Canadian Medical Association Journal 1996; 154: 1827-1834
In her essay "Medical ethics and truth telling in the case of androgen insensitivity syndrome" [full text / En bref](CMAJ 1996; 154: 568-570), Anita Natarajan argues that women who bear this syndrome should not be told of their condition. Her argument, crafted from a scholarly, philosophical base, is seriously flawed in human terms.
As a social worker with more than 35 years' experience and a woman who has borne androgen insensitivity syndrome (AIS) for 63 years, I find this essay deeply disturbing.
I know from my social work experience that secrecy as a method of handling troubling information is primitive, degrading and often ineffective. Even when a secret is kept, its existence carries an aura of unease that most people can sense.
As well, this essay confirms for me the inappropriate expectations of physicians in our society. People have traditionally expected physicians to have limitless wisdom, and physicians have been ready to accept this cloak of magical expectations. This has caused as much harm to physicians as it has to their patients. Happily, today many physicians recognize the spuriousness of such expectations, and they expect their patients, with the assistance of other skilled professionals, to face the difficult questions of their medical conditions.
Many of us know of our condition and are prepared to talk about our experiences. Medical practitioners wishing to cast off the barrier of secrecy can draw on our experience and strength. Physicians and their patients with AIS can deal with this anomaly in a healthy, constructive way.
I knew there was a secret about me. Not knowing what it was, I lived under an undefined shadow of fear that diminished enjoyment of every part of my life, hobbled my enthusiasm and, ultimately, affected my success in handling life's challenges. I had innumerable hours of psychiatric treatment, which were essentially unsuccessful because my psychiatrist shared the traditional view that patients should be protected from knowledge of AIS.
Decades later, when I first heard the words "testicular feminization," I found excellent help in dealing with reality from social-work and psychological counselling and therapy. Having recently learned the more respectful term "androgen insensitivity syndrome" and discovered an international self-support group, a whole world of self-acceptance has opened up for me. My big regret is that I did not receive this knowledge and help as a youngster. I had the psychological strength to deal with my sex ambiguity, had I been given the chance. I did not have this chance. Secrets crippled my life -- secrets like those Natarajan advocates.
Natarajan's misguidedness is troubling because she is an embryonic professional with years of patient treatment in front of her. It is furthermore disturbing that her essay was judged as suitable for an award in the Logie essay contest.
I have two hopes: to bring the issues of abused AIS bearers to the attention of relevant professionals so that healthy, constructive advice and counselling, based on openness, will be available; and to see AIS bearers come to respect our uniqueness and live as fully appreciated members of this society. Together, we must all lift the cloak of secrecy.
B. Diane Kemp, MSW
Ottawa, Ont.
Iam the US representative for the AIS Support Network, which has 70 members world wide. I write not to represent the group but to relate my own experience, which demonstrates the shortcomings of Natarajan's article.
At age 11 I was told lies about AIS. This was not mere "deception" -- a convenient term employed as a licence to lie to the patient. Telling me that I had "twisted ovaries" was a lie. The lies just did not add up. And so I found myself at age 20 in a medical school library unearthing the truth. Natarajan is correct: learning the truth about AIS is traumatic. But learning the truth alone and scared in the stacks of a library is shockingly inhumane. When physicians and parents abdicate their responsibility to speak the truth they not only allow this to happen, they virtually ensure that it will.
It is almost inevitable that the patient will learn the truth. The real question is how and when we want her to do so.
When I discovered I had AIS the pieces finally fit together. But what fell apart was my relationship with both my family and physicians. It was not learning about chromosomes or testes that caused enduring trauma, it was discovering that I had been told lies. I avoided all medical care for the next 18 years. I have severe osteoporosis as a result of a lack of medical attention. This is what lies produce.
As an attorney, I would be remiss not to emphasize that informed consent laws mandate that the patient know the truth before physicians remove her testes or reconstruct her vagina. I believe that the proposed breaches of medical ethics and legal duty advocated in Natarajan's article result in legal exposure for any surgeon who is silent. But such legalities only underscore the more fundamental issue of human dignity.
The members of our support group communicate freely, and the greatest source of anxiety is not our gonads or karyotype. It is shame and fear resulting from an environment in which our condition is so unacceptable that caretakers lie. Euphemisms also deny us legitimacy and meaning. Ultimately, I believe they have the potential to destroy the patient herself.
I am dismayed that Natarajan's essay garnered second prize in an "ethics" essay contest. But the greater harm would be for any physician to follow her recommendation.
Sherri A. Groveman
San Diego, Calif.
Natarajan's essay is a classic example of dangerously misguided ivory-tower theorizing divorced from the experience of real people. Had the writer spoken to someone with the condition or become better informed about AIS, she would have realized several things that did not make their way into her prize-winning essay.
First, many people with AIS have absolutely no problem with sexual intercourse. When I was first diagnosed in the 1970s, I was told by a gynecologist that I would need to have my vagina surgically lengthened in order to have intercourse. His mistake caused me months of anguish.
Second, and much more significant, is the assumption that people with AIS will accept whatever a physician tells them and content themselves with a diagnosis that simply does not hold up. I have been in contact with dozens of women with AIS, and all of us, having been given incomplete or patently false explanations of our condition and having suspected as much right off the bat, ended up finding out the truth from medical textbooks, articles in the popular press, university classes or open-minded physicians. It is much more devastating to get this information from a book than from a sensitive and responsible medical practitioner.
In my own case, I felt I had to know what afflicted me when I was in my mid-20s, a few years after the original -- and false -- diagnosis. The geneticist who had worked on my case would tell me only that some information deemed likely to be too upsetting had not been divulged. I experienced several months of terror until I finally found a physician who would reveal the truth. Although it took some time and counselling to become entirely comfortable with it, I mainly felt enormous relief.
Natarajan states that women with AIS who learn that they have X and Y chromosomes will suffer insecurity about their sexual identity or "strong emotions that could diminish [their] sense of rationality." Wrong again. This statement conveys a shallow understanding of sexual identity. As Natarajan rightly points out, the XY chromosome pattern is "more of academic than physiologic importance" to us; the real issues are our inability to bear children, the difficulty of some women with AIS in having sexual intercourse and, most important, the feelings of isolation and freakishness engendered by an atmosphere of secrecy and deceit. For most women with AIS, the biggest source of psychic pain is the conspiracy of silence -- both before and after full disclosure -- that makes them feel alone and anxious, unable to share their feelings and concerns.
As for our male partners, there is little evidence to support Natarajan's suggestion that they may suffer insecurity about their partners' sexual identity. Many women with AIS have told their husbands and lovers about their condition. There may be men out there who cannot deal with it; however, that has not been the case with my partners or with those of dozens of other women I have contacted.
There may be cases in which disclosure should be delayed because of a patient's emotional fragility. Perhaps the full diagnosis should be withheld until the patient clearly wants to know and begins to ask probing questions. I think that adolescence is too early for full disclosure, given that it is, at the best of times, a period of self-doubt and confusion. These are issues on which physicians must make sensitive judgement calls, perhaps in consultation with other family members and counsellors with some experience in congenital anomalies.
But it is highly unlikely that a woman with AIS will go through life without insisting on finding out why she is the way she is. Better that she be told by someone with her best interests at heart, who can lead her to counselling or other support, than find out from a newspaper article or medical book.
Physicians, women with AIS and parents of children with AIS are invited to contact the AIS Support Group, PO Box 425, Postal Station C, 1117 Queen St. W, Toronto ON M6J 3P5.
Name withheld by request
Natarajan's concern about the effect that truth-telling may have on the partner of a woman with AIS is outrageous. It implies that partners may not look beyond a blood test and that any love or respect for one's partner would change because of the knowledge that she has an XY karyotype. As a partner of someone with a similar problem, I resent the implications in this article.
The author's weak attempt to justify "deception" as "not a lie" by using Webster's Third New International Dictionary, prompted me to take out mine. In the Webster's Ninth Collegiate Dictionary, the word "lie" has multiple definitions. The first is the definition given in this essay; the second is "an untrue or inaccurate statement that may or may not be believed true by the speaker;" the third is "something which misleads or deceives;" and the fourth is "the charge of lying."
The contention that deliberately speaking an inaccurate statement with the intention to deceive is not a lie loses its validity when one looks at the whole definition, not selected portions.
The statement implying that only heterosexual women are "satisfied with [their] female sexuality" and should be "protected" from the truth also bothers me. The concomitant implication that it would be acceptable to inform a lesbian is highly prejudicial. This implies that a lesbian would want to be male but would not want children or have any desire to engage in intromission, a homophobic view at best.
The argument that future autonomy could be jeopardized is negated by the dictionary definition of autonomy: "the quality or state of being self-governing," or "self-directing freedom, and especially moral independence." By withholding knowledge, physicians do not allow for the exercise of autonomy. The patient's ability to be self-governing or self- directed means that it is his or her choice to decide what to do with the information provided by the physician, not the role of the physician to decide what information is "appropriate."
If I were Natarajan, I would continue to spend time with patients with AIS, particularly those who are aware of their condition, and ask for their input to better understand their perspective.
H. Deni Tako
San Diego, Calif.
As a gynecologist with 35 years' experience in looking after women, I have faced the dilemma of diagnosing patients with AIS on three occasions. Two of my patients were sisters, and the initial diagnosis was made when the older one presented with primary amenorrhea. The main problem in her management was her mother, a very devout Roman Catholic, who was very upset because she felt that her daughter could not be married in a Catholic church because she could not produce children. I referred the mother to a very wise and knowledgeable Catholic female gynecologist, who explained that there was no reason why her daughter could not be married in a Catholic church, any more than a woman who had had a hysterectomy could not be married in a Catholic church. The younger daughter was not really interested in getting married and, as far as I know, has not married.
Much later in my practice, I had another patient with AIS. This girl was much more knowledgeable about the biologic aspects of the condition and her own physiology and had no problem accepting it or in functioning as a loving woman.
All of these patients had bilateral oophorectomies and did well. As my older patient ages she tends to have male-pattern alopecia.
I would like to congratulate Natarajan on her article; it is nice to see that the young, bright medical students are coming along so that we old-timers can pass them the torch.
Karl M. Irwin
Long Sault, Ont.
[List of letters]
[The author responds:]
Ifound it enlightening to read the thoughts of women with AIS concerning their dealings with physicians.
One reader felt that my "misguidedness" was troubling because I am an embryonic professional with years of experience ahead of me. But just as embryos grow and develop with the help of external influences, so too will I. I feel that an ethics essay competition open to medical students, rather than practising physicians, is a way to capture the thoughts of developing professionals before they undergo the years of clinical experience needed to practise medicine. I see writing an opinion essay with feedback from readers as a vital part of shaping the views of a clinically inexperienced medical student.
I used ethical and philosophical principles to argue for and against two opposing sides of an issue and expressed a preference for one side. I fully recognize that I still have a great deal of training to go through. Your constructive feedback on this complex issue has been a valuable part of my medical education.
Anita Natarajan, BSc
Second-year medical student
University of British Columbia
Vancouver, BC
[List of letters]
[The news and features editor responds:]
Two of the authors take CMAJ to task for selecting Natarajan's essay for a prize in our Logie essay contest. I believe they are wrong, and that Natarajan's superb response explains why. Her essay was selected because she presented a coherent argument and did it well. Her response to the letters indicates that she has learned from the experience, so we have achieved our main goal. Finally, had the essay not appeared neither would this correspondence, and physicians would have been deprived of an excellent debate about a rare (and rarely publicized) medical condition.
Patrick Sullivan
Editor
News and features