Saturday, 12 July 2008

It's Easier to make a Hole than a Pole

That's the infamous saying of one surgeon, when asked why so many Intersexed infants were surgically assigned as female shortly after birth.

Surgeons are not monsters, and if gender is completely malleable at birth, he has a point. It's far easier and more effective to construct approximately female genitalia from the ambiguous mess that sometimes results in some cases.

However... in some cases that means castrating a boy. Removing perfectly functional testes, simply because the third part of the triad of two testes and a penis is lacking. And worse, gender is most definitely not completely malleable, or significantly malleable at all. Some are BiGendered, but most aren't.

So how and when is gender determined? Good question. The symptoms of gender don't appear in the neurology until well past birth. But the tendencies, the pre-conditions that dictate (or at least steer) the later development come into play earlier.

Assuming that gender is set in neurology, and assuming also that it depends on hormonal environment in the womb (as indicated by other studies), we can make certain conclusions. For some Intersex conditions, those involving cell membranes and sensitivity to various hormones, any cross-gendering would happen ab initio, and likely affect the brain. For others, those involving abnormal glandular development, the timing of the cross-gendering would be too late to affect all neural development, only some. We would therefore expect two distinct groups - one with rather more likelihood of cross-gendered gender identity than the other, depending on the exact syndromes.

From Endocrine Today of July 10 2008, "Clinical news on diabetes and endocrine disorders":
Research has started to explain the correlation between specific DSDs and gender identity later in life. For example, more than 90% of patients with 46,XX congenital adrenal hyperplasia who are assigned female in infancy later identify as females. Other conditions are less concrete: among those patients with partial androgen insensitivity syndrome, androgen biosynthetic defects and incomplete gonadal dysgenesis, approximately 25% go on to suffer dissatisfaction with their assigned gender.
As expected, and as regards the science, we can express some quiet satisfaction that we've added another piece to the puzzle.

As Human Beings though, we must be devastated. For it means that even in the best of cases, where we know the odds of neural cross-gendering are low, and so can feel comfortable taking a scalpel to an innocent babies' genitalia, we get it wrong one time in ten, and surgically create transsexuality. Worse, when the situation is more fraught, our results are not much better that tossing a coin: if not for some being BiGendered, able to function adequately (if not well) in either gender role, it would be hardly better than 50/50 - 55/45 or thereabouts.

I'll quote from the 2006 Summary of Consensus Statement on Intersex Disorders and Their Management, a result of the International Intersex Consensus Conference:
Psychosexual development is influenced by multiple factors including sex-chromosome genes, brain structure, social circumstance, family dynamics, and prenatal AE (ie Androgen Effect - ZEB). AE is apparently dose related (eg, childhood play behaviors in girls with congenital adrenal hyperplasia [CAH]) and associated with development of male gender identity (patients with 46,XY cloacal exstrophy who were gonadectomized neonatally). Androgen effect on genital development apparently occurs independently of effect on neural and behavioral development; genital appearance does not predict gender-role change. Outcome cannot be predicted by degree of prenatal AE for any DSD (Disorder of Sexual Development - Intersex - ZEB), with variability relating to both prenatal and postnatal AE differences (eg, 5{alpha}-reductase deficiency [5{alpha}-RD2]).

Emerging evidence suggests direct effects of sex-chromosome genes on brain and behavior, but behavioral roles for Y-chromosome genes have not been identified. A Y-chromosome and male-typical prenatal AE does not unequivocally entrain adult male gender identity.

Sex differences in brain structures have been identified across species. Human postmortem and neuroimaging studies have found that male brains are 8% to 10% larger than female brains. Sexual dimorphism in specific neural subcomponents persists after adjustment for brain size. The limbic system and hypothalamus are important because of roles in reproduction; specific nuclei show sexual dimorphism on postmortem studies (bed nucleus of the stria terminalis, suprachiasmatic nucleus, and the interstitial nucleus of the anterior hypothalamus ), although it is unknown when these differences emerge. Relationships between structural brain differences and psychosexual development are unclear.
...
Outcome data indicate that >90% of patients with 46,XX CAH and all patients with 46,XY CAIS who are assigned female sex in infancy identify as females. Two thirds of patients with 5{alpha}-RD2 who were initially assigned female sex who virilize at puberty, and all who were assigned male, live as males. Gender-role changes are reported for approximately half of the 17ß-hydroxysteroid dehydrogenase-3-deficient patients who are raised as girls. The recommendations are to raise infants with 46,XY CAIS and 46,XX CAH as females, whereas for infants diagnosed with 5{alpha}-RD2 or 17ß-hydroxysteroid dehydrogenase-3 deficiency, a male assignment should be considered.
Which is in accordance with what I'd found, coming in from another direction, that of studying gender identity.
Sexual functioning, psychosocial adjustment, mental health, quality of life, and social participation are understudied. Attention has been toward dissatisfaction with assigned sex or gender self-reassignment. Dissatisfaction is found in ~25% of 46,XY patients with partial virilization regardless of assigned gender.
Exactly what would be expected if about a third of people are BiGendered.
Assignment should be made as quickly as thorough diagnostic evaluation permits. Influencing factors include diagnosis, genital appearance, therapeutic options, fertility potential, cultural practices and pressures, and parental views. Individual DSD outcome data regarding gender identity, quality of life, avoidance of unnecessary surgery, hormone replacement, and fertility preservation must be considered. Fertility-potential considerations include expected fertility in virilized females with a well-developed uterus and ovaries, unlikely fertility in undermasculinized males without assisted-reproduction techniques, and fertility in some patients with ovotesticular DSD. Appearance-altering surgery is not urgent. Somatosexual and psychosexual differentiation information and treatment options are needed. Balanced, technologically sound, Web-based information may be helpful.
It is important to realise that "assignment" emphatically does not mean surgical assignment, merely social assignment. Something that can be reversed, and which does not remove fertility or sensation in a quest for "normalisation".

Hopefully my posts can be classified in the main as "Balanced" and "technologically sound". That can be difficult when I've heard so very many horror stories of misguided, and in some cases, inhuman, surgery just to please parents and spare society the embarrassment of dealing with someone Intersexed.

Right now though, the standard treatment - treatment not in accordance with this consensus statement - can be described as follows:
  1. For Infants - surgery without their consent, and at the parents' and doctors' whim. Usually to female, as that's easier.
  2. As Adults - massive roadblocks or blanket prohibitions on surgery, with the patient being diagnosed as mentally ill if they don't conform to the gender they were arbitrarily assigned.
  3. For parents - little or no advice, and the power to dictate surgical alteration of their child to match their wishes for a boy or girl. Sometimes they are just told what will be done, and required to rubber-stamp it, with no knowledge or truly informed consent.
This is exactly the opposite of what should be. What we should do is:
  1. For Infants - only such surgery as is necessary for urinal functionality, to prevent pain, and to avoid cancer risks.
  2. For Adults, recognition that only they can state what gender they are, and support with advice on surgical and non-surgical options now they are in a position to give informed consent.
  3. For parents - guidance and advice on rearing, psychological support, and if need be, therapy so they don't reject or mutilate their child.
My son was born with a mild intersex condition, obviously male in genital appearance, but requiring surgery to gain urinary functionality and relieve pain. At the time, I was unaware of any of the issues, I just wanted my boy to grow up to be a normal man, rather more normal than his daddy. At age 7, he's a normal little boy, for which I thank all that is Holy. But... I now know that there was the possibility of another outcome. I should have been informed. I looked up everything I could about his particular condition, but not the issues in general. That I only did 4 years later, when my whole world changed.

I'm not about to blame parents who have to "wing it" without being given all the information they need to make a decision. I've been there myself. I can't blame the paediatric surgeons, who apart from a bad case of arrogance, are candidates for sainthood in my book. The paediatric urologist who operated on my son made the minimal possible reconstruction, allowing him to decide when older about further cosmesis, taking into account risks an benefits.

But the system has to be changed, and the doctors made aware of the terrible consequences of premature meddling. 1 ruined life in 10 is not an "acceptable outcome", when by waiting, we can remove the chance completely. 1 ruined life in 4 is not just unacceptable, but is criminal when done merely to cater to the parents' psychological hang-ups.

Further Reading: The IBIS (International Birth Defects Information System) Sex or Gender Assignment webpage.

2 comments:

Anonymous said...

<# For Infants - only such surgery as is necessary for urinal functionality, to prevent pain, and to avoid cancer risks.>

Agreed but with a few additions.

That the cancer risk be more immediate rather then a possible one in later years. Leaving the door open to saying cancer risk still leaves these infants open to being abused.

I'd also add that as they get older, not adult, that they have similar rights as the adult in choosing what is right for them. That they are a very active part in any treatment.

Rick said...

I don't have much experience in this theme, but according to some studies that I made, in the firsts getation months all we are women, if you see from a point of view the vagina is the same thing that a penis but viewed from a different structural perspective.