Intersexuality

Intersexuality is the state of a living thing of a gonochoristic species (the individuals of a species are of one of two distinct sexes, and retain that sexuality throughout their lives) whose sex chromosomes, genitalia and/or secondary sex characteristics are determined to be neither exclusively male nor female. An organism with an intersex condition may have biological characteristics of both the male and female sexes.

Intersexuality is the term adopted by medicine during the 20th century applied to human beings who cannot be classified as either male or female

Intersexuality is also the word adopted by the identitary-political movement, surged in the nineties, to criticize medical protocols in sex assignment and to claim the right to be heard in the construction of a new ones.

Language
Research in the late twentieth century has led to a growing medical consensus that diverse intersex physicalities are normal, but relatively rare, forms of human biology. Perhaps the most prominent researcher, Milton Diamond, stresses the importance of care in selection of language related to intersexuality. Foremost, we advocate use of the terms "typical," "usual," or "most frequent" where it is more common to use the term "normal." When possible avoid expressions like maldeveloped or undeveloped, errors of development, defective genitals, abnormal, or mistakes of nature. Emphasize that all of these conditions are biologically understandable while they are statistically uncommon.

Hermaphrodite
The terms hermaphrodite and pseudohermaphrodite, introduced in the 19th century, are now considered problematic as hermaphrodism refers to people who are both completely male and completely female, something which is not possible. The phrase '"ambiguous genitalia'" refers specifically to genital appearance, but not all intersex conditions result in atypical genital appearance.

Disorders of sex development
The Intersex Society of North America and intersex activists have moved to eliminate the term "intersex" in medical usage, replacing it with "disorders of sex development" (DSD) in order to avoid conflating anatomy with identity. Members of The Lawson Wilkins Pediatric Endocrine Society and the European Society for Paediatric Endocrinology accepted the term "disorders of sex development" (DSD) in their "Consensus statement on management of intersex disorders" published in the Archives of Disease in Children and in Pediatrics. The term is defined by congenital conditions in which development of chromosomal, gonadal, or anatomical sex is atypical. However, this has been met with criticism from other activists who question a disease/disability model and advocate no legal definition of sexes, no gender assignments, no legal sex on birth certificates, and no official sexual orientation categories.

Alternatives to labeling these as "disorders" have also been suggested, including "variations of sex development"

History
Intersex individuals are treated in different ways by different cultures. In some cultures intersex people were included in larger "third gender" or gender-blending social roles along with other individuals. In most societies, intersex individuals have been expected to conform to either a male or female gender role.

Whether or not they were socially tolerated or accepted by any particular culture, the existence of intersex people was known to many ancient and pre-modern cultures.

As an example, one of the Sumerian creation myths from more than 4,000 years ago has Ninmah, a mother goddess, fashioning mankind out of clay. She boasts that she will determine the fate – good or bad – for all she fashions. Enki, the father god, retorts as follows.


 * Enki answered Ninmah: "I will counterbalance whatever fate -- good or bad -- you happen to decide." Ninmah took clay from the top of the abzu [ab = water, zu = far] in her hand and she fashioned from it first a man who could not bend his outstretched weak hands. Enki looked at the man who cannot bend his outstretched weak hands, and decreed his fate: he appointed him as a servant of the king.


 * ... [Three men and one woman with atypical biology are formed and Enki gives each of them various forms of status to ensure respect for their uniqueness] ...


 * Sixth, she fashioned one with neither penis nor vagina on its body. Enki looked at the one with neither penis nor vagina on its body and gave it the name Nibru [eunuch(?)], and decreed as its fate to stand before the king."

Since the rise of modern medical science in Western societies, some intersex people with ambiguous external genitalia have had their genitalia surgically modified to resemble either male or female genitals. Ironically since the advancements in surgery have made it possible for intersex conditions to be concealed, many people are not aware of how frequently intersex conditions arise in human beings or that they occur at all. Contemporary social activists, scientists and health practitioners, among others, have begun to revisit the issue, and awareness of the existence of physical sexual variation in human beings is returning.

There are increasing calls for recognition of the various degrees of intersex as healthy variations which should not be subject to correction. Some have attacked the common Western practice of performing corrective surgery on the genitals of intersex people as a Western cultural equivalent of female genital cutting. Despite the attacks on the practice, most of the medical profession still supports it, although activism has radically altered medical policies and how intersex patients and their families are treated. Others, typically social conservatives, have claimed that the talk about third sexes represents an ideological agenda to deride gender as a social construct whereas they believe binary gender (i.e. there is only male and female) is a biological imperative.

Depending on the type of intersex condition, corrective surgery may not be necessary for protection of life or health, but purely for aesthetic or social purposes. Unlike other aesthetic surgical procedures performed on infants, such as corrective surgery for a cleft lip (as opposed to a cleft palate), genital surgery may lead to negative consequences for sexual functioning in later life (such as loss of sensation in the genitals, for example, when a Clitoris deemed too large/penile is reduced/removed, or feelings of freakishness and unacceptability) which would have been avoided without the surgery; in other cases negative consequences may be avoided with surgery. Opponents maintain that there is no compelling evidence that the presumed social benefits of such "normalizing" surgery outweigh the potential costs. (Similar attitudes are present in some cases of botched infant circumcision, in which the solution might involve intensive medical and parental efforts to reassign the male baby to a female identity, which opponents claim lead to the degrading interpretation that females are essentially castrated males. This view overlooks the embryological origin of the penis/clitoris.) Defenders of the practice argue that it is necessary for individuals to be clearly identified as male or female in order for them to function socially. However, many intersex individuals have resented the medical intervention, and some have been so discontented with their surgically assigned gender as to opt for sexual reassignment surgery later in life.

During the Victorian era, medical authors introduced the terms "true hermaphrodite" for an individual who has both ovarian and testicular gonadal histology, verified under a microscope, "male pseudo-hermaphrodite" for a person with testicular tissue, but either female or ambiguous sexual anatomy, and "female pseudo-hermaphrodite" for a person with ovarian tissue, but either male or ambiguous sexual anatomy. The writer Anne Fausto-Sterling coined the words herm (for "true hermaphrodite"), merm (for "male pseudo-hermaphrodite"), and ferm (for "female pseudo-hermaphrodite"), and proposed that these be recognized as sexes along with male and female. However, her use was "tongue-in-cheek"; she no longer advocates these terms even as a rhetorical device, and her proposed nomenclature was criticized by Cheryl Chase, in a letter to The Sciences which criticized the traditional standard of medical care as well as Fausto-Sterling's shorter names, and announced the creation of the Intersex Society of North America.

Intersexuality in popular culture
Beginning as early as 1989, intersexuality became a topic of interest for broadcast TV and radio in the United States and other countries. Jeffrey Eugenides' novel Middlesex (2002) is narrated by an intersex character who discusses the societal experience of an intersex person. The Japanese manga series I.S., first published in 2003, features intersex characters and how they deal with intersexuality-related issues and influence the lives of people around them.

Notable intersex people

 * Cheryl Chase (activist), intersex activist
 * Erik Schinegger, alpine skier
 * Jim Sinclair
 * Lady Colin Campbell, British aristocrat and author of Guide to Being a Modern Lady
 * Edinanci Silva, judoka and Gold medalist in the woman's half-heavyweight division at the Pan-American games.

Noted researchers on intersex development

 * Milton Diamond, professor of neurology, Univ. of Hawai'i at Manoa

Intersex organizations

 * AIS Support Group UK & International
 * AIS Support Group Australia
 * Intersex Trust Aotearoa New Zealand (ITANZ)
 * Intersex Society of North America
 * Organisation Intersex International

Lack of education
In high school, most emphasis is placed upon the most common XX and XY genotypes. Thus, people nowadays may be more likely to look towards the sex chromosomes than, for example, the histology of the gonads. However, according to researcher Eric Vilain at the University of California, Los Angeles, "the biology of gender is far more complicated than XX or XY chromosomes". Many different criteria have been proposed, and there is little consensus.

Disorders and scope
Sax's strict definition of intersexuality is most relevant to family practice and psychological research. Other interest groups serve different communities and concerns and so broaden the definition of intersexuality in these fields.

For instance, the Intersex Society of North America (ISNA) definition states that the following conditions "sometimes involve intersex anatomy":


 * 5-alpha reductase deficiency
 * androgen insensitivity syndrome
 * aphallia
 * clitoromegaly
 * congenital adrenal hyperplasia
 * gonadal dysgenesis (partial & complete)
 * hypospadias
 * Klinefelter syndrome
 * micropenis
 * mosaicism involving sex chromosomes
 * MRKH (mullerian agenesis; vaginal agenesis; congenital absence of vagina)
 * ovo-testes (formerly called "true hermaphroditism")
 * partial androgen insensitivity syndrome
 * progestin induced virilization
 * Swyer syndrome
 * Turner syndrome

Some people with some of these conditions (such as Kallmann syndrome and Turner syndrome) do not self-identify as intersex. See also 17-beta-hydroxysteroid dehydrogenase deficiency.

Prevalence
The prevalence of intersexuality depends on which definition is used.

According to the ISNA definition above, 1 percent of live births exhibit some degree of sexual ambiguity, approximately one in every hundred births. Between 0.1% and 0.2% of live births are ambiguous enough to become the subject of specialist medical attention, including surgery to disguise their sexual ambiguity.

According to Fausto-Sterling's definition of intersex, on the other hand, 1.7 percent of human births are intersex. She writes, While male and female stand on the extreme ends of a biological continuum, there are many bodies [...] that evidently mix together anatomical components conventionally attributed to both males and females. The implications of my argument for a sexual continuum are profound. If nature really offers us more than two sexes, then it follows that our current notions of masculinity and femininity are cultural conceits. [...] Modern surgical techniques help maintain the two-sex system. Today children who are born "either/or-neither/both" — a fairly common phenomenon — usually disappear from view because doctors "correct" them right away with surgery.

According to Leonard Sax the prevalence of intersexuality "restricted to those conditions in which chromosomal sex is inconsistent with phenotypic sex, or in which the phenotype is not classifiable as either male or female" is about 0.018%.

Ambiguous genitalia
Ambiguous genitalia appear as a large clitoris or small penis and may or may not require surgery.

Because there is variation in all of the processes of the development of the sex organs, a child can be born with a sexual anatomy that is typically female, or feminine in appearance with a larger than average Clitoris (clitoral hypertrophy); or typically male, masculine in appearance with a smaller than average penis that is open along the underside. The appearance may be quite ambiguous, describable as female genitals with a very large clitoris and partially fused labia, or as male genitals with a very small penis, completely open along the midline ("hypospadic"), and empty scrotum.

Fertility is variable. According to some, the distinctions "male pseudohermaphrodite", "female pseudohermaphrodite" and especially "true hermaphrodite"  are vestiges of 19th century thinking. According to others, the terms "male pseudohermaphrodite", and "female pseudohermaphrodite" are used to define the gender in terms of the histology (microscopic appearance) of the gonads.

"True hermaphroditism"
With some conditions of intersexuality, even the chromosomal sex may not be clear. A "true hermaphrodite" is defined as someone with both male gonadal tissue (testes) and female gonadal tissue (ovarian tissue).

In 2004, researchers at UCLA published their studies of a lateral gynandromorphic hermaphroditic bird, which had a testicle on the right and an ovary on the left. Its entire body was split down the middle between male and female, with hormones from both gonads running through the blood.

This extreme example of hermaphroditism is quite rare.

Ovotestes
Although there are no definite reports on any true hermaphroditism in humans, there is, on the other hand, a spectrum of forms of ovotestes. The varieties range, including having two ovotestes or having one ovary and one ovotestis. This is often in the form of streak gonads. Phenotype is not determinable from the ovotestes; in some case the appearance is "fairly typically female," in others it is "fairly typically male," and it may also be "fairly in-between in terms of genital development."

Intersex activist Cheryl Chase is an example of someone with ovotestes.

Other diagnostic signs
In order to help in classification, other methods than a genitalia inspection can be performed:

For instance, a karyotype display of a tissue sample may determine which of the causes of intersexuality is prevalent in the case.

Management
Management of intersexuality can be categorized into one of the following two:
 * 1) Treatments: Restore functionality (or potential functionality)
 * 2) Enhancements: Give the ability to identify with “mainstream” people e.g. breast enlargement surgery

However, there are other categorisation systems of management of intersexuality which fall into neither category.

In any case, the most common procedure is surgery.

Surgery
The exact procedure of the surgery depends on what is the cause of a less common body phenotype in the first place.

However, there is often concern over whether surgery should be performed at all. Some[41] say that surgical treatment is socially motivated and hence ethically questionable; without evidence doctors regularly assume that intersex persons can not have a clear identity. This is often taken further with parents of intersex babies being advised that without surgery their child will be stigmatized. Further, since almost all such surgeries are undertaken to fashion female genitalia for the child, it is more difficult for the child to present as male if they later select a male gender identity. 20% to 30% of surgical cases result in a loss of sexual sensation (Newman 1991, 1992).

There is often concern whether surgery should be performed at all.

S (Alice Dreger) say that surgical treatment is socially motivated and hence ethically questionable; without evidence doctors regularly assume that intersexed persons can not have a clear identity. This is often taken further with parents of intersexed babies advised that without surgery their child will be stigmatized. Further, since almost all such surgeries are undertaken to fashion female genitalia for the child, it is more difficult for the child to present as male if they later select a male gender identity. 20% to 30% of surgical cases result in a loss of sexual sensation (Newman 1991, 1992).

Conventionally, surgery is performed at birth. Intersex advocates such as Anne Fausto-Sterling in her Sexing the Body argue surgery on intersex babies should wait until the child can make an informed decision, and label operation without consent as genital mutilation.

"Mainstream" sex development
The common pathway of sexual differentiation, where a productive human female has an XX chromosome pair, and a productive male has an XY pair, is relevant to the development of intersexed conditions.

During fertilization, the sperm adds either an X (female) or Y (male) chromosome to the X in the ovum. This determines the genetic sex of the embryo. During the first weeks of development, genetic male and female fetuses are "anatomically indistinguishable," with primitive gonads beginning to develop during approximately the sixth week of gestation. The gonads, in a "bipotential state," may develop into either testes (the male gonads) or ovaries (the female gonads) depending on the consequent events. Through the seventh week, male and female fetuses appear identical.

At around eight weeks of gestation, the gonads of an XY embryo differentiate into functional testes, secreting testosterone. Ovarian differentiation, for XX embryos, does not occur until approximately Week 12 of gestation. In normal female differentiation the müllerian duct system develops into the uterus, Fallopian tubes, and inner third of the vagina. In males the müllerian duct-inhibiting hormone MIH causes this duct system to regress. Next, androgens cause the development of the Wolffian duct system, which develops into the vas deferens, seminal vesicles, and ejaculatory ducts. By birth, the typical fetus has been completely "sexed" male or female, the hormones and genital development remaining consistent with the genetic sex.

Conditions
The final body appearance doesn't always correspond with what is dictated by the genes. In other words, there is sometimes an incongruence between genotypic (chromosomal) and phenotypic sex. Although there neither no less common chromosomal sex nor mosaicism/chimerism, but just the most common types (XY or XX), less common phenotypes still appear in such cases. In this sense, the common habit in the 21st century of elevating the role of the sex chromosomes above all other factors when determining gender may be analogous to the older habit of finding "true" sex in the gonads.

This phenomenon complicates the common XY sex-determination system, because it proves that genes don't always definitely determine the sex.

Complications
Furthermore, in the cases where nonfunctional testes are present, there is a risk that these develop cancer. Therefore, doctors either remove them by orchidectomy (surgical procedure) or monitor them carefully. This is the case for instance in androgen insensitivity syndrome.

It should be noted however that there is a growing political intersex movement which argues against labeling these categories as disorders.