Truth Disclosure Discussion

This is a discussion that was published in issue no. 12 (Summer 1998) of the UK support group's newsletter, ALIAS:

Our Grade 4/5¹ PAIS 44 year-old woman member in the US (who lived as a boy from age 3 months until 22 years) emailed to the AIS Email Circle run by the US support group:

Ref 1: See “AIS Clinical Grading Scheme” in ALIAS No. 6, Winter 1996.

In February I wrote to my pediatric endocrinologist. .... the one who originally diagnosed me with PAIS as an infant, and who continued to treat me until around 1990. I wrote, in part:

In July of 1996 I got in contact with a support group for women with AIS. I was one of 21 people (10 with AIS) who attended the support group’s first North American meeting in New York, in September of 1996. No one there besides myself had a history of a reassigned gender role, but many attendees did feel that their medical management had not been ideal. A very common complaint was having been told, as it was termed, ‘The Story’ (of ‘twisted’, ‘prolapsed’, etc. ovaries), instead of the truth, which patients often discovered on their own, often in a medical library. I remember you once told me that you thought it was best for an AIS girl not to know the complete diagnosis. Well, this was the ‘number one’ complaint.

He replied, in part:

One of the reasons it is not only of interest, but useful for the pediatrician to keep in touch with his former patients is because as adults they are better able to articulate how he or she could have made life better for them. Support groups are good in a variety of ways. They let people with rare disorders know they are not the only ones. They are informative to the physicians who often must make decisions with lifelong consequences at a time when the patient can have little input. I think I have learned to be more truthful as a result of recognizing that lies backfire and, in the end, do patients and their doctors more damage than the truth.

A question I would have for you however, is when should a physician discuss with an AIS child how things came to be the way they are? Ideally, of course, the pediatric endocrinologist gives an understanding to the parents, who in turn pass it on in bits and pieces as the questions come along, and the child develops the intellectual capacity to understand and assimilate the information. Parents, however, often abdicate the responsibility and ask the pediatrician to take it on....

Perhaps, when I write back to him, I could let him know how you feel about this and what your experiences have been.

A CAIS 49 year old emailed:

Well, two of us had a discussion, following a group meeting, in which we observed that those women who found out later (e.g. in their teens through failure to menstruate) seemed, as far as one could tell, to have a better psychological outcome than those in which there had been knowledge of their problem in the family from an early age, with mysterious childhood operations, opportunities for long-term parental anxiety, cover-ups etc.² In other words, the sudden ‘in your face’ revelation, in teenage years or later, although producing a period of intense pain, anger, confusion and grief, was at least happening after the person had, with any luck, developed a core female identity and they hadn’t spent the most formative years of their childhood suspecting there was something wrong with them that was so awful and taboo that none of the adults were prepared to acknowledge and talk about it. The reasoning was, I think, as follows. Consider three situations A, B and C:

Ref 2: See also “Key Issues for AIS Women” in ALIAS No. 5, Summer 1996.

Scenario A) The parents get clues of 'trouble' (e.g. hernias) or even a diagnosis, when their offspring is an infant/child. The doctor perhaps doesn't explain things properly, thinking “Well the parents don't need to know much at this stage; it'll be some years before the child's life is materially affected by the condition – another doctor can explain more at that stage.” Or else the parents are given some information and latch onto threatening words like 'gonads' and 'chromosomes' and become haunted by them without really understanding the full story.

The result is a) confusion in parents' minds, b) irrational fears of future – will she grow a beard, or a hairy chest; does 'plastic surgery' mean she'll have to wear a hollow plastic tube to have sex (yes, parents have apparently expressed these anxieties to the support group), what the hell are we going to tell her when she grows up, and how? c) great potential/opportunities for denial, cover-ups, secrecy by parents. The child picks up on all this and starts having self-doubts – or even serious gender anxiety/confusion if they start consulting medical books in their teens and start getting more information (the famous ‘male pseudo-hermaphroditism’ thing etc.). The parents may let things drift for many years, e.g. until the late teens, not wanting to face reality.

Scenario B) The second scenario is one where there is no sign of a problem until the teenage years when no periods start. The parents would usually get this checked out and parents and daughter would, one hopes, learn the full facts more or less together (assuming the doctors/parents decide to tell the girl the truth). This is a good scenario, in as much as any AIS scenario is good. The girl’s childhood up to that point has been unfettered by doubts and menacing ogres. She has grown up with a pretty secure self-image as a normal girl. Parents and daughter, with any luck, are now 'in it together' and have to talk. The teenager's success in establishing relationships, from now on, perhaps depends to a large extent on whether she now has a chance to address, and practise talking about, the anxiety-provoking aspects of her condition with her parents, the doctor/s, and hopefully a psychologist/counsellor (and to be guided on what, and how, to tell others – friends and partners) and above all, to meet other affected girls.

We observed that at group meetings the women who'd experienced situation (B) seemed to be much more comfortable with things compared to (A) and were much more willing to speak out and acknowledge their condition in public, and were generally more secure in their identity as 'normal' women.

Scenario C) A third scenario is that the parents are told the truth by the doctors at teenage investigations but the daughter is not told the full story – but perhaps some 'twisted ovaries' yarn or that her ovaries/uterus “just didn't develop properly” or whatever. The patient perhaps wonders about this but puts it to back of her mind and goes on to have relationships, is possibly quite promiscuous (perhaps doubting something about her femininity and seeking reassurance), maybe gets married, but then finds out the truth later from other sources. At this point there is huge potential for anger at the fact that she has been lied to, but her sense of her female gender identity should be pretty secure (perhaps the most secure of all the three scenarios). She has grown up believing herself to be a woman and has in many respects proved this to herself as far as relationships go. At the point of learning the truth she is now a mature woman and perhaps able to integrate it all into her psyche more easily than she would have done at say 15 years-old – albeit with a period of very intense emotional upheaval and pain and anger at the deception and the lost time in which she could have come to terms with things.

But in cases (A) and (C), the patient is prevented from assigning some meaning to her ‘inexplicable’ condition until much too late. The common feature is the shear isolation of either a) knowing why you are different but being too ashamed and stigmatized to speak about it, or b) not being able to find out why you are different and living your whole life with doubts and suspicions.

I would hazard a guess that finding out the truth in teenage years rather than later in adulthood is also much better from the point of view of parental acceptance because they would be learning about it at the same time as their daughter and from the same source, so in theory the door would be more open for dialogue thus reducing the difficulties in getting older/elderly parents to face things later for the first time. But the main consideration must surely be that the risk of a girl/woman finding out the truth on her own and unsupported, from books, magazines, TV, the Internet etc., is just too great – and the consequences too terrible (especially for a girl in her early teens) – that parents/doctors have a fundamental duty to do the decent thing and explain everything, with proper psychological back-up and an opportunity to meet other affected people. And putting secrecy/disclosure/support aside, the over-arching factor in establishing a normal female gender role must be the degree of vaginal development and the extent to which any serious deficiency is recognized and treated.

I just know that I started wanting to know what the hell was going on very soon after my parents told me 'something' (unspecified) might be wrong when I was 10 or 11, and I started poking around in medical books in my early teens. After all, that is when things are happening to one's peers that do not appear to be happening to us, so I think really, the earlier the better.

I heard that the mother of a 7 year-old, just diagnosed, recently contacted the group. And as a result of this, the child’s 26 year-old maternal aunt has suddenly realised the reason for her own infertility etc. Is it just me .....but I think this is really awful, that a grown woman should only find out her hidden diagnosis, in a second-hand roundabout manner, as a result of a young niece being diagnosed. It makes her doctors look very weak and lily-livered. I think that the possibility of this scenario alone calls for truth disclosure well in advance of adulthood.

A CAIS 37 year-old, who only found out the truth a couple of years ago, emailed:

I am not sure that an adolescent discovery makes things any easier for the AIS patient, at least not in my case. I don't see that my parents learning of this when I was 16, instead of when I was two (when the doctors ‘repaired’ my hernias by putting my testes back inside), helped me in anyway. It caused as many lies and as much denial in my family. I grew up as alone and frightened and it took me eight years of trying, and the threat of a lawsuit, to get my medical records. No one in my family was going to come forward with this information. They have made great strides since, but would have been very content to leave me wondering and wounded. My Mom is convinced that I couldn't have handled the truth at 16. As evidence she points to the suicidal depressions I suffered in the years following. I have said that I think they were more related to being totally isolated and not allowed to talk about anything – not even the parts of my condition I did know about (infertility and no periods). She is still shocked that as an adult I have learned the full truth and gotten through it without killing myself.

I had two hernia repair operations as a child and a seriously foreshortened vagina (2.5 cm). My father’s family physician performed a pelvic exam on me at 17 .... and told my father I would never have sex without surgery and my father told my mother this, but no one ever thought to tell me. At 17, I inserted a tampon to try and control the mess of using Premarin [vaginal] cream and two-thirds of the tampon didn’t go anywhere! At 19, my first boyfriend and I tried unsuccessfully for a year to have intercourse, all to no avail.

A CAIS 65 year-old emailed:

It is good to hear the doctor raising the questions. I truly believe that doctors, as he says, should advise parents of the diagnosis in the best way he or she can – keeping in mind the shock component and their need for both time and trained counselling help. Then I feel that the doctor must pass the task of telling the child on to the parents. [For the doctor] to agree to tell the child is taking on the 'God-Like' quality too often demanded by society. Parents need lots of help to handle this task but just as the child is going to have to handle her life – so does the parent. For a doctor to agree to take on the parent's task is saying “yes this is too difficult a problem for you to handle”. The psychological effect passed on to the child is terribly debilitating. I know, because this happened to me, and it has taken me a lifetime to get over it.

The CAIS 37 year-old emailed:

_____ [CAIS 65 year-old], I think that what you propose is an ideal world. I think that the more important issue is whether or not the AIS patient gets the full information. If it comes from parents, so much the better, but it must come from someone and we have seen far too many parents drop the ball to blindly trust them with this vital task. Is it true now that informed consent laws apply even to minors? I have been lurking on an ob-gyn Internet bulletin board for physicians. One has treated two unrelated teenage girls with XY karyotypes whose parents have ‘chosen’ not to tell their daughters the truth. It's disgusting that this is still going on.

The CAIS 49 year old emailed:

I agree, and I think that AIS is so complicated biologically and psychologically that it is too risky to leave it to parents to explain things on their own. My parents just never grasped the biological facts or sought to inform themselves, never mind thought about them, or reached any sort of understanding or digest (beyond the concept of ‘a normal girl with a fertility problem’) that would have enabled them to help me (and I was far too stigmatized by my own independent discoveries and, above all, by their silence on the matter, and the obvious discomfort of the medics, to ask any questions at all).

I feel that at the time of diagnosis, the health care professionals must fully educate parents (and daughter, unless an infant/young child at the time), and provide professional psychological counselling, so that if the parents can’t or won’t get to grips with it – scientifically/medically and emotionally – then at least the child can be rescued from this nightmare situation, and be given a space in which to explore things with a professional, because if her doctors/parents don’t explain it all to her in detail (and they all talk about it until they’re blue in the face), she is going to find out all the biological facts anyway (or worse still, bits and pieces, and strange, stigmatizing terms) from sources like medical libraries and the Internet, and will be completely on her own with it.

The CAIS 37 year-old emailed:

This one frightens me a lot and I still see it amongst the parents at our support group meetings. For example, my cousin's AIS daughter is only a few years old so she has a couple of years before she has to deal with this. I wish she would use this time to process her own feelings/emotions on AIS. Until she does, she might have the medical information down, but she won't have the capacity to help her daughter emotionally because she isn't through with her own emotional denial and subsequent grieving. How can a woman who says that she “would have killed herself if she couldn't have had children” help her daughter whose problems begin with infertility and go on from there?

I have privately asked parents what their reaction is to their daughter having AIS and I have repeatedly heard things like “well this doesn't mean anything to me, but I know that it will mean a lot to my daughter.” I think they are afraid that admitting that they have any ‘negative’ emotions to this will mean that they love their daughter less or think less of her. And I think “bullshit! I can think of lots of emotions they might have over this”:

Anger, because this happened to their child and no parent wants a birth defect and a more difficult than normal life for their child.

Guilt, because they may have ‘passed this on’ and possibly some sort of survivors’ guilt because they escaped (were fertile).

Sadness, because they wanted grandchildren through this child .

Fear, because they don't know how their daughter will handle this.

Frustration, because they can't make this go away.

No birth defect happens to just one person. They affect entire families and the whole family needs help getting through this.

The CAIS 65 year-old emailed:

The doctor should use all the help available from associate professionals particularly those extensively trained in counselling (which most doctors are not) then, with a team approach, help the parents understand they are the centre of the team and sooner or later, as the child grows, she will be that centre, together with counsellors (social work, psychology, and perhaps occasionally, psychiatry) nurse, surgeon, family doctor, and any other professional the family wishes to consult. This all takes time, discussions, and especially listening; listening to hear what the parents are really saying, giving them a chance to verbalize their fears, express their anger, grief, all the stuff so they can move on, just as their child is going to have to do – move on and deal with life – well supported.

A Grade 5 PAIS 44 year-old joined in:

Parents are the big aspect here for me. My mother, if she had the right information from my doctor, could have properly discussed the issue with me. My father, unfortunately has never been able to come to terms with my PAIS. With the proper coaching a parent should start easing the way to full disclosure by 10, 11 or 12, dependent on the child. I know I was certainly able to understand and able to grasp the threads of the AIS issue (through all the baloney I was fed) by the time I was 13 or 14. Also, the timing of the diagnosis and the gonadectomy may have some bearing. If the gonadectomy was at infancy and the child is put on HRT at 10 or 11 it seems that some sort of partial disclosure is imperative. I know that with the truth things would have been much better for me at 14. It's stupid that I had to wait decades for full disclosure of my diagnosis.

A CAIS 30 year-old joined in:

I'd have to agree with ______ [CAIS 49 year-old] that by 10-11, you're starting to formulate some pretty strange ideas about sex and puberty etc. so why not give them the full picture? If the parent knows about AIS before this age, the child should be told the entire truth by this age so they can have the tools of truth to handle what lies ahead. Of course, reference to the AIS Support Group would be quite handy for literature/facts. I've found that we know much more than any doctor knows anyway. If diagnosed after 10-11, let's say on the ‘no periods’ checkup, usually done quite a bit later, sometimes as late as 17-18, then full disclosure by the doctor is required. I believe in full disclosure to ‘normal’ children about what is happening to their bodies as well, and why should AIS girls be any different? Why do people feel that by not talking about sex, it's just going to make the whole subject just disappear? Let's be real folks.

The CAIS 37 year-old emailed:

It has been brought up by a couple of people that an appropriate age of disclosure is 10 or 11. I agree to some extent. I think the girl needs to know what is not going to happen to her: periods, pubic hair, and if she asks, fertility. She also needs to be made aware that vaginal hypoplasia is a risk, especially in this world with 13-year-old girls having sex. However, I think that unless she specifically asks, that is too early for gonadal and chromosomal information. They haven't developed abstract reasoning – well, maybe AIS girls have, because we know how frighteningly brilliant AIS girls/women are. :-)

I think that their thoughts and concerns are much more concrete than Xs and Ys at that age. I think you should be careful to address only those things that are concerning her at that age. I have wondered about young AIS teenagers, less than 15 or so, being included in on very, very frank AIS adult conversations? I worry about planting worries that she might not already have come up with on her own. Maybe I am still working from the Victorian concept that ‘children’ are not sexual beings. I know better, and I understand that human beings are sexual beings all of their lives.³ It is just that most 12-year-olds aren't sexual in the same way a 17-year-old is. Also, I believe that a 12-year-old doesn't have the same coping abilities that a 17-year-old does.

Ref 3: See “Sexuality in Children” and “Puberty at Six?” in ALIAS No. 9, Autumn 1997.

The CAIS 49 year-old emailed:

That’s true, but if you already know you’ve had mysterious childhood hernia operations and all you are told at 10/11 is “probably no periods” and “probably no babies” and if you soon discover (on your own) that it’s also a case of no vagina, no pubic hair, and perhaps no breasts until mid/late teens, then (as someone in the circle so eloquently expressed it) “you f_____g want to know what’s wrong,” and it doesn’t take the brain of Einstein to deduce that everyone’s silence, and reluctance to seek a diagnosis until your mid/late teens, is because the truth is too horrendous for them to contemplate. So it’s off to the medical section of the nearest big library/bookshop....

The Grade 4/5 PAIS 44 year-old emailed:

I shall reply to the doctor in the light of your views, stressing that whoever does inform an AIS girl of this should also avoid letting her get the idea that she is somehow ineligible for marriage or a sex life. By the time an AIS girl learns the ‘bad news’ she probably has many worries about her future, so she should also be told that other AIS girls don't let it stop them from having boyfriends and husbands.

Later, a CAIS 35 year-old emailed, on making contact with the group and joining the email circle:

This being my first support group experience, I am beginning to see that part of the process of getting beyond the secrecy is a) educating yourself, and b) educating others (especially family and medical professionals). I wouldn't expect a family to necessarily master, accept and cope successfully with this challenging issue. ..... But I do think a medical professional, who has dedicated themself to helping others, should be expected to do their best. That would entail going beyond the ‘textbook’ explanation, do[ing] the necessary research, and refer[ring] any patient with AIS concerns to someone who can help, whether it be surgical, medical, or psychological help. I was impressed that the newsletters seemed to be promoting the notion that the AIS person must inform the medical professionals about what is the best route of action.