Understanding intersex

The intersex flag (learn more).
Of course, the idea that groups
need such flags is contentious.

We tend to think of human beings as dividing into either the male sex or the female sex. But biology is not that neat. The US historian Alice D. Dreger put it succinctly:

Humans come in a wonderful array of types: many sizes, many abilities, many features, and many approaches to experiencing and organising the world. Human variation is truly extraordinary. Amid all that variation, we can say with some confidence that most human beings develop one of two common sets of a particular group of organs. That is, most people possess either what is now labelled “female” or “male” sexual anatomy... Yet some people – more than is generally assumed – are born with an anatomical conformation different from “standard” male or female bodies. Their unusual anatomies can result in confusion and disagreement about whether they should be considered female or male or something else.[1]

On the normal (i.e. most commonly occurring) model, a person’s sex is determined by having either XX (female) or XY (male) chromosomes, determined at the moment of conception. This leads to possession of one or the other set of characteristic internal and external sexual / reproductive organs. The table below sets out the typical patterns:

	Male	Female
Chromosomes:	46,XY	46,XX
Phenotypic pathway:	Wolffian	Müllerian
External sex organs:	Penis, scrotum, epididymis, and testes	Mons pubis, pudendal cleft, labia majora and minora, vulva, Bartholin’s gland, and the clitoris
Internal sex organs:	Accessory organs: vas deferens, seminal vesicles, prostate gland, bulbourethral glands	Vagina, uterus, fallopian tubes, cervix, and ovary
Hormones:	High testosterone, low oestrogen	High oestrogen, low testosterone
Gamete:	Sperm	Egg

(For anatomical terms that are unfamiliar you can follow these links for male and female.)

Sex is a naturally occurring phenomenon which we humans abstract into the binary concepts of male and female. The vast majority of human beings fits completely unambiguously into one of these two categories. 

However, in practice some people are born with characteristics that do not line up in this neat way. Other arrangements are possible, and unusual anatomy (structure of body parts) and/or physiology (the functions and relations of those parts) can create confusion over how the person should be categorised. 

Such people are often called intersex. This word draws on the Latin inter or ‘between’ to mean ‘between sexes’, and has only been in use in its modern sense for about one hundred years. The English word ‘intersex’ has been around since the late 18th century, but prior to the 20th century it was uncommon and referred to relations between men and women. It was only in 1917 that Richard Goldschmidt introduced the term ‘intersexuality’ [2], in an academic paper on endocrinology interested in how the existence of ‘an intergrade between the two sexes’ in moths can cast light on how sex differentiation works. 

Today ‘intersex’ (in the broadest definition of the word) is usually used as an umbrella term for a wide range of 40+ birth conditions that pertain to sexual characteristics. Intersex people have chromosomal, hormonal or genital variations that diverge from the (statistical) norm. They may have, for example:

• ambiguous genitalia
• one sex’s external organs and the other’s internal organs
• aneuploidies i.e. chromosomes other than 46,XY or 46,XX 
• e.g. XXY or Klinefelter Syndrome (only affects men) in which there is an extra X chromosome
• e.g. XO or Turner Syndrome (only affects women) in which one of the X chromosomes is missing or damaged
• different chromosome patterns in different cells
• untypical hormone levels (a male with high oestrogen, a female with high testosterone)

There are many conditions that might be considered intersex – there is a comprehensive list on Wikipedia – and there is a stricter definition that will be discussed below. Some are obvious at birth, others don’t get noticed until puberty, and it is possible to be intersex your whole life without realising it (e.g. untypical internal organs you never know are there). They are all empirically observable conditions based on physiology, diagnosed medically: being intersex is a material reality about your body, not an identity, feeling or belief. 

Since humans (and life forms in general) are diverse and complicated, the existence of atypical cases is not surprising and should in fact be expected. Even unambiguous males and females show a great deal of variation: there are flat-chested women and big-breasted men, hairy women and relatively hairless men, tall women and short men. So asking who is intersex and who isn’t raises questions about our sex categories and how we think about what is ‘normal’. 

‘Hermaphrodites’

For centuries, intersex people were referred to as ‘hermaphrodites’. This term comes from Hermaphroditus, who in Greek mythology was the very handsome son of Hermes (the god of travellers and the messenger of the gods) and Aphrodite (the goddess of love and beauty) and is named after them both. The Roman poet Ovid in his Metamorphoses describes the rather horrible story: the nymph Salmacis falls in love with Hermaphroditus, rapes him, and prays for them to be combined into one (without the boy’s consent), a prayer which the gods answer. 

The nymph and the boy were no longer two, but a single form, possessed of a dual nature, which could not be called male or female, but seemed to be at once both and neither.[3]

This is the only full account of the myth. Hermaphroditus became a symbol of androgyny [4] wrapped up with sexual fantasy, incarnated in the Greco-Roman sculptural trope of the ‘sleeping Hermaphroditus’: figures who look female when viewed from behind but have male genitalia on the front. The Greek historian Diodorus Siculus observed a negative twist to the story:

There are some who declare that such creatures of two sexes are monstrosities.[5] 

In biology, a hermaphrodite is defined (according to the OED) as:

A person or animal having both male and female sex organs or other sexual characteristics, either abnormally or (in the case of some organisms) as the natural condition.

In some species (such as snails, slugs and earthworms) this is normal, but hermaphroditism, in the sense of fully male and female sexual organs and functions occurring in the same individual, does not exist in humans. There has never been a single observed case in Homo sapiens. Intersex people therefore are not hermaphrodites. The closest that real human beings come to hermaphroditism is ovotesticular disorder, when someone is born with both testicular and ovarian tissue – which affects just one person in 100,000 and still does not make someone hermaphroditic. 

In a 2005 paper [6], five authors argued that ‘hermaphrodite’ and the derivative terms in use at the time (e.g. ‘pseudohermaphrodite’) were ‘scientifically specious and clinically problematic’, focussed exclusively on the gonads and failed to reflect advances in the scientific understanding of sex that allowed more accurate and useful terms. Their suggestion was to refer instead to Disorders of Sexual Development, often abbreviated to DSDs. Some people use ‘Differences’ rather than ‘Disorders’ as they find it less negative, or prefer Variations of Sexual Development or VSDs for the same reason. DSDs is now the most commonly preferred term, as ‘intersex’ gets defined in different ways and is not useful from a medical/clinical perspective. The term ‘intersex’ too can be seen as inaccurate, as it implies someone who is ‘between’ the sexes, when in fact people with DSDs are either atypical males or atypical females, not ‘in-between’ or a third sex or no sex at all. 

The term ‘hermaphrodite’ is now considered outdated, derogatory and stigmatising, because:

• It does not describe intersex people correctly.
• Hermaphrodites have sometimes been seen as monstrous.
• It likens intersex people to animals that are hermaphroditic, like snails.

Some people with DSDs have tried to reclaim the term – e.g. when the now-defunct Intersex Society of North America (ISNA) titled its newsletter ‘Hermaphrodites With Attitude’. This is akin to the term ‘queer’ being reclaimed by some gays and lesbians (many other LGB people who have been on the receiving end continue to see it as hateful). Whatever one’s view of that, the least we can say is that it is inappropriate for people without DSDs to use it. 

How many people have DSDs?

Nobody’s right to be treated humanely should depend on how common their condition is. Having data however is important, not least because it is empowering for people with DSDs to know that others share their condition.

The precise frequency of specific conditions varies a great deal. To give a few examples: Klinefelter Syndrome affects around 1 in 1000 births, Androgen Insensitivity Syndrome one in 13,000 births, and Complete Gonadal Dysgenesis 1 in 150,000.

Estimating the total number of people with DSDs depends on which conditions one defines as intersex. The ISNA wrote:

If you ask experts at medical centres how often a child is born so noticeably atypical in terms of genitalia that a specialist in sex differentiation is called in, the number comes out to about 1 in 1500 to 1 in 2000 births.

That would indicate between 0.06% to 0.05% of the population. 

Twenty years ago, Anne Fausto-Sterling et al [7] noted in an influential paper that ‘no well-documented overview of the frequency of intersex exists at present’, and to remedy this they examined the medical literature from 1955 to 2000 to try and estimate ‘the frequency of deviation from the ideal male or female’. Their conclusion was that 1.7% (1 in 60, or 17 in every 1000) of all live births were intersex, where ‘intersex’ means people who do not conform to what they call a ‘Platonic ideal of physical dimorphism at the chromosomal, genital, gonadal, or hormonal levels’. This is a figure for the whole world, as far as the data allows, since the writers looked at international data, not just US data. 

Rather than seeing sex as an absolute of either male or female, they preferred to plot the sexes as bell-shaped curves – which they justified by reference to the wide variety of body shapes, sizes, and sexual characteristics of the sexes – with intersex conditions as a small overlap in-between. Their graph (reproduced below) is popular with people who argue that sex is on a spectrum (and yes, the axes are not properly defined).

This 1.7% figure has been widely reproduced – academically, on social media, and by NGOs, e.g. in Human Rights Watch’s report on intersex child surgery, on UNFE’s Intersex Fact Sheet, or on Amnesty International’s Intersex Awareness page, which says the figure is ‘comparable to the number of people born with red hair’. Likening DSDs to a familiar phenomenon like red hair makes them sound relatively common. The conclusion that is often drawn is that 17 in every 1000 people cannot be easily classified as either male or female.

However, the psychologist Leonard Sax responded that Fausto-Sterling is not using the term ‘intersex’ in the usual way, and that her 1.7% figure ‘includes conditions which most clinicians do not recognise as intersex.’ [8] By including anything that doesn’t fit into a strict ‘Platonic ideal’, Fausto-Sterling makes her definition wide enough to include people whose sex is not at all ambiguous. 

The most common condition that gets put under the ‘intersex’ umbrella, including by Fausto-Sterling, is Late Onset Congenital Adrenal Hyperplasia or LOCAH (not to be confused with Congenital Adrenal Hyperplasia or CAH, which is present at birth), which causes the adrenal gland to produce a slight excess of testosterone in either sex from late childhood or later. In males this is unremarkable, but in females the effects are more noticeable and problematic, such as facial hair growth and infertility. Sax observes: ‘From a clinician’s perspective... LOCAH is not an intersex condition.’ Firstly, intersex is supposed to be about conditions present at birth. Secondly this is a hormonal condition and the sex of the people affected is not ambiguous at all – yet it accounts for 88% of Fausto-Sterling’s estimate (1.5% out of the total 1.7%). 

Leaving out this condition leaves us with a much lower incidence of DSDs, on her figures, of 0.2% of the population. 

Sax argues that deviating from a ‘Platonic ideal’ is not a useful clinical criterion, and that Fausto-Sterling’s definition is still ‘too broad’ and confusing. In fact, the five most common conditions listed by Fausto-Sterling are not intersex in Sax’s view. 

Klinefelter Syndrome for example is a condition where males are born with an extra copy of the X chromosome (making them XXY) in at least some of their cells; but as it affects males, there is no ambiguity regarding their sex. 

He prefers a definition of intersex based upon

conditions in which (a) the phenotype is not classifiable as either male or female, or (b) chromosomal sex is inconsistent with phenotypic sex.

This is a stricter definition than the broad umbrella term I gave earlier. If we define the term in this way and discount the five non-qualifying conditions, writes Sax, the prevalence of intersex on Fausto-Sterling’s own figures emerges as 0.018% (less than 2 in every 10,000). This is very rare, and almost 100 times lower than her estimate – 50,000 individuals in the US rather than 5 million. Put another way, it means that 99.982% of human beings are unambiguously male or female – ‘unambiguous’ meaning that we can identify the person’s sex phenotypically, i.e. simply by looking at their body, without needing to consider other markers such as their karyotype. (Even people with ovotesticular disorder or OT-DSD, who have both ovarian and testicular tissue, develop in the main towards either the male or the female reproductive role, with the other as merely vestigial.)

The intersex advocate Claire Graham makes similar arguments. She criticises Fausto-Sterling’s graph for representing ‘precisely nothing that’s said’ (there are no values on the axes, so what is it charting?) and concludes:

The vast majority of the 1.7% are unambiguously male or female. These conditions prove how robust dimorphism is.

We can see from this disparity the importance of definition. Clearly atypical cases exist, but should the concept ‘intersex’ refer only to cases of ambiguous sex, or to atypical sexual anatomy more generally? If the latter, and your definition of intersex includes unambiguous males and females who happen to be anomalous or untypical in some aspect of their reproductive anatomy, what does ‘intersex’ – a word implying a position ‘between’ the sexes – even mean? There is no straightforward answer. Describing them as a ‘third sex’ (as claimed by US author and activist Hida Viloria for example) is unscientific and meaningless. Sexual reproduction requires male and female gametes: there is no third gamete (what would it even do?), and therefore no third sex. 

These choices are often as political as they are clinical. Since Fausto-Sterling believes that sex is a continuum rather than a binary (see her 2000 book Sexing the Body) and presents DSDs as intermediate values to support her claim, it is in her interest to estimate their numbers as high as possible. 

Natural variation or disorder?

As Dreger argues in her book, intersex people first came under sustained scrutiny in the second half of the 19th century, at the same time as women, homosexuals and sexuality in general were becoming categorised by (male) physicians and scientists. The impulse to assign people to the ‘correct’ box arose from a perceived need to preserve a distinction between male and female, and promote heterosexuality against homosexuality. Unusual bodies were a challenge to the integrity of categories that were seen as natural and inviolable, and so-called ‘hermaphrodites’ were a problem that needed solving. As Suzanne J. Kessler put it in her book The Medical Construction of Gender:

Genital ambiguity is ‘corrected’ not because it is threatening to the infant’s life but because it is threatening to the infant’s culture.

The perceived need to correct category violations has human consequences. The biggest issue facing DSD campaigners to this day is that infants with DSDs are sometimes operated on, with parental consent, to make their bodies conform more closely to expectations. This practice began in the 1950s and 60s in the US under the influence of the psychologist John Money, who thought that intersex people needed their sex disambiguated under his ‘optimal gender policy’. This is the one sense in which we may, in certain cases, talk of sex having been ‘assigned’ at birth. It now happens less frequently, but surgery continues to be carried out on children with atypical sex characteristics. This is opposed by DSD organisations, as it is usually not medically necessary and the child is too young to consent. This is the material consequence of seeing a DSD as an ‘other’ or ‘in-between’ state; in reality, the child is already either male or female. 

 

Recent years have also seen a movement against pathologisation. In Sexing the Body, Fausto-Sterling says that all sexual combinations should be seen as normal:

Complete maleness and complete femaleness represent the extreme ends of a spectrum of possible body types. That these extreme ends are the most frequent has lent credence to the idea that they are not only natural (that is, produced by nature) but normal (that is, they represent both a statistical and a social ideal). Knowledge of biological variation, however, allows us to conceptualise the less frequent middle spaces as natural, although statistically unusual.[9]

Or here is the British LGBTQ+ support charity Outlife:

Being intersex is not a disorder, and is a perfectly normal and healthy way to exist. Intersex variations, though they may be unknown, are relatively common, and cause no harm to your health.

This is simply misleading: some DSDs do cause harm to your health. On their webpage titled ‘What is intersex?’, the US sexual health organisation Planned Parenthood offers a slightly more measured view by including a ‘usually’: 

Being intersex is a naturally occurring variation in humans, and it isn’t a medical problem – therefore, medical interventions (like surgeries or hormone therapy) on children usually aren’t medically necessary.

The issue here is whether DSDs are disorders or just natural ‘differences’ that pose no obstacle to people living healthy lives. It is reasonable to be cautious about classifying people’s given traits as ‘normal’ or ‘not normal’. However, calling for de-pathologisation can be dangerous, because some conditions are pathological. People learn they have a DSD by way of medical diagnosis: if a child is born with unusual anatomy or ambiguous genitalia it should be medically investigated, and certain conditions require ongoing medical management. For example, Androgen Insensitivity Syndrome (AIS) may require hormone therapy, and Salt-wasting CAH can be fatal if left untreated. 

As Claire Graham advises:

Although they are diagnosed as medical conditions, and some can have other health implications, the difference itself is not unhealthy. It does not need ‘fixing’, unless there is a risk to health, impairment of function or the individual chooses to later in life.

That ‘unless’ is important. Morgan Carpenter of Intersex Human Rights Australia writes:

interventions may be necessary for physical health, notably for endocrine issues in congenital adrenal hyperplasia. Surgical interventions may sometimes be necessary to tackle elevated gonadal cancer risks or urinary issues.[10]

People’s medical needs must be addressed on a case by case basis. No one should undergo treatment that is not necessary without their consent; on the other hand, to de-pathologise DSDs entirely would be to deny medical reality and could harm people’s health. It is dehumanising and inappropriate to tell people with DSDs that their conditions do not require intervention when they themselves in certain cases are telling us the opposite. 

Instrumentalising DSDs

One motivation behind the denial of DSDs as a medical issue is – like the exaggeration of their numbers – political. Activists claiming that sex is on a spectrum frequently hold up intersex people as evidence of intermediate stages between male and female. The more common and ‘normal’ people with DSDs are, the more sex looks like a spectrum. 

I have written elsewhere on whether sex is a binary or a spectrum, but it’s important to recognise that having a DSD has nothing to do with being ‘trans’ or with ‘gender identity’. (‘Trans’-identifying people with DSDs are tiny in number, for the simple reason that they are a small demographic within another very small demographic.)

• To have a DSD is to have a physical condition. 
• To identify as transgender is to have a particular attitude to oneself. 

People who identify as transgender are confronted with their own difficulties including discrimination. However there are appropriate ways of supporting them. Intersex advocates are unhappy with their conditions being used to score points for other people’s agendas. (Follow advocate accounts on social media and this will come over strongly.) The Argentinian intersex and trans-activist Mauro Cabral, executive director of GATE, has made this plain:

Intersex is not about being trans, queer or non-binary: it’s about bodies and what happens to people who are born with them. We need to stop instrumentalising intersex to speak our truth as trans people.[11]

In 2013 the Third International Intersex Forum in Malta produced a statement, known as the Malta Declaration, in which it asserted in its final clause:

no-one should instrumentalise intersex issues as a means for other ends.

This statement was drafted by representatives of 30 intersex organisations from all continents. 

The addition of intersex as the ‘I’ to the LGBT acronym might be seen as a way of promoting awareness of people with DSDs. However, it is controversial, as DSDs are a set of physical conditions and has nothing to do with either sexual orientation or gender. Campaigning focused on identity misrepresents DSDs and can harm understanding. Morgan Carpenter observes:

Reports that frame intersex within LGBTI contexts frequently, and incorrectly, often assume that intersex people are all gay, or transgender. From an intersex perspective, the LGBT movement appears preoccupied with identity and relationship recognition, without recognition of bodily autonomy, and deeper challenges posed by sex and gender norms. Discussions within LGBT spaces may suggest that intersex groups do not want inclusion; some intersex-led organisations seek it, while others simply fear becoming overwhelmed and instrumentalised.[12]

It’s debatable whether adding intersex to the LGBT umbrella has brought any gains. The best way to show solidarity with people with DSDs is to promote their actual issues: the first thing organisations can do in this regard, notes Carpenter, is to adopt the Malta Declaration. 

Conclusion

People’s sex is important, as it is a material fact about their bodies that has implications for their lives: above all their medical needs and reproductive role, which are different for either sex. It also of course results in social distinctions being made which can be controversial or outright harmful. 

Because concepts like ‘male’, ‘female’ and ‘intersex’ are (like all words) human linguistic and social constructs, and inevitably have fuzzy edges, it can be tempting to query their value. When Alice Dreger says ‘there is no... final answer to what must count for humans as “truly” male, female, or hermaphroditic [sic]’ [13], this is true up to a point. However self-evident our concepts seem to us, they are unavoidably plastic and historical, since our societies, knowledge and technology are mutable. For example, the current understanding of ‘sex’ as a biological term and ‘gender’ as a social/cultural term only appeared in the 20th century – and if you advocate gender identity theory you will understand them differently again (and even, on some readings, flatten the two into each other).

On the other hand, there is a material, biological reality which these concepts are attempting to grasp, by reference to which 99.98% of human beings fit unambiguously into our concepts of male or female. Even the very concept of ‘intersex’ confirms the binary that people with DSDs are put into relation to. Only a tiny 0.018% proportion of people trouble those concepts, and even those can be categorised as male or female by assessing relevant criteria. This tiny amount of ambiguity tells us our sex categories are remarkably robust, compared to the concept of the colour ‘orange’, for example, whose edges are very fuzzy indeed. For them to be meaningful, it is not necessary to have two ‘perfect’ concepts with zero ambiguity – such a demanding standard is not required of other concepts. 

What people with DSDs need is the support of their families and peers, and properly funded healthcare provision to meet their medical needs. People should make choices about their own bodies, so seeking legislation to protect infants from non-consensual and medically unnecessary surgery is one of the movement’s priorities. 

In summary:

• The idea that any human being can be ‘between’ sexes is misleading. There is no middle condition. People with DSDs are still either male or female, the great majority without any ambiguity; there are some very rare conditions where determining sex is more complicated. 
• If we leave out the late-onset condition LOCAH and keep to congenital conditions, the incidence of DSDs in the population on current evidence is not the oft-quoted 1.7% but 0.2% (1 in 500).
• Cases where sex is ambiguous are extremely rare: 0.018% of births (less than 2 in every 10,000).
• The existence of DSDs does not mean that humans are not sexually dimorphic or that the sex categories of male and female are unstable, beyond an expected, empirically observable and measurable variation.
• Since there is no third sex (let alone more), sex is binary. This doesn’t mean human behaviour or gender expression or sexual attraction or identity is or ‘ought’ to be binary.
• It is necessary to tread a responsible line between overstating and understating DSDs as ‘disorders’ rather than differences: each person’s case must be judged on his or her own needs.
• People with DSDs have their own needs and priorities and should be not instrumentalised by the political agendas of others. 

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Footnotes

[1] Alice D. Dreger, Hermaphrodites and the Medical Invention of Sex (1998), p3-4.

[2] Richard Goldschmidt, ‘Intersexuality and the Endocrine Aspect of Sex’ (1917).

[3] Ovid, Metamorphoses, Book IV, translated from the Latin by Mary Innes. 

[4] These terms can get confused. Hermaphroditism (the anatomical possession of the characteristics of both sexes in one individual; impossible in humans) is distinct from androgyny (an appearance as neither or both genders, natural or cultural or a mixture of both; possible in humans). 

[5] Diodorus Siculus, Bibliotheca Historica (Historical Library), 1st century BCE, Book IV 4.6.5. 

[6] Dreger, Chase, Sousa, Gruppuso, Frader, ‘Changing the Nomenclature/Taxonomy for Intersex: A Scientific and Clinical Rationale’ (2005).

[7] Anne Fausto-Sterling et al, ‘How Sexually Dimorphic Are We? Review and Synthesis’ (2000). You can read the whole article at this link. 

[8] Leonard Sax, ‘How Common is lntersex? A Response to Anne Fausto-Sterling’ (2002). Although I find Sax’s work useful here, he also has reactionary views on sex differences. 

[9] Anne Fausto-Sterling, Sexing the Body: Gender Politics and the Construction of Sexuality (2000), p76. 

[10] Morgan Carpenter, ‘The human rights of intersex people: addressing harmful practices and rhetoric of change’ (2016).

[11] Mauro Cabral Grinspan, 2016.

[12] Carpenter, op. cit. 

[13] Dreger, op. cit., p9.