Intersexuality, a.k.a. Disorders of Sexual Development (DSD)
qualifies as intersex? How common is it?
be used to "correct" the
physical appearance of intersex newborns?
What sexual differences qualify as DSD / intersex?
In the past, there was little consensus on what unusual conditions should be classified as intersex. The Intersex Society of North America (ISNA) 10 offered a definition:
" 'Intersex' is a general term used for a variety of conditions in which a person is born with a reproductive or sexual anatomy that doesn’t seem to fit the typical definitions of female or male. For example:
- A person might be born appearing to be female on the outside, but having mostly male-typical anatomy on the inside.
- A person may be born with genitals that seem to be in-between the usual male and female types—for example, a girl may be born with a noticeably large clitoris, or lacking a vaginal opening, or a boy may be born with a notably small penis, or with a scrotum that is divided so that it has formed more like labia.
- A person may be born with mosaic genetics, so that some of her cells have XX chromosomes and some of them have XY.
Though we speak of intersex as an inborn condition, intersex anatomy doesn’t always show up at birth. Sometimes a person isn’t found to have intersex anatomy until she or he reaches the age of puberty, or finds himself an infertile adult, or dies of old age and is autopsied. Some people live and die with intersex anatomy without anyone (including themselves) ever knowing.
Which variations of sexual anatomy count as intersex? In practice, different people have different answers to that question. That’s not surprising, because intersex isn’t a discreet or natural category.
What does this mean? Intersex is a socially constructed category that reflects real biological variation." 1
The American Psychological Association currently defines "intersex" as:
"A variety of conditions that lead to atypical development of physical sex characteristics are collectively referred to as intersex conditions. These conditions can involve abnormalities of the external genitals, internal reproductive organs, sex chromosomes, or sex-related hormones. Some examples include:
- External genitals that cannot be easily classified as male or female
- Incomplete or unusual development of the internal reproductive organs
- Inconsistency between the external genitals and the internal reproductive organs
- Abnormalities of the sex chromosomes
- Abnormal development of the testes or ovaries
- Over or underproduction of sex-related hormones
- Inability of the body to respond normally to sex-related hormones." 2
How common are various forms of intersex/DSD?
In 2008, ISNA provided very rough estimates about both:
- The frequency of atypical sex chromosome structures found in a newborn's DNA and
- The frequency of irregularities in the appearance of sexual organs at birth. 3
Some of the sexual variations reported by ISNA were:
- Newborns with bodies that differ from the typical male or female: 1 in 100 births.
- Persons who receive surgery to normalize the appearance of their genitals: 1 in 750 births.
- Persons born with sex chromosomes that are neither the typical XX (female) or XY (male): 1 in 1,666 births. 3
- Klinefelter syndrome: Males born with XXY (instead of XY) sex chromosomes: 1 in 500 males. Can result in increased height, infertility, autoimmune disorders, etc. 4
- Turner syndrome: Females born with a missing or incomplete X chromosome in some or all of their body's cells: 1 in 2,000 to 2,500 births. Generally causes decreased height, infertility, and delayed puberty. It can also involve extra skin on the neck, widely spaced nipples, swelling of the hands and feet, skeletal abnormalities, heart defects, frequent ear infections, and kidney problems. 5 It was once believed that this syndrome was often associated with mental developmental delays. However, this is now recognized as false. 6
- Mixed gonadal dysgenesis (MGD): Born with an undescended testicle on one side of their body and either an improperly developed testicle or ovary on the other side. 1 person or fewer among 2,000 births. Generally, the decision on whether to raise the newborn as a male of female is based on anatomic findings. 7
- Swyer syndrome, a.k.a. complete gonadal dysgenesis (CGD): Born a genetic male with XY sex chromosomes. 1 in 30,000 births. Exhibits female appearance. Has a normal vagina, uterus, fallopian tubes and external genitalia. Typically has non-functioning ovaries called streak gonads. CGD is often not suspected or detected until the normal symptoms of puberty fail to develop in early teen years.
The frequency estimates are only approximate.
In addition, there are two syndromes that only affect genetic males and that affect their sexual development before birth and during puberty:
- Complete Androgen insensitivity syndrome: 1 in 30,000 genetic males. They are genetically male, with XY chromosomes, but their body is unable to respond to androgens (male sex hormones). As a result, they have mostly female sex characteristics, have a female gender identity, and are normally raised as females. They are infertile and have undescended testicles which are normally removed surgically to prevent cancer growth. 8
- Reifenstein syndrome (a.k.a. partial androgen insensitivity syndrome): 1 in 30,000 genetic males. They are genetically male, with XY chromosomes, but their body only partially responds to androgens (male sex hormones). They may have either male or female sex characteristics, may be raised as either male or female, and may develop a male or female gender identity. 8
Should intersex infants with ambiguous genitalia be surgically corrected?
In rare cases, fast surgical correction is medically necessary to prevent serious health complications. In some cases, where testes are undescended or where ovaries are malformed, surgery is strongly advised to prevent cancer from developing later in life. But surgery is often optional in other cases, where the newborn's genitalia simply look non-standard.
"Admin" writing on the Organization Intersex International in the United States of America [OII-USA] web site says:
"One of the reasons these surgeries persist is similar to the reason circumcision does: people have popular notions of what genitals should “look” like, and want their children to have this to fit in. However, the bigger reason is that some people still assume that, because our biological sex is not standardly male or female, our social gender won’t be either. It is this fear of an androgynous, non-binary social gender role that drives recommendations for surgery, for some believe it will lead to children and adults who 'stick out,' or suffer psychological difficulties.
I have found, in talking to dozens of intersex adults, that these fears are unfounded and incorrect..." 9
However, Larry Baskin, chief of pediatric urology at the University of California, San Francisco wrote:
There haven’t been any studies that would support doing nothing. That would be an experiment: don’t do anything and see what happens when the kid’s a teenager. That could be good, and that could also be worse than trying some intervention. ... If you have a child born with a cleft lip or cleft palate or an extra digit or a webbed neck, I don’t know any family that wouldn’t want that repaired. Who would say, ‘You know what, let’s wait until Johnny is 20 years old and let him decide?’”
Eric Vilain, professor of human genetics, pediatrics and urology at University of California, Los Angeles has a clinic treating disorders of sex development. He said that he couldn't believe that surgeons were performing genital reconstructions with so little data about long-term results. He said:
"To me it was shocking, because where I come from, molecular genetics, we’re under extreme scrutiny. If you want to show that a molecule causes something, you have to show it with a bunch of excruciatingly painful controls. And here I was looking at a lot of surgeons who were saying, ‘We think it’s good to do genital surgery early on because the children are doing better.’ So each time I would ask, ‘What’s the evidence that they’re doing better?’ And in fact the answer is there’s no real evidence. Then I’d ask: ‘What does it mean doing better? How do you measure it? Are you talking quality of life, or quality of sex life?’ And there was never any convincing answer."
Sarah Creighton, a gynecologists in the UK has studied the outcome of intersex patients -- both those who have had surgical procedures performed early in life and those who have not. She found that intersex patients who have not had surgical procedures tend to do better, She noted that no option is guaranteed or even likely to make the lives of those babies born intersex pain-free.
These are not all happy people, either. Some of them have isolated, difficult lives. Some of the surgery patients are fine, and some of them are not, and it’s very hard to separate all the things out."
If it is difficult to "separate all the things out" when the intersex person is an adult, it must be much more difficult for parents of a newborn.
A 30 minute video "Hermaphrodites Speak" is still available at the address of the former Intersex Society of North America. Eight individuals at the first retreat organized by ISNA discuss what it is like to grow up intersexed, and the experience of finally meeting other intersexed people for the first time. You can purchase it via the Intersex Society of North America address. See: http://www.isna.org It was posted on YouTube on 2012-NOV-15, where it is probably in violation of copyright. See: https://www.youtube.com/
The following information sources were used to prepare and update the above
essay. The hyperlinks are not necessarily still active today.
- "What is intersex?," Intersex Society of North America, 2008, at:http://www.isna.org/
- "Answers to your questions about individuals with intersex conditions," American Psychological Association, 2913, at: http://www.apa.org/
- "How common is intersex?," Intersex Society of North America, 2008, at: http://www.isna.org/
- "Klinefelter Syndrome," MedicineNet, http://www.medicinenet.com/
- "Turner Syndrome," Genetic Science Learning Center, 2013, at: http://learn.genetics.utah.edu/
- "Beth," "Growing up with Turners Syndrome," 2009-FEB, at: http://home.windstream.net/
- "Mixed gonadal dysgenesis," Boston Children's Hospital, 2011, at: http://www.childrenshospital.org/
- "Androgen insensitivity syndrome," Genetics Home Reference, National Library of Medicine, 2008, at: http://ghr.nlm.nih.gov/
- "Admin," "Intersex and Medically Unnecessary, Irreversible Treatments," OII-USA, 2012-JAN-17, at: http://oii-usa.org/
- The Intersex Society of North America ended operations in 2008. A new non-profit organization, Accord Alliance, composed of health care and advocacy professionals, opened its doors in 2008-MAR. It will "continue to lead national efforts to improve health care and outcomes for intersexual persons.
Copyright © 2013 by Ontario Consultants on
Originally posted: 2013-AUG-22
Last updated on 2015-JUN-14
Author: Bruce A Robinson