Would you marry someone like this?

I was joking when I said you should davka marry a carrier.

This is about my personal view; I don’t think we’re supposed to live our lives based on remote possibilities.

Do I know for sure that it wouldn’t affect my thought process if I knew?

No, but I would hope not.

Is it a good idea to have a public database listing everyone’s carrier status? Of course not, that’s not my argument.

Being a carrier of genes is less worse a problem than carrying bad middos. If the guy has a good heart, and a clear torah head, then yes I would marry them. Life is short, chances of cropping up a kid with genetic deficiencies are slimmer than not. Hashem is in charge. You just pray”

You are comparing apples with toothpaste (not even with oranges). What have good middos to do with bad genes??? It’s not an either or situation. If someone is a carrier, he or she should NOT marry and reproduce with another carrier, because, depending on the gene they carry, they are gambling with every pregnancy. And though THEY might feel it is perfectly ok to trust in Hashem (as do I), that does not mean that if you step into empty space, you’re gonna fly. Hashem CAN and DOES do miracles every day all day. But most of us are not worthy to see a miracle of the magnitude that one would require to save the life of a baby born with Tay-Sachs, Disautonomia, etc. These are ALL preventable diseases, simply by not being meshadeich two people who carry that gene. It is not fair to the unborn children, to say Gott Vet Helfen. I would marry a carrier, but only if I were not also one, and only if it required genes from both parents in order for there to be a 25% risk (which is too high in my book) that the child would be born diseased. BTW, with what is going on nowadays with gene therapy, that will BE”H eliminate these issues, some day.

oomis: +1!

As people who are born are far more likely to find shidduchim than others are, it actually helps your kids’ chances if you get married.

Doesn’t the religious community in America have Dor Yesharim? In Israel it’s something that NO ONE goes on shidduchim before doing. They basically test you for all these diseases, keep the results secret, and you can ask to match up the results of any couple, (usually it’s done even before the first meeting) and see if there is a significant risk in any of these diseases…

Ysiegel, we certainly do have Dor Yesharim in the USA, and the young man and woman’s numbers were compatible. LOTS is caused when a parent is a carrier. A Dor Yesharim test is only to determine compatibility vis a vis genetic disorders. It will not show if a child is potentially suffering (or will in the future) this dreaded disease. There is very little research and no cure whatsoever for the late onset condition since, B”H, Tay Sachs has virtually been eradicated with the advent of testing. There are approximately 200 cases reported yearly, worldwide.

The couple chose to end the shidduch.

DY: as I understand it, the Bochur/Choson had the disease and wasn’t aware prior to the Shidduch. His and his Kallah’s numbers were compatible, meaning one of them (in this case the kallah) wasn’t a carrier. I believe he made a mistake when he wrote the boy wasn’t a carrier. His parents never tested.

To clarify on A woman outside’s post- The genetics of late onset/adult onset Tay Sachs is the same as infantile Tay Sachs- the same gene- HEXA- is mutated, the affected individual has 2 mutated copies, one from each carrier parent. There are many different mutations that can occur in this gene. In the infant form, this results in the absence of the gene product hexosaminidase A, so symptoms are most severe and observed early. In the adult version, there is a deficiency in hexosaminidase A, the, but some enzyme activity is present, so that the disease progresses slowly, and at different rates in different individuals depending on the actual enzyme levels.
From your story, it seems like one of the dating partners (let’s assume the boy) has the disease, ie is homozygous for one of the mutations. Apparently, his parents were not tested, and since he was asymptomatic during childhood, he did not know he had Tay Sachs. The girl is negative, not even a carrier, which is why their numbers are compatible- if they were to marry, then their kids would all be carriers but not have the disease. So what it boils down to, is the girl willing to marry someone with what may very will be a debilitating degenerative disease. In this aspect, it is not any different from any other disease that could not have been predicted.
By the way, Dor Yesharin only works as far as known mutations. In early testing days, fewer mutations were identified and tested for. These have been expanded, also to include mutations common to non-Ashkenazik populations (not as common as the ones in Ashkenazik populations). If theoretically, someone was to develop a sporadic germ-line mutation in the gene that is not known and not tested for, they could be found compatible with a regular Tay Sachs carrier, the couple could marry and have a child with Tay Sachs at 25% probability.

B”h no personal experience, but have some knowledge of genetics and a quick read of Tay Sachs websites filled in the missing blanks for me.
Your story brings up several interesting points that people might want to think about, I’m curious how people feel.
1. Had this boy’s parents been tested and their mutation carrier status identified, they presumably would not have married and this boy would not have been born. But he has lived 20 something healthy, productive years, presumably bringing his family nachas, doing mitzvos, learning Torah. Should all of that not have happened? Reminds me of Miriam’s protest on Amram’s divorcing Yocheved…
2. From what I read, LOTS is very variable- from mild symptoms to severe degeneration, both physical and mental, at different ages. This is similar to cystic fibrosis (also tested by Dor yeshurin)- there is a range of age of onset and disease severity. In some cases, it can be a disease that one can live with, although obviously it’s not at all pleasant. It is arguably different from the infant form of Tay Sachs that is always fatal at young ages. Yet, as far as testing and negating a potential shidduch, these are treated the same. Is that right?
3. Even with Dor Yeshurin, there is no way to guarantee a healthy spouse or healthy children. There are so many more diseases out there. Has the concept of genetic testing contributed to a feeling that we can control this, as suggested by the obvious hurt/pain expressed by AWOB when the testing of the dating couple failed to reveal a serious illness?

Sorry but I don’t grasp what is being said here? Am I understanding properly that Dor Yeshurim can not test for LOTS?

Mammele, I believe he made a mistake when he wrote the boy wasn’t a carrier. His parents never tested.

Yes, the way I understand it, it wouldn’t make sense to say that he actually has Tay Sachs but isn’t a carrier.

Maybe now with CRISPR gene-editing on the horizon, genetic diseases will become a thing of the past.

“Point three can also be applied to wearing seat-belts in cars. Even with seat-belts, there is no guarantee that one won’t be injured. ”

You are right of course, but somehow when it comes to things that people don’t understand as well, emotion takes over logic.
And no one asks a potential shidduch suggestion if they regularly wear a seat belt, or whether their parents wear seat-belts, and no one is stigmatized for having not buckled up in the past, even though this could very well affect someone’s health/safety.

“Maybe now with CRISPR gene-editing on the horizon, genetic diseases will become a thing of the past. ”

Maybe…I still remember the excitement 25 years ago when viral-mediated gene therapy seemed to be the answer to genetic disease. They even had clinical trials for cystic fibrosis. Those were actually a disaster and had to be halted and the field of gene therapy has not been the same since.
CRIPSR is more efficient/better than other methods, but the same problem applies to all gene therapies- delivery to the right place, and targeting all the affected cells. Cystic fibrosis was a good disease to target because the CFTR gene needed to be delivered to the lungs, which are accessible by inhaling. The brain and nervous system are much harder to target. What is more realistic at this point in time is gene editing in the embryo following IVF. This of course opens a medical ethics mine-field of designing the perfect healthy baby….

If your not a carryer then why noy