s that classic story of the Hasidic dude walking down the street with his 42 children and someone’s like, ‘When is that going to be enough?’ He said, ‘When I have 6 million.’”

That’s what the medical community was up against as those public health students were trying to get people to take the screening tests: the Torah says be fruitful and multiply, and an entire community was playing catch up after a devastating genocide. Anything that could possibly impede that, like carrier stigma, was going to be tough to sell.

In 1983, Hasidic Rabbi Joseph Eckstein finally found a way. He lost four kids to Tay-Sachs disease, and after that, he vowed to get his fellow Jews to accept screening.

Here’s how he did it: Just don’t tell people that they are a carrier. If no one knows their status, no one has to worry about carrier status affecting their son or daughter’s marriage prospects.

Instead the man and woman are rated together, as a couple, either compatible or not. They’re never told why or why not. And all this is done without names.

They get an identification number, and then before anyone marries those numbers are cross-referenced for possible genetic diseases.

It was a remarkable success.

By 1997, the Tay-Sachs ward in Brooklyn, the one with babies and toddlers, was practically empty, and today it’s shutdown.

As genetic information technology roared into high gear in the 90s, the Dor Yeshorim panel expanded well beyond the Tay-Sachs blood test to include more than a dozen diseases.

The service has helped create countless happy healthy families. Today high school kids in Orthodox communities have Dor Yeshorim screening days, where girls and boys line up to give blood samples.

“It was like ‘okay, he’s not alive.’”

But the Dor Yeshorim way hasn’t worked for every family.

Sarah and Yossi Dworcan are an Orthodox couple living in Florida. They went through Dor Yeshorim and got the all clear, but soon after the birth of their son Eli, the couple nearly lost him to a mysterious illness.

They first noticed Eli cried in a strange way, and stranger still, he almost never stopped.

“We have pictures of taking him home, and we were really excited but all the pictures he’s just screaming,” Sarah said.

And as he cried, Eli made these constant motions with his arms and legs.

“Picture a perfect freestyle swim, that’s what it was,” Yossi said.

Then when Eli was just a few days old, Sarah was dozing briefly while feeding him. When she awoke, Eli was blue.

“I remember the moment clearly, and to me it was like, ‘okay, he’s not alive,’” she said.

When she lifted him up, the baby started vomiting blood. But by the time the family got to the emergency room, Eli had stopped throwing up and he was breathing again. Staff did some tests, then said Eli seemed fine and sent the family home. Sarah said she didn’t want to be that crazy new mom making a scene, so she left — and went to a pediatrician.

That doctor didn’t seem ready to listen to her concerns.

“He goes, ‘Mom.’ In a really stern voice, he said, ‘Mom, if you’re not strong, you’re not going to be any good for your baby,’” she said. “That shut me up. I knew that if I was going to say another word I was going to burst out crying.”

That afternoon they got a call from the hospital Eli was born in. The results of Eli’s routine newborn screening showed he had Maple Syrup Urine Disease — a potentially fatal inherited metabolic disorder.

They rushed him back to the same hospital that had sent them home that morning, only this time an entire team of specialists was waiting.

“They just were wanting to know everything. Suddenly, everything we were saying was golden to them,” Sarah said.

When the dust settled, the new parents had time to think about the genetic screening they’d had before marriage.

“My first reaction in my head was, ‘how’s that possible?’ We take care of Ashkenazic genetic diseases. We’ve taken care of that,” Sarah said.

Her husband recalled a meeting with their OB-GYN.

“Even to rewind, when Sarah was pregnant, her OB-GYN had actually offered to do a genetic test, but Sarah said, ‘nope, we already did Dor Yeshorim we’re completely covered,’” he said.

Dor Yeshorim had said they were compatible, so the pair didn’t feel the need for any more testing. Screening existed for the Maple Syrup disease indicators when Sarah and Yossi met. But they say at Dor Yeshorim, it wasn’t offered yet.

The pair say they probably wouldn’t have ended up together if they had known they were carriers. They also say they’re thankful for their family.

“At this point, I can honestly say, I wouldn’t want anything different. I wouldn’t change Yossi for the world, I wouldn’t change Eli for the world,” Sarah said.

Sarah Dworcan with her son, Eli. (Image courtesy of Sarah Dworcan)

But Sarah and Yossi do want to change how their community looks at screening; the way Dor Yeshorim keeps carrier status under wraps.

“One of the things which you actually sign over there is that you will not be tested by any other organization in order to find out what you are genetically, [what] your carrier status is,” Yossi said.

Say a couple gets a ‘Not Compatible.’ The guy could go get tested somewhere else and see he’s a carrier for disorder X. He would then also know his partner must be a carrier as well, essentially outing her status.

Sarah and Yossie are focused on the medical risks. They started an awareness group called J netic to combat stigma.

And Eli is doing better now, with regular treatment and careful medical monitoring.

“If you met him, he’s an amazing three and a half year-old boy. You would have no idea if you saw him, that there was anything wrong for him. We are madly in love with him,” Sarah said.

Eli does need a lot of attention. Sarah and Yossi whip up a very precise diet for him and he’s fed through a gastrointestinal tube.

Then there’s the question of more children.

Any child Sarah and Yossie conceive would have a one in four chance of developing Maple Syrup Urine Disease.

Sarah says aborting a fetus diagnosed with MSUD would be difficult for her under Jewish law. But newer in vitro technology that screens the embryo before it’s implanted in the body gives her some wiggle room.

They haven’t decided yet, but now they know they have options. And they hope other carrier couples will learn they do as well.

“There are ways to have healthy children even if you are carriers,” Sarah said. “And just because we went through this doesn’t mean anyone else has to.”

 

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