Huntington's and Genetic Testing : The Frontal Cortex

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Jonah Lehrer is a contributing editor at Wired. He's also written for The New Yorker, Seed, Nature, the Boston Globe and is a contributor to Radio Lab. He's the author of Proust Was A Neuroscientist. His new book is How We Decide.

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Huntington's and Genetic Testing

Category: Culture
Posted on: March 18, 2007 4:45 PM, by Jonah Lehrer

This is an unbelievably poignant story about what it's like to learn that your nervous system is fated to self-destruct. Katherine Moser, a 25 year old occupational therapist, decided to take a genetic test that would tell her whether she carried the gene for Huntington's disease:

The test, the counselor said, had come back positive.

Katharine Moser inhaled sharply. She thought she was as ready as anyone could be to face her genetic destiny. She had attended a genetic counseling session and visited a psychiatrist, as required by the clinic. She had undergone the recommended neurological exam. And yet, she realized in that moment, she had never expected to hear those words.

"What do I do now?" Ms. Moser asked.

"What do you want to do?" the counselor replied.

"Cry," she said quietly.

Huntington's is biological determinism in its purest, cruelest form. One of the interesting subplots of the article is that young people at risk for Huntington's seem to be more willing to undergo genetic testing than previous generations. (Katherine's mother, for instance, has refused to get tested. The test has been around since 1993.) I wonder why that is. And I also wonder if I would have the courage to get the test. Knowledge can be such an awful thing.

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Comments (11)

So, what do you think it was like before 1993? They knew Huntington's was inherited, but had no way to know who would or wouldn't get it.

Woody Guthrie had Huntington's. I wonder if not knowing had some effect on Arlo's devil-may-care youth.

Posted by: chezjake | March 18, 2007 8:20 PM

While Huntington's is an extreme case, due to its gravity, what about all the other diseases that can be detected through genetic testing? I have a neurological condition and just had a discussion the other day with my neurologist about whether I should have genetic testing done to know whether I have the familial form of the condition. I have a 16-year old son; if I did the testing, and knew there was a chance he'd have the same thing, both he and I would be living with this near-certainty. The doctor and I agreed that it's better that only I worry, and not him, unless he shows symptoms.

This is a question that arises often, and something that is impossible to answer. Each person has to make their own decision; or not.

Posted by: Kirk | March 19, 2007 4:24 AM

We who are married to reporters at the New York Times wish to announce that Frontal Cortex has just rehabilitated reporter Amy Harmon. Ms. Harmon, who got slammed for an earlier genetics report (I'd do one of those "this" things, showing you just when, where and why, but I don't have the equipment) is the author of the Huntington's story being fullsomely praised this week. So because it was indeed a good story, and she's finally got her Frontal Cortex laurels, we Amy Admirers are planning a little Rehabilitation Night at a local watering hole.
It's nice to know that like Daryl Strawberry, we can all
fight our way back to glory.

Posted by: Robert Krulwich | March 19, 2007 2:29 PM

I am a relative of Katherine Moser. This is the letter I sent to Cardinal Cooke and Columbia U. Amy Harmon has just succeeded in doing a lot of damage of Ms. Moser's family who had requested that she not use any names. My understanding is that Ms. Harmon said no real names would be used in the article.

Quote

I am thoroughly disgusted by this article by Amy Harmon, surely an irresponsible journalist who, by putting Ms. Moser's name in print, along with the name of a four year old child involved in a custody dispute, has exposed Ms. Moser's family to that which they did not want to know or to be known by others.

The staff at the Huntington's Disease Center at Columbia should be ashamed of themselves for testing Katherine Moser without first ascertaining her mother's status. I hope that they are now appalled to learn that Katherine chose to announce her test results from the witness stand, an announcement that seems to have been a cold, calculated move on the part of Ms. Moser to derail her mother's custody of the child. Try to imagine her mother's shock at learning, in such a public way, that she, herself, is a victim of the dreaded Huntington's disease.

I question Katherine's motives for being tested. Did she really want to know that she has the disease, or was it her motive to expose her mother, in that twisted way of thinking that Huntington's sufferers exhibit; paranoia and extreme emotion?

Further, not only has Mrs. Moser's privacy been violated but so has the privacy of Mr. James Dowd and the other unfortunate patients who were photographed at the Terence Cardinal Cooke Health Care Center. Did Mr. Dowd and the others even know they were to be featured in this article? Where are their privacy rights and where are the officials in charge of protecting them?

unquote

Posted by: Cecilia | March 20, 2007 11:24 AM

If glory is found by destroying a family and violating their right to privacy then I would say Amy is the woman to achieve it. Despite talks with this reporter about not using our family for the story on Huntington's and receiving assurances that we would not be used in the story, there was every marker pointing back to us. Where is the glory when you have "outed" someone who has tried to keep this disease a secret? Who will speak out now for the victims of this story? I am sure that when you toast your good friend you will never feel any sorrow for the pain that this woman has inflicted on innocent people but then again you are probably just as uncaring as Amy.

Posted by: denise ford | March 20, 2007 5:29 PM

To the unhappy members of Mrs. Moser's family assembled here: does Mrs. Moser not have the right to publicly confront her condition? Should she be expected to remain quiet because you wish her to do so? It seems to me that she gains great authority as advocate because of her decision to confront her fate -- though I do also understand and sympathize that you might prefer not to know yours.

Posted by: Scott Matthews | March 22, 2007 5:53 PM

Cecilia, I believe it is well-documented that Katharine attempted to tell her mother privately that she had tested positive, but Katharine's mother was not willing to speak with her (as is also stated in the article). Given that you state that it was a matter of custody of a young child, and given that Ms. Moser alleges that she herself was mistreated by that selfsame petitioner who we now know is gene positive for Huntington's Disease, the information that Ms. Moser shared in her testimony was certainly pertinent to the case. If Katharine's mother did not want to know her gene status, perhaps she should not have initiated a court battle where her ability to parent a child, and therefore her overall mental and physical health, could be called into question.

Ms. Ford and Cecilia-- According to what I have heard, many donations have been made in honor of Katharine's bravery, publicized by Ms. Harmon's article. There has been an explosion of public awareness and concern. I hope that when, in a few years, there is a cure for HD partially because of the awareness borne from Ms. Harmon and Ms. Moser's efforts, you will both be humble enough to ask for their forgiveness for maligning them as scheming and opportunistic women.

As for the "unfortunate patients", I believe that all of their families were contacted for permission to use their photographs. I'm sure their families' judgments were the best indicator of whether to use the photos, and not your privately held beliefs that Huntington's Disease is a disease of shame and secrecy.

What a blessing Ms. Harmon and Ms. Moser have been to the Huntington's Community. The fruits of their efforts are already being seen in very real and very positive ways, but I think that in a few years, we will see the true difference they have made when they can truly say that they were part of the solution, the cure-- which is coming.

Posted by: Colleen | March 22, 2007 7:31 PM

Ms. Moser did not have to use her last name for the article. She has done untold harm.

Posted by: Cecilia | March 22, 2007 8:14 PM

Colleen As a friend of Katie's, you are well aware that she received the results of her genetic test weeks before she made any attempt to tell her mother at court. As a medical professional, she is also aware of HIPAA law and she chose not to follow it. Katie had every right to make her choice to be tested and if she wanted to tell her story more power to her but she should never had used her name or revealed her mother's status. Amy had no right to include information that would point back to the family members that did not want to be included in this article. They choose to keep their privacy and that is their right. Colleen, do not preach about attitudes on Huntington's, you are not at risk for it and can not truly understand what it is to be in a family with this dreaded disease. Also, when Katie revealed the result of her test in court, she was there petioning for custody of this child as well. I feel bad for Katie since she seems to be basking in all this attention and one day it is going to hit her what her future will be. I have seen it and I shall keep her in my prayers.

Posted by: denise | March 22, 2007 8:40 PM

I would like to take this opportunity to quote from www.patientprivacyrights.org

{Many people are unwilling to take genetic tests, knowing that afterwards they might not be able to get a job or health insurance. The woman in this story was warned about these consequences but still chose to be tested for Huntington�s Chorea. First, Congress should require informed consent before genetic test results are disclosed to physicians who are actually treating the affected individuals. Second, genetic test results should never be disclosed to insurers, employers, or the other 600,000 health-related businesses and government agencies that are currently granted access to Americans� complete personal health information by HIPAA, even with consent. Genetic records in particular should never be disclosed to anyone other than physicians, because the results can be used to discriminate against the children, families, and other relatives of those who take genetic tests.

No one should be able to give consent to disclose or use genetic or other sensitive personal health information that can harm someone else�s life or opportunities in life. ~

Dr. Deborah Peel, Patient Privacy Rights

Posted by: Cecilia | March 22, 2007 9:45 PM

As always with HD nothing is simple, nothing easy. I commend Ms Moser for her courage to approach HD head on. Only those of us who face life with HD is really like, are those have a right to make these choices. Only we know what this reality is like daily. To all those who stand on the sidelines and say she should do this...she should never do that..your hypocrisy is beyond your own comprehension. MS Moser is facing HD on her own terms, in her own way, as best as she possibly can. AND THAT is heroic. So many will say oh well Im a doctor, a nurse a relative etc... I have seen HD so I know...NO you dont. Its not YOU and it will never be you inside that body, that mind.

In so many ways the test seems to change everything and yet changed nothing without a treatment or a cure.

While the war cry over privacy is important it also hearkens the desire to keep it all secret and push HD back in the dark shadows. I have watched time and a again " the family secret" hidden behind the cry for "privacy" Instead of screaming about privacy/secrecy we should be charging at HD head on. Busting stigmas, breaking down facades, pushing for acceptance, educating everyone we can that HD is a fierce cruel disease whos sufferers deserve a treatment, and a cure NOW...not a year from now, not ten years. Not when its convenient for some congressmen or some doctor to notice it and attach there name to it.

How can we ever demand treatment, demand a cure if we continue to hide something we did not deserve and can not control. A secret speaks of shame and there is no shame.

Posted by: doug | December 20, 2007 2:13 AM