We almost always have an opinion, after we meet and examine someone, as to whether or not they have the HD gene. But it turns out we don’t guess well. (Otherwise we wouldn’t need the test.) A former colleague used to say that “it’s in the eyes,” meaning her guesses as to whether people would test positive were based on what she saw in people’s eyes. But she didn’t guess more accurately than the rest of us. Parenthetically, there may be something “in the eyes.” In addition to chorea affecting eye movements, many people with HD have trouble taking their eyes off a visual target. The fancy, doctor way of saying that is that it’s difficult for them to break fixation. When they want to turn their eyes to one side, often their head turns first, and the eyes follow a moment later. But back to our guessing wrong.
One time we guessed wrong was for a man I’ll call John. He was a bright, healthy, 25-year-old man at risk for HD. His neurological and psychiatric examinations were completely normal. He was a thoughtful, gentle man, recently married, who was doing well at a demanding career. He was an Air Force officer who had recently been deployed to a USAF base in Wyoming. He had started the presymptomatic testing process while he was stationed at Scott AFB outside St. Louis, and was back for his results.
Most patients keep pretty quiet in those few minutes before hearing the test result, but John was a little more talkative. I think he was guessing his test would be normal. After all, he was in top physical condition, and a neurologist and a neuropsychiatrist had gone over him with a fine-tooth comb and found nothing abnormal. We had been guessing his test would be normal, too, until we got the results back.
“I like this new posting,” he was telling us while we were getting seated, “even though it’s a pretty heavy responsibility.”
“In what way?” my colleague Stacey asked.
“Well, having your finger on the nuclear button ... there’s not really a button, but you know what I mean ... it gives you pause. Obviously I’m not the final decision maker, but I’m one of the people who might be called upon to launch missiles in a crisis.”
I said, “hm,” or something equally brilliant, took a deep breath and tried to think quickly. “Hang on just a second, I need to get another piece of information. Stacey, could you give me a hand?” She furrowed her brow but left the room with me. I walked around the corner to an empty office and closed the door. “OK, Stacey, I’m pretty much freaked out. I don’t know why it never occurred to me before, but I wonder if maybe it’s a bad idea to tell everybody up front that we are going to respect their confidentiality 100%. I mean, obviously this guy could cause some extraordinarily serious problems if his judgment ever turns sideways, and that could happen relatively early in the illness with personality change, or even, less likely, a delusion.”
Without knowing for sure what to do, we returned to the room and I asked him whether his job required him to complete regular medical examinations. He said yes, and also described fequent required tests of reaction time and psychological functioning. That information, combined with his completely healthy status at the time, made the decision easier: I proceded and gave him the unhappy information about his test. He of course was not happy with the results, but spontaneously mentioned that he felt he was required to pass that information to his commanding officer, and would do so.
Now the above situation worked out about as well as one could hope. But it is easy to imagine a similar situation in which patients’ reasonable demand for privacy may be outweighed by other needs. Situations can easily be imagined in which a positive test for HD, even in an asymptomatic person, may have grave implications. Such situations have come up before in other contexts, of course; for instance, prominent politicians, train engineers, airline pilots, and military officers have been affected by serious illness, placing an ethical strain on their diagnosing or treating physicians. Here are a couple of examples.
... this chapter not yet finished ...
Thomas Eagleton
[references to information about the Germanwings tragedy] (Levin 2015; Miller 2016)