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What Kind of Outsider?

Flora, who started the east coast Canadian AIS Support Group in 1996, wrote the following article in early 1998 for submission to an obs/gyn journal:

It is the early 1970s, and I have just turned 19. My family doctor in small-town western Canada has finally decided to do something about my amenorrhea [absent menstruation], so I’ve been referred to a gynecologist. After a day of tests, in a hospital an hour’s drive from the town where I live with my parents, the gynecologist walks into the room I share with three other women. Making no attempt to secure my privacy, he quickly tells me that 1) I was born without a uterus and will never bear children, 2) my ovaries are ‘malformed’ and need to be removed because of a cancer risk, and 3) I lack the top third of the vagina and won’t be able to have sexual relations, but that when I become engaged, I can get the organ surgically lengthened with a skin graft from my inner thigh.

He walks out; I am left alone, in shock. I stumble to a pay phone and beg my mother to come to the hospital, and then spend the next several months in hell. With absolutely no one to talk to, and desperate for information, I actually write to the Playboy magazine adviser to ask if anyone there has heard of this strange condition or this vaginal problem; they politely reply that I should find an appropriate physician. I discovered by happy accident, six months later, that my vagina was in fact long and elastic enough for sexual intercourse. A year after the initial tests, I had my ‘ovaries’ removed and go on hormone replacement therapy.

It is six years later. Taking my cue from doctors, I have spent most of the time since my initial diagnosis pretending to others—and even to myself—that I do not have a medical condition. But now I want to know exactly what is wrong with me. Why is there no name for this condition? How did I come to be born without a uterus and with bad ovaries, and why did I have no axillary[under-arm]/pubic hair? Is something being kept from me? I go to see my geneticist. She tells me that she respects my intelligence and curiosity and therefore must acknowledge that some details of my condition have been withheld. But she is not at liberty, she says, to divulge that information, because it would upset me too much.

During the next six months I suffer much dread about those undisclosed details. Will I die young? Will I become hirsute and/or masculine? Do I already have a slow-growing cancer? I spend time in university libraries trying to determine which condition I’ve been afflicted with. I see a listing for ‘Testicular Feminization,’ but it is accompanied by a photograph of a woman with pubic hair [Ref 1] so I think, “That can’t be it.” After moving to Montreal, I see a physician for an intestinal problem, and I tell him about my medical terror. He says he’ll request my file from the gynecologist.

Ref 1: Presumably a case of PAIS rather than CAIS (see “AIS Clinical Grading Scheme” in ALIAS No. 6, Winter 1996).

The gynecologist replies by mail:

The last time I saw her was in May of this year. Up to that point in time she had made out well emotionally, however, unfortunately, she had again spoken to the geneticist who I believe raised more questions than were necessary regarding her genetic status. I attempted to reassure her regarding this. I feel it is very important that she gets considerable support and reassurance regarding her femininity.

The new doctor tells me he has learned the truth—testicular feminization, also known as androgen insensitivity syndrome (AIS)—and he doesn’t think it’s so bad. Neither do I. In fact, I am hugely relieved.

We all grow up having platitudes about the beauty and importance of Truth drilled into us. We also learn early on that, in reality, truth is often something to be avoided. However, even when it seems we’ve successfully outrun the truth, it is still lurking on the perimeters of our lives. Dealing with difficult truths is invariably painful. Sometimes it destroys people. But the truth can also be liberating. Certainly it was for me. My genetic truth taught me so much about the universe, about chance and chaos, about the marvellous inventions of life. It also gave me the gift of compassion. And it simply allowed me to know myself. I believe it would have been impossible for me to have an adulthood that was at all meaningful without having learned about AIS, as painful as it’s been to know the extent to which I am an anomaly. I would never have surmounted my sense of being an outsider without knowing just what kind of outsider I was.

I now have some empathy for the gynecologist and geneticist who felt they couldn’t tell me the truth. After all, they were following the medical orthodoxy of the time. And I suppose they did have my best interests in mind. But the gynecologist’s failure to even begin to imagine what I was thinking and feeling was staggering. Why did he assume early on that I wouldn’t be thinking about sex until I became engaged? [Ref 2] Why didn’t he at least make sure that a nurse sat with me after he first told me about my condition. Why didn’t it occur to him that I might need counselling to help me deal with all that bad news? And, later, how could he have thought that my need to know would simply evaporate if everyone continued to stonewall?

Ref 2: However, I’m hugely relieved that he didn’t rush me into vaginoplasty—clearly, he hadn’t read the literature very carefully on vaginal length and sexual relations—even back then, as I later learned, books and articles indicated that [even] when the upper third of the vagina is missing, sexual intercourse is still feasible.

My gynecologist was also acting according to social prejudices, the ones that continue to ensure that sexual anomalies (as well as gender anomalies and homosexuality) are held in fear and loathing. But by failing to tell me the truth, he amplified that stigma enormously: basically, he seemed to be saying that the truth about my body, the body I was born with and that enables me to take pleasure in life, to work, to actualize my strengths, desires and intelligence, was intrinsically bad—so bad that it must not be described.

Sure, I have had some unhappiness about having XY chromosomes and undescended testes. But that misery had more to do with the fact that I feared I was unlovable. Partly because of our cultural fear of sexual abnormality, and partly because of how the medical profession taught me to be ashamed, I worried that men who knew the truth about me would be revolted. But I have shared my secret with a number of men with whom I’ve been intimate, including two husbands, and no one has walked away in disgust or even had to struggle to accept my genetic nature. I’m convinced that some partners loved me more because of it.

A few years ago, when I had to go to Emergency because of a minor cut, I told the attending physician, a urology resident, that I was an AIS individual. He seemed aghast that I knew the truth about my condition, and was even more dismayed when I informed him that my boyfriend (now my husband) also knew. There are still some young doctors out there who want to shroud AIS in secrecy. A prize-winning article [Ref 3] that appeared in the Canadian Medical Association Journal in February, 1996, advocated that women with complete AIS not be told the truth of their condition. The essayist, a medical student who had not consulted any individuals affected by the condition, warned that disclosure could result in “unnecessary and devastating emotional and psychologic effects that will impede [the patient’s] chances of leading a normal life.” Several of us jumped in with letters to the journal [Ref 3] arguing that the truth is likely to emerge sooner or later, and that a lifetime of lying is much more likely to result in the emotional devastation about which the essayist is concerned.

Ref 3: Reproduced in “Sex, Lies and Ideology” in ALIAS No. 6, Winter 1996. See Debate/Discussions to read the journal article and subsequent discussions. See also “Truth-telling Debate” in ALIAS No. 5, Summer 1996.

In the past few years, I have addressed medical students about the issue of truth disclosure. I remind them that we live in a medically very literate culture—viewers of ER know all about blood gases, while Chicago Hope actually dealt with partial AIS in one episode. It is highly unlikely that an AIS woman will go through life without learning the truth. Often the medical students ask me what the optimal age is for telling the truth about AIS. I don’t have any easy answers. Two years ago, I discovered that there was a support group for people with AIS. It was started up in Britain in the early ’90s, and now has contacts throughout North America, in Australia and in Europe. My AIS friends and I are frequently in contact—at regular meetings, by phone and through e-mail—and one subject that comes up frequently is disclosure. There is no disagreement among us about the fact that the truth needs to be divulged to affected individuals and their families. And certainly we agree that by the time a person reaches adulthood, she needs to know about her genetic situation.

Through the support group, we have met many women who didn’t learn the truth about their bodies until they were 30, 40, even 50. Some discovered the truth in very painful ways—from university classes, TV shows, articles in the popular press (which almost invariably get things wrong). Often these women feel profoundly betrayed, because they have not had all the years of adulthood to learn to accept their anomalous genetics and physiognomy, and to process the details of their condition with loved ones.

But do we in the support group advocate telling everything to a 12-year-old child? Probably not, unless that girl was unusually precocious—and secure. We adult AISers agree that the truth about our syndrome should probably be told in stages, beginning a few years before the normal onset of puberty. Most important, with each stage of disclosure, it’s important to make the child feel that she can explore all her feelings and questions. There is no underestimating how much harm secrecy and an atmosphere of it’s-best-not-to-talk-about-this-unspeakable-thing can do. For that reason, it’s crucial to refer parents as well as the affected person to counselling, because the parents’ natural inclination will be to pretend that everything is perfect in the interests of protecting their child from pain that she needs to feel, to surmount having AIS. No one encouraged my family to discuss my condition and what infertility meant to all of us. We mourned independently, silently. When I finally began talking to my mother and brother about AIS a few years ago, it was immensely healing.

Medical practitioners also need to realize that both parents and affected children will need to ask certain questions again and again. They will have trouble keeping the facts straight in their minds. They will need to approach difficult issues many different ways. And, if possible, it’s best to equip them with an article, a support-group brochure, or something concrete that they can read over and over again, and go back to if they begin to lose their hold on the details [she gives support group details].

By the time a girl is nine or 10 and beginning to anticipate menstruation, she needs to know that she’s different and that she will not be menstruating or able to have children. If gonadectomy has not already taken place, she needs to be told about the possible need for surgery at some point in the future, and reassured that she does not have cancer. Should a child of that age be told that undescended testes are being removed? Again, unless the child is unusually precocious, a euphemism (such as sex organs, or reproductive organs) is probably more appropriate at that point. Parents and doctors should avoid telling the ‘bad ovaries’ lie. For one thing, we all have sensors that can pick up untruths—I always doubted that bad ovaries story. And telling lies will just foster a sense of betrayal when the truth is out.

Perhaps the biggest question is: when should full disclosure happen? I think the short answer is that it depends on the girl. There is a very well-adjusted 14-year-old with complete AIS in the United States who knows everything about the syndrome and has attended support group meetings [and girls of 9 and 13 in the UK]. In the summer of 1997, she was thrilled, proud and relieved to meet adult AISers who were happy, productive people, many in marriages or long-term relationships and, in some cases, with adopted children. Certainly, once a young woman starts asking questions about what really is wrong with her, it’s time for full disclosure. But physicians must tell the truth is a compassionate way. They must ensure that the affected individual and her family feel free to express all their misery and fear about the diagnosis, and to ask many questions, repeatedly. Even when there are no outward signs of stress or anxiety, they should recommend follow-up counselling. They must be sure to tell the AIS individual and her family that they are not alone, and that there is a support group at their disposal.

Recently, I asked my G.P. what she had in her files from my early diagnosis. She had the letter from my gynecologist to the physician who told me the truth, as well as the pathology report from my gonadectomy. It made me sad to read the clinical details about these parts of me that were missing—I realize that they had to be removed because of the cancer risk, but I had never had a chance to mourn the loss of the organs (testes) that had, in fact, helped feminize me. And while some doctor may have mentioned it before, I had never truly realized that I’d also once had rudimentary fallopian tubes and uterus-like tissue. Perhaps this was noted at the same time that I was told about my chromosomes and testes and it was all too much to grasp. In any case, it was very, very important for me to learn about these minuscule bits of female reproductive material that I’d once possessed. And it saddens me that doctors, so many of whom would go on about me being “genetically male,” never ensured I had this information about my vestigial female reproductive parts.

For years I told myself that, yes, I was genetically male. But now I realize that is an inaccurate description of the way I am. I now consider myself to be genetically female through an unusual route. The gene defect [on the X] that made me androgen-insensitive superseded the influence of my Y chromosome and made my body feminine, albeit without a reproductive system [Ref 4]. We are all so hung up on chromosomes. But my AIS friends and I are proof that chromosomes do not always determine gender.

Ref 4: See also “Y Gonads?” in ALIAS No. 7, Spring 1997, and “MIF Matters” in ALIAS No. 8, Summer 1997.