Host: I have heard about what is called Tay-Sachs. What is Tay-Sachs? Why would they be worried about it?
Lakshmi Mehta: The Tay-Sachs disease is a severe neurodegenerative disorder. So babies are born looking normal, but over the next few months they start deteriorating neurologically and it's a fatal disease, eventually the children will die.
Host: How soon that they die?
Lakshmi Mehta: They die within, like a year or two.
Host: A year or two.
Lakshmi Mehta: Yeah.
Host: And they have trouble with muscles, and the brain --
Lakshmi Mehta: They basically lose all their milestones and their vision, and there is no treatment for it, and no cure of it. It's an enzyme deficiency, it's called hexosaminidase, and it's a recessive disease, about one in twenty five, Ashkenazi Jewish people are the carriers.
Host: It's a --
Lakshmi Mehta: Gene.
Host: It's recessive?
Lakshmi Mehta: It is recessive.
Host: So both parents have to carry it.
Lakshmi Mehta: Both parents have to carry it and one in 25 --
Host: Are you getting people come for. People come to you, before to get married to check things up before they get together?
Lakshmi Mehta: Yes, and we actually nine Jewish genetic diseases now.
Host: So if you don't like your daughter's future husband, check him out, and you say, oh, you've got a problem here.
Lakshmi Mehta: There are different ways that screening is done. There is a program called Dor Yeshorim, actually orthodox Jewish communities, where basically as teenagers, individuals are screened. So they have this database of what who carry, and when a match is proposed for one of those individuals.
Host: You mean love isn't involved in here anymore?
Lakshmi Mehta: No, love. When the matchmaker --
Host: What if you notice that if love goes in?
Lakshmi Mehta: So the matchmaker says, this is not compatible. He won't even tell you what you carry, but he'll tell them ---
Host: Oh, they don't even know themselves?
Lakshmi Mehta: They don't know which, what gene they carry, but they are told that it's not a good match. If they're both carrying the same genetic problem.
Host: So they'll say this is not a good deal?
Lakshmi Mehta: it's not a good deal, and then he'll find you someone else whose not a carrier, maybe someone --
Host: If a kid is born and they didn't do all the test, what could they do when a baby is born to pick up Tay-Sachs?
Lakshmi Mehta: Well, there isn't any screening at birth, so if a baby is -- I think it's extremely unusual today in our area that Ashkanzi Jewish individual is not screened for Tay-Sachs. I think at some level they are all picked up, but you would have to do a blood test for an enzyme.
Host: What's the name of that enzyme?
Lakshmi Mehta: The enzyme is hexosaminidase, but rather than check the baby, I would check the parents to see if they are really carriers, and at any time, and if only if they are both carriers, would be concerned about the baby, and then the baby can have the blood test.
Host: How many Tay-Sachs are there born every year? Are that many?
Lakshmi Mehta: It's very rare now.
Host: It's less and less, because we are screening.
Lakshmi Mehta: Because of the screening, but now --
Host: So let's kind of pick, you're out of business, by not having problems.
Lakshmi Mehta: Well, actually today, don't be too surprised, sometimes we do see Tay-Sachs in couples that are mixed ethnic background now.
Host: You don't have to be an Ashkanzi Jewish?
Lakshmi Mehta: No, one in 200 non Ashkanzi Jewish people could be carriers. So it's much rarer. Less than one in 25, but if you see Tay-Sachs, actually it's more often to a couple who is of mixed ethnic background.
Host: Because sometimes you never know what your grandfather did?
Lakshmi Mehta: No, you don't, and we do have about nine other genetic disorders, for which they screening in the Ashkanzi Jewish community, and some are much rarer.
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