Living Life With a Lethal Gene.
March 17, 2007 9:34 PM Subscribe
Facing Life With a Lethal Gene. Say you're in your early twenties. For years you've seen members of your family twist and turn invouluntarily and developing dementia due to your family history of Huntington's Disease. Even if you have the gene for the disease, the symptoms are unlikely to hit until you're 50. Would you want to find out if you're going to share the same fate as your relatives, or live life out as much as you can unaware if you're going to suffer from it too?
Another touching human interest story from the NY Times which has had a bunch of these recently.
If you're considering getting married or having children, you would have a responsibility to be aware of this risk.
posted by SPrintF at 9:44 PM on March 17, 2007
posted by SPrintF at 9:44 PM on March 17, 2007
And, let's face it, you're gonna lose much motivation for that retirement fund.
posted by adipocere at 9:49 PM on March 17, 2007 [1 favorite]
posted by adipocere at 9:49 PM on March 17, 2007 [1 favorite]
I know someone who is in this precise situation. Various immediate family members, including his father and an aunt, have succumbed to the disease; he has not and will not be tested because he doesn't want his whole life to be inflected by it, to a greater degree than it already has been.
He's very impatient and motivated sort when it comes to hitting the various milestones of adulthood -- like getting married or entering a stable and successful career situation, for example -- and I think that little trinucleotide repeat he may be carrying has a lot to do with it. He's also very religious. These understandable characteristics aside, he's a pretty normal, well-balanced guy.
I should point out that it's possible to have a version of Huntington's that hits as early as your mid-thirties. The disease varies in severity, supposedly in relation to the number of nucleotide repeats in the affected gene. The Wikipedia article is pretty good.
posted by killdevil at 9:51 PM on March 17, 2007 [1 favorite]
He's very impatient and motivated sort when it comes to hitting the various milestones of adulthood -- like getting married or entering a stable and successful career situation, for example -- and I think that little trinucleotide repeat he may be carrying has a lot to do with it. He's also very religious. These understandable characteristics aside, he's a pretty normal, well-balanced guy.
I should point out that it's possible to have a version of Huntington's that hits as early as your mid-thirties. The disease varies in severity, supposedly in relation to the number of nucleotide repeats in the affected gene. The Wikipedia article is pretty good.
posted by killdevil at 9:51 PM on March 17, 2007 [1 favorite]
Would you want to find out if you're going to share the same fate as your relatives, or live life out as much as you can unaware if you're going to suffer from it too?
Introducing an obsessive level of stress over something that is probabilistically inevitable is unlikely to improve the quality of one's life.
The only reason I can imagine to do genetic testing for "lethal" genes is if I were going to have children — and even then, research is already finding treatments for HD. For example, a research lab in my department is working on RNA drug therapies to silence CBP expression, which may have potential for human treatment.
What's slowly lethal today may not be slowly lethal tomorrow.
posted by Blazecock Pileon at 9:54 PM on March 17, 2007
Introducing an obsessive level of stress over something that is probabilistically inevitable is unlikely to improve the quality of one's life.
The only reason I can imagine to do genetic testing for "lethal" genes is if I were going to have children — and even then, research is already finding treatments for HD. For example, a research lab in my department is working on RNA drug therapies to silence CBP expression, which may have potential for human treatment.
What's slowly lethal today may not be slowly lethal tomorrow.
posted by Blazecock Pileon at 9:54 PM on March 17, 2007
I'd totally want to know. A good deal of our life is spent planning for later. I'd want to know so I can not save, do what ever I want, and then off myself at the first sign of symptoms.
posted by spaltavian at 10:06 PM on March 17, 2007 [1 favorite]
posted by spaltavian at 10:06 PM on March 17, 2007 [1 favorite]
killdevil - your friend, has s/he considered pre-screened in vitro fertilization, given the religious background?
This is a serious issue in medical ethics; on one hand humans have the 'right' to breed - on the other, when said offspring have a high risk of having significant quality of life issues, is the prerogative and biological drive to procreate offset the risks of bringing about a consciousness that has a high chance of experiencing significant suffering*?
Throw in 'every sperm is sacred' and it gets wooley, indeed.
*yes, some feel that suffering is the human condition, but there's still the chance that such a life can and will experience pleasure or help humankind; but that's gambling. Is the minuscule chance of raising a potential Einstein or Hawking worth the suffering that Hawking - or any number of genetically-maladapted people - worth the risk and consequence of having a deleterious genetic makeup?
I think the crux of the matter is where to draw the line; if I knew that my child would have the physical life that Hawking lived, I'd spare it before it became concious. Loss to humanity? Definitely. Loss to that entity? - I'd rather it not have suffered. I'm of two minds though, wrt peristaltic/saltatory evolution of humanity - I guess I'm too material/physical. Given the observation that Idiocracy champions, it's moot point anyway.
Then again, if an offspring of mine was diagnosed with ADD or ADHD, or something that doesn't directly cause decreased in physical quality of life I'd be inclined to keep my said offpsring off of pharmaceutical medication. \
posted by porpoise at 10:17 PM on March 17, 2007
This is a serious issue in medical ethics; on one hand humans have the 'right' to breed - on the other, when said offspring have a high risk of having significant quality of life issues, is the prerogative and biological drive to procreate offset the risks of bringing about a consciousness that has a high chance of experiencing significant suffering*?
Throw in 'every sperm is sacred' and it gets wooley, indeed.
*yes, some feel that suffering is the human condition, but there's still the chance that such a life can and will experience pleasure or help humankind; but that's gambling. Is the minuscule chance of raising a potential Einstein or Hawking worth the suffering that Hawking - or any number of genetically-maladapted people - worth the risk and consequence of having a deleterious genetic makeup?
I think the crux of the matter is where to draw the line; if I knew that my child would have the physical life that Hawking lived, I'd spare it before it became concious. Loss to humanity? Definitely. Loss to that entity? - I'd rather it not have suffered. I'm of two minds though, wrt peristaltic/saltatory evolution of humanity - I guess I'm too material/physical. Given the observation that Idiocracy champions, it's moot point anyway.
Then again, if an offspring of mine was diagnosed with ADD or ADHD, or something that doesn't directly cause decreased in physical quality of life I'd be inclined to keep my said offpsring off of pharmaceutical medication. \
posted by porpoise at 10:17 PM on March 17, 2007
...er, keep said offspring and keep them off of pharmaceutical medication...
posted by porpoise at 10:20 PM on March 17, 2007
posted by porpoise at 10:20 PM on March 17, 2007
I'd want to know fairly early. But then after my second HIV test I actually picked up the results, and I had more reason to believe those results might not be negative. (If I'd found out I was HIV-positive I'd've lived for today like spaltavian said.)
And Blazecock Pileon, most pregnancies are unplanned. To my thinking if I thought I might carry a "lethal gene" having sex without knowing my test results would be like having sex knowing that a former lover died of AIDS without getting tested myself. As for "[w]hat's slowly lethal today may not be slowly lethal tomorrow," how long have they been looking for a cure for cancer or HIV?
posted by davy at 10:22 PM on March 17, 2007
And Blazecock Pileon, most pregnancies are unplanned. To my thinking if I thought I might carry a "lethal gene" having sex without knowing my test results would be like having sex knowing that a former lover died of AIDS without getting tested myself. As for "[w]hat's slowly lethal today may not be slowly lethal tomorrow," how long have they been looking for a cure for cancer or HIV?
posted by davy at 10:22 PM on March 17, 2007
Would you want to find out if you're going to share the same fate as your relatives, or live life out as much as you can unaware if you're going to suffer from it too?
You would certainly want to know what you would do if you had the information, before you get said information. If you're just going to pray that you don't have HD, then I would not think it's a good idea to have the test at all. But if you've got plans to borrow vast sums of money and live the next fifteen years like they're your last, then you might be better equipped, psychologically, to deal with the news.
posted by kisch mokusch at 10:23 PM on March 17, 2007
You would certainly want to know what you would do if you had the information, before you get said information. If you're just going to pray that you don't have HD, then I would not think it's a good idea to have the test at all. But if you've got plans to borrow vast sums of money and live the next fifteen years like they're your last, then you might be better equipped, psychologically, to deal with the news.
posted by kisch mokusch at 10:23 PM on March 17, 2007
It isn't the same but it is as close as I can get, if someone told you they could tell you exactly when and how you died, would you want to know, knowing that you can do nothing to change the inevitable?
Nope, I wouldn't either. The taint would be far too overbearing.
posted by fenriq at 10:26 PM on March 17, 2007
Nope, I wouldn't either. The taint would be far too overbearing.
posted by fenriq at 10:26 PM on March 17, 2007
fenriq writes "if someone told you they could tell you exactly when and how you died, would you want to know, knowing that you can do nothing to change the inevitable?"
Oh, hell yes! It would be a great comfort, not having to worry about dying until then, and knowing how long I had to finish the things I want to get done.
posted by orthogonality at 10:35 PM on March 17, 2007 [2 favorites]
Oh, hell yes! It would be a great comfort, not having to worry about dying until then, and knowing how long I had to finish the things I want to get done.
posted by orthogonality at 10:35 PM on March 17, 2007 [2 favorites]
if someone told you they could tell you exactly when and how you died, would you want to know, knowing that you can do nothing to change the inevitable?"
Hell yes!
That would rock. Ahh the freedom from worry!
posted by lazaruslong at 10:41 PM on March 17, 2007
Hell yes!
That would rock. Ahh the freedom from worry!
posted by lazaruslong at 10:41 PM on March 17, 2007
Well, I already have reason to suspect I'll eventually die by suicide, whether I'm terminally ill/damaged or not. (All those jealous husbands are so stupid.)
posted by davy at 10:49 PM on March 17, 2007
posted by davy at 10:49 PM on March 17, 2007
As for "[w]hat's slowly lethal today may not be slowly lethal tomorrow," how long have they been looking for a cure for cancer or HIV?
I don't believe that the need for testing for HIV is the same as the need for testing for HD. The epidemiological implications and societal impact of the spread of HIV are on a fundamentally different level from HD.
HIV spreads quickly through exchange of blood, vaginal secretions and semen, and progression of AIDS can take as little as two years. HD is a fairly rare affliction, spread only through reproduction to the next generation, giving a symptom progression time of 30-50 years between generations.
In any case, there are very few cures for diseases, but there are plenty of treatments and management therapies. With cancer, for example, you have chemo patients go into remission at higher rates than in the past, and with HIV you have positive folks on retroviral cocktails, surviving longer than when it first showed up in the early 1980s, when people would die within a couple years.
Research into genetically HIV-resistant people has shown a receptor mutation (CCR5) that points to a vulnerability in HIV's infection process and may lead to a vaccine. Genetic therapies are targeting genetic changes in tumor cells, providing "magic bullet" therapies. There are lots of irons in the fire with respect to HIV and cancer.
As to whether the availability of cures for various diseases are down to the complexity of those diseases or the fact that a cure is a less profitable business model than a management-based treatment, who knows. Depends how cynical one is, I guess.
But certainly the RNA therapy I mentioned (and others) has stemmed off HD in model organisms like mice and fruit flies. This may have application for humans in future trials.
With respect to HD, given the research out there, I'm optimistic that treatments will be available within 10-15 years. Thus, testing for a disease that would have a longer progression time, if I had it, would introduce unnecessary stress in my life. I would be concerned for my offspring and would want them to have the knowledge, but as for myself, I don't know that knowing would add anything positive unless I knew treatments would be available around the corner.
posted by Blazecock Pileon at 10:49 PM on March 17, 2007 [1 favorite]
I don't believe that the need for testing for HIV is the same as the need for testing for HD. The epidemiological implications and societal impact of the spread of HIV are on a fundamentally different level from HD.
HIV spreads quickly through exchange of blood, vaginal secretions and semen, and progression of AIDS can take as little as two years. HD is a fairly rare affliction, spread only through reproduction to the next generation, giving a symptom progression time of 30-50 years between generations.
In any case, there are very few cures for diseases, but there are plenty of treatments and management therapies. With cancer, for example, you have chemo patients go into remission at higher rates than in the past, and with HIV you have positive folks on retroviral cocktails, surviving longer than when it first showed up in the early 1980s, when people would die within a couple years.
Research into genetically HIV-resistant people has shown a receptor mutation (CCR5) that points to a vulnerability in HIV's infection process and may lead to a vaccine. Genetic therapies are targeting genetic changes in tumor cells, providing "magic bullet" therapies. There are lots of irons in the fire with respect to HIV and cancer.
As to whether the availability of cures for various diseases are down to the complexity of those diseases or the fact that a cure is a less profitable business model than a management-based treatment, who knows. Depends how cynical one is, I guess.
But certainly the RNA therapy I mentioned (and others) has stemmed off HD in model organisms like mice and fruit flies. This may have application for humans in future trials.
With respect to HD, given the research out there, I'm optimistic that treatments will be available within 10-15 years. Thus, testing for a disease that would have a longer progression time, if I had it, would introduce unnecessary stress in my life. I would be concerned for my offspring and would want them to have the knowledge, but as for myself, I don't know that knowing would add anything positive unless I knew treatments would be available around the corner.
posted by Blazecock Pileon at 10:49 PM on March 17, 2007 [1 favorite]
A little spooky an article, because of the young woman's last name. It's not that common, and I may have worked with her father in the mid 80's. I hope it's not the same family.
Knowledge can be a dangerous thing. Once, I was told I had 18 months to live. That was over 20 years ago. But it was really painful at the time. Good to know how wrong they were. (I'm fully recovered from the issue, long since).
But something like this? Ignorance only makes total sense, to me, if you're not playing the reproductive game. I'd not want to have children, unknowing whether I was offering this gene to them. The only alternative I can see would be invitro screening, without recieving knowledge of the results. Ms. Moser's mother was not unreasonable. (God, I hope it wasn't my friend's wife, I liked her).
posted by Goofyy at 1:29 AM on March 18, 2007
Knowledge can be a dangerous thing. Once, I was told I had 18 months to live. That was over 20 years ago. But it was really painful at the time. Good to know how wrong they were. (I'm fully recovered from the issue, long since).
But something like this? Ignorance only makes total sense, to me, if you're not playing the reproductive game. I'd not want to have children, unknowing whether I was offering this gene to them. The only alternative I can see would be invitro screening, without recieving knowledge of the results. Ms. Moser's mother was not unreasonable. (God, I hope it wasn't my friend's wife, I liked her).
posted by Goofyy at 1:29 AM on March 18, 2007
Another touching human interest story from the NY Times which has had a bunch of these recently.
I think that little paper is really going to make a name for itself someday.
posted by jjg at 1:30 AM on March 18, 2007
I think that little paper is really going to make a name for itself someday.
posted by jjg at 1:30 AM on March 18, 2007
I had a girlfriend who had this in her family (grandparent) and she obsessed over getting tested. she was practically already living her life as if she did have the gene, and life would effectively end before she was 40. her father wouldn't get tested because his life insurance policy (which was for a ridiculous amount) would've been cancelled/phased out, and he basically didn't want to know. one in a long list of issues that father-daughter team clashed over. Last I heard, she never got tested, and had a couple of kids (this was a big consideration for her. 50% chance of passing that on was terrifying to her).
this link, however, is a great read, and I thank you.
posted by Busithoth at 6:24 AM on March 18, 2007
this link, however, is a great read, and I thank you.
posted by Busithoth at 6:24 AM on March 18, 2007
I would be concerned for my offspring and would want them to have the knowledge
But not concerned enough to avoid having offspring in the first place? I can understand the "I don't want to know" thing as long as it only affects you, but if you're thinking about getting married, let alone having kids, you have a moral obligation to find out. To allow someone to commit to you for life when said life may be over in a couple of decades after a long, agonizing period of decline is unconscionable; to take the risk of passing it on to kids is vile.
posted by languagehat at 6:32 AM on March 18, 2007
But not concerned enough to avoid having offspring in the first place? I can understand the "I don't want to know" thing as long as it only affects you, but if you're thinking about getting married, let alone having kids, you have a moral obligation to find out. To allow someone to commit to you for life when said life may be over in a couple of decades after a long, agonizing period of decline is unconscionable; to take the risk of passing it on to kids is vile.
posted by languagehat at 6:32 AM on March 18, 2007
My grandfather died of Huntington's when my mother was in her early teens. He was estranged from the family, so she only knew him as he was dying. Whenever my family would talk about him, they always implied he died from alcoholism (he was a traveling magician, so it made a certain amount of narrative sense), and it was only after my mother had passed the typical age for the disease to manifest that she told me the truth.
I'm so thankful she delayed telling me for so long. I was such a melodramatic little twit when I was younger that I don't think I would have accomplished anything (Who cares about Chemistry homework - I'm going to DIE!).
On the other hand, if she had developed the disease, I don't know if I would have respected her choice. I can't imagine both losing my mother and knowing that I might go through the same ordeal. And then being blindsided by it, knowing that my parents had known all along that such a thing might happen. This sort of foreknowledge turns tragedy into betrayal.
And yet, most days, I'm pretty glad that I was born. But then, the risk paid off.
posted by bibliowench at 7:43 AM on March 18, 2007
I'm so thankful she delayed telling me for so long. I was such a melodramatic little twit when I was younger that I don't think I would have accomplished anything (Who cares about Chemistry homework - I'm going to DIE!).
On the other hand, if she had developed the disease, I don't know if I would have respected her choice. I can't imagine both losing my mother and knowing that I might go through the same ordeal. And then being blindsided by it, knowing that my parents had known all along that such a thing might happen. This sort of foreknowledge turns tragedy into betrayal.
And yet, most days, I'm pretty glad that I was born. But then, the risk paid off.
posted by bibliowench at 7:43 AM on March 18, 2007
Cool story. I can't understand how you would not want to know whether you had it or not. How could you have kids knowing that you might be passing along the gene to them?
posted by octothorpe at 8:10 AM on March 18, 2007
posted by octothorpe at 8:10 AM on March 18, 2007
Tricky territory; personally, whether I got tested or not, I'd probably get sterilized. I'd be terrified of passing something like that along to my kids even if I personally escaped the odds. And I don't see how "not knowing" works; in my mind, I would constantly be wondering anyway...every muscle cramp would make me wonder "is this it??" and I'd go crazy. Better to know and to have time to come to grips with that.
And yeah, for me...probably lay in a supply of of bye bye drugs.
posted by emjaybee at 8:46 AM on March 18, 2007
And yeah, for me...probably lay in a supply of of bye bye drugs.
posted by emjaybee at 8:46 AM on March 18, 2007
On a (somewhat) related note, the Machine of Death short story contest.
posted by John of Michigan at 8:48 AM on March 18, 2007
posted by John of Michigan at 8:48 AM on March 18, 2007
Of course one should find out. The more known folks with your genetic ailment, the more research dollars are going to be directed to a cure. Also the larger population sample to study to find potential therapies. If you are 20 years old today, even if you have the variant that strikes in your 30s you have 10-15 years for a medical breakthrough. The same is through for cancer and alzheimers. Also you have a chance to make preperations if they don't find a cure. You can decide how your going to die, where you are going to hospice, how to protect your asests for your heirs, etc. Quit being such a wuss, we're all going to die, having more certainty around the most probable cause and timing is a blessing.
posted by humanfont at 9:10 AM on March 18, 2007
posted by humanfont at 9:10 AM on March 18, 2007
Humanfont: I concur.
While I do not have Huntington, I do have Marfan syndrome^, which, if untreated, is likely to terminate you around the age of 35, as your aorta ruptures. I have had my heart surgery, so my life expectancy is back to more or less normal. (I'm now 36)
Here knowledge is definitely a good thing - while Marfan cannot be cured, at least some of the deadlier symptoms can be alleviated, if treated in time. Plus, you have the knowledge to not pass it on to a new generation (my own Marfan was the result of a mutation).
There are a lot of nastier genetic disorders out there and Huntington is certainly one of them. Still, from my perspective, I would support the notion that full knowledge would always be preferable. Even if you are going to die young (for some years I expected to die around 35), knowing this allow you to act accordingly. Willful ignorance will not accomplish much, especially if you can observe the disorder in family members - I would find the doubt more unnerving than certainty. Best case, you're healthy. Worst case, your fears have been confirmed, but at least now you can face them.
posted by bouvin at 9:54 AM on March 18, 2007
While I do not have Huntington, I do have Marfan syndrome^, which, if untreated, is likely to terminate you around the age of 35, as your aorta ruptures. I have had my heart surgery, so my life expectancy is back to more or less normal. (I'm now 36)
Here knowledge is definitely a good thing - while Marfan cannot be cured, at least some of the deadlier symptoms can be alleviated, if treated in time. Plus, you have the knowledge to not pass it on to a new generation (my own Marfan was the result of a mutation).
There are a lot of nastier genetic disorders out there and Huntington is certainly one of them. Still, from my perspective, I would support the notion that full knowledge would always be preferable. Even if you are going to die young (for some years I expected to die around 35), knowing this allow you to act accordingly. Willful ignorance will not accomplish much, especially if you can observe the disorder in family members - I would find the doubt more unnerving than certainty. Best case, you're healthy. Worst case, your fears have been confirmed, but at least now you can face them.
posted by bouvin at 9:54 AM on March 18, 2007
But not concerned enough to avoid having offspring in the first place?
If I know there's a 50% chance my kids have the allele, and I know that there is a 30-50 year delay before progression of the disease for them, assuming they are unlucky, then given those odds it is safer to expect HD treatments to be available for them, than, say, for myself.
In sum, if I was having children, it is a less risky proposition for their survival than it was for my parents having me.
Luckily we don't yet live in a GATTACA-style world, so I don't have to worry about the Eugenics Police entering into my decision-making process.
posted by Blazecock Pileon at 10:44 AM on March 18, 2007
If I know there's a 50% chance my kids have the allele, and I know that there is a 30-50 year delay before progression of the disease for them, assuming they are unlucky, then given those odds it is safer to expect HD treatments to be available for them, than, say, for myself.
In sum, if I was having children, it is a less risky proposition for their survival than it was for my parents having me.
Luckily we don't yet live in a GATTACA-style world, so I don't have to worry about the Eugenics Police entering into my decision-making process.
posted by Blazecock Pileon at 10:44 AM on March 18, 2007
it is safer to expect HD treatments to be available for them, than, say, for myself.
Wow. First you imagine a beautiful fantasy world in which HD is cured, then you blithely place your potential kids in it and wash your hands of any moral issues. Impressive.
Check it out: they were saying cancer would be cured any minute now half a century ago. Still no real progress. Oh well, the dream is beautiful, let's live in it—reality sucks.
I don't want any Eugenics Police either, but I expect people to make responsible decisions for themselves. I'm constantly reminded of how overoptimistic I am about humanity.
posted by languagehat at 11:15 AM on March 18, 2007 [2 favorites]
Wow. First you imagine a beautiful fantasy world in which HD is cured, then you blithely place your potential kids in it and wash your hands of any moral issues. Impressive.
Check it out: they were saying cancer would be cured any minute now half a century ago. Still no real progress. Oh well, the dream is beautiful, let's live in it—reality sucks.
I don't want any Eugenics Police either, but I expect people to make responsible decisions for themselves. I'm constantly reminded of how overoptimistic I am about humanity.
posted by languagehat at 11:15 AM on March 18, 2007 [2 favorites]
If you must have children (and adoption or donated egg/sperm is not an option), pre-natal screening is progressing nicely, and works well for many genetic disorders. This is not an area that I would leave to Nature, or the expected progress of Science. Eugenics is a really, really bad idea as state policy, but I think we should be responsible with our own genetic material. You shouldn't (knowingly) gamble on your children's health.
posted by bouvin at 11:19 AM on March 18, 2007
posted by bouvin at 11:19 AM on March 18, 2007
Part of me really wishes that these tests would not be allowed until there was at least one treatment available to help or fix the situation (i know it's not good to think that, and i usually always come down on the freedom of important information).
Like for the breast cancer genes there are remedies available, but for many diseases the testing to id them has way way too far outpaced available solutions.
posted by amberglow at 11:51 AM on March 18, 2007
Like for the breast cancer genes there are remedies available, but for many diseases the testing to id them has way way too far outpaced available solutions.
posted by amberglow at 11:51 AM on March 18, 2007
(and i know i certainly would want to know--i'd demand it--even if it messed up my life entirely)
posted by amberglow at 11:52 AM on March 18, 2007
posted by amberglow at 11:52 AM on March 18, 2007
First you imagine a beautiful fantasy world in which HD is cured, then you blithely place your potential kids in it and wash your hands of any moral issues.
Languagehat, I think you are wildly, badly, wrongly misinterpreting what I am saying. Take a breather and re-read another time, perhaps.
posted by Blazecock Pileon at 11:59 AM on March 18, 2007
Languagehat, I think you are wildly, badly, wrongly misinterpreting what I am saying. Take a breather and re-read another time, perhaps.
posted by Blazecock Pileon at 11:59 AM on March 18, 2007
For a minute, I thought this was going to be a post about It's a Bird, by Steven Seagle.
posted by carmen at 2:32 PM on March 18, 2007
posted by carmen at 2:32 PM on March 18, 2007
Wow. First you imagine a beautiful fantasy world in which HD is cured, then you blithely place your potential kids in it and wash your hands of any moral issues. Impressive.
There's a difference between an unrealistic fantasy and hoping for a positive outcome which, given the rate of current research advances, is far from impossible. Everyone must set his own threshold for refusing to have offspring given some chance of some genetic problem arising in the future... to force your threshold on them is just the route to Eugenics Police again. Yeah, there are going to be people who will make truly irresponsible decisions, but assuming they are not being flippant about it, it still wouldn't be my place to object.
(Yeah, I'm going to regret saying this when Languagehat is Commissioner of Eugenics Police in 20 years, I know.)
posted by Krrrlson at 3:03 PM on March 18, 2007
There's a difference between an unrealistic fantasy and hoping for a positive outcome which, given the rate of current research advances, is far from impossible. Everyone must set his own threshold for refusing to have offspring given some chance of some genetic problem arising in the future... to force your threshold on them is just the route to Eugenics Police again. Yeah, there are going to be people who will make truly irresponsible decisions, but assuming they are not being flippant about it, it still wouldn't be my place to object.
(Yeah, I'm going to regret saying this when Languagehat is Commissioner of Eugenics Police in 20 years, I know.)
posted by Krrrlson at 3:03 PM on March 18, 2007
if someone told you they could tell you exactly when and how you died, would you want to know, knowing that you can do nothing to change the inevitable?"
I really don’t know. There is on the one hand the freedom that comes from that sort of knowledge. On the other hand, each successive moment framed in the light of that knowledge might become more trivial and less valuable somehow. Most of us live our lives without the knowledge of how we will die and that fact is part of what contributes to our sense that each day we have is precious, that our time spent with our friends and family is precious. If I knew my allotment of time, I’m sure my goodbyes wouldn’t be as sweet because I could be certain that they wouldn’t be my last.
Maybe my real thought, though, is that life and the time you have to live it would, in the case of having the knowledge of when that time would end, not be less valuable than it would otherwise but would perhaps be of a more determinate value than it would otherwise, that is you would be able to more accurately say what each moment is worth and thusly have a better sense of how you want to spend that time. As things stand generally, our lack of knowledge leaves us unable to allocate clear determinations of value to the time we have, except perhaps in retrospect.
posted by inconsequentialist at 3:09 PM on March 18, 2007
I really don’t know. There is on the one hand the freedom that comes from that sort of knowledge. On the other hand, each successive moment framed in the light of that knowledge might become more trivial and less valuable somehow. Most of us live our lives without the knowledge of how we will die and that fact is part of what contributes to our sense that each day we have is precious, that our time spent with our friends and family is precious. If I knew my allotment of time, I’m sure my goodbyes wouldn’t be as sweet because I could be certain that they wouldn’t be my last.
Maybe my real thought, though, is that life and the time you have to live it would, in the case of having the knowledge of when that time would end, not be less valuable than it would otherwise but would perhaps be of a more determinate value than it would otherwise, that is you would be able to more accurately say what each moment is worth and thusly have a better sense of how you want to spend that time. As things stand generally, our lack of knowledge leaves us unable to allocate clear determinations of value to the time we have, except perhaps in retrospect.
posted by inconsequentialist at 3:09 PM on March 18, 2007
Languagehat, I think you are wildly, badly, wrongly misinterpreting what I am saying. Take a breather and re-read another time, perhaps.
That's very possible, in which case I apologize. You might help a brother out by hinting at how I should be interpreting it, though.
There's a difference between an unrealistic fantasy and hoping for a positive outcome which, given the rate of current research advances, is far from impossible.
Not really. "Far from impossible" = "unrealistic fantasy," to my mind. I mean, it's far from impossible that advanced space aliens are visiting us, so why not pin your hopes on their saving our asses from our mistakes? Once again, I have no truck with Eugenics Police (as an anarchist, I'm not fond of police of any kind), but I would hope people would consider a 50% chance of an early death sentence (give or take the unknown but presumably low chance of a miraculous cure being developed in the near future) a sufficient reason not to procreate.
posted by languagehat at 4:02 PM on March 18, 2007
That's very possible, in which case I apologize. You might help a brother out by hinting at how I should be interpreting it, though.
There's a difference between an unrealistic fantasy and hoping for a positive outcome which, given the rate of current research advances, is far from impossible.
Not really. "Far from impossible" = "unrealistic fantasy," to my mind. I mean, it's far from impossible that advanced space aliens are visiting us, so why not pin your hopes on their saving our asses from our mistakes? Once again, I have no truck with Eugenics Police (as an anarchist, I'm not fond of police of any kind), but I would hope people would consider a 50% chance of an early death sentence (give or take the unknown but presumably low chance of a miraculous cure being developed in the near future) a sufficient reason not to procreate.
posted by languagehat at 4:02 PM on March 18, 2007
"Far from impossible" = "unrealistic fantasy," to my mind. I mean, it's far from impossible that advanced space aliens are visiting us, so why not pin your hopes on their saving our asses from our mistakes?
I guess where we differ is that I would not equate the expected probability that current disease research will yield positive results in a 10-20 year timeframe with the expectation that aliens will solve these problems for us in the same span. Given progress in biomedical research in the last 50 years, I would be very surprised if there weren't miracles yet coming in our lifetimes. Call it informed optimism?
posted by Blazecock Pileon at 6:11 PM on March 18, 2007
I guess where we differ is that I would not equate the expected probability that current disease research will yield positive results in a 10-20 year timeframe with the expectation that aliens will solve these problems for us in the same span. Given progress in biomedical research in the last 50 years, I would be very surprised if there weren't miracles yet coming in our lifetimes. Call it informed optimism?
posted by Blazecock Pileon at 6:11 PM on March 18, 2007
Blazecock, one problem with your optimism is that only reasonably widespread diseases get funding for research. If whatever genetic disorder you happen to have is rare[1], you are out of luck. There has be a sufficiently large market for a cure to be developed (baring freak breakthrough discoveries of course).
[1] Or its sufferers are too poor.
posted by bouvin at 7:10 PM on March 18, 2007
[1] Or its sufferers are too poor.
posted by bouvin at 7:10 PM on March 18, 2007
Given progress in biomedical research in the last 50 years, I would be very surprised if there weren't miracles yet coming in our lifetimes. Call it informed optimism?
I wonder if most of that progress hasn't been essentially more about identifying things than actually curing things. We've learned more and more about how things develop and where and how our brains and bodies develop and work, and we can operate in tinier places than before and keep people alive much longer than before, but i haven't seen that many big cures or major new vaccines -- the HPV vaccine is perhaps the only new big one in my memory, and cervical cancer is not at all as big a killer as many others.
posted by amberglow at 9:54 PM on March 18, 2007
I wonder if most of that progress hasn't been essentially more about identifying things than actually curing things. We've learned more and more about how things develop and where and how our brains and bodies develop and work, and we can operate in tinier places than before and keep people alive much longer than before, but i haven't seen that many big cures or major new vaccines -- the HPV vaccine is perhaps the only new big one in my memory, and cervical cancer is not at all as big a killer as many others.
posted by amberglow at 9:54 PM on March 18, 2007
First you imagine a beautiful fantasy world in which HD is cured, then you blithely place your potential kids in it and wash your hands of any moral issues.
Languagehat, I think you are wildly, badly, wrongly misinterpreting what I am saying. Take a breather and re-read another time, perhaps.
posted by Blazecock Pileon at 1:59 PM on March 18
I'm reading you exactly the same way.
If there is a 50% chance of you passing it along... it should be absolutely unconscionable to procreate, to the point of being criminal in my mind.
There was a family that lived up the street from us a few years ago that had a 6 year old child, then had another baby, and the baby died within 1 year. Turned out to be a severe genetic defect, and there was a 50/50 chance of the same happening with another child.
In that situation, I would be sterilized the following day. And quite honestly, that is the only acceptable option. I want to say "in my opinion" but really... I think this transcends "opinion". It approaches a moral obligation.
They elected to have another baby, who also perished. And not a pleasant passing either, one of those ventilator assisted wasting away deaths.
They became criminals in my estimation if through nothing else but negligence. People are found to be criminally negligent in all sorts of ways every day.
Would you let them go to a nursery school and start executing kids based on the results of a coin toss? If the answer is no, then that should also answer the question of if they should be allowed to procreate or not.
posted by Ynoxas at 9:56 PM on March 18, 2007 [1 favorite]
Languagehat, I think you are wildly, badly, wrongly misinterpreting what I am saying. Take a breather and re-read another time, perhaps.
posted by Blazecock Pileon at 1:59 PM on March 18
I'm reading you exactly the same way.
If there is a 50% chance of you passing it along... it should be absolutely unconscionable to procreate, to the point of being criminal in my mind.
There was a family that lived up the street from us a few years ago that had a 6 year old child, then had another baby, and the baby died within 1 year. Turned out to be a severe genetic defect, and there was a 50/50 chance of the same happening with another child.
In that situation, I would be sterilized the following day. And quite honestly, that is the only acceptable option. I want to say "in my opinion" but really... I think this transcends "opinion". It approaches a moral obligation.
They elected to have another baby, who also perished. And not a pleasant passing either, one of those ventilator assisted wasting away deaths.
They became criminals in my estimation if through nothing else but negligence. People are found to be criminally negligent in all sorts of ways every day.
Would you let them go to a nursery school and start executing kids based on the results of a coin toss? If the answer is no, then that should also answer the question of if they should be allowed to procreate or not.
posted by Ynoxas at 9:56 PM on March 18, 2007 [1 favorite]
(and prevention has skyrocketed--but that's an id thing too--we've learned which foods and which activities cause or help prevent things--we haven't cured those things tho)
posted by amberglow at 9:57 PM on March 18, 2007
posted by amberglow at 9:57 PM on March 18, 2007
Ynoxas, it's not up to you to decide that--it's up to the parents. Many millions of people should not have kids (for all sorts of reasons) but they do. Should they be prevented as well? Just those who carry genetic markers? Who decides? Is a history of breast cancer in both sides of the family enough to do it? Just one side? A 50% chance of a kid having the disease? More? 25%? ...
posted by amberglow at 10:00 PM on March 18, 2007
posted by amberglow at 10:00 PM on March 18, 2007
Moreover, how can you decide if it is better for the child to have lived and died, or to have never existed? When is a tragic life worse than no life at all?
Would you let them go to a nursery school and start executing kids based on the results of a coin toss?
Overlooking the faulty analogy for the moment -- would you let some random dude decide when it is criminal for you to procreate?
posted by Krrrlson at 10:53 PM on March 18, 2007
Would you let them go to a nursery school and start executing kids based on the results of a coin toss?
Overlooking the faulty analogy for the moment -- would you let some random dude decide when it is criminal for you to procreate?
posted by Krrrlson at 10:53 PM on March 18, 2007
If there is a 50% chance of you passing it along... it should be absolutely unconscionable to procreate, to the point of being criminal in my mind.
Hypothetical: Your family has a history of nearsightedness and lives in a society which hasn't yet invented eyeglasses (or some technology to see better).
Not being able to see is fatal, as there are predators and other dangers you need to be able to see to avoid.
Society doesn't want the burden of invalids who get hurt because they can't see, and so the society writes into law that your parents can't have kids because there's a history of bad eyesight in your family.
That's pretty much the equivalent being established here. The technology isn't available to keep the offspring alive, so you want to write laws that decide who get to have children.
posted by Blazecock Pileon at 11:53 PM on March 18, 2007 [1 favorite]
Hypothetical: Your family has a history of nearsightedness and lives in a society which hasn't yet invented eyeglasses (or some technology to see better).
Not being able to see is fatal, as there are predators and other dangers you need to be able to see to avoid.
Society doesn't want the burden of invalids who get hurt because they can't see, and so the society writes into law that your parents can't have kids because there's a history of bad eyesight in your family.
That's pretty much the equivalent being established here. The technology isn't available to keep the offspring alive, so you want to write laws that decide who get to have children.
posted by Blazecock Pileon at 11:53 PM on March 18, 2007 [1 favorite]
Fantastic article. I commend this woman for taking life by the horns, so to speak and finding out what time she has left to do all she wants!
I do always find it shocking when parents have kids regardless of the chances of them having a fatal and debilating disease, however.
posted by agregoli at 9:33 AM on March 19, 2007
I do always find it shocking when parents have kids regardless of the chances of them having a fatal and debilating disease, however.
posted by agregoli at 9:33 AM on March 19, 2007
I think you have to excuse previous generations--most knowledge about diseases and cancers were summed up as "it runs in the family". It's only very recently that people knew for sure the chances of passing things on (or that they were definitely genetic at all), and even then as you see with the healthy Aunt in the story, it's still a crapshoot.
Adults now are the first group growing up with enormous knowledge--but no remedy (much like women now who have the breast cancer genes, etc). They'll be figuring this out, and making their own choices--at least they're armed with knowledge if not cures/vaccines.
posted by amberglow at 12:02 PM on March 19, 2007
Adults now are the first group growing up with enormous knowledge--but no remedy (much like women now who have the breast cancer genes, etc). They'll be figuring this out, and making their own choices--at least they're armed with knowledge if not cures/vaccines.
posted by amberglow at 12:02 PM on March 19, 2007
Not being able to see is fatal, as there are predators and other dangers you need to be able to see to avoid.
Society doesn't want the burden of invalids who get hurt because they can't see, and so the society writes into law that your parents can't have kids because there's a history of bad eyesight in your family.
As someone with terribly bad eye-sight, even I can tell that this equivalent is, in fact, not an equivalent.
Having terribly bad eye-sight, in your hypothetical predatory world would decrease their chances of survival due to external factors. These individuals would not increasingly disabled due to internal progression of disease - but if we take your example at face value, they probably don't have sufficiently advanced medicine that would save many of the badly-injured poor-eyed ones. So yes, you'd have a small sub-set of people who get injured, and then have to be cared for the by community. But they can still be, far more likely than not, productive or useful in other ways - storytellers, etcetera.
The eyesight in itself, doesn't cause them harm.
Rather the case against reproduction mentioned above has a strong ethical component, which you neglect - that of the high likelyhood that someone with HD (or an equivalent disease, say, cystic fibrosis) will pass it on to their progeny, dooming them to painful and short lives. I don't think one has to be quite willing to advocate laws against procreation (for reasons amberglow elucidated - a chance, even a sizeable one, is not certainty) to find procreation with a high risk of a horrible disorder to be morally questionable.
posted by canine epigram at 1:03 PM on March 19, 2007
Society doesn't want the burden of invalids who get hurt because they can't see, and so the society writes into law that your parents can't have kids because there's a history of bad eyesight in your family.
As someone with terribly bad eye-sight, even I can tell that this equivalent is, in fact, not an equivalent.
Having terribly bad eye-sight, in your hypothetical predatory world would decrease their chances of survival due to external factors. These individuals would not increasingly disabled due to internal progression of disease - but if we take your example at face value, they probably don't have sufficiently advanced medicine that would save many of the badly-injured poor-eyed ones. So yes, you'd have a small sub-set of people who get injured, and then have to be cared for the by community. But they can still be, far more likely than not, productive or useful in other ways - storytellers, etcetera.
The eyesight in itself, doesn't cause them harm.
Rather the case against reproduction mentioned above has a strong ethical component, which you neglect - that of the high likelyhood that someone with HD (or an equivalent disease, say, cystic fibrosis) will pass it on to their progeny, dooming them to painful and short lives. I don't think one has to be quite willing to advocate laws against procreation (for reasons amberglow elucidated - a chance, even a sizeable one, is not certainty) to find procreation with a high risk of a horrible disorder to be morally questionable.
posted by canine epigram at 1:03 PM on March 19, 2007
They'll be figuring this out, and making their own choices--at least they're armed with knowledge if not cures/vaccines.
I simply cannot imagine the pain that would come with knowing the odds, having a child anyway, and then having that child end up with the disease. Everyone makes their own choices in this world, but playing this particular gamble comes off as purely selfish to me.
posted by agregoli at 1:07 PM on March 19, 2007
I simply cannot imagine the pain that would come with knowing the odds, having a child anyway, and then having that child end up with the disease. Everyone makes their own choices in this world, but playing this particular gamble comes off as purely selfish to me.
posted by agregoli at 1:07 PM on March 19, 2007
well, if they can test for it in utero, the women can abort if they want. Giving birth itself can be seen as pretty much an entirely selfish thing if you want--disease or not. Why should we want to pass on our genes? Why are there so many unwanted kids all over the world languishing while people spend thousands and thousands on fertility treatments? It's evolutionarily(?) selfish in itself to bear kids, no?
posted by amberglow at 1:47 PM on March 19, 2007
posted by amberglow at 1:47 PM on March 19, 2007
That article reads like my childhood.
My mother was diagnosed 14 years ago. So she started preparing. Getting all her affairs in order, going for testing, researching options.
All that time, my sister and I were constantly reminded that we had a chance at it.
At first, mom was optimistic. She hoped her kids didn't have it. She respected our decisions to not get the test, to not know, to not have that genetic shadow hanging over our heads.
Then Grandpa died. Not solely from his Huntingtons, but definitely related. He fell, and his body just couldn't get up.
He couldn't walk straight. He shook when he was asleep.
After that, mom started harping on the kids to get tested. She wanted to know. We still didn't.
Every year, she talks more about death. We get wills giftrwapped. Christmas dinner is peppered with "how's life" and "I want to be buried here".
Anyone I'm dating gets the "Huntingtons" talk during holidays. It's always methodical. She's got her speech memorized. She wants grandchildren, but doesn't want them to have her heritage.
This last Christmas, I got the standard t-shirt I won't wear, a card from my Uncle, and a blood test kit, to ship my DNA off to a DNA bank for Huntingtons, with my sister getting a matching present. Mom even wrapped it.
It's still sitting on a bookshelf, gathering dust. Because I don't want to know. My sister just sent me this article. And I read it. We both agree, it sounds like Mom. Her kit is untouched too.
I don't like the fatalistic attitude I've seen come over my Mom since her diagnosis. So for that reason, I'm not getting tested. If and when I get to the point where I want children, I'll cross that bridge. But I see how all consuming this has become to my mom, and I don't want that for me, or those around me.
posted by fnord at 3:50 PM on March 19, 2007 [1 favorite]
My mother was diagnosed 14 years ago. So she started preparing. Getting all her affairs in order, going for testing, researching options.
All that time, my sister and I were constantly reminded that we had a chance at it.
At first, mom was optimistic. She hoped her kids didn't have it. She respected our decisions to not get the test, to not know, to not have that genetic shadow hanging over our heads.
Then Grandpa died. Not solely from his Huntingtons, but definitely related. He fell, and his body just couldn't get up.
He couldn't walk straight. He shook when he was asleep.
After that, mom started harping on the kids to get tested. She wanted to know. We still didn't.
Every year, she talks more about death. We get wills giftrwapped. Christmas dinner is peppered with "how's life" and "I want to be buried here".
Anyone I'm dating gets the "Huntingtons" talk during holidays. It's always methodical. She's got her speech memorized. She wants grandchildren, but doesn't want them to have her heritage.
This last Christmas, I got the standard t-shirt I won't wear, a card from my Uncle, and a blood test kit, to ship my DNA off to a DNA bank for Huntingtons, with my sister getting a matching present. Mom even wrapped it.
It's still sitting on a bookshelf, gathering dust. Because I don't want to know. My sister just sent me this article. And I read it. We both agree, it sounds like Mom. Her kit is untouched too.
I don't like the fatalistic attitude I've seen come over my Mom since her diagnosis. So for that reason, I'm not getting tested. If and when I get to the point where I want children, I'll cross that bridge. But I see how all consuming this has become to my mom, and I don't want that for me, or those around me.
posted by fnord at 3:50 PM on March 19, 2007 [1 favorite]
can you get her to therapy about it, fnord, or group therapy for all of you? She has to learn to fit the knowledge into the rest of her life without dwelling or ruining her life (and yours), no? Does she know how much this affects you guys?
posted by amberglow at 5:04 PM on March 19, 2007
posted by amberglow at 5:04 PM on March 19, 2007
She does know how it affects us. Neither my sister or I are very quiet about it.
Both of us have been to therapy, and talked about it.
Unfortunately, Mom is in a different city, so we don't see her that often.
I'll suggest the group therapy to her. She has a degree in psychology, focusing on therapy, so that's probably something she'd go for.
posted by fnord at 6:10 PM on March 19, 2007
Both of us have been to therapy, and talked about it.
Unfortunately, Mom is in a different city, so we don't see her that often.
I'll suggest the group therapy to her. She has a degree in psychology, focusing on therapy, so that's probably something she'd go for.
posted by fnord at 6:10 PM on March 19, 2007
Agreed, amberglow - having kids is inherently selfish to me - but it still stuns me the lengths that particular brand of selfishness can go to.
posted by agregoli at 9:08 AM on March 20, 2007
posted by agregoli at 9:08 AM on March 20, 2007
or maybe there's a support group or something like that she could give a try, fnord?
(or you 2 could confront her privately at some holiday when you're all together, but that prob wouldn't help--she has good reasons (or thinks she has), and that might override the impact it has on you 2 and the rest of the family, so she prob feels justified in her dwelling on it and changing bec of the knowledge, etc)
/armchair psych : >
posted by amberglow at 8:11 AM on March 21, 2007
(or you 2 could confront her privately at some holiday when you're all together, but that prob wouldn't help--she has good reasons (or thinks she has), and that might override the impact it has on you 2 and the rest of the family, so she prob feels justified in her dwelling on it and changing bec of the knowledge, etc)
/armchair psych : >
posted by amberglow at 8:11 AM on March 21, 2007
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posted by portisfreak at 9:36 PM on March 17, 2007