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Friday, April 20, 2012


Someone I know was recently diagnosed with Huntington's Disease, sometimes known as Huntington's Chorea, and it got me thinking about some really perplexing moral dilemmas posed by the disease.  Huntington's, for those of you who do not know [there is a long and very informative article on Wikipedia, if you are interested] is a genetic disorder that afflicts between 5 and 10 persons per 100,000 worldwide, with Europeans much more likely to have it than Asians or Africans.  It is genetically dominant and not sex-linked, which means that if either parent has the gene on one chromosome [and hence will eventually come down with the disease, since it is dominant], a child has a 50% chance of having the disease as well.  If each parent has the gene on one chromosome, the probability is 75%, and if either parent has the gene on both chromosomes [a rarity] the probability of each child being affected is 100%.  Note that because the genetic disorder is dominant, it is not possible to inherit it from parents neither of whom develops the disease [assuming they live long enough.]

The disease causes increasingly severe motor problems, cognitive problems, and eventually dementia, leading to death.  Although there are cases of the disease appearing in childhood, it typically becomes manifest in young or middle-aged adults [which is to say, at or after child-bearing age].  There is no cure, and although drugs can to some extent ameliorate the symptoms, especially in the early phases of the disease, its progress is inexorable.  Life expectancy after the initial manifestation of the disease is about twenty years.

The moral dilemmas are obvious:  Should parents who know that they are gene carriers tell their children?  Should they have their children tested?  Should adults who know they are at risk for being carriers [and hence eventually sufferers from the disease] have themselves tested before they have children, and if they are carriers, should they have children at all?  Is someone who knows that Huntington's runs in the family obligated to tell a partner before marriage or procreation?  Does that person have a responsibility to be tested before entering into a committed relationship that might result in children? 

There is virtually no medical advantage to be gained for the individual from genetic testing, since knowing that one has the gene does not make it possible to do anything at all to lessen the inevitability of developing Huntington's.  To tell a child or young adult that he or she has the gene is to give that person a very hideous death sentence, albeit one that may not be imposed for several decades.  But knowing that one has the gene would allow a man or woman to deliberate about whether to have children, inasmuch as there would be a 50/50 chance of passing the disease on to each child.

One of the peculiarities of a genetic disorder like this is that it can be eliminated from the human genome in one generation if every carrier chooses not to reproduce.  Although an incidence of one per ten thousand [more or less] is very low, in a population of three hundred million, one would expect to find perhaps 30,000 persons who have manifested or will manifest Huntington's at any given time.  From a Public Health perspective, eliminating such a genetic disorder from the human genome would seem to hold out the prospect of avoiding a very great deal of human suffering.

So, there are the dilemmas [and many more, which I am sure my readers can conjure up.]  I have to confess that I have very few secure moral intuitions about this matter.  The one thing that seems obvious to me is that anyone who knows that he or she is at risk for carrying the gene has a moral responsibility to be tested before contemplating procreation.  It also seems to me clear [although perhaps not quite as clear] that such a person ought to inform anyone with whom he or she is considering entering a relationship that might lead to procreation.

I would be very curious to know what folks think about this.


Aliyah said...

Just some thoughts about the following:

"To tell a child or young adult that he or she has the gene is to give that person a very hideous death sentence, albeit one that may not be imposed for several decades. But knowing that one has the gene would allow a man or woman to deliberate about whether to have children, inasmuch as there would be a 50/50 chance of passing the disease on to each child."

Telling a child or young adult that he has Huntington's is not giving him the death sentence. Nature does that. You are simply informing him of the sentence.

And now the dilemma seems to be amenable to solution depending on the moral theory appealed to. Utilitarians may argue that the unhappiness caused by this knowledge is not outweighed by any benefit, and that therefore he should not be told. If he is not in a position to cause harm to others by his having the disease (by, for instance, passing it on), then ignorance seems morally supported.

However, when he reaches adulthood and begins to consider a family, Kantian considerations seem to dictate that he be told, since such ignorance would be culpable. He needs to be able to respect the rational nature of his spouse in choosing risks. He also needs to consider the possible offspring and the harm it may cause them (and their families, ad infinitum).

Superfluous Man said...

Professor Wolff, I feel sure you have read or have heard that Woody Guthrie had this disease. Arlo, Woody's son has been touring the country with his children and others and at one of his concerts, I saw in the paperwork or the website some mention of a charity that has been established for this family. Even though most families are not lucky enough to have family that has enough members who are musically talented, at least enough to be able to generate some legacy income that could help family members with Huntington's disease, this family would probably be able to give us some direction in how we might as a society approach the kind of ethical issues that you raise.

Although I don't expect Arlo to show up here tomorrow, I think if we are looking for answers to this kind of question, we might find it helpful to seek out the kinds of questions and answers that families that are already dealing with these kinds of ethical and moral issues have already confronted. As far as I know, I am lucky enough to have not had to deal with this kind of issue, but on the other hand, everyone potentially has something like this that would causes them to want to know, or not know, the answer to some of these questions before they arise. For instance, my grandmother had Alzheimers. If Alzheimers is hereditary and we can know with certainty who it will affect, should we tell people before it starts affecting how they behave and think and remember?

I recall that when I was much younger I had a teacher who developed early Alzheimers. When told of this diagnosis, she committed suicide a few days after she was told. What responsibities and duties does society have to protect people from information about themselves, if it has such a responsibility and duty at all? Those are difficult moral questions and I make no pretense of having an answer. However, I think most reasonable people agree that we do not want to have another repeat of the horrific Tuskegee syphillis experiment, and although the questions you pose are not directly related to all of the questions raised in that inquiry, indirectly there are at least some questons that are raised whenever a person is told or is not told they have any type of disorder, whether it is caused by disease or caused by a hereditary condition. The Tuskegee experiment even involved questions that should have been adressed under the Nuremburg code, which the US signed after World War II. Perhaps then, the Nuremburg Code is the first place we should look before undertaking such an endeavor.

Superfluous Man said...

Excuse me Professor, the United states did not sign the Nuremberg Code as there were no signatories, but it was incorporated into the Code of Federal Regulations, which offers us a very good reason to admit that Federal Regulations can and often do serve a fundamentally worthy and necessary purpose.

David Auerbach said...

One work-around for the procreation issue is to have genetic testing on the embryo and abort selectively. This is already done; and there's the nicely complex case of people who do that to produce a healthy bone marrow donor for a diseased sibling. (the disease at issue there is Fanconi's anemia.

Robert Paul Wolff said...

As I expected, this little post has generated some very interesting comments. I did know about Woody Guthrie [an icon from my youth]. It is his widow, marjorie, according to Wikipedia, who established the foundation. One wonders about Arlo. If Huntington's can be tested for in utero early on, it seems to me that it would be a good argument for aborting the embryo. Would a mother want to bear a child who would, she knew, develop such a disease, with 100% certainty? I would have thought not, though that is not my call to make.

NotHobbes said...

I believe there is an obligation to inform a potential partner and be tested before considering having children.
In the event of a positive test result and still forging ahead to plan a family smacks of utter selfishness; wholly inconsiderate towards an as yet unborn child, purely to fulfil one's own emotional desires.
Personally, I could not live with myself, knowing I could have inflicted a premature and agonising death upon my own children

Superfluous Man said...

On Woody's Wkipedia page, there is information about the foundation created by Marjorie. That foundation is now called the Huntington's Disease Foundation of America. There are other various foundations and charities around Woody and his living children, Arlo, Nora and Joady Ben. Something of an extensive family tree, apparently incomplete, can be found here:

I'm not sure if any of the living children have Huntington's but previously some in the family line have died from it according to his Wikipedia page, some of them children of Woody's children. I am assuming there have been advances in technology along the way, but whether any of the other family members now have or will succumb to the disease is not publicly known.
Arlo is something of a political anomaly. His wikipedia page says he is a registered Republican and supports Ron Paul, although on his website, he comes out in favor of the Occupy movement ("Occupy Everything"), is aware of the plight of young people in this country, and is also strong supporter for unions. One might conclude that at some point he has felt burned by taxes, although that is speculation, but in some ways he appears to be a political novice, at least at how wealth has come to be distributed the way it has in this country. On the other hand, it's hard to determine what motivates any particular person.

I added this information since the Guthrie family is the most well known American family to be plagued with this disease.

High Arka said...

What a good, thoughtful post.

Requiring the telling of a partner in procreation seems wrong, but in a moral sense, one should tell the partner if there's an understanding that they're going to be investing their genetic material in offspring.

Which raises another question: is choosing an individual to have children with something that one should do in order to increase the chances of the offspring having a successful life?

Probably not. Eugenics by any other name is still eugenics. If partners begin weeding each other out based on current interpretations of existing conditions, then they will be playing god just as much, in effect, as if they killed afflicted infants and then chose different partners later.

Who's to say when the condition may be curable? Who's to say what positive form the condition may one day take, which we cannot anticipate now?

What incredible treasures of the human race might grow with that condition, who would not have been born if their parents had decided to purchase a blond-haired, blue-eyed, tall, good-looking, Huntington's-free child instead?

And all that said, asking someone if they have it, or anything similar, is not, in and of itself, "wrong." You are accurate, Dr. Wolff, to pose it as a question of personal choice, rather than of social control.

Unknown said...

There are many people who know that their statistical chance of carrying the Huntington's allele (based on their parents' genotypes) is 50% but do not want to know for sure whether they carry it or not. If they want kids, a viable option is IVF with preimplantation diagnosis. That is, grow an embryo in vitro and figure out whether the embryo carries the disease allele before implanting in the mother's uterus. If the embryo carries the unwanted allele, simply don't implant. This will insure that the implanted embryo will not carry the disease allele, and no one but the doctor reading the genotyping results will have to know whether either parent carries the allele. It's easy, no one has to know for sure whether they carry the disease allele, and it's a less traumatic procedure than terminating a pregnancy after amniocentesis or what have you (while I support abortion, it's obviously a procedure that's best avoided, if possible).

High Arka said...

And a more exposed form of eugenics rears its head.

Eugenics: "the applied science or the bio-social movement which advocates the use of practices aimed at improving the genetic composition of a population."

Unknown, do you argue with the terminology, or do you believe that eugenics is justified in certain cases?

formerly a wage slave said...

I think RPW hit an acute point when he wrote that if those who carried the gene in one generation chose not to reproduce---and he did not intend thereby either to legislate or to advocate compulsion---The disease would disappear, thereby " avoiding a very great deal of human suffering."
Genetic testing makes sense when there is realistic hope of thereby lessening suffering.

In his book "The Lives to Come" Philip Kitcher claimed that overnight discoveries of cures for illnesses are rare---medical progress is slow. So it is unlikely either that Huntingtons will take on a benign form or be cured any time soon. Of course, so far as " playing god " goes, anyone who procreates is doing that, but insofar as one can prevent suffering in the future, it is not a matter of hubris, but rather compassion. That last claim, I admit, is too general to capture, say the difficulty entailed by the prospect of bringing someone into the world with the promise of only ? Fifteen, or? Twenty or X good years, and then how many years of deterioration and suffering? I do think, however, that one lesson of Kitcher's book is that someone who is realistically faced with such questions needs to sit down, and talk about, and think about them in detail, thinking about what's invoved in detail.

High Arka said...

That assumes a lot of things. Firstly, it assumes that our understanding of diseases is so accurate that we can eliminate them simply by cutting certain people out of the breeding pool.

Right now, you believe that of course, our understanding is accurate. But what if it's not? What if bloodletting is not an effective cure? What if a harmless passenger virus is not responsible for immune deterioration?

Actively meddling in evolution based upon the current popular scientific trends may not be the best way to decide which children should be born and which should not be.

Joanne said...

I have met many wonderful individuals and families who are living with the challenges of Huntington's disease. The conclusion I have come to is that I need to respect each person's decision about all of the many issues; testing, telling, potentially having at risk children and many more. We all have genetic issues that if we were to be aware of would probably give us reason to struggle with many of these same issues.

I did not see anyone comment on the potential of discrimination should a test prove someone to be gene positive. Of course the test doesn't tell them when they might become symptomatic yet having knowledge that you have the gene could lead to education, employment, insurance and other types of discrimination.

There are so many issues to deal with and I am continually heartened by the courage I see in families with Huntington's disease. It is my privilege to be a part of a local group, Philly Cure HD, working to offer support to these families.

Erin Krause said...

My mom had non diagnosed Huntington's disease. I remember my grandma having-and dying- from it. I chose to get tested so I could live my life in the best way possible. It was an extremely difficult decision. I am negative. But it is ignorant to imply it's only a death sentence. In essence, it is. But it becomes your life. And it makes you fight for those who can no longer fight for themselves. It's easy to say what you'd do in a situation you will never be in.

Jon Smith said...

Huntington's disease is an inherited (genetic) condition that affects the brain and nervous system.
huntingtons disease

Sarah Foster said...

I stumbled upon this while looking for HD blogs, and found it interesting.

In my case, the fathers of my progeny were BOTH aware that I was at risk for the disease and that there was a chance that it could be genetically transmitted.

Knowing that I have HD, I do feel guilty for having passed this risk on to my three sons. But, while I support abortion, I am so glad I did not abort these three wonderful human beings, even happier than I am that I was not aborted by my mother.

Whether or not they are HD positive, they are three important gifts to the world. The oldest is realizing his potential as a leader, and I'm forever grateful for the help he has received from his father's side of the family which made this possible. The middle is likely to find a cure for HD if he continues to feed his amazing scientific and mathematically voracious mind. And the youngest son will either be a history professor or a famous comedian, or maybe even both.

So yes, their lives are worth the guilt. I don't feel like I've introduced more impurity to the world, more genetic inferiority. These children are going to change the world for the better. And I look forward to grandchildren.

Hope you are well. SF