Intersex people

INTERSEX PEOPLE: What You Should Know and Why You Should Care Presented By Veronica Drantz, PhD And Intersex Spokespersons Alex & Mugsie Prepared for H.E.R. Day at the Center on Halsted June 11, 2011

Famous Intersex People
Carolyn Cossey XXXY (Klinefelter’s)
Julia Child XY (CAIS)
Caster Semenya (CAIS?)

Intersex People Are Natural
"There is a spectrum of sexual identity and intersex people bridge this spectrum. We have a unique vision to offer the world and we have existed since the dawn of humanity."
Hida Viloria – intersex activist
XX (CAH)

The Myth: “The Gender Binary”
Gametes are binary (eggs or sperm)
Organisms that make the gametes are not binary!

23 Pairs Human Chromosomes XX (Female), XY (Male) Figure 25-2 Karyotype of chromosomes from a normal male. The chromosomes have been stained with Giemsa's stain, which produces a characteristic banding pattern.

Genetic Sex Determination
Typical male has Y chromosome
Typical female has no Y chromosome
Figure 25-1 Basis of genetic sex determination. In the two-stage meiotic division in the female, only one cell survives as the mature ovum. In the male, the meiotic division results in the formation of four sperms, two containing the X and two the Y chromosome. Fertilization thus produces a male zygote with 22 pairs of autosomes plus an X and a Y or a female zygote with 22 pairs of autosomes and two X chromosomes. Note that for clarity, this figure and Figures 25–6 and 25–7 differ from the current international nomenclature for karyotypes, which lists the total number of chromosomes followed by the sex chromosome pattern. Thus, XO is 45, X; XY is 46, XY; XXY is 47, XXY, and so on.

Y Chromosome Has Testis-determining Region (Gene)
Is a sex-determining gene on the Y chromosome in placental mammals and marsupials (Y chromosome evolved from X chromosome)
Initiates male sex determination
Located near tip of short arm of Y chromosome
Codes for SRY protein
SRY = Sex-determining region Y
aka testis determining factor = TDF
Causes gonad to differentiate into testis
SRY protein is DNA-binding regulatory protein
Acts as transcription factor
Initiates transcription of cascade of genes necessary for testicular differentiation

Multiple Genes Determine Gonad Differentiation
MALES: SRY upregulates SOX9 activating expression of steroid genesis factor 1 (SF1) and other genes responsible for testes differentiation and INHIBITING expression of WNT4
FEMALES: WNT upregulates DAX1 (when inhibited); DAX inhibits SOX9 expression and upregulates downstream target genes that induce ovarian differentiation
http://quizlet.com/3597081/x-and-y-chromosomes-flash-cards/

Genes on Sex Chromosomes Are Expressed in Brain of Embryo
XX and XY embryonic rodent brains develop differently before gonads develop (so hormone influence is not a factor)
Laura L. Carruth, Ingrid Reisert & Arthur P. Arnold. Sex chromosome genes directly affect brain sexual differentiation NATURE NEUROSCIENCE 5, 933 - 934 (2002)
Phoebe Dewing, Tao Shi, Steve Horvath, Eric Vilain Sexually dimorphic gene expression in mouse brain precedes gonadal Differentiation MOLECULAR BRAIN RESEARCH 118 (2003) http://www.shb-info.org/sitebuildercontent/sitebuilderfiles/4_vilain_et_al.pdf

“Organization – Activation” Mechanism
“ Prenatal hormone theory”
Organization before birth
Activation at puberty

Sexual Differentiation of Internal Genitalia Figure 25-4 Embryonic differentiation of male and female internal genitalia (genital ducts) from wolffian (male) and müllerian (female) primordia.

Sexual Differentiation of External Genitalia Figure 25-5 Differentiation of male and female external genitalia from indifferent primordial structures in the embryo.

Sexual Development in Mammals Figure 25-6 Diagrammatic summary of normal sex determination, differentiation, and development in humans. MIS, müllerian inhibiting substance; T, testosterone; DHT, Dihydrotestosterone

Androgen Hormones Are Made in Adrenal Glands and Gonads http://www.carolguze.com/text/442-6-sexual_differentiation.shtml 17  -HSD =17-beta hydroxysteroid dehydrogenase

Male Testosterone Levels Figure 25-8 Plasma testosterone levels at various ages in human males.

Mechanism of Steroid Hormone Action Brain Figure 25-18 Schematic diagram of the actions of testosterone (solid arrows) and dihydrotestosterone (dashed arrows).

Puberty = “Activation” Alteration in typical women Female Secondary Sex Characteristic Cause Genitalia Enlargement of breasts, uterus, vagina Estrogen Voice larynx retains prepubertal proportions; high-pitched voice Lack of testicular androgens Hair less body hair, more scalp hair, flat-topped pubic hair pattern Lack of testicular androgens Pubic and axillary hair Androgen Mental Interest in sex Androgen Body Conformation Narrow shoulders, broad hips, thighs that converge, arms that diverge (wide carrying angle), female distribution of fat in breasts & buttocks Lack of testicular androgens Skin Sebaceous glands more fluid (inhibit acne) Estrogen

Activation at Puberty
Tanner scales
http://upload.wikimedia.org/wikipedia/commons/thumb/0/0c/Tanner_scale-female.svg/240px-Tanner_scale-female.svg.png
http://upload.wikimedia.org/wikipedia/commons/8/8f/Tanner_scale-male.png

Male Is Altered Female !!!
Mammalian body plan is inherently female
Every individual’s body plan is a variation on the female theme
Default (female)
Fully altered (male)
Partly altered (intersex)
Everyone falls on some point of the continuum from female (gynemorphic) to male (andromorphic)
This sexual body plan is organized by presence or absence of steroid hormones during the critical period of development
Sexual systems are activated later by steroid hormones during puberty

What Is a Female? A Male?
Primary sex characteristic
Type of gonads
Female gonad is ovary – produces eggs
Male gonad is testis – produces sperm
Develop in fetus
Testosterone & its derivatives are “directional”
Secondary sex characteristics
Produced by gonadal hormones
Androgens
Estrogens
Develop during puberty
Hormones are “activational”

Sexual Identity & Orientation – Nature or Nurture?
The genitalia are obviously organized before birth, and we obviously do not learn or choose our genetic sex, gonadal sex, hormonal sex, or somatic sex
But what about sexual behavior? Sexual identity? Sexual orientation? Innate or learned/chosen?
Is the brain, like the body, organized by the presence or absence of sex hormones before birth?
Evidence for Organization-Activation Mechanism
David Reimer story
Intersex people (AIS, CAH, cloacal exstrophy)
Brain work (nonhuman mammals, humans)

John Money "Neutrality-at-Birth“ Theory
“ Sexual behavior and orientation as male or female does not have an innate, instinctive basis”
Money, J. Hermaphroditism, gender and precocity in hyperadrenocorticism: Psychologic findings. BULLETIN OF THE JOHNS HOPKINS HOSPITAL 96:253-264 (1955)
Postulate 1: Individuals are psychosexually neutral at birth
Postulate 2: Healthy psychosexual development is intimately related to the appearance of the genitals
No evidence to support this theory (s erious flaws in Money’s statistical and research methods)
*Cappon D, Ezrin C, Lynes P. Psychosexual identification (psychogender) in the intersexed THE CANADIAN PSYCHIATRIC ASSOCIATION JOURNAL 4:90-106 (1959)
Psychologist

Milton Diamond “Sexuality-at-Birth" Theory
Prenatal genetic and hormonal influences predispose at birth to a male or female sexual identity
Inherent sexuality provides built-in "bias“ with which the individual interacts with environment; sexual behavior and thus gender role, are not neutral and without initial direction at birth
Organization – Activation Mechanism
Evolutionary view
Biologist

1959 – Breakthrough at University of Kansas
Phoenix CH, Goy RW, Gerall AA, Young WC. Organizing action of prenatally administered testosterone propionate on the tissues mediating mating behavior in the female guinea pig. ENDOCRINOLOGY 65:369-382 (1959)
Milton Diamond
Felt that fellow scientists were too cautious failing to link their animal findings to human situation
Decided to write essay challenging psychosexual neutrality theory

Diamond Versus Money
Diamond challenges Money - 1965
Diamond, Milton. A critical evaluation of the ontogeny of human sexual behavior. QUARTERLY REVIEW OF BIOLOGY 40:147 – 175 (1965)
Over the years, animal work accumulated showing “determining influence” of prenatal hormones
Money responds to challenge - December 28, 1972
Symposium of American Association for the Advancement of Science “Sex Role Learning in Childhood and Adolescence”
Man & Woman, Boy & Girl: the differentiation and dimorphism of gender identity from conception to maturity . Baltimore, MD: John Hopkins University Press, 1972. 311 p. (Depts Psychiatry and Pediatrics, John Hopkins Univ. Sch. Med., Baltimore, MD)

The David Reimer Story “Identical Twins Case”
“ Nature-Nurture Experiment”
Circumcision accident destroyed John’s penis; Dr. Money consulted
John is “assigned” as Joan
Same nature : same genetics, womb
Different nurturing : raised as different genders
Touted as a success by John Money, this “Nurture Over Nature” case report became the foundation of standard care for treatment of certain intersex conditions, micropenis, and accidental penile amputation in infancy

Colapinto, John. As Nature Made Him - The Boy Who Was Raised As A Girl HarperCollins (2000)

Joan/Brenda Becomes John/David
“ Joan’s turning point occurred at the age of 14, when she, on her own initiative, began living as a boy, John. John recalls how soon thereafter he finally learned the truth, “In a tearful episode following John’s prodding, his father told him of the history of what had transpired as an infant and why. John recalls: ‘All of a sudden everything clicked. For the first time things made sense and I understood who and what I was.’”
Beh HG, Diamond M. An Emerging Ethical and Medical Dilemma: Should Physicians Perform Sex Assignment on Infants with Ambiguous Genitalia? MICHIGAN JOURNAL OF GENDER & LAW 7: 1-63, 2000

Colapinto, John. As Nature Made Him - The Boy Who Was Raised As A Girl HarperCollins (2000)

John Money tells medical community that Joan/Brenda is a happy girl/woman and then claims to lose track of her
Milton Diamond finds Joan/Brenda living as David!
When David discovered his case was medically famous and that thousands of intersex babies had suffered his plight, he cooperated with Milton Diamond and “went public”
Milton Diamond, Ph.D. & H. Keith Sigmundson, M.D. Sex Reassignment at Birth: A Long Term Review and Clinical Implications. ARCHIVES OF PEDIATRIC & ADOLESCENT MEDICINE 151:298-304 (1997)
Money’s view is now discredited!
David Reimer (1965-2004)

Intersex People Curtis Hinkle - Founder Organization Intersex International

Intersexuality
Incidence: 1.7%*- 2.5% ???
Intersexual people are as natural as the rest of us
They are nature’s experiments providing information about sexual development
Only in recent years have we been able to track intersexual people over their lifetime and find out about their sexual identity and orientation
They illustrate how no definition of female or male is always “satisfying”
* Fausto-Sterling, Anne. SEXING THE BODY: GENDER POLITICS AND THE CONSTRUCTION OF SEXUALITY. Basic Books (2000)

Sex Has Many Levels
Genetic Sex – Chromosomes (X & Y in mammals only)
Gonadal Sex – Ovaries/Testis
Hormonal Sex – Estrogen/Testosterone
Somatic Sex – Body anatomy/physiology
Psychological Sex - Sexual identity
and then there is
Sexual orientation
Primary sex characteristic

Unequal Crossing-over Between X and Y During Paternal Meiosis
Translocation of SRY gene from Y to X chromosome
XY Female
Swyer syndrome
Absence or mutation of SRY on Y produces females with gonadal dysgenesis
XX Male
XX male syndrome
SRY gene in one or both X chromosomes, produces infertile males

Variations of Genetic Sexes
Nondisjunction of chromosomes during meiosis in parent’s gonad
XO Female (Turner Female)
XXX Female (Superfemale)
XXY, XXXY etc. Male (Klinefelter Male)
XYY Male (Jacob’s Syndrome)
48XYXY
http://www.genetic-diseases.net/down-syndrome/

Turner’s Syndrome
Karyotype is 44 autosomes plus XO
Ovarian agenesis or gonadal dysgenesis
Gonads rudimentary or absent
Female external genitalia
Short stature, other congenital abnormalities
No maturation at puberty
Luria, Z., Friedman, S., and Rose, M.D. HUMAN SEXUALITY. New York: John Wiley & Sons (1987)

Klinefelter Syndrome
Karyotype 47XXY
Most common sex chromosome disorder (1 or 2 cases/1000)
Male phenotype
Hypogonadism
Seminiferous tubule dysgenesis
Reduced or absent spermatogenesis
Variability in presentation is related mainly to the timing and amount of androgen deficiency
Increased FSH levels (indicates low inhibin B from Sertoli cells)
The more supernumerary sex chromosomes that exist, the more likely are detrimental physical and mental findings
Cryptorchidism 3X more frequent
Taller, greater leg growth ( 5 and 8 years)
Tendency for central obesity
Minority born with small penises; testosterone cream caused penis growth; however, normal size penis in only 77% by end of puberty; in contrast, testes normal size at birth but fail to grow normally
Gynecomastia 30% to 90% of cases
May have diminished body and facial hair, female pubic hair pattern, small phallus, poor muscular development, and progressive disproportion in leg and body length, feminine fat distribution
Fertility becomes increasingly doubtful as age advances; however, before infertility is predicted, sperm analysis is required because paternity has been documented
Forbes, C.D. and Jackson, W.F. A COLOUR ATLAS AND TEXT OF CLINICAL MEDICINE. England: Mosby-Wolfe (1993) http://www.aurorahealthcare.org/yourhealth/healthgate/images/si55551770.jpg

Klinefelter Syndrome & Gender Expression
Infrequently discussed in medical descriptions of KS are individuals’ concerns with gender expressions and feelings
An unknown percentage of persons who have KS experience androgynous or feminine feelings that can develop at an early age
Some people who have KS consider themselves to be transgendered , others intersexed, and others transsexual; in one study, investigators concluded that all of their KS patients viewed their personalities as dual male and female
A host of investigators similarly reported cases of men who had KS who transitioned to live as women or who harbored aspects of gender dysphoria
Carolyn Cossey, a “James Bond girl,” was raised as boy, but changed to live as a girl at young age, and became a famous model; her karyotype was found to be XXXY
Shirley Ratcliffe Arch Dis Child 1999; 80 :192–195

Sex Chromosome Mosaics
A genetic mosaic is a creature whose body is built of a mixture of cells of two or more different genotypes
Fusion of different embryos to form chimera
Nondisjunction or mutation in stem cell undergoing mitosis in early embryo creating two genetic kinds of cells
Mosaic intersex types
46XY/47XXY
46X/46XY

Androgen Insensitivity Syndrome
Caused by mutations in the gene for the androgen receptor
Recessive X-linked single gene syndrome (mother is carrier)
Genetic males - 46 XY
Testes (abdominal or inguinal) secrete testosterone
No uterus or other internal female genitalia (MIS worked)
Male internal genitalia undeveloped (Testosterone did not work)
Female secondary sex characteristics develop (eg, breasts)
At puberty testosterone from testes is turned into estrogen by enzyme (aromatase) in peripheral tissues
Often discover their condition when they fail to menstruate (No uterus)
(Julia Child – CAIS)
B rain

CAIS People Feel Like Women! Luria, Z., Friedman, S., and Rose, M.D. HUMAN SEXUALITY. New York: John Wiley & Sons, 1987. Forbes, C.D. and Jackson, W.F. A COLOUR ATLAS AND TEXT OF CLINICAL MEDICINE. England: Mosby-Wolfe (1993)

Partial AIS (2-5): Cannot Predict Sexual Identity
Grade 1: normal masculinization in utero
Grade 2: male phenotype with mild defect in masculinization (eg, isolated hypospadias)
Grade 3: male phenotype with severe defect in masculinization—small penis, perineoscrotal hypospadias, bifid scrotum or cryptorchidism
Grade 4: severe genital ambiguity— clitoral-like phallus, labioscrotal folds, single perineal orifice
Grade 5: female phenotype with posterior labial fusion and clitoromegaly
Grade 6/7 female phenotype (grade 6 if pubic hair present in adulthood, grade 7 if no pubic hair in adulthood)
Numbered I through 7 in order of increasing severity (more defective masculinization ) Adapted from Quigley CA, DeBellis A, Marschke KB, El-Awady MK, Wilson EM, French FS. Androgen Receptor Defects: Historical, Clinical, And Molecular Perspectives. ENDOCRINE REV, 1 6:282; (1995) with permission. In: Diamond, Milton and Watson, Linda Ann. Androgen Insensitivity Syndrome And Klinefelter’s Syndrome: Sex And Gender Considerations. CHILD ADOLESC PSYCHIATRIC CLIN N AM 13: 623—640 (2004)

Complete AIS & Gender Identity
39 subjects:
100% lived as women and believed that it was the best decision for them; however, this was not a simple solution for all
“ Acceptance of assignment does not mean that assignment has been correct. It just means that most are able to adapt and live with the handicap; however, they might have preferred other options”
* Indicates to me that androgen receptor is required for “alteration” of human gender identity brain region(s). Diamond, Milton and Watson, Linda Ann. “Androgen insensitivity syndrome and Klinefelter’s syndrome: sex and gender considerations” Child Adolesc Psychiatric Clin N Am 13 (2004) 623—640 published statements from ALIAS, an AIS newsletter. Agree Disagree “ I don’t think I am any different in feeling than if I were born XX, feel very female.” 82%* 18% “ All my efforts over the years in presenting a female persona have left me completely exhausted. I might just as well have had a mastectomy, cut my hair short and lived as a celibate man. It would actually have been easier I think.” 10% 90% I have to “work at being a woman” 56% (dressing in a feminine way or using cosmetics or hair styles in a way to signal “female” unambiguously, altering selection of clothes; 30% did above much of the time) 44% Considered suicide 62% 38% Attempted suicide 23% 77%

Partial AIS & Gender Identity
18 subjects:
Often ambivalent about assigned gender
67% believed that the gender in which they were raised was best for them, whereas the others voiced reservations
“ Gender switch” occurred at mean age of 33, range 18-46)
* Now angry about castration, vaginal reconstructions surgery, and somatic feminization by estrogen treatment since puberty **Attempted before switching Diamond, Milton and Watson, Linda Ann. “Androgen insensitivity syndrome and Klinefelter’s syndrome: sex and gender considerations” Child Adolesc Psychiatric Clin N Am 13 (2004) 623—640 PARTIAL AIS 8 raised as boys 4 live as women 10 raised as girls 2 live as men* Considered suicide 61% Attempted suicide 17%

Congenital Adrenal Hyperplasia (CAH)
21  -hydroxylase enzyme deficiency (vast majority)
Other enzyme deficiencies (rare)
Continuum of CAH conditions
Classical
Salt-wasting
Simple virilizing
Nonclassical
Phenotype correlates with genotype and reflects residual activity of milder mutation
3  -HSD = 3-beta hydroxysteroid dehydrogenase; 17  -HSD = 17-beta hydroxysteroid dehydrogenase http://www.carolguze.com/text/442-6-sexual_differentiation.shtml

Five Degrees of Virilization Affecting the Urogenital Sinus and External Genitalia in Females Hines M, Brook C., Conway, G.S. Androgen And Psychosexual Development Core Gender Identity, Sexual Orientation And Recalled Childhood Gender Role Behavior In Women And Men With Congenital Adrenal Hyperplasia (CAH). J SEX RES, 41: 75-81 (2004)

Congenital Adrenal Hyperplasia (CAH) Luria, Z., Friedman, S., and Rose, M.D. HUMAN SEXUALITY. New York: John Wiley & Sons (1987) Forbes, C.D. and Jackson, W.F. A COLOUR ATLAS AND TEXT OF CLINICAL MEDICINE. England: Mosby-Wolfe (1993)

Frequency of CAH
Figure 4. Frequency of nonclassical and classical 21-hydroxylase deficiency in comparison with other autosomal recessive disease incidences
Hines M, Brook C., Conway, G.S. Androgen And Psychosexual Development Core Gender Identity, Sexual Orientation And Recalled Childhood Gender Role Behavior In Women And Men With Congenital Adrenal Hyperplasiz (CAH). J SEX RES, 41: 75-81 (2004)

CAH (Adrenogenital Syndrome) Luria, Z., Friedman, S., and Rose, M.D. HUMAN SEXUALITY. New York: John Wiley & Sons (1987)

Sexuality of CAH Women
More male-typical play behavior as children*
Less satisfaction with the female sex of assignment and less heterosexual interest*
CAH girls who show the greatest alterations in childhood play behavior may be the most likely to develop a bisexual or homosexual orientation as adults and to be dissatisfied with the female sex of assignment*
“ The finding of greater same-sex fantasy … is consistent with the prenatal hormonal hypothesis”**
“… excess prenatal androgens predispose some women to the development of same-gender sexual orientation”***
“ Dose-response relationship of androgens with sexual orientation” - a study of women with various forms of CAH****
*Hines M, Brook C., Conway, G.S. Androgen And Psychosexual Development Core Gender Identity, Sexual Orientation And Recalled Childhood Gender Role Behavior In Women And Men With Congenital Adrenal Hyperplasia (CAH). J SEX RES, 41: 75-81 (2004)
**Veniegas, Rosemary C. Biological Research on Women's Sexual Orientations: Evaluating the Scientific Evidence. J SOCIAL ISSUES, (2000)
***Pattatucci, A. M. L., & Hamer, D. H. Development And Familiality Of Sexual Orientation In Females. BEHAVIOR GENETICS, 25: 407-420 (1995)
****Meyer-Bahlburg HF, Dolezal C, Baker SE, New MI. “Sexual Orientation in Women with Classical or Non-Classical Congenital Adrenal Hyperplasia as a Function of Degree of Prenatal Androgen Excess” ARCHIVES OF SEXUAL BEHAVIOR 1: 85-99 (2008) ,

Hypospadias
These common conditions are often not regarded as intersex condition.
But they are!

Cloacal Exstrophy
Rare, complex defect of the entire pelvis and its contents
Associated with severe phallic inadequacy or phallic absence in genetic males
For about 25 years, neonatal assignment to female sex has been advocated for affected males
Bladder Extrophy and absence of the penis green arrows: everted bladder; blue arrows: the scrotum; yellow arrows: umbilcal cord

Prenatal Androgen - Major Factor in Development of Sexual Identity
Genetically and hormonally male-born children may identify as males despite being raised as females and undergoing feminizing genitoplasty at birth
Kayla’s story
Reiner, William G. and Gearhart, John P. Discordant Sexual Identity in Some Genetic Males with Cloacal Exstrophy Assigned to Female Sex at Birth. THE NEW ENGLAND JOURNAL OF MEDICINE, 350:333-341 (2004)

The Sexual Brain
Amygdala
Part of Limbic System
Genesis of emotions & emotional expression
Hypothalamus
Homeostasis
Neuroendocrine Control
Instinctive Drives & Behavior
Hunger
Thirst
Sleep
Body Rhythms
Sex
Netter, Frank H. The CIBA COLLECTION OF MEDICAL ILLUSTRATIONS. Vol I. Nervous System. Part I. Anatomy & Physiology. New York: CIBA (1983)

Sexual Behavior and Gonadal Function Controlled by Different Regions of Hypothalamus in Mammals Figure 25-28 Loci where implantations of estrogen in the hypothalamus affect ovarian weight and sexual behavior in rats, projected on a sagittal section of the hypothalamus. The implants that stimulate sex behavior are located in the suprachiasmatic area above the optic chiasm (blue area), whereas ovarian atrophy is produced by implants in the arcuate nucleus and surrounding ventral hypothalamus (red). MB, mamillary body

Summary - Core Sexuality
The scientific story explains everyone!
“ The preponderance of evidence seems to indicate that the theory of organization-activation for the development of sexual behavior is certain for non-human mammals and almost certain for humans"
Milton Diamond. Clinical implications of the organizational and activational effects of hormones. HORMONES AND BEHAVIOR 55:621–632 (2009)
Pivotal work on human anterior hypothalamus:
LeVay, S. A Difference In Hypothalamic Structure Between Heterosexual And Homosexual Men. SCIENCE, 253: 1034–1037 (1991)
Zhou, J.N. Hofman, M.A. Gooren, L.J. and Swaab, D.F.. A Sex Difference in the Human Brain and its Relation to Transsexuality. NATURE, 378: 68-70 (1995)
Kruijver, Frank P. M., Zhou, Jiang-Ning, Pool, Chris W. Hofman, Michel A.,. Gooren, Louis J. G And Swaab, Dick F. Male-To-Female Transsexuals Have Female Neuron Numbers In A Limbic Nucleus. J CLIN ENDOCRINOL METAB, 85: 2034-2041 (2000)
Garcia-Falgueras, Alicia, Swaab, Dick F. A Sex Difference In The Hypothalamic Uncinate Nucleus: Relationship To Gender Identity. BRAIN, (Nov 2, 2008)

Organization-Activation Theory
“ The fetal brain develops during the intrauterine period in the male direction through a direct action of testosterone on the developing nerve cells, or in the female direction through the absence of this hormone surge. In this way, our gender identity (the conviction of belonging to the male or female gender) and sexual orientation are programmed or organized into our brain structures when we are still in the womb”
Garcia-Falgueras A, Swaab DF. Sexual hormones and the brain: an essential alliance for sexual identity and sexual orientation PEDIATRIC NEUROENDOCRINOLOGY 17: 22-35 (2010)

Critical Periods of Genitalia and Brain Are Different
“ However, since sexual differentiation of the genitals takes place in the first two months of pregnancy and sexual differentiation of the brain starts in the second half of pregnancy, these two processes can be influenced independently, which may result in extreme cases in transsexuality.”
“ This also means that in the event of ambiguous sex at birth, the degree of masculinization of the genitals may not reflect the degree of masculinization of the brain.”

Core Sexuality: Nature - Not Nurture!
“ There is no indication that social environment after birth has an effect on gender identity or sexual orientation”

Three Dimensions of Core Sexuality
Somatic Morphology (X axis)
Sexual Identity (Y axis)
Sexual Orientation (Z axis)
Everyone occupies a point in this three-dimensional space! Gynemorphic Andromorphic Gynecentric Androcentric Gynephilic Androphilic

Sex  Gender
Sex is biological
Gender is cultural
Gender “traits” differ from culture to culture and from time to time
Castor Semenya Controversy over whether this South African eighteen-year old should be allowed to compete as a woman continues

The “Gender Binary”
We live in a “binary gendered” culture with two genders only
Woman
Man
Many cultures recognize more than two genders

NORTH AMERICA
Two-spirits – Native American Berdache – Illiniwek (Illinois) Muxes – Zapotec People, Oaxaca, Mexico
Thanks to Lorelei Erisis http://transprov.wordpress.com/2009/05/21/simple-list-of-third-gender-and-gender-variant-groups/ Two-spirits Muxes

SOUTH AMERICA
Travestís – Brazil Bichas Viados Guevedoche – Dominican Republic Quariwarmi – Incan
Thanks to Lorelei Erisis http://transprov.wordpress.com/2009/05/21/simple-list-of-third-gender-and-gender-variant-groups/ Viados Travestís

EUROPE
The Sworn Virgin – Balkans Catamites – Ancient Greece Mollies – Modern England Tertium genus hominum (a third human gender) “eunuchs” – Ancient Eastern Mediterranean
Thanks to Lorelei Erisis http://transprov.wordpress.com/2009/05/21/simple-list-of-third-gender-and-gender-variant-groups/ Sworn Virgin Sworn Virgin Catamites

AFRICA
Ashtime – Maale culture of Southern Ethiopia Mashoga – Swahili-speaking areas of the Kenyan coast, particularly Mombasa Mangaiko – The Mbo people, Democratic Republic of the Congo Sḫt (”sekhet”) – Middle Kingdom of Egypt (2000-1800 BCE)
Thanks to Lorelei Erisis http://transprov.wordpress.com/2009/05/21/simple-list-of-third-gender-and-gender-variant-groups/ Mashoga

MIDDLE EAST
The Kurgarûs – Sumer kur.gar.ra ur.sal Zenanas – Arab Xanith or Khanith – Oman
Thanks to Lorelei Erisis http://transprov.wordpress.com/2009/05/21/simple-list-of-third-gender-and-gender-variant-groups/

ASIA
lyha – Mohave Hijras – India, Pakistan & Bangladesh also known as: Aravani/Aruvani or Jogappa Hijira (alt. sp.) – India Sādhi Kotis Sadhin – The Gaddhi in the foothills of the Himalayas
Thanks to Lorelei Erisis http://transprov.wordpress.com/2009/05/21/simple-list-of-third-gender-and-gender-variant-groups/ Hijras

ASIA continued
Basivi – Madras (area of India) Tritiya-prakrti (third-nature) – Indic culture of premodern India Ubhatobyanjanakas – Buddhist Vinaya Pandakas Kathoey – Thailand Pandaka – Ancient Buddhist Societies
Thanks to Lorelei Erisis http://transprov.wordpress.com/2009/05/21/simple-list-of-third-gender-and-gender-variant-groups/ Kathoey Kathoey

ASIA-PACIFIC POLYNESIA
Fa’afafine – Samoa Polynesia Fakaleiti – Tonga Mahu Wahine – Hawaii Mahu Vahine – Tahiti Whakawahine – Māori Akava’ine – Cook Islands
Thanks to Lorelei Erisis http://transprov.wordpress.com/2009/05/21/simple-list-of-third-gender-and-gender-variant-groups/ Fa’afafine

INDONESIA
Waria Kwolu-aatmwol – “Sambia” community in the eastern highlands of Papua New Guinea PHILIPPINES bakla – Tagalog Bayot – Cebuano Agi – Ilonggo Bantut – Tausug Binabae Bading Lakin-on
Thanks to Lorelei Erisis http://transprov.wordpress.com/2009/05/21/simple-list-of-third-gender-and-gender-variant-groups/ Waria bakla

Disordered or Just Different?
Gender binary has permeated medicine
The medical profession has pathologized and stigmatized gender-variant peoples
Lesbian, gay, bisexual people
Transsexual people
Intersex people

Medical Profession’s Treatment of LGBT People Homosexuality was removed from the list of mental disorders by the World Health Organisation in 1990 1952 (DSM-I) Sociopathic Personality Disorders 1968 (DSM-II) Sexual Deviation 1970 Gay rights activists storm APA annual convention 1972 APA annual meeting –first-ever panel of non-patient homosexuals” and Dr. Anonymous 1973 (DSM-II Revision) Deletion of Homosexuality Substitution of Sexual Orientation Disturbance (Homosexuality is not illness but supposedly discomfort with being persecuted as a homosexual is an illness) 1980 (DSM-III) Gender Identity Disorder (GID) Ego-dystonic Homosexuality (formerly SOD) 1987 (DSM-III Revision) Homosexuality omitted entirely (Ego-dystonic homosexuality/SOD removed. It’s normal to not want to be persecuted & empirical data to support diagnosis is lacking) )-> 2000 (APA Position Statement) Ethical psychiatrists should stop conversion or “reparative therapies”

Medical Quackery Continues
Genital normalization surgeries on newborns continue!
DSD = Disorders of Sexual Development
OII objects to “disorder” terminology
http://www.gopetition.com/petitions/solidarity-with-the-intersex-community.html
Milton Diamond recommends Variations of Sex Development
http://adc.bmj.com/content/91/7/554/reply#archdischild_el_2460?sid=437e97e7-049d-42c8-b60f-6d8d02dd31c1
http://adc.bmjjournals.com/cgi/eletters/91/7/554

Intersex Problems Are Socio-cultural
“ The basic problems faced by the intersexed are socio-cultural in nature and not medical and are a result of the dogmatic fundamentalism inherent in the current binary construct of sex and gender”
“ Some intersexed individuals are subjected to genital mutilation in childhood as a result of this totalitarian, sexist oppression”
Curtis Hinkle - Founder Organization Intersex International http://www.gopetition.com/petitions/solidarity-with-the-intersex-community.html

Phall-O-Meter
Whatever happened to “informed consent?”
Whatever happened to “first, do no harm?”
Sharon E. Preves. INTERSEX and IDENTITY The Contested Self Rutgers University Press (2003)

OUTCOMES: Ambiguous Genitalia “Normalization” Surgery
John Money’s lab follows up on some of their intersex cases
Admit that follow-up studies are very limited; hence, the need for this study
Intersex types studied
46, XY
Quigley grade 2-4, average 3.5
AIS, gonadal dysgenesis, others
Turns out that roughly half were assigned/raised as boys, half as girls
Written questionnaire and physical exam
Pigeon; CJ, Wisniewski, AB, Gearhart JP, Meyer-Bah burg, HFL, Rock, JA, Brown, TR, Canella, SJ, Market A, Ngai KM, Money J, Berkovitz GD. Ambiguous Genitalia With Perineoscrotal Hypospadias in 46,XY Individuals: Long-Term Medical, Surgical, and Psychosexual Outcome PEDIATRICS 110:10p (2002)

Genital “Normalization” Surgery - Dismal Outcomes
23% of participants (46XY, Quigley grade 2-4, average 3.5, roughly half were assigned/raised as boys, half as girls) were dissatisfied with sex of rearing
Indicating “general predictions cannot guarantee future gender development for any single case”
This figure could be as high as 44% (if all non-participating patients were also dissatisfied)
Majority (62% men, 67% women) sought counseling concerning condition
Mean surgeries: men 5.8; women 2.1
Half were dissatisfied with body image
Two-thirds were dissatisfied to some degree with sexual function
Researchers never asked: What if we did nothing?
Migeon; CJ, Wisniewski, AB, Gearhart JP, Meyer-Bahlburg, HFL, Rock, JA, Brown, TR, Casella, SJ, Maret A, Ngai KM, Money J, Berkovitz GD. Ambiguous Genitalia With Perineoscrotal Hypospadias in 46,XY Individuals: Long-Term Medical, Surgical, and Psychosexual Outcome PEDIATRICS 110:10p (2002)

Diamond, M. Sex, gender, and identity over the years: a changing perspective CHILD AND ADOLESCENT PSYCHIATRIC CLINICS OF NORTH AMERICA 13:591-607 (2004) NEW STANDARDS OF CARE FOR INTERSEX PATIENTS American Academy Pediatricians year 2000 British Association Pediatric Surgeons year 2001 #1. General moratorium on sex assignment cosmetic surgery No surgical moratorium (In1999, AAP decided that surgical moratorium was “unrealistic” because it was hypothesized that parents would not accept it) No surgical moratorium #2. Moratorium should not be lifted unless and until studies show outcomes are positive Recognized need for more research and greater candor and honesty Recognized need for more research and greater candor and honesty #3. Efforts should be made to undo effects of past physician deception and secrecy No call back to families or individuals that had previous treatment No call back to families or individuals that had previous treatment Response to intersex birth “ Social emergency” Informed consent includes “possibility of non-operative management” Gender assignment All virilized females (CAH or maternal androgen) should be girls (because of retained fertility) Gender assignment on individual basis; may include cultural considerations Clitoral surgery Infants raised as girls “will usually require clitoral reduction” “ There is a strong case for no clitoral surgery in lesser degrees of clitoromegaly” Penile surgery PAIS infants “in whom a very small phallus mandates a female sex of rearing” should have testes removed The risk of malignant testicular changes in AIS is small

Pediatric Policy Is Reprehensible
2006 Consensus Statement - chose term “ Disorders of Sexual Development”
Dismiss “sexual identity” issue: “Structure of the brain is not currently useful for gender assignment”
Factors they say influence their decision on gender assignment
Diagnosis
Genital appearance
Surgical options
Need for lifelong replacement therapy
Potential for fertility
Views of family
Circumstances relating to cultural practices
Collaboration with participants in International Consensus Conference on Intersex organized by Lawson Pediatric Endocrine Society and European Society for Paediatric Endocrinology Lee PA, Houk CP, Ahmed SF, Hughes IA. Consensus statement on management of intersex disorders PEDIATRICS 118:488-500 (2006)

Pediatric Policy Must Change
Pediatricians use parental distress & prejudice to justify damaging surgery
Lee PA, Houk CP, Ahmed SF, Hughes IA. Consensus statement on management of intersex disorders PEDIATRICS 118:488-500 (2006)
Clitoral reduction is “standard clinical procedure” (Dr. Dix P. Poppas, Panel at Weill Cornell Medical College)
http://www.cornellsun.com/section/news/content/2010/10/05/weill-medical-college- says-poppas%E2%80%99-surgical-procedure-standard
Pregnant women treated with dexamethasone to prevent “ behavioral masculinization” (same-sex attraction and tom-boy behavior) in CAH girls
http://www.starobserver.com.au/news/2010/07/15/opposition-to-genital-drugs/27947
Meyer-Bahlburg HF, Dolezal C, Baker SE, New MI. “Sexual Orientation in Women with Classical or Non-Classical Congenital Adrenal Hyperplasia as a Function of Degree of Prenatal Androgen Excess” ARCHIVES OF SEXUAL BEHAVIOR 1: 85-99 (2008) ,
Let’s follow Columbia’s example
Diamond, M. Sex, gender, and identity over the years: a changing perspective CHILD AND ADOLESCENT PSYCHIATRIC CLINICS OF NORTH AMERICA 13:591-607 (2004)
Europe has ethical principles for intersex treatment
Wiesemann, C., Ude-Koeller, S., Sinnecker, G. H. G., & Thyen, U. Ethical principles and recommendations for the medical management of differences of sex development (DSD)/intersex in children and adolescents EUR J PEDIATR 169:671–679 (2010)

INTERSEX People in Germany - Historic Public Consultation and Dialog
INTERSEX people in Germany have been invited to participate and observe proceedings in an historic public consultation and dialog “on the situation of people with intersexuality [sic] in Germany”. The event has been organized by Deutscher Ethikrat - The German Ethics Council – and its participants include ‘experts’ in intersex, medical people, lawyers, parents of intersex people and some intersex people themselves
June 6 th , 2011 http://oiiaustralia.com/13790/intersex-people-germany-experts-deutscher-ethikrat-consultatio/

Poor Medical Treatment of Intersex People
Over 50% are misdiagnosed!
Minto CL, Crouch NS, Conway GS, Creighton SM. XY females: revisiting the diagnosis BJOG: an International Journal of Obstetrics and Gynaecology 112:1407–1410 (2005)
Intersex people have their own physiology and health concerns that are not being addressed by physicians
Holterhus P-M, Bebermeier J-H, Werner R, Demeter J, Richter-Unruh A, Cario G, Appari M, Siebert R, Riepe F, Brooks JD, Hiort O. Disorders of sex development expose transcriptional autonomy of genetic sex and androgen-programmed hormonal sex in human blood leukocytes BMC GENOMICS 10:292 (2009)

Intersex People Now Also Crazy!
Special subtype of gender incongruence in DSD is recommended by Zucker’s committee for the DSM-V!
OII responds: “We see no need to further medicalise and stigmatize intersex people by referring to them as necessarily disordered (DSD) and where mistakes in assignment have been made, we see no value in medicalising and stigmatizing them further by applying another form of disorder called ‘gender incongruence’”
http://www.intersxualite.org/DSM5.html

Common Concerns - LGBT and I
Not “Adams” or “Eves.” Do not fit the “gender binary”
Persecution and discrimination because of sexual difference that is innate
No place in the culture; no public representation

More Common Concerns - LGBT and I
Pathologized as “disordered” (rather than “different”)
Cruel and unnecessary medical “treatments”
Medical damage is based on belief that sexuality is learned
Real medical needs not met by medical profession

More Common Concerns - LGBT and I
“ In the closet” (secrecy & shame)
Must “come out”
Need to politically organize
Civil rights issues based on gender expression
Many intersex people share “sex reassignment” issues with trans people
Transsexuality is a subtype of (brain) intersex

Discrimination Against Intersex People Is a Socio-cultural Disorder
Intersex people are natural variations.
Intersex people are different, not disordered
The suffering of intersex people is not intrinsic to their condition; rather it is imposed by the binary-gendered society/culture

Intersex People Forsaken by Religion and Medicine
Organized religion and the medical profession have been part of the socio-cultural problem and continue to be a problem
The scientific message that “core sexuality is innate” needs to reach the religious communities, medical community, educators, and parents

Human Rights Issue: To Be Who We Are
People should be able to express themselves wherever they feel comfortable on the feminine-masculine continuum without having rights taken away or medical alterations forced upon them to maintain those rights
All of us, not just the Adams and the Eves, have the birthright to be who we naturally and innately are

Basic Human Right: To Be Who We Are!

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Intersex people

by Veronica Drantz on Jun 17, 2011

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Intersex people — Presentation Transcript