Trust me I’m a patient

First published in Wavelength, May 1999, pp. 8-10.

[Please note that there has been some  progress in the treatment of AIS women since this was first written. For more information visit the website for the Androgen Insensitivity Syndrome Support Group. This is an extremely informative site and is a valuable resource for patients, families and doctors.]

Thousands of women in the UK are genetically ‘male’. For many of them it is a ‘dark secret’. Many others do not even know.

Lied to by doctors and parents, operated on without informed consent, treated as freaks for medical research – these XY women are now finding their own voice and their own identity.

Androgen Insensitivity Syndrome, or AIS, is a form of intersexuality where a foetus with XY (i.e. ‘male’) chromosomes develops a female body. Such XY women usually have internal testes and no uterus, but externally the only signs of the condition are the lack of pubic and underarm hair and the absence of periods.

It is likely that more than 1 in every 20,000 ‘male’ births are affected, but it is quite probable that many women with XY chromosomes are never diagnosed, and of those women who have been diagnosed many never know because they are never told.

Last year [1998] the General Medical Council issued new guidelines which require all doctors to be open and frank with their patients. But women with AIS have faced a long history of lies, secrecy and half-truths. For these women the new guidelines may mean the truth at last, but it is doubtful that it will be the truth, the whole truth and nothing but the truth.

The general belief is that for a woman to find out she has ‘male’ chromosomes would be too great a shock for her to bear. Consequently, with the best interests of the patient in mind, it has been a common practice for doctors not to disclose the true nature of the condition. When parents are told, they too get drawn into the conspiracy of silence. The result is a web of lies from parents and doctors – the very people we are expected to trust most.

It is highly likely that this policy of secrecy has caused more damage than the condition itself. As one woman said:
I have experienced more emotional pain about the fact that my family didn’t tell me the truth than about either my gonads or my chromosomes.

Knowledge that there is a secret is knowledge enough, and AIS patients are as able as anyone to pick up on the tell-tale clues of hushed conversations and embarrassed looks. One patient figured out there was something wrong in hospital when she was examined by over a hundred doctors and interns. Another woman only found out from her mortgage company which had queried her medical records as part of the application process.

With no information coming from doctors or parents, AIS patients are forced to rely on their own resources – typically medical libraries and increasingly the internet – and the haphazard process of self-diagnosis.

One AIS woman, now in her 40s, had already self-diagnosed when she was told of her condition at age 18:

It’s not that I was told the truth that caused problems, because I had been trying to come to terms with it completely alone for a number of years. It was the fact that it was delayed so long. I took on-board the very powerful message that this was something so unbelievably rare, and so shameful and taboo-laden that it could never be talked about within the family, and we were left to grapple with our own unspeakable monsters. I had been convinced that all the adults in my life – parents, doctors, even teachers – knew exactly what was wrong with me but were doubly punishing me for some unknown reason, by not telling me the facts and not telling me that they were aware I had found out, thus imprisoning me with my own unutterable discoveries.

The usual course of management for the condition is to remove the testes and to put the AIS patient on lifelong hormone replacement therapy. Without full disclosure it is at least a matter of debate as to whether agreement to such treatment constitutes informed consent. (The testes are often described as ‘twisted ovaries’ and their removal is explained as a ‘hysterectomy’.) The same might also be said of the other medical procedures that AIS women have experienced – displayed as freaks in front of doctors, subjected to medical photography, and having blood and tissue (often labial tissue) samples taken for research.

An AIS woman in her 30s recalls:

My decision to drop all medical care was the direct result of the humiliation and obfuscation I had been subjected to over the previous years. Junior hospital doctors were paraded past my vagina when I was 14 or 15. Not once was I asked how I felt about any of what had transpired. I was a patient with a terrible secret that even the doctors and my own mother couldn’t, or wouldn’t, discuss.

Yet in those cases where the patient has been told (or has found out) the diagnosis, she often comes to know more about the condition than the doctor. Moreover, what is known (and more importantly how it is known) results in major disjunctions in the perspectives of doctor and patient:

                                                           DOCTOR                           PATIENT

                             PROBLEM          Not fully male                      Not fully female

                                  CAUSE          Faulty gene                         Presence of unwanted hormone

                            CONCERN           Gender identity                    Coital adequacy, fertility

           EXPERIENCE OF AIS            Vicarious via patient             Lived, first-hand

                              POLICY             Secrecy                               Disclosure

Doctors see a failure to virilise and trace the problem back to a ‘faulty’ gene, but XY women experience a failure to fully feminise. They trace the problem back to the presence of an unwanted hormone which inhibits the development of uterus and tubes.

Language makes the problem worse. Why should bits of biochemistry such as chromosomes and hormones be labelled as ‘male’? Doctors see the male label and worry about gender identity, but AIS individuals experience a female body and worry about infertility and sexual adequacy.

Therein lies the major difference. For doctors, AIS is a condition to be known about only via their own or other patients, but for XY women it is a lived experience.

In 1993 the Androgen Insensitivity Syndrome Support Group was formally established in the UK and has done much to persuade the medical profession that it is disclosure and not secrecy that is in the patient’s best interests. The group provides support for affected individuals and families and aims to break the taboos and secrecy that surround the condition. The group is now also active in the US, Canada and Australia.

My own personal involvement is as the father of a 12 year old AIS girl. Just before her first Christmas she was operated on for an inguinal hernia caused by internal testes. Against the medical orthodoxy of the time, we as parents decided not to have the testes removed and to bring her up in full knowledge of her condition from as early an age as possible. My first reaction on being told of her condition was that of a proud parent: “Wow, I always knew she was special.” It was this positive attitude which I hoped to take to the support group.

Over the past five years I have been able to see the group grow from very uncertain beginnings when individual and collective self-confidence were low, to a point where it has a membership of over 200 affected families and individuals, charitable status, and is being consulted by the Middlesex Hospital [part of the UCLH Hospital Trust, London] which is setting up a specialist intersex unit.

This is clear recognition of the group’s expertise in the field. More particularly, it shows how, through individual and collective action, they have established their own realm of ‘experiential’ expertise at the same time as using and questioning existing medical knowledge. Now, both sides can be seen as commanding areas of expertise: the medical/technical and the lived/experiential.

But there is still much to be done. Indeed, researchers into the psychosocial aspects of the condition are hampered by the very secrecy that should be investigated. One research group was unable to approach patients through their doctors because so many doctors refused permission on the grounds that they did not want their patients to know the true diagnosis.

Access to medical records should help counter doctors’ secrecy, but patients will only look if they know there is something to look for. And although the General Medical Council’s new guidelines may now promote openness, many doctors will find it difficult to volunteer information as a first step in the long and difficult process of secrecy reversal. (Just how do you tell someone you have betrayed their trust for several years?)

With the prospect of possible disciplinary action for not being frank and open, burying secrets even deeper may be seen as an attractive easy option. Each new diagnosis is an opportunity for a new honesty, but there will be countless women and children, already diagnosed, who will continue to labour under the weight of a fearful ignorance.

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