…and this is a 
A problem with normal
Category:
Posted on: August 17, 2008 12:12 PM, by PZMinion
MAJeff here.
LisaJ's Danio's (hangover error) posts about Usher Disease (I and II), as well as my own syllabus preparation for the upcoming semester, have gotten me thinking about issues of intersexuality. In particular, her noting of the geographic issues related to the prevalence of various forms of Usher disease reminded me of the concentration of five-alpha-reductase deficiency in parts of Turkey, Papua New Guinea and the Dominican Republic.
Some folks are probably asking, "What is this intersexuality thing?" Basically, it's a range sexual development disorders in which people's bodies develop in such a way as to place them in a "border region" of sex. Hermaphrodism is what people usually think of, but there is a wider range of conditions, including hypospadias and congenital adrenal hyperplasia.
If any of you have read the novel Middesex you already have an idea of what I'm talking about with five-alpha-reductase deficiency. People with this condition are genetically XY, but during fetal development something happens such that in many people the testicles may not descend, the scrotal sac may not fuse, and the penis can appear more like a clitoris (such an ambiguous thing is often called a microphallus). Because of these developmental issues, people with this condition are given a female gender designation at birth. Once puberty hits, though, the testicles descend, the penis may enlarge, the "labia" fuse to form a scrotum, and other male secondary sex characteristics appear. One of the things I find so interesting about this particular condition is the way that it has been routinized in the patterns of life and cultural systems in parts of the Dominican Republic. The people living in these areas have their own term for the condition, "guevedoce" ("eggs/balls at 12").
In class, I often use a video produced by the Intersex Society of North America, an organization that shut its doors this years in favor of a different advocacy organization, the Accord Alliance. In particular, this segment of that video talks about, and interviews, someone who identifies as a guevedoce, as well as his family. (YouTube won't allow it to be embedded.)
It's this issue of how people with various conditions are integrated into social life that is my primary concerns. One of the things intersex activists have been challenging for the past decade or so is infant genital surgery. When children with some sexual development disorders are born with ambiguous genitals they are quite literally made to fit into one of the existing gender categories. "Fixing" them means surgery to make their genitalia more closely resemble "normal" genitals. If the phallus falls inside the middle range, where it's "too long" for a clitoris or "too short" to be a penis, well, it's snip-snip time. Many of the decisions to engage in surgery are based not on medical necessity, but social preference. Questions such as, "Will he be able to stand to urinate?" or "Will her partners be turned off by such a large clitoris?" or "How will the parents deal with looking at such a strange body while changing diapers?" can become more important issues when determining whether to operate than such things as "Will cutting part of the phallus off affect this child's sexuality later in life?" (Ann Fausto-Sterling has an excellent discussion of these issues.)
Not surprisingly, surgeries do affect folks. Many report a loss of sensitivity from having such operations performed on them. (As one of my students once said to the other women in the class about the possibility of having half a clitoris and no sensitivity, "Wouldn't it just make you tense all the time!") It's more than loss of sensitivity, though. There are often other complications that require more than one surgery. Ongoing pain or recurrent infections are not uncommon.
This is one of those spaces where I get all anti-normalization. These people's bodies are being normalized--they are being reconstructed so they fit within normative assumptions about what genitalia must look like based on statistical averages. And, it's done without their consent. Intersex activists have been successful in increasing awareness in the medical profession, but there are still issues. Many of these flow from the gender order we have in this society. The problem with such medically unnecessary genital surgeries isn't these babies' bodies, but social beliefs about what those bodies are supposed to look like.
Comments
Posted by: Thanny | August 17, 2008 12:34 PM
I'm definitely on the side of preventing parents from mutilating their children's bodies for cosmetic reasons (and that includes circumcision).
Posted by: Paul Burnett | August 17, 2008 12:46 PM
"The problem with such medically unnecessary genital surgeries isn't these babies' bodies, but social beliefs about what those bodies are supposed to look like."
Why is male circumcision okay in parts of the Western world, while at the same time many in the Western world criticize other parts of the world that practice female circumcision?
And it's not just genital mutilation that's "normal" - excess fingers or toes are snipped off, as are external vestigial tails.
Posted by: SC | August 17, 2008 12:46 PM
I'm disappointed in you, MAJeff. You really need to start associating with a better class of people. ;)
Posted by: LisaJ | August 17, 2008 12:47 PM
Great post Jeff. This is a very interesting subject. I can understand how parents of a child who is born with such a condition must feel alot of pressure to make their child appear 'normal', and how difficult this must be to deal with when the child is first born. However, I absolutely agree that parents should not allow their children to be mutilated in this way. I just don't understand how someone could think that deciding for your child if they are going to be a boy or a girl is in any way a good idea. Just let the child be and let them grow up into the person they really are.
Posted by: Nemo | August 17, 2008 1:22 PM
Standing to urinate is a highly overrated ability.
Posted by: Mystyk | August 17, 2008 1:35 PM
This sounds a lot like Androgen Insensitivity Syndrome, in that the child is genetically male but appears somewhere in a range from 75% male up to 100% female in appearance. The biggest difference I see is that AIS seems to have a much higher rate of female gender identity (well over 90%), and 5-alpha seems to have more issues with changes due to puberty.
Posted by: Michael Drake | August 17, 2008 1:36 PM
Interesting that being normalized also means being denatured. This fact should have caused heads to explode.
Posted by: Tabby Lavalamp | August 17, 2008 1:48 PM
Paul Burnett wrote:
While both are wrong and originally based in religion, this is still comparing apples and oranges. If male circumcision involved removing the glans, then we can talk. As it is, removing the foreskin for the most part doesn't even come close to the mutilation involved in female "circumcision" (though in some areas just the clitoral hood is removed, and that's the only time it's a fair comparison). But when partial or complete removal of the clitoris is involved, the comparison starts to fail, and the multilation only gets more horrific from there, depending on where it's practised.
Posted by: wright | August 17, 2008 1:49 PM
If I were a parent of such a child, my question would be: what options offer my child the best chance of physical and emotional health? I would want to read the relevant literature, talk with intersexed people and their families, as well as physicians in the relevant specialties.
These are the kind of inquiries that can only be done in an open, humane society. Parents of intersexed children and intersexed adults need answers, or at least enough information so they can ask the right questions.
Thanks, MAJeff, for being part of that informed dialogue.
Posted by: Breakfast | August 17, 2008 1:53 PM
Well, to be totally fair: it's not done in the name of something as faceless as 'statistical averages'. Statistical averages are certainly the cause of our belief in a bifurcation of genders in the first place -- but those beliefs, and the norms that go with them, obviously have a rich and well-established cultural life of their own. It's not so easy to just thumb your nose at all that. Not easy in terms of situating oneself as a person, certainly not easy in terms of being accepted or even comprehended by others. Which I'm sure you're well aware of, anyway.
Posted by: Muffin | August 17, 2008 2:03 PM
As someone with two intersexed friends, I can only agree, wholeheartedly. Both my friends went under the knife at birth for no other reason than that they didn't appear "normal" enough, and both are deeply unhappy with their assigned gender nowadays. One is in the process of transitioning (f2m); the other is unable to do so due to unrelated medical conditions, and the fact that she'll never be what she really is, so to speak, has left her heartbroken.
It's an utter shame what has been and still is being done to people, all without their knowledge, their consent, or even so much as a reason.
Posted by: maxi | August 17, 2008 2:09 PM
Great post. Exremely thought-provoking!
Posted by: Akheloios | August 17, 2008 2:10 PM
What if the glans of the penis was originally an example of a clitoris that was appeared in men and was obviously selected for, or vice versa.
Normalization means that were actively muddying the waters of selection by making interesting genital configurations look exactly like the boring normal kind.
Who knows what kind of incredibly pleasurable genitalia we could adapt in a few hundred generations if we stopped this blind adherence to a narrow ideal of body image.
I for one would welcome a new race of humans with our tails back and new doubly pleasurable sex toys. We just have to breed with the right people, and if they are mutilated before we can choose, then we're stuck with the boring average human forever :'(
Posted by: Neural T | August 17, 2008 2:16 PM
Good timing. A documentary about David Reimer aired just a few weeks ago. It's a fascinating tale. Really drives home the message that, contrary to feminist critiques of the 1970s, the mind is not a blank slate with regard to gender identity.
I've been fascinated by the intersex phenomenon for a long time. It is (or should be) an unfortunate problem for people who assert that the genders were created by God. No, sex and gender are adaptive reproductive strategies that can fail in development just like everything else. They are not immutable. They don't even have clean boundaries.
Posted by: Breakfast | August 17, 2008 2:20 PM
It's...uh...it's because of...sin!
Probably the mothers had naughty thoughts while the kids were in utero or something. Or, no -- the fetus did!
Posted by: Daniel | August 17, 2008 2:44 PM
This blog, written by a rocket scientist, is a fascinating collection of information, both personal and scientific, regarding intersex, transsexualism and related psychosocial and psychosexual issues.
The author began to spontanteously transform from M to F in 2005 even though she is XY genotype.
It is erudite and heartfelt. Just read the posts about the passport issue. You won't know whether to laugh, weep or crawl into a ball and rock gently in a corner - an amazing person.
Posted by: Fernando Magyar | August 17, 2008 2:56 PM
MaJeff,
Let me begin by putting my cards on the table face up. I'm a 55 year old heterosexual male in a great relationship with a wonderful hetero female. I grew up in Brazil where all forms of sexuality are in general not as big a deal as they are here in the USA. However as the father of a kid with Aspergergs I truly hate the term "normalization" wherever and however it may apply. BTW my grandfather was a doctor and he convinced my parents to have me circumcised at the ripe old age of six, I still have vivid memories of how painful it was to urinate through the bandages...
You say:
My question to you is how can we even begin to convince people that this is not like fixing a cleft palate, let alone that we might literally be crippling these people and therefore keeping them from experiencing the full pleasure of their sexual potential?! How the hell do you get it through the heads of those who pretend to be in a position to dictate morality to the rest of us that they are unequivocally and fractally wrong?
Posted by: Abbie | August 17, 2008 2:58 PM
It's really nice to see this issue brought up on Pharyngula.
Posted by: Kelly | August 17, 2008 2:59 PM
Dear David,
Your post oversimplifies a very complex issue. First of all, the term "intersex" is no longer used. Now, the preferred term is "disorders of sex development" or "DSD". "Intersex" is seen as ambiguous and stigmatizing and has been rejected by the affected communities and the medical community at large. http://adc.bmj.com/cgi/content/abstract/adc.2006.098319v1
Second, each disorder must be evaluated individually. The surgical issue affects each DSD differently. For instance, with congenital adrenal hyperplasia, the vast majority of women so affected have no gender dysphoria, and live normal female lives. They do not consider themselves to be "intersex" and do not relate to the discussions about genital surgery. They have a uterus, vagina and ovaries, but no vaginal opening and differing degrees of clitoral enlargement. Most adult women with CAH are happy that their parents chose surgical reconstruction and would not wish a similarly affected child to live with significantly gender atypical genitals, while expressing a desire to be very conservative in the surgical procedures. Clitoral reduction is discouraged and must be conservative when done, but not necessarily the creation of a vaginal opening. So, the issue is less whether it should be done, but the extent of the surgery and whether it preserves clitoral sensation. Self-esteem and overall mental health can be negatively impacted by significant genital differences in CAH and inability to engage in sexual intercourse.
In CAH, there are medical reasons for creating a vaginal opening, as proper childhood steroid treatment will allow the child to menstruate, and she needs a vaginal opening for the proper flow of blood. Moreover, reflux of urine into the vagina may cause infections, which can be life-threatening to those with CAH.
In addition, these women are fertile as females and most are heterosexual.
So, a more in-depth discussion of the topic may be warranted.
http://www.caresfoundation.org/productcart/pc/surgery_considerations_cah.html
Posted by: Greta Christina | August 17, 2008 4:00 PM
Excellent piece, on an under-discussed topic. Thanks!
Posted by: Helioprogenus | August 17, 2008 4:11 PM
Have they done studies where they follow a group of intersex individuals from birth who didn't have reassignment surgery and then psychologically assess them to determine their satisfaction in life? If you can also do a comparative study on those with gender reassignment, we can have a better understanding on the actual differences with the quality of life in both groups. In the defense of doctors and parents who feel it necessary for reassignment, they're doing it in hopes that the quality of life of the individual will turn out to be significantly better. With a comparative study, which I'm sure has been done (but I'm not aware of), these facts could be readily relayed to the parents. Otherwise, it comes down to fix them, or fix society.
Posted by: Jparenti | August 17, 2008 4:20 PM
I've always been fascinated with gender-identity issues and the way they're approached in our society (usually with blunt-force ideals and little in the way of reason). Great post!
As far as circumcision goes, I still haven't figured out how anyone equates the relatively benign procedure performed on males with the mutilation of female genitalia that is practiced by certain tribes in Africa and elsewhere. It really isn't the same thing at all. The disgusting practice of destroying a woman's body should be realized and talked about by our society, so that it can be ended once and for all. Male circumcision, I don't know. It can be botched, I know, but I prefer it, simply because of hygenic reasons. I'm quite happy with my lack of foreskin, thank you! But maybe it should be left until the man decides whether he wants it or not, around the teen years, rather than being forced at birth.
Posted by: David Marjanović, OM | August 17, 2008 4:28 PM
I disagree.
Posted by: David Marjanović, OM | August 17, 2008 4:31 PM
What hygienic reasons?
What about the protection against infection that the foreskin provides?
And thirdly, why are only Muslims, Jews and Americans circumcised in the western world?
Posted by: Pablo | August 17, 2008 4:34 PM
Logical error on line 1!
Given that I have never had a foreskin (since I can remember), I have a hard time comparing my current hygenic situation to what it would be like with one. Actually, check that - it's not hard, it's impossible. Same for you. You may be happy without, but how do you know you wouldn't be happier with?
Moreover, even if it is cleaner, it's good that we don't use the same approach for other problems. To prevent cavities, we brush our teeth. We don't pull them. We don't amputate toes to prevent pernichia, we teach proper foot care.
But when it comes to the peepee? Chop it off!!!!!
Posted by: mayhempix | August 17, 2008 4:41 PM
Middlesex was one of the best books I had read in many years.
Whereas the ideas of normalization appear to have grown out of a need to "protect" these individuals from society, it is clear that it is society that wanted protection and instead needs to be normalized to the acceptance of the various degrees of sexuality.
Posted by: Gregory Kusnick | August 17, 2008 4:45 PM
That cuts both ways. You can't ask infants if they want surgery, but neither can you ask them whether they're willing to be poster children for broad-spectrum sexual tolerance. There are negative consequences to either choice, and whichever way the parents decide, it's necessarily done without the child's consent. There's no way around that.Posted by: khan | August 17, 2008 4:48 PM
I'm not sure where I fit on the spectrum.
I am physically female and heterosexual, never wanted children.
Apparently I "think like a man", and always assumed to be male on the internet.
I have never wanted to "be a man", have just wanted to be taken seriously even though I lack a penis.
Since menopause my dream have been various combinations of hetero, homo, and bi.
Maybe we (as a species) should stop trying to cram all our diversity into teeny little stereotypes.
There is genotype, phenotype, sexual orientation, secondary characteristics, social behavior, and dislike of housework.
Posted by: Jackal | August 17, 2008 4:53 PM
What the hell is it about most cultures that gender roles have to be so well defined and limited? I look extremely feminine, but I feel fairly androgynous. At 19, my mother's involvement with LGBT rights groups caused me to reconsider my sexual orientation. I had been defacto straight, but on a scale of 1 - 10 with 1 being hetero, I'm probably a 4. I HATE when people tell me or anyone else how to live their personal lives when it's not hurting anyone, and I especially hate people being forced into a specific gender identity. Ladies and gentlemen, this is what we call sexism, and it is a detriment to our society. I could go on, but I feel that it's all been said before, especially in this group.
Posted by: Matt Heath | August 17, 2008 5:15 PM
If the PZminions had a permanent group blog it would be the best thing on the interwebs. Another great post.
Posted by: wazza | August 17, 2008 5:16 PM
personally, if I was going out with a girl and we got to the point where we were just about getting each others' pants off and she said "We need to talk", I'd freak out a little (mostly over "does she not like me enough?" but also just about every possibility in the space of about five seconds)
bringing these conditions into the open and normalising them would go a long way towards removing the possibility of that particularly head-sploding situation by making it possible to talk about it at the just-friends stage
and that's my horny young male point of view.
Posted by: Aphrodine | August 17, 2008 5:21 PM
That was a fascinating read. Thanks for posting it.
Posted by: khan | August 17, 2008 5:21 PM
What do horny young males think about sex with a woman 20 or more years older?
Posted by: Danio | August 17, 2008 5:40 PM
Bravo, MAJeff! Awesome post on a very interesting topic. Given that these individuals are genetically male (XY) and most develop male secondary sex characteristics in puberty as a result of surging testosterone, it makes absolutely no sense to me to feminize them on the basis of superficial criteria (penis? clitoris? Where's the imaginary line between the two?). It's disappointing, but not surprising, that SCIENCE takes a back seat to popular opinion once again in the determination of how to treat these cases. The doctors serving these populations should be advocating more strenuously for the long term well-being of their infant patients, IMO.
Posted by: Peter Ashby | August 17, 2008 5:45 PM
I agree with you MaJeff. The problem is that people see the massively skewed distribution of sexuality and anatomy in humans as a simple binary because they encounter 'difference' so little. So many of us are simply 'normally' heterosexual that anything different is merely characterised as 'aberrant' that people don't think about fitting it into a distribution.
You see this in the politics of Gays where in some cases there is hostility to those who wish to see themselves as Bisexual. The wish to see a dichotomy (Gays and Straights) is again so strong that those who bridge between either get ignored or pressured into 'choosing' sides*. Our psychology has a lot to answer for.
*yes, I know much of it is driven by competition for scarce public resources, the criteria for which is very ignorant of things like Bisexuals and so there is no box they can fit in.
Posted by: Nick Gotts | August 17, 2008 5:48 PM
On male circumcision, I'd say "don't cut bits off people without a very good reason", but I understand there is increasingly strong evidence that male circumcision reduces the chances of contracting HIV, because the virus is particularly good at getting into the body via the cells of the prepuce. Anyone with medical knowledge have comments on this?
Posted by: SEF | August 17, 2008 5:57 PM
Intersex people (and other babies!) should definitely be left unmutilated to make any such decisions over what they really are and should be for themselves, later. What I've mostly found is that people don't even know what intersexuality is and that it happens at all. The mutilations and cover-ups just add to that public ignorance - as well as damaging the individuals concerned.
Posted by: SEF | August 17, 2008 6:03 PM
@ Nick #36:
Circumcision is nowhere near as effective (at reducing the chances of contracting HIV) as wearing a condom is! Plus the condom also protects a female from an infected male. Circumcision can only really provide a false and very one-sided sense of security. Just the sort of thing religious folks apparently love ...
Posted by: Nick Gotts | August 17, 2008 6:11 PM
SEF@38,
I see your point, but on the other hand, once you're circumcised you don't have to have the operation again every time you have sex, while you do have to put on a condom every time - and there are often circumstances where a woman lacks the power to insist on the man wearing one. From an epidemiological point of view, it's crucial to reduce the mean number of infections passed on per infected person to below 1. There is the question of "risk compensation" though (e.g. wearing a motorcycle helmet apparently increases the risks taken by the rider, though not enough, in that case, to cancel the advantage of wearing one), so the net outcome isn't clear.
Posted by: JoJo | August 17, 2008 6:20 PM
Nick Gotts #39
Back when I was young, dumb, and horny* I always wore a condom, even though I was circumcised as an infant. While HIV was not a concern in the late 60s and early 70s, other STDs and pregnancy were.
*Not like now, when I'm creeping into geezerhood, dumb, and nothing like as horny.
Posted by: Curtis E. Hinkle | August 17, 2008 6:30 PM
Ambiguous Medicine and Sexist Genetics: A Critique of the DSD Nomenclature
By M. Italiano, M.B.B.S. (A.M.) and Curtis E. Hinkle
© Aug. 8, 2008
Online at: http://www.intersexualite.org/sexist_genetics.html
Many intersex persons around the world and their allies are concerned about the new nomenclature, DSD or "Disorders of Sex Development", which has been endorsed by the Chicago Consensus (1) to replace the term "intersex". We believe that the categories proposed are not only demeaning, but also scientifically flawed.
The age of chromosomes
The DSD nomenclature uses chromosomes, instead of gonads, as the most important classifier of an individual's sex, such as "46,XY DSD" and "46,XX DSD". This is no more helpful than using male pseudohermaphroditism or female pseudohermaphroditism which was based on gonads. (2) Instead of male pseudohermaphroditism and female pseudohermaphroditism, the new DSD nomenclature proposes "46, XY DSD" and "46, XX DSD" as replacements for the former taxonomy.
Furthermore, what was called true hermaphroditism is now dichotomized to fit more neatly within the binary. True hermaphroditism used to be called "true" because it meant that an individual had both ovarian and testicular tissue and gonads (ovaries and testicles) were considered to be the "true" determiner of one's sex. Of course the word "true" was problematic because it suggested that all other forms of "hermaphroditism" were not legitimate, only "pseudo conditions". Also, using the term "hermaphrodite" as a word to describe a person with an intersex variation has often been criticized as insulting and inaccurate. However, by replacing true hermaphroditism with "ovotesticular DSD", we still have another problem. The DSD nomenclature now wishes to divide "ovotesticular DSD" (formerly true hermaphroditism) into "46, XY ovotesticular DSD", "46, XX ovotesticular DSD", or "chromosomal DSD" (of "46,XX/46,XY" chimerism or "45, X/46,XY" mosaic types). In effect, it gives an individual in the latter case two types of DSD, an "ovotesticular DSD", and a "chromosomal DSD". Also, we see the division based on chromosomes, which again exposes the preeminence of chromosomes as the "true" markers of an individual's sex. Further, by combining "ovostesticular DSD" with a chimeric or mosaic karyotype, as it does, it also fails to provide a clear classification of so-called "ovotesticular DSD" which has 3 or more cell line types, isochromosomes, inversions, or ring chromosomes in the karyotype.
For individuals who have both 46,XX in some cells and 46,XY in other cells, and who are referred to as having a "chromosomal DSD" of "46,XX/46,XY(chimerism)" type, it is not uncommon for them to have male anatomy only (3) or female anatomy only (4) and they may also be fertile. In this new nomenclature they would be "diagnosed" as having a "chromosomal DSD" despite any practical relevance for them. Furthermore, although the DSD nomenclature is intended to be representative of congenital conditions, there are individuals who have become 46,XX/46,XY because their twin's cells make up part of their own karyotype (5), or because an individual who is 46,XX received a bone marrow donation from someone who is 46,XY, as well as by many other means (6). In fact, a pregnancy may also lead to "false positives" for a DSD since fetal cells end up in a woman's bloodstream. (5)
Likewise, individuals with a 45,X/46,XY karyotype are listed as having a "chromosomal DSD", but with a parenthetical "mixed gonadal dysgenesis" or "ovotesticular" DSD. This is also confusing since many 45,X/46,XY individuals do NOT have mixed gonadal dysgenesis or ovotesticular tissue. Again, some have only typical male or female anatomy (some being fertile as such), and the XO cells are known to disappear during various stages of development. (7) Thus, predicting this type of "chromosomal DSD" in prenatal screening has been demonstrated to be hampered by a high rate of erroneous results, has provided unnecessary cause for alarm (by projecting birth defects which do not exist), has led to unwanted elective abortion, and is considered a serious problem in clinical genetics. (8)
Another problem is that the DSD proponents have misunderstood basic genetics (or intentionally distorted the information) and have assumed that XY chromosomes indicate that testicular tissue is expected. This assumption leads to another error in the new taxonomy because when gonadal dysgenesis is classified as a "46,XY DSD", (see Table 2 in reference 1 http://aappolicy.aappublications.org/cgi/content/full/pediatrics;118/2/e488/T2 ) DSD proponents refer to it (parenthetically) as "testicular dysgenesis". This is misleading and ambiguous because many individuals with 46,XY gonadal dysgenesis actually have OVARIAN dysgenesis. (9) It has been known for over 30 years now that in the presence of an unaltered Y chromosome, but in the absence of substances which would cause testicular differentiation and development, that ovaries start to form, not testicles. (reviewed in ref. 9). It is therefore deceptive to classify 46,XY gonadal dysgenesis as 46,XY testicular dysgenesis because testicular dysgenesis is the result on some occasions but at other times the result is ovarian dysgenesis. The type of treatment indications for dysgenetic testicular tissue may differ from that of dysgenetic ovarian tissue, and thus may unnecessarily confuse clinicians. Furthermore, the preeminence of chromosomes in this taxonomy is apparent and the idea that XY chromosomes somehow are the real "male" sex marker is the result of sexist genetics which produces more ambiguous medicine.
A basic problem with the DSD nomenclature is that it divides all the "disorders" into groups based on what are erroneously known as "sex chromosomes". (10) This sexist interpretation of genetics, typical throughout this new nomenclature, leads to ambiguous medicine because there are individuals who have male anatomy only but have what appears to be XX chromosomes and are diagnosed as having a "46,XX DSD". Likewise, there are individuals who have female anatomy with what appears to be XY chromosomes and are diagnosed as having a "46,XY DSD". If these apparent XY individuals have a piece of the Y chromosome missing, (such as would include the SRY testis determining gene) they are still referred to as having a "46,XY DSD", which is factually impossible since they are not XY, but X plus only part of the Y. Likewise, someone who is called XY (but in reality has an extra copy of an X chromosomal gene called DAX1) is also put in the category of having a "46,XY DSD", even though this is impossible, since they are not XY, but are instead X (PLUS another piece of an X)+Y. Likewise, individuals who appear to be XX, but are actually XX (PLUS the Y chromosome-specific SRY gene) are listed as having a "46,XX DSD" and a disorder of gonadal (ovarian) development, both of which are technically inaccurate. The fact that the DSD proponents (1) have put a note next to some conditions which indicates whether a deletion or addition of some X or Y chromosomal material exists, further demonstrates the inconsistency of their listing these conditions in the binary categories of "46,XY DSD" or "46, XX DSD" and not that of "chromosomal DSD." In these regards, the DSD terminology is in violation of the principles and accepted diagnostic nomenclature used by clinical and molecular cytogeneticists. (11) Why didn't the DSD proponents put these in the "chromosomal DSD category"? One apparently needs an entire extra "sex chromosome" or to be lacking one, in order NOT to be put in the binary "EITHER XX or XY" category.
The DSD nomenclature is ambiguous and sexist in its understanding of genetics and it appears that this is necessary in order to preserve an "artificial binary". People who have portions of the X or Y chromosome missing or added are neither XX nor XY. The DSD system again here is flawed. Technically, CAIS individuals do not have a so-called "46,XY DSD" (even though the proponents state that they do) because the androgen receptor gene on the X chromosome is altered so that, in fact, they are only "X"Y. The androgen receptor is certainly involved in sex development. Thus if it is not there or is altered, it is ambiguous and misleading to call these individuals XY. It is equally ambiguous and misleading to call CAIS individuals "genetic males". Yes, they have the SRY gene and a typical Y chromosome, but the X linked gene sequences for androgen "action" are not something that they "have". The same is true for an XY individual who has a female anatomy only, unaltered X and Y chromosomes, but an alteration on one of the many genes on one of the so-called "non sex chromosomes" (autosomes) which are certainly sex determining.
Sophia Siedlberg, Genetics Advisor to the Organisation Intersex International, came up with a polygenic model which explained the role of genes, not chromosomes, in sex determination. (12) This model has been misappropriated by others who don't know how to interpret it correctly. We can be quite sure, that barring an environmental cause (such as a teratogen), if we have an XY individual who does not appear to be a male, but instead appears female or intersex, that this person CANNOT be a "genetic male", "chromosomally a male", "genetically a male" and vice versa for individuals who have XX chromosomes. How do we know this? By the simple rule of basic genetics, that
GENES (+ environment) = PHENOTYPE (observable trait)
Thus, the DSD model based on "sex chromosomal" divisions has failed. By using the umbrella term "development", it has also misapplied the knowledge base from the field of (sex) "differentiation" and conflated it with that of "development". (13) It is ambiguous and sexist (in that it prescribes what sex one should be and not what sex one is and it perpetuates gender and sexist stereotypes based on chromosomes). It promotes confusion and oppression. It is NOT scientific. It simply uses scientific terminology in such a way that is confuses those who have little knowledge of genetics and biology. In so doing, it victimizes intersex people while offering "unlimited immunity" to medical and psychological professionals who continue FORCED sex assignments, FORCED sex reassignments, and FORCED gender expression expectations.
DSD makes the central health issue one's sex
A second big problem with the DSD Consensus is that it largely ignores the health issues of intersexed individuals. With its emphasis on "sex" divisions based on chromosomes, they have persons with non-intersexed conditions like labial adhesions, cloacal exstrophy of the bladder and absent penis in an otherwise typical male, (or absence of a vagina in an otherwise typical female), mixed in with endocrine conditions, such as congenital adrenal hyperplasia, or mixed in with other organ system conditions, such as Smith-Lemli-Opitz Syndrome, and Turner's syndrome. These are then categorized as "sex development disorders", thus taking this "distant commonality" of one symptom, i.e., sex, and placing all of these disparate conditions as a disorder of one's sex, while the predominant health issues become categorically "secondary" and likely to be ignored by clinicians.
DSD lacks clinical relevance
Even without considering the fact that the DSD Consensus largely ignores health issues, its taxonomy is in many cases irrelevant for the purposes of clinicians, especially those with subspecialties. An XX male with testes, a penis, and no female reproductive organs, who finds out at the age of 30 that his chromosomes are atypical after an infertility check, is in the same category as an otherwise typical female with ovaries and a uterus who has vaginal atresia. Both have a "46,XX DSD". The same holds true for a male, typical in every way but with isolated hypospadias (classified as having a "46,XY DSD"), whose clinician finds that they have given their prior patient, an XY female with streak ovaries, uterus, and vagina who has given birth after embryo donation the same diagnostic classification of "46,XY DSD". Again, ambiguous diagnoses lead to ambiguous treatment implications and vice versa. This is ambiguous medicine.
Gender conformity based on sexist genetics
With disorders of sex development, which sounds like "sexual development" (and can be confused with psychosexual development or psychosexual disorders), we now see a pathologizing of gender, gender identity, gender role, sexual orientation, and its ties to (re)assignment. People with a so-called DSD, especially in the binary XX or XY categories, are expected to conform in the above categories according to a binary gender expression, as indicated by the expectations of the DSD category, as well as the whim of the person who enforces the assignment or re-assignment. Those who reject such enforcement can be labeled mentally disordered, and treatment can be instituted or re-instituted at the whim of professionals, and this can be enforced legally.
DSD is about ambiguous medicine, sexist genetics, body control, and mind control. It certainly is not a client centered consensus statement. The fact that almost no intersex people had input into this consensus is glaringly evident.
In effect, we have moved from the "age of gonads" to the "age of chromosomes" even though it has been established that "sex chromosomes" as portrayed do not determine one's sex. (10) This is based on prescriptive notions about genetics, not a descriptive understanding of the role of chromosomes in sex determination. Genes, not "sex chromosomes", determine sex, and most of the genes involved are not on the X and Y chromosomes. They are on the autosomes.
It appears to the authors of this article that the DSD nomenclature misinterprets genetics based on a sexist, binary male/female model and in so doing, it has erroneously pathologized and stigmatized intersex people in order to try to preserve the heterosexist male/female hierarchies that justify the oppression of many classes of people, not just those who are intersexed.
REFERENCES
1) Hughes, I.A. et al. Consensus statement on management of intersex disorders. J. Ped. Urol., 2006, 3:148-162.
Available online at: http://aappolicy.aappublications.org/cgi/content/full/pediatrics;118/2/e488/T2
2) DamianiI, D. & Guerra-Júnior, G. As novas definições e classificações dos estados intersexuais: o que o Consenso de Chicago contribui para o estado da arte? Arq Bras Endocrinol Metabol. 2007, 51: 013-7.
Available online at: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-27302007000600018
3) Gencik, A. et al. Chimerism 46,XX/46,XY in a phenotypic female. Hum. Genet., 1980, 55: 407-408.
4) Sudik, R. et al. Chimerism in a fertile woman with a 46,XY karyotype and female phenotype: Case Report. Hum. Rep., 2001, 16: 56-58.
5) Schoenle, E. et al. 46,XX/46,XY Chimerism in a Phenotypically Normal Man. Hum. Genet., 1983, 64: 86-89.
6) Ford, C.E. Mosaics and Chimaeras. British Med. Bull, 1969, 25:104-109.
7) Chang, H.J. et al. The phenotype of 45,X/46,XY mosaicism: an analysis of 92 prenatally diagnosed cases. Amer. J. Hum. Genet., 1990, 46: 156-167.
8) Robinson, A. et al. Prognosis of prenatally diagnosed children with sex chromosome aneuploidy. Am J. Med. Genet., 1992, 44: 365-368.
9) Wachtel S.S. & Simpson J.L. Sex Reversal in the Human. In Wachtel S.S. (Ed.) Molecular Genetics of Sex Determination., 1994, 287-309. Academic Press, Inc.
10) Italiano, M The Scientific Abuse of Genetics and Sex Classifications. Manuscript published July 17, 2008 © Organisation Intersex International.
Available online at: http://www.intersexualite.org/Genetics_1.html
11) Schaffer, L.G. & Tommerup, N. ISCN 2005: An International System for Human Cytogenetic Nomenclature (2005): Recommendations of the International Standing Committee on Human Cytogenetic Nomenclature., 2005. Karger, S.C. Publ.
12) Siedlberg, S. The Gender Genital Gene Genie. Manuscript published 2001.
Available online at: http://www.gender.org.uk/chstnuts/ggg.htm
13) Italiano, M. Some problems with the new terminology for intersex. Manuscript published July 13, 2008 © Organisation Intersex International.
Available online at: http://www.intersexualite.org/Terminology.html
Posted by: Mane | August 17, 2008 6:59 PM
@ Nick #36:
TBH, I don't really believe these studies, I mean, in the past, circumcision has been the cure for just about everything they could think of, but it's really just a stupid justification for a barbaric practice.
Posted by: Pablo | August 17, 2008 7:06 PM
Recall that the African studies of AIDS transmission involved following circumcised adults for 6 months after they were circumcised. It is not clear how much of the difference in the rates of HIV infection were due to differences in transmission, and how much is due to the fact that recently circumcised men are not apt to be having a lot of sex, at least immediately.
That is my interpretation of the problems.
Moreover, it is not clear as to how they translate to the US, where things are different in terms of education and condom use.
Posted by: pipsqueak | August 17, 2008 8:29 PM
Another awesome PZMinion post.
One thing that interests me here is how closely the issues MAJeff raises mirror those of people with all kinds of physical disabilities (note I'm not saying that intersex people are disabled).
My mum did some research with children and teenagers with disabilities a few years ago and a recurring theme was their distress at being put through lengthy and often painful treatments to make them appear more "normal". i.e. instead of being left to scoot about in a wheelchair, they might undergo several rounds of surgery so that they could kind of walk. This would make them appear more normal, but actually leave them less able to keep up with their able-bodied friends.
Posted by: Nick Gotts | August 17, 2008 8:43 PM
Mane@42,
You could well be right. But exactly because I find it counter-intuitive, and wish it not to be true, I'm not willing to dismiss the studies, or forget them. Time will tell - probably.
Posted by: SEF | August 17, 2008 8:50 PM
@ Pablo #43:
There's also the possibility that, if some of them were having circumcision as adult men (ie rather late as these things go) on the assumption it would protect them, they were necessarily more aware of the reality of HIV-AIDS and the risks of having sex than many in the control group - who might have had little education or even mis-education on the subject (there are some horrific examples of the various lies and disinformation put out by religious "authorities"). Hence the mindful group could well be taking fewer risks than some random control group.
It's rather like some of the supposed health benefits misattributed to vegetarianism actually being down to that subset being somewhat more health-freaky overall (as they have to be to survive on an otherwise nutrition-poor diet) than the general population from which a typical control group might be drawn.
There would have to be good evidence that the people conducting the circumcision-HIV studies took care to ensure they matched the subjects in the groups for their level of (relevant) education and their relative risk-aversion (such that their behaviour other than over the circumcision decision was as similar as possible).
Posted by: Shadow | August 17, 2008 9:02 PM
As one of my students once said to the other women in the class about the possibility of having half a clitoris and no sensitivity, "Wouldn't it just make you tense all the time!"
Which brings up another kind of interesting point, really, which is that just because someone is fully one gender (physically and emotionally) and content that way, doesn't mean all the parts are going to work 'properly'. Frankly, I'd be a lot happier without that particular bit at all, because it would keep my partners from assuming it's pleasurable to have it touched and going straight for it. It isn't, and even yelling, "How many freaking times have we had this conversation about staying away from that thing?" doesn't seem to discourage it.
But then, I'm a little more on the asexual/anorgasmic side of hetero (I have a definite orientation, but little real interest in the act and no ability to get off in the traditional fashion), and all I ever hear is, "Oh, honey, you just haven't been done right yet. Someday, someone will be able to 'fix' you."
*Sigh* Not broken. Just wired differently.
Posted by: Nick Gotts | August 17, 2008 9:06 PM
SEF@46,
Evidence that vegetarianism is a "nutrition-poor diet"?
Posted by: Alan Kellogg | August 17, 2008 9:50 PM
Daniel, #16,
I've known Zoe for some time now. I first met Alan through his contributions to the RPG APA Alarums and Excursions in the late 70s, and rediscovered him again when I learned about his blog, A.E. Brain
His journey began when his GP started him on Lipitor for cholesterol. The Lipitor worked, stopping cholesterol production entirely, and testosterone production as well. Along with eliminating fat deposits in the abdomen, which held years of estrogen production. In short, Alan found himself flooded with estrogen, and starting a sort of secondary puberty. There were a few months where Alan and his doctor were wondering just what the hell was going on. so Alan was referred to a endocrinologist, who ran a few tests. Alan learned the results of those tests when his doctor greeted her with, "Ms. Brain, I think we've learned what's been going on."
Since then she has gotten a new I.D. as a woman, a new name --- Zoe Ellen Brain, and even a new Australian passport. Zoe is now working on her PHD, has a wife and child (married status doesn't change because of a sex change under Australian Federal law), has blogged about her experiences, and blogs about space travel, rockets, neat stuff, and intersexed and transexual issues. She's well worth keeping track of, and she can give you a more comprehensive account of her adventure than I ever could.
And to hype this Zoe Brain comment on Google, I'm going to repeat Zoe Brain a few times in a blatant attempt to get Zoe Brain over here to comment. BTW, when you do visit Zoe Brain's blog have a look at the lady up at he upper left hand corner. That's Zoe Brain. Zoe Brain is a cutie, and I'm saying that to embarrass Zoe Brain. :)
Posted by: clinteas | August 17, 2008 10:24 PM
//But then, I'm a little more on the asexual/anorgasmic side of hetero (I have a definite orientation, but little real interest in the act and no ability to get off in the traditional fashion)//
Shadow,No 47 :
There is no such thing as an "asexual/anorgasmic side of hetero",there is of course "different wiring" to every individual,and half the fun is to figure out which buttons to press on a person,isnt it(at least for me).
"Not interested in having my clit assaulted" is not an exclusively hetero thing tho by any means,why would it be limited to heterosexuals with a clit(if thats the bit you were referring to) ?
Posted by: flame821 | August 17, 2008 11:28 PM
I have to side with the "wait and see and let the child/patient have a say"
As I recall (over my 20 years in medicine) the operating phrase in these sorts of cases was "It's easier to dig a hole than build a pole" so the majority of the cases were assigned a female persona. A huge problem when they reached adolescence and secondary characteristics made themselves known.
Posted by: Shadow | August 18, 2008 12:14 AM
#50:
Oh, I didn't think it would be. I don't think I was explaining myself well. *Scratches head* Lemme try again.
The question that was put forth pertaining to women and how having a partial clitoris and lack of sensitivity might make them a little nuts just reminded me of some statements that have been made to me on occasion. It's ineresting to me that, among that people I have experience with personally (because I honestly don't know what's going on in the world at large), even those who appear to otherwise have a decent amount of understanding and acceptance as far as gender issues go don't always seem to extend it to other aspects of sexuality. So while it might not phase them to hear of an individual might desiring reassignment surgery - or that an individual who'd had surgery performed at birth might want to correct issues stemming from that later on - it does seem to throw them to be presented with a woman who would, were it possible, potentially be interested in having one specific gender-related portion of her anatomy removed for the sake of feeling disconnected to and unhappy with it. I've been told in no uncertain terms that I'm 'sick' and ought to seek therapy, or that I'd be sorry afterwards because it would destroy my ability to experience sexual pleasure or have an orgasm (well, gee, I don't have them anyway, so). It's not the only thing I'd like to see gone (I'm almost phobicially freaked out by the fact that I'm technically able to reproduce), but it's the one that's the least expected, I guess.
In kind of a similar vein, I've been questioned as to why I'm not interested in dating, why I'm not interested (enough) in sex, told that my lack of drive is either abnormal or a result of denying myself - and that in either case, I ought to (again) seek professional help so that I can open up and experience the sort of pleasure I deserve. I absolutely understand that 'asexual' and 'heterosexual' are not necessarily terms that can or ought to be used together; it's just that I'm lacking a word to properly express myself without having to go into spiels of detail. So I'm also quite sympathetic to the fact that none of the concepts I've described are restricted to women or heterosexuals - I was speaking purely of my own experiences.
And of course, I have no reason whatsoever to believe that the speaker in the mentioned classroom would be of similar opinion to the individuals I've encountered - it just happened to spark the thought that I find it interesting that even as we're learning more about alternatives to 'standard' humanity and (hopefully) becoming more open to allowing their expression, we still seem to have expectations for the members of the 'normal' group that don't necessarily fit any better.
Posted by: Zoe Brain | August 18, 2008 12:35 AM
I added a welcome post to those here, and a few links to some of the articles most relevant to this post.
---
Daniel - re #16 -
Thanks! That's such a glowing review, would you mind if I quoted it?
---
Kelly - re #19
10% of those 46xx individuals born with CAH identify as male. Many of the rest don't identify as "Intersexed", and rather resent that label. They're just partly masculinised women, not poor gender-confused freaks (their words, not mine - I belong to a CAH support group).
As a general rule, the best practice would appear to be:
a) Neonatal Minimal surgery to ensure urinary continence etc, freedom from pain, and reduce immediate (rather than potential long-term post-puberty) cancer risk.
b) No other neonatal surgery until an age of informed consent is reached. In particular no surgery without consent that would either remove sensation or compromise fertility.
c) Then allow the patient to say what gender they are - M, F, N - and how they wish their body to be configured surgically and/or hormonally. They should be informed of potential cancer risks and other dangers of all alternatives.
---
Shadow #47
Almost a mirror image of how I felt prior to transition. I had male peripherals and female device drivers, so functionality was limited. I could please, but not be pleased.
The surgery put the right nerves in approximately the right places. So although some sensitivity has to be lost in any surgery, effectively it's been increased dramatically.
Women vary. About 1 in 5 are anorgasmic. Rather more than that get no kick out of clitoral stimulation, unless accompanied by other things, and sometimes not even then.
---
Alan K - re #49
You succeeded. In both getting me to comment, and in causing me to blush for the first time in living memory.
I thought after having a non-volitional sex change, *nothing* could embarrass me. I was wrong.
Oh BTW we tried using Lipitor again to see what would happen in a 3 month experiment - the condition wasn't repeatable. So the current working hypothesis is having both masculinising NC-CAH and feminising CAIS Intersex conditions simultaneously, symptoms triggered by Lipitor. But we're still guessing. The treatment for NC-CAH works though, so to be pragmatic, that's all that matters.
Posted by: clinteas | August 18, 2008 12:59 AM
Hm,I wonder,GPs give out Lipitor like lollies these days.Sounds like a few non-related random events had to coincide to cause what it did to Zoe,still,if you give it too enough people.....
I was aware of the number of 1 in 5 for anorgasmic females,didnt think it was purely attributable to a hardware problem tho.
@ Shadow,
thanx for clarifying that,I assumed that was how you meant it.
And dare I say that this:
//It's not the only thing I'd like to see gone (I'm almost phobicially freaked out by the fact that I'm technically able to reproduce), but it's the one that's the least expected, I guess.//
would seem to indicate a few non-organic issues,that might be accessible to some sort of exploration through therapy.
But not my business at all,of course.
Posted by: Shadow | August 18, 2008 2:07 AM
@clinteas:
Well, I am a diagnosed head case or three, so... -g- I just don't believe that that's the sole root of the quirks downstairs. And I'm not so terrified of reproducing that I can't function sexually in the ways that do interest me - I'm able to say, "Okay, I can take appropriate precautions and reduce the chances," and I'm responsible enough to make sure I do. It'll freak me out if I sit and think too much about it, but it's not something I have a habit of dwelling on (whereas there are triggers for other anxieties I have a much harder time with). I do feel a definite disconnect from the ability and the organs, almost as if they're incidental to my makeup rather than a necessary part of being female, and I've occasionally wondered if I'd feel more akin to the way I see myself in my head if they were gone - but it's not something that's of so much importance that I feel I need to be that altered to reflect it (but I thoroughly support the right of others to be modified in any way they see fit in order to be comfortable in their own skin).
And really, I've had such negative experiences with therapy (for other reasons) in the past that I'm a big fan of managing things myself when I can. I'm told that some of the "not okay, needs to be fixed" lines aren't as firmly drawn now, but I've been so much better off since I drew the "not doing this anymore" line that I'm not willing to risk it.
Posted by: clinteas | August 18, 2008 2:11 AM
Shadow,
Im sure youve been through all this a million times with yourself and others,I really shouldnt attempt to comment I guess....
Whatever works for you and makes you happy !
Posted by: Shadow | August 18, 2008 2:31 AM
No worries. -g- I'm pretty willing to discuss with people who are reasonable about the whole thing, and the mere fact that I got a, "Whatever works for you," from you suggests you're a whooole lot more reasonable than some I've come across.
Posted by: Alan Kellogg | August 18, 2008 3:04 AM
Zoe Brain, #53,
You can blush, you can have romantic thoughts. You can have romantic thoughts, wild crazy monkey sex is in your future.
As many a researcher into human sexuality has learned, a large part of sexual response is imagination. You can see yourself in a pleasurable sexual situation it eases a lot of problems. Blushing shows that you can see yourself in pleasurable sexual situations
Posted by: tina | August 18, 2008 4:21 AM
While people with conditions like CAH and AIS might not present with gender dysphoria in appreciably higher numbers than the average "normal" population, people with Klienfelter's Syndrome (47, XXY) *do* have gender issues in higher than average numbers...which shouldn't really be much of a surprise if chromosomes have anything to do with gender ID- if "normal" means you have to pick only two, then someone with an XXY genotype could be either an XY male with and extra X, or an XX female with an extra Y.
But despite this possibility and well documented gender incongruities in many XXY people (who are almost all assigned as male based on that single Y), standard treatment is supplemental testosterone to force them into being as physically male as possible...which is fine if the individual's gender ID is male, but for those whose innate sense of gender is androgynous or female, it is about the worst possible thing that could be done to them.
The saddest part of all of this is how criminally little attention is given to intersexed people's innate sense of their own gender, not just in individual treatments but in medical literature and research...with very rare exceptions, there are practically no protocols that deal with helping IS people with associated gender dysphoria or for fixing failed IS "normalizations" where the individual knows that their birth assignment was wrong (because doctors are extremely unwilling to admit making such mistakes, let alone admitting that they happen often enough that standard protocols need to be developed)...these people more often than not are forced to take the same route as non-IS (at least by current standards) transsexuals which means seeking and accepting a diagnosis of having a psychological disorder...even though the DSM says that GID can only occur in the absence of an IS condition.
These people have to deal with the same kinds of medical, legal and societal hurdles that transsexuals do, and the biggest societal hurdle is the fact that the very same people in positions of authority and power who are the most adamant about forcing "normalization" involving hormone therapy and genital reassignment surgery on non-consenting children as a means of reinforcing the allegedly natural gender binary they consider 'God's Plan', are often the same people who are most adamantly against anyone *willingly* getting hormone therapy and genital reassignment surgery as adults in an attempt to align their gender ID and physical body, because "God doesn't make mistakes"...and this holds true not just for run-of-the-mill transsexuals but even