Textbook Discussion

These three sections ('Reading the Words', 'Hearing the Words' and 'Saying the Words') from ALIAS No. 9 are based on an email discussion in 1997 between two adults in their 40s, one in the UK with CAIS and the other in the USA with PAIS grade 4 (assumed female until 3 months, then raised as a boy – with mastectomy – until 23 when she changed to the female role):

Reading the Words

The CAIS woman wrote:
John Money’s ‘five universal exigencies’ [Ref 1] or ‘pressing human necessities’ (pairbondage in particular), were mentioned in an early newsletter [Ref 2]. He mentions ycleptance as being the other exigency, apart from pairbondage, that has particular significance for patients with a birth defect of the sex organs. He explains that ycleptance is a recycled Elizabethan term which comes from the verb to clepe, meaning to name, call or style. He says, “Human beings have named and typecast one another since recorded time. The terms range from the haphazard informality of nicknames to the systematic formality of biomedical nomenclature and diagnostic terms that prognosticate our futures and shape our lives. They affect our self-image, and all too often can be brutally stigmatizing.”

Ref 1: Money J: Five Universal Exigencies, Indications and Sexological theory. J. Sex Marital Ther., 10: 229-238, 1984.
Ref 2: See “Psychological Support” in ALIAS No. 1, Spring 1995.

Whilst on holiday in a large city, my CAIS sister and I took the opportunity (who wouldn't) to do a quick survey of a few of the many gynaecology books in a large academic bookshop. We were looking, for example, at what was included for our condition in the index, how it was referred to in the main text, what stigmatizing/outdated terms were used, whether the complete and the incomplete or partial forms (CAIS and PAIS) were given recognition, whether truth disclosure and psychological counselling were advocated, whether vaginal hypoplasia was covered, whether the need of unaffected XX relatives to know of their possible carrier status was mentioned, etc., etc. In the following summary (which concentrates on CAIS) you can generally assume that if I don’t mention a topic from the above list, then it probably wasn’t dealt with.

Symonds [Ref 3] only uses the outdated, and some would say inaccurate/stigmatizing term ‘testicular feminization syndrome’ (I’ll call this ‘TFS’). Leader, Bennett and Wong [Ref 4] also use only TFS, and refer to such patients as being “sexually infantile”[Ref 5]. Smith [Ref 6] likewise refers to TFS, (and to male pseudo-hermaphroditism), without mentioning AIS at all. Govan, Hart and Callander [Ref 7] use TFS as their primary index entry and state in the text that patients have “a tendency to eunuchoid proportions” but they do mention counselling. Llewellyn Jones [Ref 8] uses both terms (AIS and TFS) as index entries, as does Pernoll [Ref 9]. Chamberlain [Ref 10] uses TFS as the main index entry (with AIS also) and stresses psychological management and teamwork. Studd [Ref 11] uses the term AIS alone (well done!) and mentions psychological support (very well done!!). Sakala [Ref 12] uses AIS in the main text but has no index entry for the condition at all. Selzter and Pearse [Ref 13] include both AIS and TFS in the index and are amongst the very few to mention the complete/partial forms. Magowan [Ref 14] uses ‘Androgens, insensitivity’ in the index, uses ‘TFS (AIS)’ in the text and mentions the complete and partial forms. Rynan, Berkowitz and Barbieri [Ref 15] use TFS in the index and mention ‘AIS (often referred to as TFS)’ in the text.

Ref 3: Essential Obstetrics and Gynaecology, 2nd Ed., 1992, Churchill Livingstone.
Ref 4: Handbook of Obstetrics and Gynaecology, 4th Ed., 1996, Chapman and Hall.
Ref 5: Presumably a reference to lack of some secondary sex characteristics, e.g. pale underdeveloped nipples and lack of pubic hair?
Ref 6: Gynaecology in Primary Care, 1997, Williams and Wilkins.
Ref 7: Gynaecology Illustrated, 4th Ed., 1993, Churchill Livingstone.
Ref 8: Fundamentals of Obstetrics and Gynaecology, 1994.
Ref 9: Current Obstetric and Gynaecological Diagnosis and Treatment, 7th Ed., Appleton and Lange.
Ref 10: Gynaecology by Ten Teachers, 16th Ed., 1995, Chamberlain.
Ref 11: Progress in Obstetrics and Gynaecology, 1996.
Ref 12: Obstetrics and Gynaecology, 1997, Williams and Wilkins.
Ref 13: Women’s Primary Health Care Office Practice Procedures, McGraw Hill.
Ref 14: Churchill’s Pocketbook of Obstetrics and Gynaecology, 1997, Churchill Livingstone.
Ref 15: Kistner’s Gynaecological Principles and Practice, 6th Ed., 1995, Mosby.

Berek, Adashi and Hillard [ Ref 16] use AIS in the index and use ‘AIS (formerly TFS)’ in the text. They give comprehensive coverage in the text then state that “these patients are pseudo-hermaphrodites” and that “patients are usually tall with a eunuchoidal tendency (long arms with big hands and feet).” Grudzinska and Beedham [Ref 17] use only ‘TFS (XY female)’ in the index and state that “most experienced clinicians would not reveal the karyotype in view of likely psychological effects.” They recommend “counselling and support for the parents, the individual and partners in later life” (but seem to be referring to infertility counselling only).

Ref 16: Novak’s Gynaecology, 12th Ed., 1996, Williams and Wilkins.
Ref 17: Treatment and Progress in Obstetrics and Gynaecology.

Mishel, Davajan and Lobo [Ref 18] use both AIS and TFS in the index and recommend that “patients with AIS are told that they have an abnormal sex chromosome, without specifically mentioning a Y chromosome because it is commonly known that an XY karyotype indicates maleness.” I would say that this is actually inaccurate because they imply, I think, that the Y chromosome is abnormal in AIS. It isn’t. If there’s any chromosomal abnormality to be mentioned, it’s on the X chromosome [Ref 19]. “In addition” they say, “because psychologically and phenotypically these individuals are females, the term gonads should be used instead of testes. However, they should be told about their infertility ....” They recommend that “.... if necessary, progressive vaginal dilation or skin-graft vaginoplasty should be performed when the patient approaches the age of sexual function.”

Ref 18: Infertility, Contraception and Reproductive Endocrinology, 3rd Ed., 1991, Blackwell Scientific.
Ref 19: See “Y Gonads” in ALIAS No. 7, Spring 1997.

My overall impression is that, even in very recently published books, a) outdated and extremely stigmatizing terms (like ‘testicular feminization’, ‘male pseudo-hermaphrodite’, ‘eunuchoid proportions’ and occasionally some really awful ones like ‘sexually infantile’) are still heavily used, b) very few authors mention the fact that there are two forms of the condition with different issues applying, c) secrecy, deception and paternalism are still advocated, d) vaginal hypoplasia is very rarely mentioned (let alone any psychological support for this problem in its own right, or any treatments, surgical or non-surgical), e) counselling, when mentioned at all, often refers to counselling a patient about her infertility only. I also get the impression that many medics use the word 'counselling' to mean the conveying of minimal amounts of diagnostic information by a doctor rather than facilitating psychological support, or talking by the patient [Ref 20].

Ref 20: See “Can/Should Doctors Counsel?” in ALIAS No. 1, Spring 1995.

In other words, everything possible seems to be done (by inclusion or omission of terms/topics) to make patients feel even more stigmatized, freakish and alone, whether as a result of their own furtive bookshop forays, or by making sure that they are not subject to the appropriate attitude from the next generation of textbook-reading doctors. I think that medical book authors need to know that bookshops do not keep endocrinology and gynaecology books under lock and key, and that their readers are not just doctors but patients and parents who've been starved of information [Ref 21].

Ref 21: See “A Mother’s Story in ALIAS No. 7, Spring 1997, for example.

The PAIS woman replied:
Agreed. What is supposed to happen when someone with AIS changes doctors and starts getting a completely different story from the one she's used to? Your book survey reveals the same material as was used 20-25 years ago. Maybe most of these authors have little actual experience with AIS, so they just regurgitate what they've read themselves. Here is a list of terms used for our condition and my opinions of them:

Androgen Insensitivity Syndrome (AIS) – The best term for the condition. It is accurate and descriptive without being stigmatizing.

Testicular Feminization Syndrome (TFS) – I don't mind this term too much when it is used by medical personnel. It does, however, contain the T-word, which creates a potential for misinterpretation when medically unknowledgable people hear it.

Male pseudo-hermaphroditism (MPH) – The most stigmatizing one of all! The ‘male pseudo’ part implies that the ‘pseudo-hermaphrodite’ is not even ambiguous sexually, but is ‘really’ a male.

I object to MPH much more when it is used as a specific term for AIS than when it is used as a generic term for (partially or completely) anatomically demasculinized gonadal males [Ref 22], including those with AIS. There is a need for such a generic term that is more concise than this mouthful of gobbeldygook. Last year I saw MPH used as the specific term for AIS in ‘The Family Doctor’, a CD-ROM that came with my computer. I thought this was inappropriate and reflected poor research on the part of whoever wrote it. Maybe a CD-ROM called ‘The Pediatric Endocrinologist’ would have better information. MPH was also used in an article mentioned in the last newsletter [Ref 23]. I didn't really like to see this term, but I approved of every other aspect of the article, and even considered sending the authors a letter telling them how good and useful it was.

Ref 22: i.e., various conditions resulting from either a) decreased androgen production, b) abnormal androgen metabolism, or c) normal androgen production/metabolism but with hormone resistance (e.g. AIS).
Ref 23: Costa et. al., Management of ambiguous genitalia in pseudo-hermaphrodites: new perspectives on vaginal dilation. Fertility and Sterility, Vol. 67, No. 2, Feb. 1997.

The CAIS woman wrote:
Paediatric intersex expert Dr. David Grant [Ref 24] has commented that “[The terms] female pseudo-hermaphroditism (FPH) [Ref 25] and male pseudo-hermaphroditism (MPH) [Ref 26] are now largely obsolete and Prof. [Ieuan] Hughes would like to replace them with ‘virilized female’ and ‘undervirilized male’ as he thinks these terms are less offensive and easier to understand.” I actually heard Prof. Hughes [Ref 27] suggest this, at a meeting of the British Society for Paediatric Endocrinology in Sept. ‘96, and claiming that this proposal came out of patient support groups having told him they didn’t like/understand the older terms. Well, what can I say! Can you see parents/patients rushing to use these terms? There’s no way, as a CAIS woman, that I’m going to refer to myself as an ‘undervirilized male’!

Ref 24: Hon. Snr. Lecturer, Institute of Child Health, 30 Guilford St., London WC1N 3JH. Personal communication. Dr. Grant is now deceased.
Ref 25: Female chromosomes, with uterus and ovaries but masculinized external genitalia, e.g. CAH (Congenital Adrenal Hyperplasia).
Ref 26: Male chromosomes, with testes but feminized external genitalia, e.g. AIS.
Ref 27: AIS genetic expert and Prof. of Paediatrics, Addenbrooke’s Hospital, Cambridge, CB2 2QQ.

The PAIS woman commented:
Well said. And I wouldn't call the terms female and male pseudo-hermaphroditism obsolete either, as they are still widely used and they are useful terms in clinical communication, as there is little debate about their meanings. Using terminology that has been around for 20-30 years does make it a lot easier to refer to older literature and to communicate without confusion, even though they are also stigmatizing. I suspect that in Prof. Hughes' mind FPH and MPH are offensive because of the ‘hermaphrodite’ part, not because of the ‘pseudo’ part. I don't mind terms like ‘hermaphrodite’ that imply ambiguity. I think that the idea of changing my gender role was in the back of my mind for a long time, even when I was a child, and being ambiguous, in a way, made me eligible to do this. The new terms Prof. Hughes proposes may be less offensive to genetic females with only slightly masculinized genitalia, and to genetic males with only mildly demasculinized genitalia, who live in the gender role concordant with their genetic sex, but to someone in our situation they are potentially more offensive. Also, his terms sound like they refer only to pubertal effects, not prenatal ones. I have usually seen ‘masculinization’ refer mainly to prenatal development, and ‘virilization’, mainly to pubertal development of secondary sexual characteristics.

The CAIS woman wrote:
My view is that TFS and MPH are outdated as medical terms and are stigmatizing to AIS patients, but I acknowledge that one needs to be able to cross reference newer terms (AIS, intersex etc.) to these older terms. I’d like to see the older terms (if they have to be quoted at all) being relegated to brackets in the main text of any paper/textbook; something like “.... androgen insensitivity (testicular feminization syndrome or male pseudo-hermaphrodite in some older literature)” and being banned altogether from titles, headings, and indexes. It would also be nice if the word ‘syndrome’ could be dropped as this has a nasty ring to it.

Our US representative commented:
I have to admit I don't like the word ‘hermaphrodite’ because it implies an ambiguity not merely in biological sex but also to my mind in gender identity (perhaps I am reading something into the word that is not implied) and I can recall as a child classmates using the word in a most derisive way. There is also the fact that many doctors insist on distinguishing between ‘true hermaphrodite’ [Ref 28] and ‘pseudo-hermaphrodite’ and have become a bit testy if I don't use the ‘pseudo’ prefix in relation to myself. While I might disagree with the theoretical underpinnings which distinguish the categories, or the very need for two categories, for myself I don't feel a keen interest in disabusing them of their categorization when there is so much else about which I wish to disabuse them.

Ref 28: In which there is both male and female gonadal tissue.

The CAIS woman said:
My psychotherapist pointed out today that I seem to favour the use of the term 'testes' as opposed to 'gonads' in talking about AIS – in spite of the fact that ‘testes’ could be construed as stigmatizing in someone with a female phenotype – and yet I am apparently not happy with the use of terms revolving around 'eunuch'. I said that this was because the latter has very negative, freakish connotations except possibly in the world of the much-heralded castrati opera singers. I suppose, if I'm honest, it’s to do with a fear of being somewhere in the middle. Maybe 'testes' is more definite and circumscribed, and less amorphous than ‘gonads’, even though it is in opposition to my phenotype, as it were. I think it’s also to do with the stigma attached to stunted development, congenital abnormality, that sort of thing. To me, the word ‘gonad’ conveys the idea of something incomplete, unformed, and primitive, whereas ‘testis’ suggests a fully-developed organ.

The PAIS woman replied:
I don't like the word ‘eunuch’ either, probably because it implies a sexless person – someone incapable of, or ineligible for a sex life. I think that the precise definition of ‘eunuchoid proportions’ is having the distance from the pubic symphysis to crown of head shorter than half total height (legs disproportionately long) and having an arm span greater than one's height (arms disproportionately long). You know that Leonardo Da Vinci drawing of a man standing inside a square with outstretched arms? I think it is supposed to illustrate ‘ideal’ proportions – arm span equal to height, which is exactly double the symphysis to crown distance. Maybe someone like you or I should think up a better term for the body proportions that result from prolonged growth of long bones than the one derived from the ‘E-word’. I don't see this as that big a problem myself, though.

The CAIS woman replied:
Yes, and we could start by putting Leonardo [da Vinci] straight on a few things ["Man is the Measure of All Things"]. But I really think these book authors should stop wasting space banging on about ‘eunuchoid extremities’ in AIS (stigmatizing, and not generally true – as the members of our group show) and use it instead to recommend truth-disclosure with psychological back-up.

The PAIS woman wrote:
About 'gonad' versus 'testis' – gonad is a respectable, medically accurate word with a clear meaning. I don't see anything wrong with its use as an alternate term for testis in AIS [once testis has been mentioned]. Doctors should, however, realize that there is a big difference between not unpleasantly overemphasizing a fact, and being evasive or even secretive about it. A lot of textbooks don't really make this distinction. If a doctor uses the G-word instead of the dreaded T-word because it seems a bit less emotionally charged, that's okay. If s/he uses it in order to withhold information that the AIS patient doesn't have, or isn't supposed to have, then that's dishonest.

The CAIS woman wrote:
I think that the term ‘gonad’ is accurate/truthful in as much as that in anatomical parlance it is used to cover both ovaries and testes, but in the realms of embryology it refers to the primitive undifferentiated state, prior to the formation of ovaries or testes.

The PAIS woman replied:
Yes, there is a bit of a problem with a generic term like ‘gonad’ – it can be used to make extremely misleading statements which are, nevertheless, literally true. At our New York meeting in Sept ‘96, an AIS woman referred to her ‘gonadectomy’, rather than her ‘orchidectomy’ or ‘testes removal’. I don't think she was being obscure, evasive, or inaccurate [since she knew about, and could therefore equally well have used the word ‘testes’ in relation to her condition]. This is the sort of context in which I do approve of the use of the word ‘gonad’ – when information is not being concealed. I disapprove just as much as you of sheltering patients from the facts of an AIS diagnosis.

I also don't agree with that gynecologist who said, in relation to AIS “.... it is also inappropriate for us to use the word testicles. I think we should use the word gonads [Ref 29].” This particular rationale seems to suggest that since these glands don't function as normal testes do, we can claim that they are not testes. We'll just call them gonads and avoid the whole issue. He is possibly making a case for considering the testes in AIS to be something like the undifferentiated streak gonads in Swyer [Ref 30] or Turner Syndromes, which they are not. Stretching the truth in this way just allows him to mislead without calling it a lie. That does seem to be the problem with the advice in some books to avoid stigmatizing language. It can be misinterpreted to mean avoiding the truth itself. The real reason why [male] doctors worry so much about this whole issue is because of the stigma attached to being a feminine male or a male homosexual. Never mind that this stigma shouldn't apply to AIS women (and maybe shouldn't even exist at all); most men are very uneasy with this.

Ref 29: See “Y Gonads” in ALIAS No. 7, Spring 1997.
Ref 30: See “Swyer Syndrome” in ALIAS No. 8, Summer 1997.

Whatever terms they use, the unspoken message should be ”Your feelings are important, that's why I try to be considerate and tactful” and not “If I said this in plain English, you'd freak out, so I have to use evasive baby-talk instead.” The ideal goal should be for a girl with AIS to assimilate the truth in such a way that none of these words hold any real terror. The whole concept of someone with AIS as a woman who lives a happy life in spite of having testes and XY chromosomes is one I have been able to assimilate in a very positive way. When I read about this for the first time at age 18, it seemed a lot better than what I was going through at the time. That's why I think it can be good for a girl with AIS to learn her diagnosis early. That way she can get the pain and disappointment over with and build a positive self-concept based on reality, not on expectations that will ultimately be shattered. Here is what I found in some new endocrinology texts:

The text by Brook and Marshall [Ref 31] is very condensed and incomplete – more of a study guide than a textbook. It describes CAIS only, and says nothing about treatment. They say that “The patients are tall because they are genotypically male.” [Ref 32]

Ref 31: Essential Endocrinology, Charles G. D. Brook & Nicholas T. Marshall, 1996 ISBN 0-632-03622-2 Blackwell Science.
Ref 32: See articles on growth in ALIAS No. 2, Summer 1996.

Becker [Ref 33] refers to ‘Complete Androgen Insensitivity’ and says that “it may be inadvisable to inform these phenotypic females of their karyotype, because the psychological implications may be devastating.” ‘Psychologically devastating’ seems to be a popular phrase with endocrinologists. In a 1970 article in Clinical Pediatrics, of which I am the subject, it says that my pubertal breast development was ‘psychologically devastating’ [since I was being raised as a boy]. I see this as the rhetorical tactic of substituting intensity of emphasis for persuasiveness of logic. Becker also states that “Occasionally the arms and legs are disproportionately long and the hands and feet disproportionately large.” In relation to PAIS they say “External genitalia consist of .... [and a] perineal orifice that resembles a vagina (i.e. a pseudovagina)”, another example of a ‘pseudo’ word being used in a stigmatizing way. I was very disappointed with the book’s misleading, incomplete and out-of-date information, particularly in relation to PAIS.

Ref 33: Principles and Practice of Endocrinology and Metabolism, Kenneth L. Becker, Ed., J.B. Lippencott, ISBN 0-397-51404-2.

Greenspan and Strewler refer to the ‘Syndrome of Complete Androgen Resistance and its Variants (Testicular Feminization)’ under ‘End organ resistance to androgenic hormones (androgen receptor defects)’. They say that “Therapy .... involves affirmation and reinforcement of their female gender identity” and “.... the individual whose psychosexual gender is dissonant with chromosomal sex need not have any psychologic catastrophe as long as the sex of rearing is accepted with conviction by the family and others during the critical early years.” [Ref 34] It is stated that "In most cases, vaginal constructive surgery is not required."

Ref 34: Not necessarily so. Carried to excess, this may be exactly what closes off avenues for openness, discussion, exploration – and for acceptance/resolution through meeting others – as far as the affected person is concerned.

Cowan and Seifer [Ref 35] refer to Complete Androgen Insensitivity (no ‘Syndrome’) and suggest “Patients and their parents must be counseled regarding the fact that the patient will not menstruate, nor will she have children.” Like most of these books, this one does not straightforwardly and explicitly confront the question as to whether the patient should be told the complete truth about her AIS. It covers vaginoplasty in the section on Rokitansky Syndrome, saying that “The gold standard for surgical correction of vaginal agenesis is McIndoe [skin graft] vaginoplasty.” I would call it the baseline, not the gold standard. They also mention newer techniques such as tissue expanders in the labia minora to create skin for lining a neovagina [Ref 36], and the Vecchietti method [Ref 37]. They recommend that “In patients with a vaginal depth of 3-4 cm, dilation therapy is the treatment of choice.” They also mention PAIS, saying that it is 1/10th as common as CAIS, but say nothing about therapy or management.

Ref 35: Clinical Reproductive Medicine, Bryan D. Cowan, David B. Seifer, Lippencott-Raven, 1997 ISBN 0-392-5486-8.
Ref 36: This tissue expansion method was pioneered, we believe, at Leeds General Infirmary, by Prof. Richard Lilford (now moved to West Midlands NHS Executive).
Ref 37: See “Vecchietti Video” in ALIAS No 5, Summer 1996, “Vecchietti Re-visited” in ALIAS No 6, Winter 1996, "3rd US Meeting" in ALIAS No. 13, Autumn 1998 and "A Vote for Vecchietti" in ALIAS No. 14, Spring 1999..

The section on AIS by Gooren, in Bardin’s book [Ref 38], had the best and most complete information (which is not saying much). It uses the term ‘complete androgen resistance’ and advises that “It is a good approach to unfold the truth stage by stage” and that “.... information should be explained to the patient .... using an age-appropriate explanation of the syndrome and treatment plan.” He says that “Further psychiatric consultation may be appropriate in situations in which the family or the patient has severe difficulty adjusting to the diagnosis. In this era patients have access to their own medical files, or they may turn to other sources for information on their syndrome. For those it may be better to learn the facts .... from their physician than from their files or from a medical encyclopedia.” While he recommends truth disclosure, the author seems to have some reservations about this (he says that “No reference should be made to the gonads as testes”) and advocates a very cautious and tactful attitude. He says “Undue and severe psychological trauma may be produced by the physician who informs the patient that she is, in fact, a male or has sexual characteristics of both sexes.”

Ref 38: Current Therapy in Endocrinology and Metabolism, C. Wayne Bardin, M.D., Mosby, 1997, ISBN 0-8151-2016-8.

He also says that “Some have advocated an early removal of the testes on the grounds that it is easier to obtain the parents' consent early in life than to have to confront the adolescent child with removal of her gonads.” One might argue that this advocates compromise of medical decisions for the sake of diagnosis concealment which is ethically really questionable. There would be less of a problem here with a fully-informed patient. On vaginal hypoplasia he recommends that “Dilation is the intervention of first choice.” Surgery is said to be needed in rare cases. About pressure dilation, there is also this coy remark that “Psychosexual counseling is needed during this period, as .... the girls may equate the procedure with masturbation, particularly when the dilator is obtained from a non-medical source.”

The CAIS woman asked:
Do you mean that perhaps she should be told it’s OK to have a bit of fun as part of the ‘treatment’ and thus increase her motivation to continue with it?

The PAIS woman replied:
No, I was just amused at the way they went round the houses to avoid using the word ‘dildo’.

Bardin also covers ‘Partial Androgen Resistance’. Overall, the AIS coverage is comprehensive, accurate and informative. What he says about truth disclosure is generally very sensible. This is the book I would want my endocrinologist to read.

Contd. below......

General ALIAS Refs:
See also “Classify, Classify” in ALIAS No. 4, Spring 1996.

Hearing the Words

The CAIS woman wrote:
I've seen suggestions that before a certain age a child cannot think conceptually and that there's no point explaining complex medical conditions in sufficient detail to qualify as 'truth disclosure' until the person has, say, covered biology/genetics in school classes. It seems sensible to plan truth disclosure to take account of such developmental milestones?

The PAIS woman responded:
I think that even a young child could assimilate the full truth about an AIS diagnosis if it was presented the right way [Ref 39]. Most children don't have enough background information about biology and genetics, so this would have to be provided also, but it could be done. Maybe in some situations, making a later age of disclosure an ironclad law could be harmful. I would guess that full disclosure by mid teenage is about right most of the time, but the child should get some information [about their condition] before then, integrated into the sex education that any child should receive. There shouldn't be any one big moment of revelation. However, this question should be decided empirically, not by armchair speculation.

Ref 39: See also last paragraph in “Period Pain” in ALIAS No. 8, Summer 1997.

Some of the quotes from your bookshop survey about psychological counseling are kind of interesting, notably the one from Grudzinska and Beedham about “counseling and support advised for .... partners in later life”. It must be really nice to have a physician who is so helpful and deeply involved in one's life that he'll take care of explaining things to your boyfriend! My impression is that, in real life, they don't even have the time to explain anything properly to parents, or to patients themselves. Anyway, by the time someone is old enough to have romantic partners she should long since have been provided with the words she can use to handle these situations herself. Instead, most of these books advise keeping the patient in at least partial ignorance, which makes her dependent on the ‘counseling and support’ they recommend, but which few doctors are actually ready to supply.

From the patient’s point of view, the best option is not to have a life so messed up that one needs support in order to face one’s ‘special situation’. I feel that such ‘supportive’ counseling is a waste of time, and that counseling should be businesslike and oriented towards providing information, not ‘hand-holding’. What I believe in is for a counselor to promote the patient's self-reliance and ability to cope with whatever situations he or she will encounter. They can do this best by providing accurate and complete information about his or her condition and treatment options, and about sex in general. Unfortunately, most counseling professionals are not geared to do this. Child clinical psychologists with specialized knowledge of AIS and related conditions, and of sex problems and their treatment, are very rare.

Also, there is a taboo on talking about sex with children, and people rationalize keeping them in ignorance as being necessary for their emotional well-being, when, in fact, it is more likely to be harmful. Counselors are unlikely to get into talking about erotic arousal, and sensation or orgasm with a child. They might fear getting into trouble with the parents or hospital authorities, or even being prosecuted under laws intended to protect children from pedophiles. Even when I saw counselors as a young adult (aged 21-22), I perceived an attitude of prudish avoidance of these topics on the counselors' part. It seemed this way to me even though I was shy and puritanical myself.

The current approach seems to be to avoid dealing with sexual problems until the person becomes an adult, even if the problem has by then become more complicated. One could call this protecting children from what they are not yet ready for, but one could also call it shrugging off a responsibility so that someone else can deal with it later. I think I've mentioned before that some of the overemphasis on gender identity in such conditions possibly stems from prudish avoidance of the real issue – sexuality – in dealing with a child.

The CAIS woman responded:
By ‘overemphasis on gender identity’ do you mean the ‘mythologizing of CAIS by overemphasising the patient's femininity’ that we discussed on a previous occasion [Ref 40], i.e., the way in which medics/parents throw up a smoke-screen of gender identity rhetoric/reassurance in order not to have to talk with youngsters about sex/sexuality?

Ref 40: See “MIF Matters” in ALIAS No. 8, Summer 1997.

The PAIS woman replied:
What I meant when talking about ‘mythologizing’ etc. was just that there does seem to be this idea going around that AIS women are more attractive, and behave in a more feminine way than other women. Whether true or not, I think that these ideas are popular because a) they make the syndrome seem more remarkable and b) they reassure those people who are uncomfortable with the idea of an XY woman. But what I’m saying here is that by making gender identity the justification for whatever management/treatment is applied to a child, doctors can talk in terms of the existence of, or the achievement of cosmetic normality – whether the child looks, or can be made to “look normal, like other boys (or girls)” – rather than having to talk in terms of ability to have sexual intercourse.

Perhaps I feel this way because of the frustrating time I had with my counselors. I told them, truthfully, that my goal and purpose in changing to be a girl was to end up having a sex life with a male partner. I didn't care a lot any more whether I was supposed to be a girl or a boy, except that I thought my life would be simpler, and my goal easier to achieve, as a girl (I think it has been). They seemed to feel this was the wrong reason for a gender role change, and that a better reason would be a deep desire welling up inside me to wear dresses instead of pants[trousers], or something similar.

I have a bit of a grudge against the whole profession of clinical psychology. The counselors I saw a couple of times as a child didn't even try to provide the kind of information about my condition that might have done me some good. When I was a young adult, though, after I had done all the dirty work of gathering information and figuring out what my options were, they were full of suggestions and advice (lots of it bad). However, there is one member of this profession whom I do respect and admire – John Money. When I was 22, and deciding to transition from male to female, I got a clandestine look at my male PAIS cousin's medical files. I had time to read the whole thing, including the verbatim transcript of John Money’s interview with my cousin in which he told him everything about intercourse, erotic sensation, homosexuality, transsexualism, etc; even that, as a male, one of his options would be to use a strap-on artificial penis. All this, translated into language an 11-year-old could understand.

In several of his books [Ref 41], Money mentions the need for counselors who identify themselves specifically as pediatric sexologists, and who specialize in the sexual problems of children. The most important qualification for someone who specializes in sexological counseling is complete and detailed medical knowledge [Ref 42]. I used to hear, in response to the same inquiry, “I'm an endocrinologist“ (meaning “therefore I'm not the one to ask about your social problems”) and “I'm not an endocrinologist” (meaning “therefore I don't need to know about the details of your syndrome”). It would have been helpful to have had a counselor who was, in a sense, also an endocrinologist. I do have to comment that my endocrinologist was better at counseling than my counselor was good at endocrinology.

Ref 41: Many of John Money’s books on sexual development/problems are written for a wide readership. His recent books are available from Prometheus Books, 59 John Glenn Drive, Amherst, NY 14228-2197, USA. Tel: (716)-691-0133 or (800)-421-0351, Fax: (716)-691-0137. Email: PBooks6205@aol.com. Ask for catalogue. See also “Books” in ALIAS No. 1, Spring 1995.
Ref 42: John Money recommends that “Children with birth defects of the genitalia need, in the language of childhood, a cutting doctor, a needle doctor and a talking doctor. The talking doctor may be either a psychologist or psychiatrist (provided he or she has specialty training in sexual medicine and psycho-neuroendocrinology) whose services should be available on demand throughout childhood and adulthood.” Money J: Psychologic Considerations in Patients with Ambisexual Development. Seminars in Reproductive Endocrinology, 5:3, August 1987.

The CAIS woman wrote:
I couldn’t agree more. I think my life too could have been very different if I'd had the sort of early frank explanations and open discussion that Money advocates. This is one the aspects of his published work that I particularly liked when I first came across it a few years ago (have a look at what he says about ‘pairbonding’, in “Psychological Support” in ALIAS No. 1, Spring 1995). My prospects/options for sex and pairbonding – with a 1 cm vagina – were nowhere near the agenda of those who investigated/cared for me, whereas it has always been my number one issue/anxiety. It has always affected my female identity, and core sense of who I am, far more than genes/gonads/infertility, which is what everyone around me was probably worrying about.

I too believe that medical knowledge is important in a counsellor – to be able to understand the links between mind and morphology, as it were, although a few others with AIS I’ve spoken to disagree, saying it’s all about feelings and that medical knowledge is not relevant. I guess they would argue that in a case of CAIS there is usually no wish to change gender role, so the medical/surgical aspects of that do not apply, and the phenotype is already female – so why would medical knowledge be important? I would argue that this is ignoring the internal anatomical situation and the important question that might be in the mind of a CAIS youngster as to how she came to be as she is, or even in some cases “How am I supposed to be the 'normal woman' everyone is telling me that I am, if I can't have intercourse due to the vaginal hypoplasia which no-one is willing to acknowledge and discuss with me.”

Girls with vaginal hypoplasia have a lot of anxiety about how well-equipped they are for a sex life. They have no reference point, no standard, against which to evaluate their status (since, unlike with boys, the normal range is not visibly discernable) and they need a lot of motivation and support if they are to use pressure dilation as a vaginal extension procedure. I think that properly trained medically-aware psychosexual counsellors or ‘sex therapists’, who can talk about the mechanics of sex, are needed here. Also, how could one discuss with a counsellor one’s feelings about the stigmatizing terminology, or what terms to use in explaining things to others, if they are not familiar with the medical concepts and jargon themselves?

The PAIS woman wrote:
A textbook that I referred to earlier [Ref 43] said that “Additionally, with the parents out of the room, it is important to discuss the patient's sexual history with her. Often, these girls have attempted intercourse and the results have been so dismal that their ability to function sexually may be impaired. Patients should be informed that there are several methods, both surgical and non-surgical, which can be used to create a vagina and that she should be able to achieve normal sexual function.” It's good that the author recommends addressing this issue, but I think there is a better way to go about it. If I were the physician, I'd say something like: “Another girl I know with the same condition as you decided to have intercourse with her boyfriend, but it didn't work. She was very embarrassed, and afterwards she worried a lot about her vagina, until I told her about the different ways of treating this problem, which I'd like to tell you about right now.” In this way, the doctor can convey all the information the patient needs to know without having to make her feel like a political prisoner under interrogation. This is what Money calls the 'parable technique'.

Ref 43: Bryan D. Cowan and David B. Seifer: Clinical Reproductive Medicine, Lippencott-Raven, 1997, ISBN 0-392-5486-8.

Another issue is the way counselors tend not to volunteer information or bring up a topic unless the patient has already mentioned it. If the patient thinks a topic is taboo, and that the counselor is avoiding it for this reason, then communication about that particular thing is just not going to happen. I was recently reading a book chapter by S.W. Baker on ‘Psychological management of intersex children.’ [Ref 44] He says, “The first step is to discover what the child already knows.” I see this as a somewhat flawed approach, because the concerns which a child is likely to avoid bringing up may be the very ones where help is most needed.

Ref 44: In Natalie Josso: ‘The Intersex Child.’

The CAIS woman wrote:
I was in exactly that situation, of feeling that everything about my condition was far too taboo to bring up voluntarily – with anyone, even my parents. And if I’d ever had the luxury of a psychology professional asking me what I knew, I would have completely clammed up. After all, I‘d had to find out all the most significant aspects of my condition on my own from books (XY chromosomes, testes, no uterus/vagina, ‘infantile nipples’, ‘eunuchoid proportions’, ‘intersex’, ‘male pseudo-hermaphrodite’, ‘hairless pseudo-female’, ‘sexually infantile’ etc. etc; and the whole mechanism of how it all happens – no doctor ever tried to explain that to me). I’d started by looking up ‘hernias’ in my parents’ medical dictionary when I was young enough to need to stand on a high kitchen stool to reach the bookshelf, and ended up diagnosing myself as having testicular feminization syndrome in my mid teens, a number of years in advance of my diagnosis by a doctor.

The mere fact that I was forced to do this on my own told me that it was all far too much for the adults around me to handle and that dialogue would thus be completely out of the question. I felt compelled to protect them, by not confronting them or letting on that I knew all these things. Whilst I had read all those emotionally-loaded and stigmatizing words in secret, and knew that they applied to me, I had never heard anyone speak these awful words (and knew I never would, since they were obviously part of a secret language shared only by doctors) never mind uttered them myself. I had neither voice nor vocabulary. So I kept quiet, even after my diagnosis; in fact for 30 years or so. It really was the ‘unspeakable monster’ and the ‘elective mutism’ that Money describes so well.

Contd. below......

General ALIAS Refs:
See also “The Taboo on Talking” in ALIAS No. 1, Spring 1995, “A Search for Support” in ALIAS No.4, Spring 1996, “Vorsprung durch Technic?” in ALIAS No. 7, Spring 1997.

Saying the Words

The CAIS woman wrote:
It’s almost unbelievable that in middle-age I should find myself agonizing over what, if anything, to tell my first ever real boyfriend about my condition. And that someone with whom I was emotionally involved had to be the very first human being to whom I’d ever articulated the words; and so late in life. In the emotional breakdown precipitated by my eventual disclosure of the truth to him – some two years after the early failure of the relationship – I also found myself bringing up the subject of my medical condition with my parents for the first time, only to find that my father couldn’t understand the medical basis of it at all and my mother wouldn’t even try, still refusing to acknowledge my condition as being anything other than a fertility problem.

After another 4 years, in which I’ve had some counselling/psychotherapy, I now feel as if I’ve just started to crawl towards the walls of the deep pit that all this landed me in at the age of 10 or so, but it’s really too late to have had any significant effect. And I have to say that by far the most powerful agent for self-acceptance has been the support group meetings where I have finally realised that I am not a one-off, an isolated freak of nature. I have always felt very strange indeed in the days immediately following each gathering (kind of ‘grounded’ and more integrated, and yet also elated). Nothing that has ever passed between a therapist and myself has had such a profound effect.

Then, on being invited to make a presentation at a medical conference overseas, I had many sleepless nights. Not so much over ‘revealing all’ to a crowd of clinicians (although that prospect was bad enough) but more over whether, what, and how to tell an old and trusted friend from university days, with whom I might stay, about the real reason for my visit (and for not having had boyfriends, for not having got married etc., etc.). I was trying to tell myself it was an opportunity to practise opening up to someone and trusting them with the information, but I was then to hear my mother anxiously advise “well, all you say is that you couldn’t have children and that you’ve had a hysterectomy.” How can I get through to her that infertility has been the very least of my problems/issues and that I’ve never told anyone that I’ve had a ‘hysterectomy’, and never will – because it’s just not true – and I’m fed up with all the half-truths, and cover-ups, and denials, and pretending that I’m something I’m not.

At a time when my contemporaries are in a third stage of life, handling their problem teenagers, I’m only just starting to deal with existential and inter-personal limitations that are far more fundamental than the question of whether or not one is able to have children of one’s own, and for which a personal survival strategy should have been sorted out, and rehearsed with non-critical people, when I was a teenager myself.

I recently came to the realisation that because I’d never actually had the wherewithal to say the words “I have AIS” out loud in front of another human being, or to admit to having had testes, I’d never been able to claim ‘ownership’ of my condition. Well, I might perhaps have mumbled my diagnosis incoherently, fighting back the tears, when occasionally in adulthood a new doctor, surrounded by an army of students, would ask “Well what do we have here then, perhaps you’d like to tell us about your problem?” but I had never actually articulated the words spontaneously and clearly, and felt reasonably OK about it – until the third support group meeting in March 1996. This was a very momentous event in my life. I was in my late 40s.

AIS appears to be a clinically well-known, albeit rare condition and appears in many textbooks. Many describe it, accurately, as a failure of male development yet wax lyrical about the supposed super-female qualities of such patients (conveniently forgetting at that moment about her lack of internal organs) and advocating secrecy and deception; so that it is, in fact, a myth that is broadcast to the lay public. One or two authors employ the quite reasonable label ‘XY woman’ but few doctors will use this term in front of parents/patients. Patients are left with no vocabulary with which to acknowledge and talk about their condition beyond the over-worked, simplistic and inaccurate notion of ‘a normal female with a fertility problem’. The condition thus has no validity in the public domain, it is invisible – with many doctors/parents pretending that it doesn’t exist.

I’ve just filled out a questionnaire to take part in an HRT clinical trial. I decided to answer the questions as if I was a regular XX post-menopausal woman, and answered “No” to “Have you had a hysterectomy?” [Ref 45] and to “Do you have any medical problems/conditions?” and I made up a fictitious date for when my periods stopped. I decided that if doctors can keep telling me I’m just a normal infertile woman then I can claim the same when they are seeking important information from me.

Ref 45: This is a truthful answer. It was a gonadectomy, or more specifically, an orchidectomy that I had, aged 24, and not a hysterectomy.

The significance of all this hit me fully a year later, when I started re-visiting the stigmatizing terminology in the current medical textbooks. By writing in this way about patients behind their backs (or so they think) doctors keep the ownership of the condition, and the language used to describe it, well and truly in the clinical realm and stop it being acknowledged in the eyes of society as a condition that actually occurs as a natural phenomenon. The immensely stigmatizing terms – probably handed down from one generation of textbook-writing doctor to another, with no thought as to their real value or that they might describe real people – were a very large contributing factor to the intense shame and self-hate I have always felt since first reading them in my early teens.

One of the support group’s UK members quoted Cheryl Chase [Ref 46] as having talked about “.... the pattern in which no individual who is able to talk back to you can be labelled a hermaphrodite [Ref 47]. Hermaphrodites are allowed to exist only in the abstract, as objects, not as subjects.” The point being, the UK member surmises, that the infant, who cannot talk back, is an object of interest and perhaps of intervention rather than a subject in all of this. And that when a doctor is confronted by an older individual who is able to talk back as a subject, there is a propensity to deny that the individual concerned is a hermaphrodite or intersexed, even if the medical records used from the infancy of the person employ these terms!

Ref 46: Founder member of ISNA (The Intersex Society of North America), PO Box 3070, Ann Arbor, Michigan 48106-3070, USA. Tel: (734) 994 7369, Fax: (734) 994 7379, Email <info@isna.org>.
Ref 47: Using the term ‘’hermaphrodite’’ as synonymous with ‘intersexed’.

In a summary (sent to ISNA members) of ALIAS No. 6, Cheryl says: “Several letters criticize physicians' refusal to consider vaginal depth as a problem. One institution produced a guidebook for parents of girls with Complete AIS which emphasized, “.... The only major problem these patients face is therefore the relatively common one of infertility.” In fact, an AIS specialist who spoke at a recent AIS Support Group meeting assured the AIS women and their families that “Patients with CAIS are not intersexed.” [Ref 48] Intersexuality seems to be like the Cheshire cat where physicians are concerned. They find the subject fascinating, the patients most interesting, the presentations bizarre. But whenever a physician addresses an intersexual or a family, he will assert that the person addressed is not intersexed. It is our impression that medical definitions of intersexuality are constructed with the specific purpose of defining it away.”

Ref 48: See “External Emphasis” in ALIAS No. 6, Winter 1996. This specialist in fact writes about CAIS in a paper entitled ‘Male Pseudo-hermaphroditism: Clinical Management, Diagnosis and Treatment’ (Hormone Research, 38 (suppl 2):77-81, 1992).

I feel that I have indeed suffered, as a result of a denial, or ignorance, of the intersexual aspects of my CAIS on the part of those who cared for me. There was so much emphasis on normality based purely on external appearance – and certainly the term ‘intersexed’ was never spoken, to me, or to my parents.

The PAIS woman asked:
Do you mean that you would have felt better if you had conceptualized what was wrong as an intersex disorder, rather than viewing it as just being an ‘imperfect female’? As offensive as some may imagine the term ‘intersex’ to be, I know of about a dozen people who want it to be applied to them, but not a single person to whom it could apply who doesn't.

The CAIS woman replied:
I conceptualized it as an intersex condition from a very early age, but the only way my parents have ever conceptualized it is as ‘an infertile but otherwise normal female’. My mother has always freaked out and refused to listen, at the mere mention of chromosomes or abnormal development or anything like that, so we could never talk – about any aspect of my condition. It's the way my parents have always denied reality, partly through ignorance due to the fact that the situation was never explained properly to them (or if it was, they didn't take it in) and instead were brainwashed by the medics' ceaseless exhortations about how normal I was, or would be, as a female. A separation of CAIS from PAIS in the medical literature – as if they were totally unconnected disorders – also helps, I think, to foster this notion that everything's OK if you look OK on the outside and that CAIS has nothing to do with intersexuality.

I think it’s time we all developed some interest, involvement, and perhaps pride, in our true status, and decided what terminology we want to be used. I want to bring the current terminology out into the open, and to empower affected people to acknowledge that these terms are being used in the literature and in their medical records to describe them behind their backs, but with a lot of footsying around and paternalism being advocated, when it comes to doctors talking face-to-face with patients/parents. We need to decide whether or not this is appropriate. I want to encourage written material like ALIAS that blurs this linguistic divide between doctors and patients. I know of several AIS women who have booked extended appointments with their doctors to go through their notes and ask for stigmatizing terminology to be changed. We need to take responsibility for our own bodies and collaborate with the medics in writing the user manual in plain English.

We asked our US representative if she thought we might cause offence with these 3 articles. She replied: I think this is brilliant! The reason I'm so keen on it is that I know for myself that [now] each time I hear myself talk about things it takes some of the sting out of it, and it is the history of having said it before that makes it easier to say it again. Having a lexicon to express one's identity, and becoming comfortable with such expression is, to my mind, a critical component of the healing process. As I've told you many times, my favorite quote from ALIAS No. 1 was the statement about “saying out loud the words that you thought would remain locked inside your mind, circling in your head forever”. That one passage provided a cathartic self-awareness. When I first encountered the passage it so perfectly articulated exactly how I felt that I cried from a place so deep inside that I never knew it existed.

Dr. David Grant commented:
Although deep debate on historical terminology can be very confusing, I think it is great that you are exploring the available vocabulary. One of the great problems is that there are no good terms which patients can use to describe their medical problem to their family and friends, and as a result it gets swept under the carpet.”

The PAIS woman commented:
I use ‘ambiguous genitalia’ and ‘AIS’ to describe my medical problem to family and friends, and that seems to work pretty well.

General ALIAS Refs:
See also “Period Pain” and “Too Explicit for Words” in ALIAS No. 8, Summer 1997.

AISSG