Residual Dried Blood and New Born Screening in Minnesota

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by Courtney Jarboe, Bioethics Program Student

In Minnesota, residual dried blood (RDB) samples collected for newborn screening had been stored, retained, and used for research without parental consent. It had been presumed that the Minnesota Department of Health (MDH) had the statutory authority to do so. In 2011, the Minnesota Supreme Court ruled (in the case of Bearder v. Minnesota) that the newborn screening program was subject to the Genetic Privacy Act (2006) (pdf), which requires written consent for secondary uses of genetic information. This ruling led to revisions of newborn screening legislation and the destruction of 1.1 million newborn screening RDB samples. Since the legislative dust has settled, MDH has begun aggressive educational campaigns to rebuild trust and awareness between parents and healthcare providers in Minnesota and address information gaps in the public’s understanding of the newborn screening program and the associated research.

In February of this year, I received a letter from the Citizen’s Council for Health Freedom (CCHF), an organization that actively supported the nine families in Bearder v. Minnesota who sued to stop the use of RDB samples for research. (see image) The letter details CCHF’s concerns about the MDH newborn screening program. The letter also suggests that MDH can use the RDB and the associated child’s DNA without parent consent. Included with the letter were a letter addressed to MDH and a copy of the MDH ‘Directive to Destroy’ form.

CCHF disclosed in late March that they distributed the letter to roughly 10,000 parents across the state based on birth certificate records. Within just a week of CCHF’s distribution of the letter, MDH received 59 directives to destroy RDB samples and related results. This was more than the total number of requests in the previous 5 months before these letters were sent out.

I was particularly interested in this letter because of my master’s project on Minnesota’s newborn screening program. Because this wasn’t my first exposure to the newborn screening program in Minnesota, I realized that I might be able to help clarify some of the aspects of this letter that other parents should be aware of. First, CCHF claims that, “Consent requirements mean ownership claims. But now, only if you object will the State release ownership claims to your baby’s DNA.”  However, CCHF is asking parents to complete “the official state opt-out form” and return the “I Did It” postcard. However, this is misleading as the form is actually the ‘Directive to Destroy Newborn Screening Samples and Test Results’ form. CCHF does not inform these parents that they could submit a request to MDH to return the remaining samples back to them. The ‘Directive to Destroy’ may not be the best choice for every family. What if that family should avoid destroying the sample due their medical history?

CCHF also claims “Without consent, the law allows research to be conducted on your child.” After Bearder v. Minnesota, this claim is simply not true. MDH stores and retains residual dried blood samples and test results from infants that participated in the newborn screening program as of August 1, 2014. These samples are used for quality assurance testing and the development of new tests for the screening panel, but the law does not allow research to be conducted on these samples. If they should wish to do research, researchers and MDH must obtain written informed consent from parents in order to use samples (Parental Consent for Research Use of Newborn Screening Blood Spots and Test Results).

Parents who have questions about their state’s newborn screening program practices should consult with their primary care provider or state’s newborn screening program office. If their provider cannot provide the answers, the state’s newborn screening program should have staff available to assist them. For more information about Minnesota’s newborn screening program, visit the state’s website. Information about other state programs, including contact information can be easily found on Baby’s First Test.

Jarboe Minnesota Letter (1) Jarboe Minnesota Letter page 2 (1)

[The contents of this blog are solely the responsibility of the author and do not represent the views of the Bioethics Program or Union Graduate College.]


Three’s a Crowd

by Theresa Spranger, Bioethics Program Alumna (MSBioethics 2012)

There is a new IVF (In Vitro Fertilization) procedure being developed in the United Kingdom.  The procedure aims to prevent diseases of the Mitochonidria.  These include certain types of Muscular Dystrophy that are genetically passed down from the mother.  This procedure would introduce DNA from a third parent by the transfer of the nucleolus from a donor egg.   99.8% of the genes of the resulting child would be from the biological mother and father.  Approximately 0.2% would come from the donor woman’s egg.  This would result in the child having 3 biological parents.

The whole idea is an interesting one and it’s a noble goal to want to protect these children from Mitochondrial disease.  However, I think it’s a “slippery slope.”  Yes, yes, there it is…the standard conservative argument for everything.  I hate making it as much as you probably hate reading it, but I think it applies in this case.

When you talk about genetically modifying humans (which is what we are talking about here), you open some very scary doors.  If a person’s genetics can be modified to correct a genetic disease at conception, could they be modified to ensure the child will be tall?  Or, to ensure she will have blue eyes?  The potential is “designer children,” or children whose genetic makeup has been specifically chosen.

I can already hear some of you out there: “That will never happen.”  “No one is going to pay that kind of money to pick out their child’s eye color.”  And so on, and so on.

For those who are skeptical of the idea, please allow me to give you an example of who this technique could be marketed to:

Kim Kardashian – a woman with more money than brains, whose maternal instinct didn’t stop her from naming her daughter North West.  Could you honestly tell me that someone like Ms. Kardashian wouldn’t want to create her “perfect” child?  Or, that no company would allow her to do this for the right price?

There is an epidemic in our society right now of “trophy babies.”  Some parents have babies and forget that they are tiny people.  That those children need to be nurtured and taught, and that one day they are going to need to be functional adults.  I think the ability to have “designer children,” would only make this trend worse.  It would allow those with enough money to choose qualities in their child like a little girl picks a doll from the American Girl store.  I don’t think this would be good for the child or society.

What if the “designer baby” trend then really took off?  What would society look like?  Naturally, there would be the haves and the have-nots.  People who could afford it would select for: intelligence, athleticism, beauty, etc., and the gap would widen between socioeconomic classes in our country.

As much as I would love to see a cure for mitochondrial diseases, any time we manipulate a person’s genes we are playing with fire.

[This blog entry was originally posted in a slightly edited form on Ms. Spranger’s blog on September 17, 2013. Its contents are solely the responsibility of the author alone and do not represent the views of the Bioethics Program or Union Graduate College.]

Of DNA and Databases

by Theresa Spranger, Bioethics Program Alumna (MSBioethics 2012)

You have the right to remain silent, but your DNA can and will be used against you…

On Monday. The United States Supreme Court decided to allow DNA to be taken at the time of arrest from those accused of “serious” crimes. The DNA sample is taken by a cheek swab and the information is then held in a database. The sample is run against others in the database, some of which are unknown samples that were collected at crime scenes. Occasionally, there is a match and the new DNA sample can help the police to solve one of their cold cases.

The case that went to the Supreme Court is that of Alonzo King. Mr. King was arrested on an assault charge and his DNA swabbed at the time of arrest. The DNA matched that of an unsolved rape case and King was charged with this rape. The problem arose when King pled guilty to a lesser crime than the assault. Under current Maryland law, the police would not have been allowed to take his DNA for the crime for which he pled guilty. King’s attorneys argued that because he was convicted of a lesser crime the DNA evidence should not be permissible in the rape case.

In a 5/4 decision that rocked party lines, King’s rape conviction was upheld. Kennedy, the notorious swing vote on the court, wrote the majority opinion. This opinion was that collecting DNA was like fingerprinting upon arrest and not a violation of the person’s rights. He was joined by Justices: Roberts, Thomas, Alito, and Breyer.

The dissenting justices were Ginsberg, Kagan, Sotomayor, and Scalia. Scalia wrote the dissent and argued that the ruling was too vague, the precedent the court was setting was dangerous, and collection of DNA has high potential for future misuse.

I agree with the dissent and believe the court made a mistake with this decision. It’s not that I would like Mr. King roaming the streets to rape again, but I think some of the justices neglected to look at the bigger picture. My main issue is with the saving of information, DNA information is not like having your fingerprints on file. Your DNA is a map of you and we have no idea how this information could be used in the future. We learn more and more about DNA and genetic makeup every day and the more we learn the more cautious I become about sharing my genetic information.

What about the DNA sample itself? Is this retained along with the database of information? It would be one thing to have a database, but another entirely to have the physical sample. Scientists can do amazing things and all indications point to more incredible discoveries in the world of genetics. This should give us pause when discussing the creation of a central database for anyone.

A further issue with this ruling is its vagueness. The court states that DNA gathering is permissible in cases of “serious” crimes. What does this mean exactly? What must someone be accused of to lose their right to control the use of their DNA? Drunk driving, shoplifting, protesting? Or is it truly for violent criminals? Murderers, rapists, etc.?

Taking the sample upon arrest however, flies in the face of our nation’s presumption of innocence. Keep in mind that not all of those arrested are criminals. Who knows, perhaps one day you will be in the “wrong place at the wrong time” and your genetic information will be on permanent file without your consent.

Making decisions based on what we know about DNA today is never a good idea, genetics and manipulation of genes is an ever changing field and we need to be making decisions into the future. I certainly want to give the police every advantage when catching dangerous criminals, but not at the risk of my or other innocent American’s personal privacy or freedom.

[This blog entry was originally posted in a slightly edited form on Ms. Spranger’s blog on June 6, 2013. Its contents are solely the responsibility of the author alone and do not represent the views of the Bioethics Program or Union Graduate College.]

Kudos to This American Life

by Michelle Meyer, Bioethics Program Faculty

A few weeks ago, I blogged about a recent episode of This American Life (TAL), “Dr. Gilmer and Mr. Hyde,” about the quest of one Dr. Gilmer (Benjamin) to understand why another, beloved Dr. Gilmer (Vince), had brutally murdered his own father after hearing voices that compelled him to do so. The episode ends (spoiler alert) with the revelation that Vince suffers from Huntington’s, a rare, neurodegenerative disease that causes progressive physicial, cognitive, and psychological deterioration.

Listeners, it seemed to me, could naturally conclude from the episode that it was Vince’s Huntington’s that had caused him to murder his father. That might or might not be true in this particular case. Huntington’s can cause behavioral and mood changes, including irritability, aggression and belligerence. It can also cause (less often) psychosis. But even if Huntington’s caused Vince to murder his father, or somehow contributed to the murder, the extreme violence that Vince displayed — strangling his father, then sawing off his father’s fingertips to preclude identification — is in no way typical of the Huntington’s population as a whole. And so what most troubled me most about the episode was its failure to note just how rare this kind of extreme violence is among those with Huntington’s, just as it is very rare among human beings generally. And so I wrote to TAL, requesting a clarification.

I’m happy to report that the TAL producer for the episode, Sarah Koenig — who had not intended to suggest any causal link between Vince’s murder of his father and his Huntington’s, much less between murder and Huntginton’s more generally — has issued a clarification on the show’s blog, and promises to make a similar clarification in the episode itself, should they ever re-air it. Kudos to TAL, and many thanks to Sarah for being incredibly gracious in our exchanges.

One clarification deserves another. In my earlier blog post, I also worried that some listeners might  conclude that Vince’s father was similarly driven to commit horrific acts of sexual abuse on Vince and his sister because he, too, was (presumably) suffering from Huntington’s (an autosomal dominant genetic disease). Although I think that a listener who didn’t know better could reasonably conclude that Huntington’s causes people to become sexual predators almost as easily as they could conclude from the episode that Huntington’s causes people to become murderers, nothing in the episode suggests that Sarah, Benjamin Gilmer, or anyone else at TAL believe that Huntington’s causes sexual abuse, or that they intended for listeners to reach that conclusion. I regret anything in my earlier post that suggested otherwise.

Again, I’m very grateful to Sarah and everyone else at TAL for hearing me (and other listeners) out and for agreeing to make the clarification — and just in time for HD Awareness Month!

[The contents of this blog are solely the responsibility of the author alone and do not represent the views of the Bioethics Program or Union Graduate College.]

Lara Croft: Cancer Activist

by Sean Philpott, Acting Director of the Center for Bioethics and Clinical Leadership

In an Op-Ed piece published in Tuesday’s New York Times, actress Angelina Jolie revealed publicly that she had undergone a prophylactic double mastectomy — removal of both breasts — in order to reduce her risk of developing cancer.

Ms. Jolie had a reason to be concerned. Genetic tests showed that she carried a mutation in a gene known as BRCA1, a change in her DNA that greatly increased the likelihood that she would develop breast or ovarian cancer sometime during her life. Cancer-causing mutations in the BRCA1 gene (or a related gene known as BRCA2) are rare, but account for a majority of familial cases of breast and ovarian cancer seen in the US.

Ms. Jolie likely inherited this mutation from her mother, who died of cancer at 56. Based on her test results, doctors estimated her lifetime risk of developing cancer at approximately 87%, probably at an early age. By contrast, the average woman in the US has a lifetime risk of 12%, with diagnosis usually coming later in life.

The decision to remove both breasts could not have been an easy one, particularly for a starlet who is famous for playing buxom femme fatales in movies like Lara Croft: Tomb Raider, Mr. & Mrs. Jones, and Salt. Ms. Jolie admits as much in her Times article. A prophylactic mastectomy doesn’t completely eliminate her risk of breast cancer, only reduces it by about 10-fold.

She is also at increased risk of developing ovarian cancer, but elected not to have her ovaries removed. A prophylactic oophorectomy, as that procedure is known, is an invasive procedure with long-lasting physiological effects, including early menopause, cardiovascular disease, osteoporosis, and loss of sexual function.

With recent advances in reconstructive surgery, there was no need for Ms. Jolie to go public. She wouldn’t have been the only Hollywood star to get breast implants, just one of the few that had a medical reason for doing so. Barring release of her medical records, a serious breech of privacy, no one would have been the wiser.

So why speak out? According to the actress, she wrote about her experience so that other women could benefit. Specifically, so women with a familial history of cancer could get tested for mutations in the BRCA1 and BRCA2 genes and, if necessary, to “take action.”

Having a spokeswoman like Angelina Jolie increase public awareness of breast cancer is good. It is a laudable goal, but it also one that worries me. Women who look to Angelina as a role model might rush to be tested for cancer-causing genes. However, the results of genetic testing have profound consequences — physically, psychologically and for future insurance coverage. In addition, the tests in question are very expensive. A single test costs approximately $3,000, and may not be covered by existing health insurance plans. Many women simply cannot afford to do what Angelina did.

These exorbitant testing costs are due to the fact that a Utah-based company called Myriad Genetics has patented both the BRCA1 and BRCA2 genes. Myriad currently holds a monopoly on testing for breast and ovarian cancer-causing mutations. The legality of this monopoly had been questioned, most notably in a US Supreme Court case challenging a private company’s right to patent human genes. But until the Court’s ruling in October, the company has every legal right to charge what it believes the market will bear.

Given this, only women with a clear familial history breast or ovarian cancer should be tested. But figuring who has such a history is not an easy task. As many as one in eight women in the US will develop breast cancer at some point in their lives, making it likely that most people will have a sister, mother, aunt or grandmother with a diagnosis. People can have as many as two, three or even four female relatives with cancer. But most of these cases will not be associated with mutations in BRCA genes. It takes a trained genetic counselor or skilled physician, using a detailed family tree, to know for sure whether or not a woman is a potential carrier of a mutant gene.

Moreover, for those unlucky few who do carry a mutant copy of BRCA1 or BRCA2, a prophylactic mastectomy or oophorectomy may not be the answer. Ms. Jolie made a carefully considered and informed decision, in consultation with a highly trained team of doctors, to undergo this radical procedure. But there are other less effective but less expensive and less invasive options, including tamoxifen or regular monitoring, that may be the better choice for many woman (particularly those that lack the savvy and resources of Angelina). I’d hate to think that they rushed to have their breasts removed simply because their favorite starlet had done the same.

None of these concerns I voice is meant to take away from what Angelina has done. Speaking publicly about her decision is a courageous thing to do. But the take-home message for women is far more nuanced than get tested and get treated.

[This blog entry was originally presented as an oral commentary on Northeast Public Radio on May 16, 2013. It is also available on the WAMC website. Its contents are solely the responsibility of the author alone and do not represent the views of the Bioethics Program or Union Graduate College.]

Portrait of a Litterer

by Theresa Spranger, Bioethics Program Alumna (MSBioethics 2012)

Heather Dewey-Hagborg is an American information-artist who, for her latest project, roams the streets of New York looking for cigarette butts, chewed gum, or strands of hair.  She collects the samples, extracts DNA, and has the DNA analyzed in a lab for certain genetic characteristics (race, gender, eye color, etc.).  From these results she uses a computer program and 3D printer to create a 3-demensional image of the person’s face.  Ms. Dewey-Hagborg says that her sculpture is more of a loose representation of the person rather than an exact portrait.  The capability to create an exact portrait may be available in the future, but at this time the analysis needed for that is too sophisticated for our technology.

The idea came to Ms. Dewey-Hagborg from a single strand of hair she found stuck in a cracked piece of glass.  She wondered whose hair it was and what that person looked like.  Ms. Dewey-Hagborg has spent the past year designing the computer program and creating her 3D portraits.  She calls her project “Stranger Visions.”

This project sparks many interesting ethical thoughts and issues.  We shed DNA everywhere we go, every time we scratch an itch, sweep our hand through our hair, lose an eyelash, etc.  The “Stranger Visions” project brings up some interesting thoughts on the use of discarded genetic material.  For instance, how could this technology be used by: a government, scientist, or police force?

Could the police force use this technology in their investigations?  Perhaps it could be used instead of or in conjunction with sketches from witness descriptions?

Scientists could use the information for statistical analysis and profiling.  An oversimplified example of this could be the title of this post, “Portrait of a Litterer.”  Litter could be collected as this artist has done, but then analyzed to profile and answer some questions about those who litter.  Who is more likely to litter: men or women?  Are litterers more likely to have blue eyes?  And so on…

Certainly, the usefulness of the littering example is questionable, but the idea could certainly be transferred to other areas, the possibilities are endless.  The question however: is this type of use ethical?  Do we want statisticians to be able to collect genetic information for profiling?  What rights do you have to samples of your DNA if they are no longer attached to your person?  A host of questions spring to mind, questions with few good answers.  It seems evident to me, that our discussion in society is falling behind technology in the area of genetics.

In my opinion, we need to quickly catch up in our discussion of genetics.  As technologies become more and more sophisticated, the use of our personal genes could become a forefront issue for our society.  We cannot help leaving traces of ourselves behind us for anyone to gather and do with as they please.  So, how do we prevent our genetic information from being used for something against our will?  Is there a way to adequately legislate on this issue?  Should we legislate?  Do we own the hair, skin, saliva, etc. that contains our genetic information?  The questions and strange possibilities this project presents to us are truly endless.

While I believe this project is fascinating and harmless in itself, it should spur us to deeper thought and conversation on this issue of genetic material: ownership, and use.  It should also encourage you to find a trash bin for your gum and/or cigarette butts lest you find your face on an art gallery wall…

Want to know more?   Here are links to some interesting articles…

[This blog entry was originally posted in a slightly edited form on Ms. Spranger’s blog on May 9, 2013. Its contents are solely the responsibility of the author alone and do not represent the views of the Bioethics Program or Union Graduate College.]

This American Life and Stigma

by Michelle Meyer, Bioethics Program Faculty

UPDATE: This American Life has made a clarification. Please see Prof. Meyer’s latest blog post for more.

Let me begin by saying how much I absolutely adore This American Life (TAL). I listen to it religiously. I particularly had been looking forward to the most recent pocast episode of TAL: Dr. Gilmer and Mr. Hyde. As the episode’s blurb teases, “Dr. Gilmer and Mr. Hyde” concerns a doctor — Benjamin Gilmer — who takes over the rural South Carolina practice of Vince Gilmer (no relation). Vince is no longer available to see patients because he is serving a prison sentence for killing his father. As Benjamin gets to know Vince’s — and now his — patients, he forms a picture of Vince that’s at odds with his status as a convicted murderer. How could this doctor who was so devoted to his patients have so brutally murdered his own father?

This episode is right up my alley. True crime? Check. Forensic psychology? Check. The intersection of law and medicine? Yes, please. So when I awoke yesterday morning at 5 am and couldn’t go back to sleep, I eagerly cued up the podcast. The episode recounts, in TAL’s  typically-riveting fashion, the story of Benjamin’s search for the truth behind Vince’s murder of his father. I enjoyed every minute of the episode until the last five minutes or so, when I became troubled by one critical omission.

Spoilers  follow after the jump; listen to the episode first.

Hopefully, you’ve listened to the episode. You should; it’s good. But if not, here’s the gist, based on my memory of listening to the episode casually in the wee hours of the morning (read: some details may well be off).

The day in 2004 that he killed his 60-year-old father, Dalton, 41-year-old Vince picked him up at a psychiatric hospital to transport him to a nursing home. It’s not clear what his father suffered from, but whatever it was, he was dependent on a walker or wheelchair, required help with basic daily functions like dressing, and was on antipsychotic medications. Vince would later say that before dropping him off at the nursing home, he had promised his father that they would go kayaking at a favorite lake several hours away, and he had brought a kayak in the truck for that purpose. But his father had sexually abused his sister and him from the ages of 6 and 3, respectively. (The TAL reporter says that it’s the most horrific case of abuse she’s ever heard. Vince’s sister would later confirm the abuse, although she disappeared before the trial and hasn’t been seen or heard from since.) And somewhere en route to the lake, his father had made an inappropriate sexual comment or grope, and a compelling voice in his head told him to kill his father. He used a piece of rope to strangle him, tugging harder and harder until the man stopped struggling. He then moved the body to the bed of the pick up and drove around for hours trying to decide what to do with the body. Eventually, he took it to Virginia, where he used a small saw to to amputate the tips of all ten of his father’s fingers before dumping the body, to prevent it being identified. He then drove home. The next day, he went to work, where he told everyone that he had taken his father home, but that Dalton had wandered off and he had filed a police report. He acted perfectly normally the rest of that week, seeing patients and socializing with colleagues.

Until the police called on him. The body had been quickly discovered (still warm, in fact). And it had been quickly identified (Dalton’s shirt was stamped with a name tag, “D. Gilmer,” that Vince himself had arranged for). And police, not surprisingly, quickly decided that Vince was their guy. Vince told police that he had brought his father home to live with him, and that his father had gone outside to play frisbee with the dog before wandering off, something police knew couldn’t be true. Police say Vince wasn’t scared or agitated. Instead, he threatened to have the detective’s badge. Police also learned that he had a one-way ticket to Alaska scheduled for around the time of the killing. And he had stopped paying his father’s bills at the psychiatric hospital, and owed over $200,000. Before police could get an arrest warrant, Vince fled.

When police caught up to him, he finally confessed. Despite mounting a defense that hinged on severe cognitive disability, he insisted on representing himself at trial, where his behavior, according to court transcripts and observers, was a model of disordered thinking (his court-appointed attorney described it as akin to “watching someone try to commit suicide with a butter knife”). His defense centered on serotonin depletion. He had been on an SSRI to increase his serotonin, after struggling with anxiety for years, but had abruptly stopped taking it a day or two before the murder, rather than tapering it off as he surely knew he was supposed to do. As some do, he reacted very badly, he says, becoming extremely irritable, hearing compelling voices, and generally feeling “mentally retarded.” In the courtroom and, at least occasionally, in prison as he awaited trial, Vince displayed odd grimaces and shaking. He repeatedly asked prison officials for 80 mg of Celexa, uncrushed.

But when prison officials videotaped him in the prison courtyard, they noticed that his twitches and shaking seemed to conveniently come when prosecutors were in sight and go when they were not. And the rest of Vince’s story was fishy, too. He claimed that the killing wasn’t premeditated, but the plan to go kayaking made little sense given Vince’s father’s immobility, the fact that he had not brought a walker with him in the truck, and the fact that it was getting dark. And Vince had come to pick up his father prepared with both the rope and the saw (sometimes referred to in the episode as pruning shears). The jury took an hour to conclude that he had been faking his symptoms and found him guilty of 1st degree murder. The judge sentenced him to life in prison without the possibility of parole.

Benjamin can’t accept the idea that the kindly family doctor he’s come to know through their shared patients suddenly turned into a premeditate murderer of his own father. He begins to investigate, and variously pursues both Vince’s own hypothesis, serotonin depletion, as well as personality change due to a traumatic brain injury Vince suffered about a year before the murder. But in the end, Benjamin, on a hunch, wonders if Vince — and, for that matter his father — might have been suffering from a rare genetic disease, Huntington’s. Once called Huntington’s Chorea (for the dance-like movements most sufferers exhibit), Huntington’s Disease (HD) is perhaps best known as having killed Woody Guthrie. As the TAL reporter says, HD is “a horrible condition, one of the worst, like a cruel trifecta of Alzheimer’s, Parkinson’s, and Lou Gehrig’s Disease rolled into one.” Its progressively severe physical, emotional and cognitive symptoms invariably lead to death, generally within about 20 years. HD is caused by an excessively long trinucleotide repeat (CAG, in particular) on the huntingin gene, on the short arm of chromosome 4. Genes code for proteins, and excessively long strings of trinucleotides code for excessive amount of protein, where it wreaks havok in the brain. Anyone who has 40 or more CAG repeats will get HD within a normal lifespan, and each of their children has a 50% chance of inheriting the allele (which may or may not either contracted or expanded below or above the parent’s CAG repeat length).

By this point in his investigation, Vince has been moved to a psychiatric ward within the Virginia prison system after he threatened to commit suicide, and Benjamin and the TAL reporter suggest that give him Vince the genetic test for HD, which they do via a simple blood draw, apparently after a psychiatrist counsels him. It comes back positive: one copy of Vince’s huntingtin gene has 43 CAG repeats. (One oddity is that it appears that the diagnosis was emailed to Benjamin, who then, some weeks, later, broke the news to Vince by phone, portions of which were recorded and included in the TAL episode. HIPAA, anyone? And of course, as TAL notes, Vince’s diagnosis suddenly means that his (missing) sister is at 50% risk of having HD, and her kids are at 25% risk. One wonders what they think of that information being public.)

In terms of the mystery of why Vince killed his father, the episode pretty much ends there. HD can lead, among other things, to serotonin depletion, so it’s possible that, in a way, Vince’s theory of his own crime was right all along. Indeed, following the diagnosis, his psychiatrist has prescribed the exact 80 mg of Celexa that he had been requesting for 9 years, and he reports feeling less anxious. The final few minutes of the episode turns to the injustices done to Vince, and how they might be redressed. Benjamin and the TAL producer rightly note that at the very least Vince, who exhibited symptoms of HD at the time of the killing, should never have been allowed to represent himself. As for releasing him from prison, University of Virginia School of Law’s Richard Bonnie suggested that instead of making that argument in court, they pursue compassionate release.

I’ll leave others to comment on the criminal law aspects of the case. I want to talk about something else. The implication of the episode (and its unfortunate title) seems to be that Vince Gilmer killed his father because he has HD. TAL speculates, not unreasonably, that Vince likely inherited HD from his father. And so the epsiode also implies that Dalton Gilmer may have brutally abused his children because he, too, had HD. Either or both of those things may well be true. But although the occasional incidents involving people with HD who kill themselves or others make for splashy news and riveting human interest stories, the fact is that the vast majority of people with HD are not dangerous to themselves or others. Although psychosis is one of many possible manifestations of HD, most don’t experience that particular symptom and, again, of those who do, very, very few end up harming themselves or others.

People with HD are instead much more likely to be the victims of violence. They were burned at the stake as witches in Salem and sent to the gas chambers during the Holocaust, for instance. Less dramatically, they are routinely turned away from public accommodations or arrested because their chorea is mistaken for drunkenness. Many who are at risk for HD choose not to be tested, not only because they don’t want to know, but also, in many cases, because they fear the consequences of an HD diagnosis for their employment and insurance status (GINA notwithstanding), on top of the risk that others will respond to them with irrational fears and prejudices.

Thanks to highly popular portrayals of HD like that in “Dr. Gilmer and Mr. Hyde,” the public has associations with HD that the already-horrible reality of the disease doesn’t bear out as typical, and they will likely project those associations on those with or at-risk for the disease. As TAL’s explanation of the basics of HD during the episode suggests, many, perhaps most, TAL listeners, educated though they are, likely know little or nothing about HD. The episode offers a needlessly misleading introduction to the disease that has the potential to harm those already suffering from it.

Although, as noted above, I have some qualms about how Vince was tested and about the privacy implications for his relatives of so widely broadcasting his diagnosis, I don’t object to telling the story of a person with HD who killed his father — not even to the telling of the story of a person who filled his father because he has HD. In its own way, Vince Gilmer’s story is an illustration of how we can and should do much better in diagnosing and managing HD and its sequelae (along with similar illnesses). The episode tells the story of a doctor falsely painted as evil, when really he was just sick.

But in the course of exonerating Vince Gilmer of culpable murder, TAL paints everyone with, or at risk of, HD as extremely dangerous. Responsible science communication requires that Vince’s story, as important as it is, be much better contextualized to reflect how unusual it is. I have written to TAL to ask that they consider adding a brief comment to that effect to the podcast version of the episode, to any rebroadcasts of it, and to its blog. I’ll update this post if I hear back from them.

[This blog entry is adapted from one originally posted on the blogs Bill of Health and The Faculty Lounge on April 18, 2013. Its contents are solely the responsibility of the author alone and do not represent the views of the Bioethics Program or Union Graduate College.]