5-alpha-reductase is an enzyme converting weaker testosterone into more potent dihydrotestosterone (DHT). When there is little of this enzyme, the baby develops as a girl.  At puberty, though, testosterone production usually rises and can cause ‘virilization’, physical development as a male. Puberty blocking hormones can be used until the child is ready to decide, for themselves, which way they wish to develop at puberty. As there have been cases where the child naturally migrates to a male role, conservative approaches would incorporate listening to the child.

https://ghr.nlm.nih.gov/condition/5-alpha-reductase-deficiency

See also Virilization

Most people with 17-beta hydroxysteroid dehydrogenase 3 deficiency are born with external genitalia that appear typically female. In some cases, the external genitalia do not look clearly male or clearly female. Still other affected infants have genitalia that appear predominantly male, often with an unusually small penis (micropenis) or the urethra opening on the underside of the penis.

It is reported that about half of these individuals adopt a male gender role in adolescence or early adulthood, so childhood gender assignment surgeries are discouraged.

https://ghr.nlm.nih.gov/condition/17-beta-hydroxysteroid-dehydrogenase-3-deficiency#definition

See also Ambiguous Genitalia, Hypospadias, Micropenis

This issue is caused by a shortage of the 21-hydroxylase enzyme.  When 21-hydroxylase is lacking, substances, that are usually used to form cortisol and aldosterone, build up in the adrenal glands and are converted to androgens. These androgens can lead to a genetically 46 ‘XX’ child to develop male appearing physical structures during fetal development.

https://rarediseases.info.nih.gov/diseases/5757/21-hydroxylase-deficiency

See also Androgens, Congenital Adrenal Hyperplasia

This is a birth variation where the outer genitals do not have the typical appearance of either a boy or a girl. It is often the first sign many doctors or parents see that indicate their child is of an intersex background.

Ambiguous Genitalia can be caused by a variety of conditions and rarely need immediate surgical intervention as long as the child is able to excrete and urinate without complications.

DISCLAIMER: The author of this section benefited from the Americans with Disabilities Act protections for intersex individuals during the 1990s.  As such, the author lists their understanding of the legal protections provided by the Act, but is not a legal scholar, nor attorney, so this section should not be construed as legal advice.

Intersex are covered by the ADA under Section 12102 as those suffering from a medical condition that impairs “a major life activity” which explicitly includes “… endocrine and reproductive functions”.

While Section 12211 denotes that “…homosexuality and bisexuality are not impairments and as such are not disabilities under this chapter.”  Its language excluding transexualism uses the phrase “gender identity disorders not resulting from physical impairments” which does not remove intersex people from protection under the ADA.  Fortunately, LGBT are now protected from workplace discrimination under the 2020 Supreme Court decision “BOSTOCK v. CLAYTON COUNTY, GEORGIA”.

Before the ADA went into effect in 1990, major businesses were able to require all job applicants to affirm they could pass a physical examination.  As having an intersex condition was traditionally deemed as being a physical variation disqualifying one from passing a physical examination, companies implicitly barred us from gaining employment at most major employers.  While an intersex person, at the time, could lie about their medical background and claim to be able to pass a physical examination, they could then be terminated from their job for lying on their job application if their intersex background later came to light.

The Americans with Disabilities Act changed that by requiring that businesses could only disqualify people for not passing a physical examination when the disqualifying condition directly impaired one’s ability to perform their job.  Further under Section 12112, businesses are barred from requiring their own medical examination and making further inquiries into an individual’s condition beyond what the employee and their medical providers wish to share.

To assure protection under the ADA, this author joined a Fortune 500 company in 1994 and soon after notified Human Resources of their being intersex.  By so doing, protected status was gained under the ADA and I was protected from managers and coworkers who found out about, and coerced me for, my intersex background.  In fact, three different managers who assumed they could terminate me, or force me out of their division of the business, months later found themselves out of a position, instead, over an eight year period.

Upon notification of one’s intersex status, Human Resources is to create a medical file with that information and protect that information from typical employee record access, limiting it to the equivalent of a ‘need to know’ status for H.R. and management.  During my eight years, two Human Resources people lost their positions for violating those protections.  If an employee does not notify Human Resources of their medical intersex background, then they may not qualify for protections under the ADA on the basis of the employer claiming that they did not ‘know’ the employee was intersex.  They may have suspected, believed, heard a rumor, etc., but unless directly notified by the employee, protections under the ADA likely will not be in place for subsequent discrimination attempts.

Unfortunately, the Americans with Disabilities Act does curtail protections for disabled, and thus intersex, individuals for jobs “in sensitive positions” within, or for, the Department of Defense, the Nuclear Regulatory Commission, and the Department of Transportation.  So an intersex person may wish to avoid such jobs until greater legal protections are put in place.

The ADA also exempts Private Clubs and Religious Organizations from honoring the disability law, and thus any related protections for intersex people.

While the Americans with Disabilities Act denotes that States are not exempt from the provisions of the ADA, it does defer enforcement when working for the Congress of the United States to the ‘chief official’ of each department and committee chair.  As such, the quality of protections an intersex person my find could depend on the results of the most recent congressional election.  The executive and judicial branches of the federal government are not mentioned within the ADA and, as such, the author has no knowledge of what protections or exclusions one may find while working for those entities.

One final word of caution: Section 12205 of the ADA allows the winning party of any lawsuit to recoup attorneys’ fees, litigation expenses, and other costs at the discretion of the judge.  So if you bring a lawsuit in good faith and win, you would be in good shape.  But if you bring a lawsuit and lose, and the judge didn’t feel you should have brought the case, you may find yourself paying the legal bills of a fortune 500 company…!

If you would like more details about the author of this section’s experience, please use our contact page, here.

https://www.ada.gov/pubs/ada.htm

An androgen is any natural or synthetic steroidal hormone that regulates the development and maintenance of male characteristics.  They do so by binding to androgen receptors of invertebrates.  This includes the embryological development of the primary male sex organs, and the development of male secondary sex characteristics at puberty. Androgens are created in the testes, the ovaries, and the adrenal glands.

See also Androgen Insensitivity Syndrome

Androgen insensitivity syndrome is a rare condition that is best known for affecting the development of the genitals and reproductive organs for a child with ‘XY’ genes. An ‘XY’ child born with AIS is deemed genetically male, but the external appearance of their genitals may be female or somewhere between male and female.

Women also respond to androgens and it is possible for ‘XX’ children to be born with limited or no response to androgens, as well, though this most often goes unrecognized as it causes little conflict at puberty.

AIS has common subcategories:

CAIS – Complete Androgen Insensitivity Syndrome results in someone who looks completely female and has no response to androgens.

PAIS – Partial Androgen Insensitivity Syndrome results in someone who has some, but a significantly reduced, response to androgens.

MAIS – Mild/Male Androgen Insensitivity Syndrome results in someone who looks and is functionally male, but has a reduced response to androgens.

https://www.nhs.uk/conditions/androgen-insensitivity-syndrome/

See also Androgens, Estrogen Insensitivity Syndrome (EIS)

A person with 46 ‘XY’ genes, which usually corresponds to a male sex, is born without testes. Within a few weeks of fertilization, the embryo develops rudimentary gonads (testes), which produce hormones responsible for the development of the reproductive system.

If testes fail to develop within eight weeks, the baby will develop female genitalia. If testes begin to develop but are lost or cease to function between eight and ten weeks of gestation, the baby will have ambiguous genitalia when born. However, if the testes are lost after fourteen weeks, the baby will have partial male genitalia with the notable absence of gonads.

https://medlineplus.gov/ency/article/001185.htm

See also Ambiguous Genitalia, Swyer syndrome

Aphallia refers to being born without a penis, in a patient with otherwise typical male anatomy.

https://en.wikipedia.org/wiki/Aphallia

People that have two different sets of DNA are called human chimeras.  This happens when a woman is pregnant with fraternal twins, but the embryos combine into one fetus.  If both sets of cells share the same general set of sex chromosomes, either ‘XX’ or ‘XY’, the resulting child will appear with typical sex characteristics. If the embryos had differing sex chromosomes, the competing cell development can result in a visible variation in external genitalia as well as internal organs.

In the world of DNA testing to confirm paternity and maternity, it has been found that chimeraism can confuse the test results.  One such story is of Lyndia Fairchild: https://en.wikipedia.org/wiki/Lydia_Fairchild

https://en.wikipedia.org/wiki/Chimera_(genetics)#Humans

See also Ambiguous Genitalia, Mosaicism Involving “Sex” Chromosomes.

Clitoromegaly, or macroclitoris, is an uncommon enlargement of the clitoris. It is congenital or acquired, most often as a result of exposure to excess androgens in fetal life, infancy, or adolescence. It is often associated with Congenital Adrenal Hyperplasia (CAH).

https://isna.org/faq/conditions/clitoromegaly/

See also Congenital Adrenal Hyperplasia, Female Genital Mutilation

See Androgen Insensitivity Syndrome.

Within medical circles, this model of patient care became popular for intersex babies starting in the 1950s. In this model, parents are urged to quickly decide on a gender assignment for their intersex baby. It was theorized that a child would identify with whatever gender the parents raised them as.

If a child was found to be deviating from the assigned gender, the parents were encouraged to verbally and physically ‘correct’ the child and enforce stereotypical gender norms.

In practice, this model often resulted in life altering, unnecessary genital surgeries, irreversible hormone treatments, and lying to the child about their medical history. This methodology was also called the Hopkins model.

https://isna.org/faq/concealment

See also Gender Assignment, Gender Identity, Informed Consent, John Money, Patient-Centered Model

Congenital adrenal hyperplasia is a group of rare inherited autosomal gene disorders characterized by a deficiency of one of the enzymes needed to make specific hormones. CAH effects the adrenal glands located at the top of each kidney.

https://www.mayoclinic.org/diseases-conditions/congenital-adrenal-hyperplasia/symptoms-causes/syc-20355205

See also 5-alpha Reductase Deficiency, 17-beta Hydroxysteroid Dehydrogenase 3 Deficiency, 21-Hydroxylase Deficiency

A condition in which one or both of the testes fail to descend from the abdomen into the scrotum.

DSD was coined in the early 21st century to provide an alternative term for children born with intersex traits.  Originally standing for ”Disorders of Sex Development” it has transitioned to ”Differences of Sex Development” as often times these variations are natural, and not needing immediate medical intervention, if any at all.

See also Intersex

EIS is caused by a variation in estrogen receptors which leaves the body unable to use, or with a significantly reduced response to, estrogen. The effects of this are similar to a congenital estrogen deficiency, caused by a defect in aromatase, the enzyme responsible for the body’s ability to use estrogens. This latter condition is referred to as ‘Aromatase Deficiency’.

https://en.wikipedia.org/wiki/Estrogen_insensitivity_syndrome

See also Androgen Insensitivity Syndrome (AIS)

Female genital mutilation, also known as female genital cutting, is the cutting or removal of some or all of the external female genitalia in a child assigned female at birth, or a child to be assigned female after the procedure.

https://www.who.int/news-room/fact-sheets/detail/female-genital-mutilation

See also Clitoromegaly, Intersex Surgery

Gender is how one feels on the inside, typically male or female. This may or may not match their phenotype, or how they appear on the outside. This is a social and cultural role as well as an identity and there is a wide spectrum of how one can feel.

Things that can sometimes, but not always, contribute to gender are genetics, prenatal or possibly postnatal hormones, social, cultural, and environmental influences.

Gender presentation can differ from culture to culture, sometimes with some aspects of one culture being the opposite in another culture. One such example is the Wodaabe.  A nomadic tribe in Africa, they hold an annual fertility festival called Gerewol, where men put on makeup, do their hair and dress in their finest to attract a female mates.

https://en.wikipedia.org/wiki/Social_construction_of_gender

See also Phenotype

Labeling a baby as a boy or girl. This does not require surgery.

See also Gender, Gender Identity

A group of professionals who, without knowledge of the child’s personality, interests, or gender leanings, conclude amongst themselves what sex a child with ambiguous genitalia should be surgically made. These boards are often assembled at Hospitals and can include endocrinologist, urological surgeons, ethicist, psychologist and clergy of a religion the child has not yet embraced.

Often times these committees are used to pressure parents of intersex children to allow a surgeon to perform a sex assigning surgery, despite there often being no urgent reason for such surgery. The parents, faced with a group of professionals with imposing titles, feel intimidated into accepting a surgeon’s desire to perform these surgeries. Professionals invited to participate in these committees have reported speaking out against such surgeries, only to be out-voted, but then have their name and title used as someone agreeing on the need for surgery.

See also Intersex Surgery

An individual’s personal feelings about their gender. A person may have a different gender identity than the gender assigned to them at birth. For intersex individuals, a wrongly assigned, paperwork gender has far less emotional impact than a wrongly, surgically assigned sex.

See also Gender, Gender Assignment

Gonad is generic term used to describe both testes, ovaries, or an ovotestes. It is especially useful when a structure might not appear typical.

See also Mixed Gonad Dysgenesis, Ovo-Testis, Streak Gonads

Gonadal dysgenesis is classified as any congenital developmental disorder of the reproductive system in a male or female. It is the uncommon development of the gonads in an embryo, with expected reproductive tissue replaced with sometimes functionless, fibrous tissue, termed ‘streak gonads’.

Often gonadal dysgenesis is associated with Swyer Syndrome.

https://en.wikipedia.org/wiki/Gonadal_dysgenesis

See also Mixed Gonad Dysgenesis, Ovo-Testis, Streak Gonads, Swyer Syndrome.

Minimally developed gonad tissue present in place of testes or in place of ovaries.

https://pubmed.ncbi.nlm.nih.gov/503377

See also Gonad, Gonadal Dysgenesis, Gonadectomy / Orchiectomy, Mixed Gonadal Dysgenesis, Streak Gonads

See also Gonad, Intersex Surgery

A mythological being that is both fully female and fully male. This is physiologically impossible, and also considered a stigmatizing term.

While some intersex people have reclaimed this term, it should never be used by sex-typical people, much as derogatory racial terms can be used by members of that race, but not by members of other races.

https://en.wikipedia.org/wiki/Hermaphrodite

See also Intersex, Pseudohermaphrodite

See Concealment-Centered Model

Chemicals created in endocrine glands that regulate physiology and behavior. Sex hormones include androgens, estrogens, and progestogens, among others.

https://en.wikipedia.org/wiki/Hormone

See also Androgens, Androgen Insensitivity Syndrome, Estrogen Insensitivity Syndrome

The practice of weighing all options, considering evidence, alternatives, and potential risks in order to make a truly informed medical decision. Such decisions are best made over a period of time and not in a rushed fashion.

https://www.ama-assn.org/delivering-care/ethics/informed-consent

See also Gender Committee / Gender Selection Committee, Intersex Surgery, Revision Surgery

The term intersex is used to describe someone who is born with variations in sex characteristics. These could include chromosomes, genitals, gonads, or sex hormones which do not fit the typical definitions for male or female bodies.

Often a cosmetic surgical intervention on children which became favored for babies with uncommon genital appearances.  Initially performed and developed in the early 20th century, such surgeries gained popularity by the 1960s and a wall of unresearched rationals have been created to support an ‘urgent need’ for these cosmetic surgeries.

After over fifty years of such surgeries being performed on children without their personal consent, the lifelong consequences have come to light.  These include mis-assigned genders, nerve damage causing lifelong pain & dysfunction, and childhood scarring that does not grow with the rest of the child’s body necessitating frequent ‘revision surgeries’.  These revision surgeries further damage & reduce a child’s original, natural tissue and emotional trauma from such surgeries are caused when the need is not truthfully explained to the child.

See also Gender Committee, Revision Surgery.

A psychologist in the 1950s who theorized that gender was completely dependent on “nurture.” He advocated for genital surgery on intersex infants and raising them as girls.

His theories have not born-out over the subsequent decades and have resulted in many surgically misgendered children who have had to deal with life long gender dysphoria, depression, sexual dysfunction, bowel and urinary incontinence, and frequent pain from nerve damage.

A patient centered model is now preferred where an intersex child is allowed to grow up and make their own choices on gender and surgical options.

https://en.wikipedia.org/wiki/John_Money

See also Gender Assignment, Gender Identity, Informed Consent, Concealment-Centered Model, Patient-Centered Model

Karyotype is a test that gives a picture of all of the chromosomes present in a single one of your cells. When checking for intersex traits, your doctor may look specifically at the sex chromosomes of a karyotype which are typically XX or XY, but can have many variations, such as XXY, XXXY or others. This test doesn’t provide an exact diagnosis, but it can provide clues to where to look for more information.

See also Sex Chromosome Variations

 Klinefelter syndrome, also known as 47 ‘XXY’, is the set of symptoms that result from two or more ‘X’ chromosomes in males.  The primary features are infertility and small, poorly functioning testicles.  Often, symptoms may be subtle and many people do not realize they are affected.

https://rarediseases.org/rare-diseases/klinefelter-syndrome/

See also Sex Chromosome Variations

Leydig cell hypoplasia is an autosomal recessive condition in which the LHCGR gene does not function typically which leads to a difference in sex development.

Two types of LCH have been defined (Toledo, 1992).  Those with type I, have complete inactivation of LHCGR, and may have a typical female phenotype with 46,XY chromosomes, low testosterone and high LH levels. They can achieve secondary sex characteristics with hormone replacement.

Those with type II, caused by partial inactivation of the gene, can have a phenotype (appearance) anywhere along the spectrum of development. 46, XX Females with inactivating mutations in the LHCGR gene may have difficulty with follicular development and ovulation.

https://rarediseases.info.nih.gov/diseases/3244/leydig-cell-hypoplasia

See also Androgens, Differences Of Sex Development

Micropenis is a medical term for a penis, usually diagnosed at birth, that is well under the normal size range for an infant.  In every other way, including structure, appearance, and function, a micropenis is like any other healthy penis.  As the penis is fully functional, childhood corrective surgery is not recommended as such surgical scarring often does not grow with the child as normal tissue does. Such childhood surgeries could result in more serious complications as the child grows up.  It is recommended that, barring any other life threatening physical issues, “corrective” cosmetic surgery be held off until adulthood when the affected person can participate in informed consent.

https://en.wikipedia.org/wiki/Micropenis

See Androgen Insensitivity Syndrome.

This is when a chromosomal variation causes a child to be born with two different gonads: An undescended testis and a dysgenic (malformed) ‘streak gonad’. It is one of the most common disorders of sexual dysfunction and the second most common cause of ambiguous genitalia.

See also Ambiguous Genitalia, Mosaicism Involving “Sex” Chromosomes, Streak Gonads

Most people have 46 chromosomes in each of their cells, and two of those 46 chromosomes are commonly referred to as “sex chromosomes”.  Most girls and women have two ‘X’ sex chromosomes, most boys and men have an ‘X’ sex chromosome and a ‘Y’ sex chromosome.

People with “mosaic” chromosomes have differing sets of chromosomes in groups of cells, and sometimes organs.  As one has some cells having 46 ‘XX’ genes and other cells having 46 ‘XY’ genes, it’s said the body is made up of a varied set of colored tiles, making a mosaic.

See also Chimeras

MRKH is a congenital disorder of the female reproductive system. Girls with MRKH have normal ovaries and fallopian tubes, an absent or incomplete vagina, no cervix and either an underdeveloped uterus (uterine remnant) or no uterus at all.

https://ghr.nlm.nih.gov/condition/mayer-rokitansky-kuster-hauser-syndrome#description

See MRKH: Mayer-Rokitansky-Küster-Hauser syndrome

This is a rare medical term to describe a woman who hasn’t given birth to a child. It has been used in medical files as a placeholder for women lacking reproductive capability.

An ovo-testis is a gonad with both testicular and ovarian aspects. In humans, ovo-testes are an infrequent anatomical variation associated with gonadal dysgenesis. For invertebrates that are normally hermaphroditic, such as most gastropods (snails and slugs), an ovo-testis is a common feature of the reproductive anatomy.

https://en.wikipedia.org/wiki/Ovotestis

See also Gonadal Dysgenesis, Mixed Gonadal Dysgenesis, Streak Gonads