ABC's NATIONAL RADIO HEALTH REPORT  

Summary:
Boy or girl? This is one of the first questions new parents are asked. But sometimes it's not so clear. This program looks at the wide variety of intersex conditions, and the dilemmas faced by doctors, patients and parents when they have to make the choice - boy or girl. At what stage in the child's life should the choice be made? And does the choice have to be made at all?

Transcript:


Rae Fry: Hello, and welcome to the Health Report. I’m filling in for Norman Swan today, and we’re looking at something that’s a hot issue in medical circles, but that the rest of us don’t hear much about: intersex conditions.

At the core of the debate is surgery on babies and very young children. For decades, this has been conventional medical practice for children born with sexual organs that are in between male and female. It sounds barbaric, and patient groups and some surgeons are calling for the practice to be stopped. They say surgery should be delayed until the child is old enough to consent.

But as we’ll hear, surgery or no surgery is not the only issue. Intersex conditions include a wide variety of internal and external, genetic and hormonal characteristics. Many aren’t visible at birth. And every individual affected is unique.

Chris Somers: My name is Chris Somers. I’m considered an intersex person, and I see myself as an androgyne which is neither male nor female but a complex mixture of all. My life has been perhaps rather extraordinary in some respects. I was born in Kenya and brought up in both Kenya and Sudan and UK, and went to a boys’ boarding school for ten years, and during that time I developed characteristics that would be deemed as female, and as a result of that in a boys’ school, it was considered in for a dig to have a good tease of Chris. I developed breasts when I was about 13 years old, and had a bilateral mastectomy when I was 16-1/2, not because I wanted to, but because society out there would not accept me.

Rae Fry: Chris became a photographer and eventually migrated to Australia. She/he now lives in Perth and is doing postgraduate studies in education.

In spite of the early surgery, Chris only got a diagnosis at age 27, when in hospital with an unrelated illness.

Chris Somers: I’d had a number of different tests done on me to find out what this other thing was. They said to me, ‘There’s something that you’re not telling us, what is it?’ And I finally owned up to the fact that I’d had a bilateral mastectomy, and the surgeon who had carried that out had done a very good job, because it was almost imperceptible, and it was only after much more scrupulous checking over of my body that someone noticed the minute scars, and they carried out a karyotype which is an analysis of the genetic structure of my body and found that I had the 47XXY chromosomes.

Rae Fry: 47XXY is Klinefelter’s Syndrome. Many wouldn’t call it an intersex condition, since the penis is average in size, and unlike Chris, most people with Klinefelter’s identify unambiguously as men. Often they don’t know about their genetic makeup until they try and have children and find they’re infertile. Most have lower than average testosterone levels, but it’s not always visible. There’s a lot of variety in how the genes are expressed, physically.

Chris Somers: When people see me, they see a male primarily, because on the outside of me I’ve got a beared, and male-pattern baldness. This has all been basically medically induced because I take testosterone every other week. This over time, of course, I have a broken voice, and my voice broke actually two years ago for about the fifth time, where I have no register now. A few years ago I had an ultrasound; that ultrasound revealed that I have ovaries, an internal collapsed vagina, the possibility of a uterus, though I think that is perhaps not the case. When I was a younger person, in my mid-20s, occasionally people used to ask me ‘Oh, by the way, what sex are you, Chris?’ I used to say, ‘Well, anything you like’, and whereas I might be saying that semi-jokingly, I was also extremely serious, but at the same time I was very scared of revealing too much, because of the consequences of that revelation.

Rae Fry: Chris Somers, from Perth.

Now some such conditions, unlike Chris’s are revealed when a baby is born, with genitals that aren’t obviously either male or female.

Associate Professor Garry Warne is an endocrinologist, a hormone specialist, at Royal Children’s Hospital in Melbourne. He says about one in every 4,500 babies are born this way, in Victoria, he sees about one a month.

Garry Warne: I might be called to a maternity hospital where a baby has been born, and no-one in the room is able to tell what the sex is, so that the penis might be too small to be really called a penis, there might be a cleft but not one that would be typical for a girl, the urinary opening might be in the wrong place for either a boy or a girl, and perhaps we can feel either no testes at all, or perhaps one testis.

Rae Fry: This is just one example. And you might have heard of the term ‘hermaphrodite’, which technically means someone who has both ovaries and testicles.

That’s quite rare, and doesn’t reflect the huge variety of interesex conditions.

Garry Warne: The first group is the one where they look atypical on the outside. There are a number of causes of that, the most common one is an altered gene that causes the adrenal glands to over-produce a male hormone, and if the baby is a genetically female baby, she’ll be born with masculinised genitalia.

Rae Fry: So that’s congenital adrenal hypoplasia?

Garry Warne: That’s correct. Another one is where there’s a mixup of the chromosomes, and some of the cells in the body contain male chromosomes, and some of the other cells have a missing X-chromosome. In that situation the gonads don’t differentiate properly into either ovaries or testes, and they may produce a bit of male sex hormone and cause some development in the male direction, but it’s not completed.

Rae Fry: That’s called gonadal dysgenesis. And in another condition, called androgen insensitivity syndrome, or AIS, the baby is genetically male, XY, and able to produce testosterone, but the tissues don’t respond to it. This can be complete, so the baby looks female but has no uterus, or partial, with ambiguous external organs.

For parents, it’s a shock when their baby is born.

Garry Warne: Problems that parents have are confusion, guilt, sadness, anxiety about how they will relay this problem on to their family and their friends outside the hospital, and also fears about what that might mean for the future.

Rae Fry: Garry Warne thinks that just how traumatic it is for parents depends on how they’re told. But many health professionals don’t have the experience or the training to know what to say.

Garry Warne: Well I remember hearing a story from one young woman, she was a single mother, the baby was born in a country hospital, and first of all they took the baby out of the room and didn’t say anything to the mother about why they were whisking the baby away. So she was left there, thinking the worst, and then later on, some hours later, a doctor came back and tried to explain to her what was wrong and the doctor burst into tears because he didn’t have the words for it. The mother actually felt compassion for the doctor, because at least, you know, the doctor was able to appreciate the difficulty of the situation so that she wasn’t angry about that. But subsequently, a nurse brought the baby to the mother and she was relieved to find that it was only a genital problem, because she’d begun to think that either the baby had died or it had something dreadful like a cerebral haemorrhage and would be handicapped forever. So she was relieved to learn that it was only a problem of ambiguous genitalia.

Rae Fry: But panic, rather than relief, is the usual response.

Knowing that everybody will be asking, boy or girl? the pressure on parents and doctors to decide things one way or the other is enormous. And for many years, conventional practice has been to operate while the child is still a baby.

Milton Diamond is Professor of Anatomy and Reproductive Biology at the University of Hawaii.

Milton Diamond: If a child is born with ambiguous genitalia the most common way that’s been dealt with is to say, ‘Well it’s very difficult to build a functioning penis for this child, so let’s make it into a female by constructing surgically, a vagina, something that looks like a vagina.

Rae Fry: The thinking behind early surgery is that it’s important, socially and psychologically, to create a clear gender identity.

Milton Diamond: The theory was that children come to know who they are, as male or female before the age of two. They also were trying to placate the parents, and often it quite disturbed them, they want children that look normal, so they say ‘Doc, what can we do?’ and the doctor has an easy solution for a difficult problem.

Rae Fry: In the medical literature, the popularity of the ‘easy solution’ rested on a classic case. It was called the John/Joan case, pseudonyms for a patient who was born an average male. But when John was being circumcised, the surgeon had a nasty accident.

Milton Diamond: The cautery burnt the boy’s penis so that it basically sloughed off. The child was left without a penis. The solution that was recommended was to reconstruct that child into a female and bring it up as a girl. Well the clinical reports that came out afterwards was that John, who now became Joan, was doing fine, and accepting the reassignment.

Rae Fry: Now you caused quite a stir by following up later in life, so tell me what happened to John?

Milton Diamond: Well basically, as he was growing up, he rejected the girl’s clothing, he rejected living as a girl, got into fights with all his peers, so they didn’t accept him as a girl, and he didn’t accept himself as a girl, he became reclusive. Basically, eventually, he said, ‘I’m not going to live this way, I’m going to either live as a boy, as I think I am, or I’m going to commit suicide.’

Rae Fry: And so at 14, Joan or John started hormone therapy and eventually he was reassigned as a man. Milton Diamond reports that he’s now happily married and is the adoptive father of his wife’s three children.

Now like John, some of the children who had had genital surgery as a baby or a young child, were not happy with the results.

Until the late ‘60s or so, surgery for say congenital adrenal hyperplasia usually involved almost complete removal of the enlarged clitoris. Since then, better techniques have been developed that preserve as much of the sensitive tissue as possible, but there are few long-term studies on how successful they are.

Milton Diamond, and a number of intersex patient advocacy groups in the United States, the United Kingdom, and Australia, have called for a moratorium on paediatric surgery unless it’s needed for good medical reasons. Otherwise, they say, it should be delayed until the child is old enough to consent.

Milton Diamond: These are cosmetic surgeries, they’re not medically needed. Now those who do it say, ‘Well yes, it is medically needed because the parents are quite disturbed or the child will feel bad growing up with ambiguous genitalia.’ Well, my comments to that are first, it’s the individual that has to live with those genitals then he or she may not like what’s been constructed for them, and that’s what happens. These people, when they become adults, complain that they’ve had their genitals restructured in ways that they don’t approve of.

Rae Fry: Professor Milton Diamond from the University of Hawaii.

Milton Diamond’s recommendations to delay surgery has not been adopted wholesale by the medical profession. At first, it’s hard to understand why not. Except when surgery is needed to prevent, say, recurrent urinary tract infections, and apart from the difficulty of growing up different, why shouldn’t kids be left physically intact so they can later more easily change their minds about their bender, if they wish.

But Dr Sonia Grover is not so sure. She’s a gynaecologist and another member of Garry Warne’s team at the Royal Children’s Hospital in Melbourne. She treats older girls with intersex conditions at the Hospital’s Centre for Adolescent Health. And she thinks that some of their problems are not because they’ve had surgery per se, but because less experienced surgeons have sometimes done the work. And it shows.

Sonia Grover: How the clitoral reduction has been done and where the clitoris ends up being sited, so sometimes it’s not quite sited in what would be a normal anatomical position; the labia majoris or the big lips are often wrinkly in kids who are born with ambiguous genitalia and if the surgery’s done well then the wrinkly appearance can also be corrected just as part of the rest of the surgery is being done, it gets resolved as well. And then how the vaginal opening area is corrected seems to be one of the biggest problem areas. So that when I look at the outcomes, or read the papers regarding outcomes in other parts of the world, I can imagine that the women with those outcomes will be having uncomfortable intercourse because the vaginal opening is inadequate with a bridge of skin partly obstructing the way, no wonder it’s painful.

Rae Fry: If surgery in children is done well, for example, by her colleague, leading paediatric surgeon John Hutson, Sonia Grover believes that it might be more successful than if it’s done at a later age.

Sonia Grover: Because the operating distances to correct are now much larger, it’s actually not necessarily particularly easier for me to make the corrections that I’ve watched the paediatric surgeon here in Melbourne do with relative ease in little children.

Rae Fry: So it’s harder to do because the patient is bigger?

Sonia Grover: I think so. It’s possibly too early for me to make any definitive statement on that, and I’m also conscious that it’s counter to what other people are saying elsewhere in the world, that we shouldn’t be doing it on little kids, and we should be doing it when they’re older. I’m not sure. I’m not sure that I’m failing in terms of sexual function outcome but I feel like I have to work much harder to get a good outcome than the paediatric surgeon, John, who does it as a baby.

Rae Fry: Given that patient advocacy groups are requesting that surgery be delayed where possible, medically, how can you justify advocating early surgery?

Sonia Grover: I have to think very hard about what we’re doing. And one of the difficulties in this is that this surgery is so variable, and I’m looking at outcomes of John’s surgery and saying, ‘Well I think I’m seeing good outcomes’. And then I also know that there are other substantial factors that influence outcome. If you have had during your medical visits to hospital through your childhood, and it doesn’t happen now, but it did happen 25, 30 years ago, as standard practice, the repeated looking occurred and sometimes repeated examinations of genital areas. Now that can’t have a good impact on a young girl who’s developing and in fact I’ve had some of my young women who are now in their early 20s who’ve said to me, ‘Look, having sex is a bit like having a medical examination. You lie there with your legs open.’ Now if they’ve got such clear recollections of uncomfortable experiences, and that may not be uncomfortable physically but emotionally uncomfortable, that is going to have a lousy impact on how they’re going to function down the track. So when we say the outcomes of surgery elsewhere haven’t been good, it’s not just surgery we’re talking about, we’re talking about surgery and what happened to all those consultations over the years. So it’s not just an anatomical outcome we’re talking about. If you feel lousy about your body, if you’re convinced your body is abnormal, then you’re not going to be relaxed when you’re having intercourse, and if you’re not relaxed and comfortable with yourself, you don’t get aroused, you don’t lubricate, it’ll be dry and therefore it will be painful. So was it the surgery that made it painful, or was it the fact that the whole thing was too scary and terrifying, that you didn’t have a good experience sexually.

Rae Fry: The bottom line is that we don’t have good enough research to make good decisions. The studies that have been done suggest most intersex people are satisfied with the gender they were assigned, though maybe not with their equipment.

But research in this area is difficult. You need long-term studies, patient numbers are relatively small, and bad past experiences mean that many, understandably, are unwilling to participate.

So the team at Royal Children’s, along with researchers from the Murdoch Children’s Research Institute, are about to embark on their own study.

Garry Warne: Our plan is to try to follow up every single patient that was treated in one hospital, the Royal Children’s Hospital in Melbourne, over the past 30 years, and find out from them how many are satisfied with the outcome, how many are not, if they’re not, what are the reasons and also to find out what could we be doing more in terms of establishing services for adults that would make life better for those people now.

Rae Fry: Even for older children and adults, consenting to surgery is not a simple issue. Chris Somers’ father, who was in fact a doctor, did ask Chris whether she/he wanted the mastectomy.

Chris Somers: He said, ‘Well look, Chris, we can do something about this if you wish.’ I said, ‘I don’t really wish it at all, but how am I going to live in a society that is not going to accept me with my breasts and my penis’. So I therefore decided, in order to survive without perhaps becoming a suicide case, that the only way for me was to conform.

Rae Fry: The experience has left its mark on Chris’ artwork.

Chris Somers: In a number of my images one will see the human breast is constantly referred to. If you like, it is a celebration of that which I lost and it is also a celebration of the feminine within me, and trying to regain that part of the equation.

Rae Fry: Chris Somers.

Occasionally, intersex conditions are diagnosed before birth, through amniocentesis, or occasionally, by an ultrasound.

For example, a screening check of Down’s Syndrome might pick up the extra X chromosome that occurs in Chris’ condition, Klinefelter’s syndrome. Which raises the issue of what parents are told that might affect their decision about whether to have the baby.

Last year a British study found that some parents who talked to doctors and nurses, before or instead of a genetic counsellor, were given misinformation about Klinefelter’s, for example that their baby would be mentally retarded. In fact the evidence suggests a very small lowering of average IQ, which often won’t make a difference in day-to-day life.

Associate Professor, Garry Warne.

Garry Warne: I was very interested in that study, and the message is clearly that we need more genetic counsellors, and they themselves need more specific training. But I fully support that and I would promote genetic counselling as a discipline in Australia.

Rae Fry: Medical practice in intersex conditions is changing. It’s less likely these days, for example, that a boy born with a very small penis, called a micropenis, will be surgically reassigned as a girl. This is because of research from Johns Hopkins and other universities showing that while size does matter, functionality maters more.

Garry Warne: The impression that I have is that assigning someone as female just because they had a very small penis, is a disaster. My own personal practice would be never to assign a child like that female.

Rae Fry: In the past, some patients weren’t told of their condition for years, sometimes not at all. These days there’s more openness, which is important not only ethically, but because of ongoing health issues, like an increased risk of cancer and osteoporosis.

Sonia Grover: For the women who have XY material in their gonads, there is probably a 30% risk of malignancy by the time they’re about 30 years of age. So it is significant and if there’s a tumour, it ends up with a cancer that can kill you. So for that group, it’s very clear-cut. Again, there’s room for debate about the women who have got androgen insensitivity syndrome. The risk of those gonads, those testes, becoming malignant, is far, far lower, so the need to remove the gonads in that group is not as urgent.

Rae Fry: Now when you remove the gonads, the testes or ovaries, you also remove of course their capacity to produce hormones; what ongoing issues does that raise?

Sonia Grover: Well that’s one of my concerns with the AIS group, that we know that many teenagers, no matter what condition we’re talking about, can be non-compliant as teenagers.

Rae Fry: They don’t take their medication?

Sonia Grover: They don’t take their medications, because it’s part of growing up, being dependent, rebelling. So for people with androgen insensitivity syndrome who’ve had their gonads removed at an early age, we need to make sure that they’re taking their hormones during their adolescent years, and it’s a very critical time, because during your adolescent years, is when you’re making your bones strong, and if you don’t have oestrogens around at that time, then you end up with osteoporotic bones.

Rae Fry: There’s still a lot of disagreement about what determines gender identity: whether someone feels male, female, or a bit of both. But whether or not a child has surgery, most patients, and doctors, including Milton Diamond, still agree that a child should be labelled as one or the other, boy or girl, because gender in our society is so central to how we relate socially.

Milton Diamond: If you bring someone up as a boy, or you’re bringing someone up as a girl, they simultaneously are learning the other gender as well. You’re really learning both at the same time, and it makes the ability to switch or be neutral, easier when you’re an adult. Would our society expect a certain type of rigidity? Yes, the first question that’s generally asked when a child is born is, ‘Is it male or female?’ Maybe it’s the second one, the first one ‘Is Mom and baby healthy?’ But right up close, they’re going to ask –

Rae Fry: Boy or girl?

Milton Diamond: Right. And you say, ‘Well you know, there’s a medical situation and we’re not sure yet.’

Rae Fry: So you recommend that the parents are just quite open about that?

Milton Diamond: Well when I say, I don’t think they have to broadcast it from the top of the building, but I think it’s not something to be shameful, they have not been bad parents, this is a biological variation that occurs. If they start dealing with it in shame, that shame is transmitted to the child, and the child grows up with shame for something that he or she did not do.

Rae Fry: The Royal Children’s team thinks that parents have to decide for themselves what they tell people. They say that parents should be given full information about the child’s condition, and told that not having surgery is a valid option. If surgery is chosen, they believe it should be left to a small number of expert surgeons, maybe just three or four nationwide.

Whatever happens, they believe that good psychological and social support is crucial. And Sonia Grover believes that in some cases, it may be best to leave the question open. Boy or girl?

Sonia Grover: For some people it’s going to be easier to sit on the fence because they’re really not sure where they belong. Whether that’s the wise decision to be doing with little children all the time is one of the things that we need to answer from our study. I mean if our study shows that the vast, vast, vast majority of people are in fact happy with the gender alignment that is given to them, then I’d actually ask the question ‘Well of the few that we’ve made the wrong gender assignment to, could we have retrieved that situation earlier, but how different is the rate of that occurring to trans gender, transsexual issues occurring in the rest of the population?’ Because we know that people without any medical diagnosis can look like a male, and in fact decide they really feel like they’re woman inside. So we tolerate that in that context and so we should, and I think we need to have the broad-mindedness and openness to do the same in our patients with medical conditions. The important thing is that the conversation occurs, and the openness occurs. But that applies whether this person’s got a medical condition or not. If you don’t feel comfortable with who you are, then we should be talking.

Chris Somers: There are some of us who perceive ourselves to be bisexual, there are a number who see us as heterosexual, male to female relationships. Others who see themselves as I like I do, if I’m making up to a woman, gosh, I keep on checking myself as to whether I’m actually a lesbian. It’s a dilemma, because the language doesn’t allow for a difference in understanding as to who we are. For example, if one is to look at various forms, such as the driving licence, what sex are you, male or female? I always enter that, first thing last, which asks me about my sex, I circle both boxes, male and female and I put the word ‘androgyne’ and I say ‘Contrary to popular belief, there are people who are a mix of sexes and genders, and in this case could be called androgynous or intersexed. If you have any problems concerning this, please contact Professor Milton Diamond or Professor Lou Landau in the United States, and/or in Australia for further details’. And it always raises a few eyebrows, but the interesting thing about that is that they do ask me, and then they get very serious and actually say, ‘Well, we’re really glad to have been opened up to this one.’

Rae Fry: Chris wishes she/he had had a choice of oestrogen instead of testosterone, or as well as it. And would like to see Australia set up a major research treatment and education centre with an embracing view of sex and gender.

Chris Somers: And at birth, I would also like to see that the medical profession is not too quick to whip out the scalpel and rearrange the child that does not fit their pre-existing position of what a human is supposed to be or look like. There is far too quick decision made to rearrange us into the format that they presume is the correct one, when in fact it may precipitate a disaster.

Rae Fry: Chris Somers from Perth. And I would like to thank Chris for speaking to me for the program.

I’m Rae Fry, and Norman Swan will be back next week with new findings about folate and Alzheimer’s disease.

References:
Somers C. and Felicity Haynes, ‘Intersex: Beyond the hidden a-genders’, in Unseen Genders: Beyond the Binaries, edited by Felicity Haynes and Tarquam McKenna, Peter Lang Publishing, New York, 2001, pp 29-40

Abramsky L. et al, What parents are told after prenatal diagnosis of a sex chromosome abnormality: interview and questionnaire study. British Medical Journal Vol 322, 2001, pp 463-466

Wisniewski A.B. et al, Congenital micropenis: long-term medical, surgical and psychosexual follow-up of individuals raised male or female. Hormone Research Vol 56, 2002, pp. 3-11

Kipnis K. and Milton Diamond, Pediatric ethics and the surgical assignment of sex. Journal of Clinical Ethics Vol 9, 1998, pp 398-410

Professor Milton Diamond
Department of Anatomy and Reproductive Biology,
Pacific Center for Sex and Society,
University of Hawaii at Manoa,
Honolulu, Hawaii 96822,
U.S.A.

Chris Somers
International Foundation for Androgynous Studies,
PO Box 1066,
Nedlands, Western Australia 6909

Associate Professor Garry Warne
Department of Endocrinology and Diabetes,
Royal Children's Hospital Melbourne,
Parkville, Victoria 3052

Dr. Sonia Grover
Consultant Gynaecologist,
Royal Children's Hospital Melbourne,
Parkville, Victoria 3052

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