Home Page
About this Site
Contact Us

What is AIS?
Complete AIS
Partial AIS
Related Conditions

Group Literature
Group Meetings
Raising Awareness

AIS in Articles/Books
Personal Stories

Obtaining/Facing Diagnosis
Orchidectomy (Gonadectomy)
Vaginal Hypoplasia
Genital Plastic Surgery
Information for Parents

Patients' Charter
Recommended Clinicians
Research Studies
Fertility Advances

Informationen in Deutsch
Información en Español
Information en Français
Informazioni in Italiano
Informatie in het Nederlands
Informacje po Polsku
Information på Svenska
Információ Magyarul
Eestikeelne Informatsioon

Links to Other Sites

Once a Dark Secret

The following anonymous article was published in the ‘Personal View’ column of the British Medical Journal, Vol 308, p542, 19 Feb ‘94. The BMJ online version of the article is available here but please note that the contact details (Shirburn Ave., Mansfield, Notts) quoted there for AISSG UK (at the end of the second of the two letters that follow the article) are out of date:

We'd love to be in touch with the author of this article if they felt willing to contact the UK group, merely because it seems a key article in the emergence of intersex conditions out of the shadows, probably being the first time an XY woman's words had appeared in print. Your identity would be kept confidential.

It has taken 12 years for me to admit to having testicular feminisation syndrome, but having reached this stage I consider it important to share my perspective on the condition and the counselling that is essential for those in a similar position. If the following narrative seems melodramatic it is unintentional, but it puts my case into perspective and provides a context for my recommendations for managing the condition.

At the age of 15 I was told by a school doctor that I would be "starting my periods very soon"- I did not. I saw my general practitioner who read through my clinical notes. I had been treated for hernias as a child. He referred me to a gynaecologist who examined me thoroughly and quizzed me about my sex life – a traumatic experience for a naive and sheltered virgin. I was told that I would need an operation to remove the gonads. I was studying O-level biology and was surprised by this reference but there were more important things to consider at the time. I saw myself as female and came to think of gonads as the medical profession's generic reference for my ovaries. In hospital a nurse told me that surgeons did not remove ovaries from girls my age. I met my new consultant and in front of his medical staff and nurses he told me that they were not ovaries and that I was sterile. The operation followed and I began taking hormone replacement therapy drugs normally prescribed for menopausal women.

I am highly intelligent and my suspicions were aroused and an element of confusion set in. I went to the university's medical library and read several books on gynaecology and the truth of my condition became clear. I shudder when I think of that aftemoon.

I sought an interview with my consultant who confirmed my diagnosis and I asked for counselling. The next six years were spent in periods of deep depression with frequent desires for suicide. My consultant did what he could but there were several events which increased my despair. I was admitted to hospital for adhesions and a nurse asked me if I had previously had a penis. I was seen by a psychiatrist in his office in a maternity hospital with a heavily pregnant doctor participating. I was seen by a psychosexual counsellor who tried to take me apart and reconstruct my psyche, a process which nearly caused a mental breakdown.

These, however, are things in the past and I have now accepted the condition but there remains issues that need to be addressed.

I am chromosomally male, a pseudo-hermaphrodite. These two phrases pervaded my rational thought. I did not think I was female. I did not think I was male. I did not know what I was.

The medical profession's understandable interest in rare cases underlined my feelings of freakishness: I was introduced as an "interesting case"

There was a reluctance to explain clearly and unambiguously to me the nature of the condition and its manifestations. Allied to this was the ignorance of the nursing staff and their ill advised comments.

My condition is now being managed superbly. I have a sensitive and sensible consultant who over the past year has enabled me to deal with this problem and my recommendations are as follows:

Mine was a dark secret kept from all outside the medical profession (family included) but this is not an option because it both increases the feelings of freakishness and reinforces the sense of isolation. It also neglects the need for the counselling of siblings.

I spent a large part of my early 20s feeling repugnance towards myself and I have to work hard to push it to the back of my mind.

Two responses, one from a doctor and one from a patient, were published in the BMJ, Vol 308, pp 1041-1042, 16 April ‘94 under the following title and subtitles:

Gender Identity in Testicular Feminization

[Response 1] Phenotypically, anatomically, legally and socially female

Editor - The personal view on testicular feminization expresses poignantly the difficulties facing someone who has to come to terms with the condition. The author was clearly distressed to learn that she was "chromosomally male, a pseudohermaphrodite," and highlights the essence of the problem in asking "What makes a person female?" Doctors make a fundamental mistake in cases of testicular feminizaion if they concentrate on the chromosomal definition of sex -- a definition that is unhelpful to the patient.

Ther are many definitions of a person's gender: genotypic (chromosomal pattern); gonadal (testis or ovary); phenotypic (including body habitus and genital anatomy); hormonal (oestrogen or androgen effects on the target tissues); legal (birth certificate, passport, etc.); and, most importantly, social, which includes how we see ourselves, how others see us, the approach to sexual relationships, and everyday matters such as which changing room to use and which schools we may go to. Discordance among these definitions of gender may lead to problems in sexual identity. But patients with testicular feminization have a high degree of concordance in their sexual identity: they are phenotypically, anatomically, hormonally, legally and socially female.

To explain the diagnosis by telling the patient that she is fundamentally male but with abnormal sexual development may, to the purist, be embryologically correct but does nothing to help: it simply destroys a most fundamental part of a person's identity -- gender. That is unnecessary. The explanation offered should begin from the understanding that the patient is female, and it should build on that assumption. The need for gonadectomy will ultimately require that the gonadal and chromosomal sex be explained, but care must be taken to do this in a way that does not disturb the overall gender identity. There are conditions in which gender assignment is sometimes difficult (for example, congenital adrenal hyperplasia), but testicular feminization is not one of them.

James A.O. Ahlquist, Radcliffe Infirmary, Oxford, OX2 6HE.

[Response 2] Be honest and open with sufferers

Editor - I should like to endorse the recommendations of the writer of ‘Once a Dark Secret’ concerning the androgen insensitivity syndrome (testicular feminization syndrome). It’s not that my gynaecologist told me the truth that angers me (I’d used medical libraries to reach a self-diagnosis anyway) but that neither I nor my parents were offered any psychological support but were left to flounder in our own separate feelings of shame and taboo.

I believe young girls with AIS would be happier not knowing of their male genetic/gonadal status, but a conspiracy of silence cannot guarantee blissful ignorance. The human psyche will be drawn to the truth of things that threaten it, even if it means severe psychological pain. Unless parents can talk openly at the time of diagnosis with a professional counsellor (not a doctor) and are given information -- on what/when to tell their child, contacts with other sufferers, sources of counselling/psychotherapy, the timing of gonadectomy /HRT, vaginoplasty techniques etc. -- they will become imprisoned by their own feelings, leading to isolation and inaction. The client's condition will be regarded as a lost cause, or something too terrible to ever mention in the family, let alone to outside professionals who otherwise might have helped. Those parents who forbid disclosure possibly seek to protect themselves and will probably also fail later to take action which could be vital to their daughter. This could be far more damaging than truth disclosure in a caring, supportive environment, with time to dissipate the threat by discussion.

Paediatricians should also be alert to vaginal agenesis in the complete form of AIS. I lived alone in fear and gender confusion with my self-discovered deficiency from age 10 (after my parents had told me a neonatal hernia repair had indicated "a problem, with no likelihood of menstruation/babies") until late diagnosis at 18. I am angry that the complications (stenosis) of one technique only (McIndoe) were then repeatedly used by clinicians to deny me a vaginoplasty and hence the right to express my sexuality in young adulthood (the ‘stenosis-free’ Baldwin technique has been around since 1904). I am now 46, and after two late vaginoplasty operations (McIndoe and Williams) still have only a short, partly ‘externalised’ vagina. Today’s high-profile women's medicine still cannot guarantee one of the most fundamental attributes of womanhood. Infertility has been the least of my problems. Dr John Money, a world expert on the psychology of sexuality and sexual disorders, cites ‘pairbondage’ (parent-child and sexual lover-lover bonding) as the first of the five ‘universal exigencies’ or pressing human necessities, and emphasises the “enormous anxieties felt in this area of life by young people with birth defects of the sexual organs”. The ability to have babies is not included within any of the five categories. The immediate failure of my first real relationship has at last forced me to seek psychological help, after a lifetime of unnecessary secrecy, shame, delayed action and great damage to my personal/sexual identity and self-esteem.

[Contact details of some support groups were given, including the (now out of date) details for the AIS Support Group]