麻豆传媒

CHERYL CHASE has a mission. She鈥檚 fighting one of society鈥檚 strictest
conventions鈥攖hat there are men and there are women, and that鈥檚 all. In a
way it鈥檚 a very personal crusade, because Chase was born intersexed, with sex
organs that were neither wholly male nor wholly female. She was raised as a boy
for the first 18 months, but the doctors felt they couldn鈥檛 leave her that way.
Because it was easier to make her look female rather than male on the outside,
that鈥檚 what the surgeons did. They removed her testicular tissue and remodelled
her genitals long before she could offer any opinion on the subject. And she
doesn鈥檛 think someone should have been allowed to make that decision for
her.

But this is not just a personal complaint. It鈥檚 a battle she鈥檚 fighting on
behalf of a remarkably large number of people. Around one in 2000 babies in the
Western world have some kind of surgery because their bodies don鈥檛 fall neatly
into the categories of male and female. That鈥檚 more babies than are born each
year with Down鈥檚 syndrome or cystic fibrosis.

Chase founded the Intersex Society of North America (ISNA) and, along with a
growing number of supporters, is campaigning to stop the practice of surgically
鈥渘ormalising鈥� babies born with intersex conditions or ambiguous looking
genitalia. 鈥淭here is no demonstrated benefit to the patient,鈥� she says. 鈥淎nd
there are plenty of instances of people speaking out to say that this has harmed
their sexual function or has harmful emotional consequences.鈥�

Genital surgery like this has been kept largely hidden and secret. But now
intersex management is becoming one of the most talked about subjects in
medicine. It鈥檚 an ethical minefield. There are big disagreements on where to
draw the line between medical necessity and the purely cosmetic. And how should
you balance the immediate anguish of parents against the future anguish of
patients? It鈥檚 the distraught new parents, grief-stricken and worried about
their child鈥檚 future, that the physicians have to deal with. This has led to
accusations that surgery is done purely for the parents鈥� benefit, to make them
more comfortable with the child鈥檚 sex. And now more and more patients are coming
forward to say they are not happy with what happened to them as children.
Clinicians are realising that they must take a long, hard look at whether
surgery to make the genitals look normal is solving anything.

Jay Hayes-Light, director of the UK Intersex Association, believes it isn鈥檛.
鈥淲hat seems to be happening is that society鈥檚 so-called standards are colluding
with medicine to make sure that people fall into one of two bipolar positions,鈥�
he says. 鈥淵ou are either male or female and you cannot be in between. We are
actually a community of people who were swept under the carpet, because the
surgeries aim to make us blend in.鈥�

In a world where the most common first question asked of new parents is 鈥渂oy
or girl?鈥�, it鈥檚 amazing how many variations there are
(see 鈥淐ommon intersex conditions and genital anomalies鈥�).
Problems include anomalies in the genital structures, often an enlarged clitoris
or a very small penis. Sometimes the penis or vagina don鈥檛 form at all, or the
urethra may open in the wrong place. Some conditions are called intersex, where
the chromosomes and phenotype don鈥檛 match, often leading to a blurring of the
male and female anatomies.

Currently, if a girl鈥檚 genitals are masculinised by exposure to hormones in
the womb, doctors are most likely to recommend feminising surgery before the age
of one to create a vagina and reduce the bulk of the clitoris. If a boy鈥檚 penis
is underdeveloped, and doesn鈥檛 show any signs that things will change with
hormone treatment, they may suggest that reassigning him as a girl is the best
option. In some cases it鈥檚 not at all clear which sex a child will identify with
in the future. Take Jamie, for example. This was a child born with one
undescended testicle, a phallus larger than a clitoris but with none of the
features of a penis, no vaginal opening and a rudimentary ovary and uterus.
Jamie鈥檚 birth certificate must still state male or female.

But whether it鈥檚 a slight clitoral reduction, creating a vagina or
reassigning a gender, surgery should wait, activists say, until the child is old
enough to decide for itself. Sure, make as informed a decision as possible about
which sex the child is most likely to adopt, and name, dress and rear the child
as a boy or a girl, they say. But keep the child鈥檚 options open in case you
choose wrong.

鈥淥ne of the worst things that can happen is that, say, a penis is removed and
the testes taken out and that at age eight the kid insists that he鈥檚 a boy,鈥�
says Anne Fausto-Sterling, Professor of Biology and Women鈥檚 studies at Brown
University, and author of Sexing the Body, about our biological and
cultural views on gender. 鈥淭hat could have been prevented if he鈥檇 been allowed
to be raised with some level of ambiguity for some period.鈥�

The patients coming forward are also pointing out that surgery causes pain,
scarring and lack of sensation, and removes the chance that improved treatments
may someday make them fertile. Nor does early surgery eliminate the need for
follow-up operations, doctors are finding. Early surgery should only be used if
there鈥檚 a real medical necessity, believes Fausto-Sterling. 鈥淎nd by medical
necessity, I mean life or death,鈥� she says.

鈥淲e shouldn鈥檛 be doing surgery just to make the genitalia look normal,鈥�
agrees Philip Ransley, a paediatric urologist from Great Ormond Street Hospital
in London. But reserving it for life and death situations is too idealistic.
Some conditions, such as congenital adrenal hyperplasia, CAH, in which prenatal
exposure to androgen hormones masculinises an XX fetus, can lead to malformation
of the urethra, which sometimes joins to the vagina. 鈥淲ho is going to look after
these patients when they get stone formation in the vagina, recurrent urinary
infection and are incontinent of urine?鈥� he asks.

And there are cases where Ransley believes that leaving things alone makes
matters worse. If the decision is to raise someone as female, but they have
testes, he believes you have to act quickly before testosterone can masculinise
the child further. 鈥淚f you do nothing, you will undoubtedly create a disaster,鈥�
says Ransley. Moreover, he points out, the gonads in some intersex patients seem
to be at a higher risk of becoming cancerous. 鈥淭here are risks that you may lose
the patient, that they may disappear and therefore carry a substantial cancer
risk with them perhaps unknowingly,鈥� says Ransley. Undescended testes often
cause hernias too, which have to be dealt with surgically.

Everyone agrees that some surgery is medically necessary, but we know next to
nothing about the risks and benefits of most surgery for intersex conditions.
Plenty of patients have come forward to tell their horror stories, but there
have been no systematic studies to say whether doing nothing is more often good
or bad for patients. The intersex advocates say they wish they鈥檇 been given a
choice, but it鈥檚 not at all clear how many would have been happier if they鈥檇
been left alone. Ransley believes that the psychological anguish of leaving a
child is 鈥渙utstandingly awful鈥�. And he thinks that the effects of parents鈥�
anguish should not be underestimated. 鈥淚f the parents are unhappy then they
rapidly transmit that feeling to the child.鈥�

鈥淭he way things have been managed is a response to a real anguish of parents
and children dealing with feeling different,鈥� says Polly Carmichael, a clinical
psychologist at the Great Ormond Street Hospital. 鈥淚鈥檝e been surprised about how
disturbed they are in the difference in appearance from their peers. It鈥檚
something that really affects their sense of themselves and their self-esteem.鈥�
She gives an example of a boy with a very small penis with the urethra opening
on its shaft. 鈥淧eople at school are calling him a girl,鈥� says Carmichael.

When Justine Schober, urological surgeon at the Hamot Medical Center in Erie,
Pennsylvania, presented the first follow-up study of 20 boys and men who had
escaped surgery for a condition called micropenis at a conference back in 1988,
it provoked an uproar, she says. The study suggested that the most common way of
managing the condition鈥攂y surgically reassigning the boys as
girls鈥攚as misguided. She found those who had escaped feminising surgery
were happy with that decision. They reported living fulfilled lives, the older
ones reported good sex lives, normal marriages, and one was a father.

But the picture is not always so clear-cut. Sarah Creighton and Catherine
Minto from University College London Hospitals have been working with a group of
ten British women who escaped surgery for intersex conditions. 鈥淭hese are not
all happy people either,鈥� says Creighton. 鈥淪ome of them have isolated, difficult
lives. Some of the surgery patients are fine and some of them are not and it鈥檚
very hard to separate all these things out.鈥� And these two studies are just
about the only systematic follow-up studies that have been done to date.

The medical world certainly agrees that more research is needed, and the
North American Task Force on Intersexuality, a group of physicians and
representatives from patient support groups, as well as a working party set up
by the British Association of Paediatric Surgeons, are beginning to identify the
research questions and methods. But Schober, a member of NATFI, says: 鈥淚t鈥檚
going to take us years to sort out the questions. We don鈥檛 really know the
answers yet, but we are making efforts to get those answers. The decision they
are making is so profound for that child in the long run. It is the very core of
their being for the future.鈥�

Sue Elford, who chairs the CAH support group, has a son with CAH and now
spends a lot of time talking to new parents. 鈥淚f it had been my daughter I would
willingly have had everything made 鈥榥ormal鈥�, as they call it, as soon as
possible. But now, you know, it frightens me because it often isn鈥檛 the best
thing.鈥� She thinks that if parents are told the full implications of surgery,
how little is understood about the long-term benefits, the scar tissue, and
potential for lost sensation, they will be better armed to make good decisions
for their children and more likely to realise that appearances aren鈥檛
everything. 鈥淭hey should be given all the facts, which I feel isn鈥檛 always
done,鈥� says Elford.

And too often, doctors push parents to decide too soon, say intersex
advocates. 鈥淭hey speak to the parents while they鈥檙e still in emotional crisis
and the parents OK the surgery at that point,鈥� says Chase鈥攅ven though the
operations generally happen later in the first year. Parents need time to
adjust, she says.

The problem has been compounded by the veil of secrecy that almost always
surrounds an intersex child. 鈥淭he thing that causes the most trauma is not
finding out the truth but rather finding that they have been lied to,鈥� says
Alice Dreger, an ethicist at Michigan State University who serves on the board
of ISNA. 鈥淲hat it says is that your condition is so shameful we can鈥檛 even speak
of it. It鈥檚 like the way cancer used to be treated.鈥�

So, in a way, the arguments surrounding surgical intervention for intersex
are just a start. Even after surgery, a large number still see their sexual
identity as intersex rather than male or female, according to a new study by
Schober. Medicine can鈥檛 鈥渃ure鈥� intersex. Society is going to have to learn to
understand and accept it and allow people to be who they are, rather than who we
think or expect they should be, advocates say. 鈥淧art of the solution is by
people just speaking out about their lives,鈥� says Chase. 鈥淚t鈥檚 been shocking to
parents because they haven鈥檛 heard of it. The more that intersex people are
willing to come out and talk about themselves, the less surprising it is to
parents when their intersex child is born.鈥�

• Congenital adrenal hyperplasia:
  An excess of masculinising androgen hormones
  can produce an enlarged clitoris and fused labia in girls. CAH is the most
  common cause of ambiguous genitals (1 in 10,000 births, though estimates that
  include the mildest forms are as high as 1 in 100).
• Androgen insensitivity syndrome:
  The fetus has testes and X and Y
  chromosomes, but its cells don鈥檛 respond to testosterone, so the baby will
  appear completely female (around 1 in 20,000 births). In partial AIS (between 1
  in 13,000 and 1 in 130,000, depending on definition), there is some response to
  testosterone, and ambiguous genitals may develop.
• Micropenis:
  The penis is underdeveloped, sometimes because of low hormone
  levels (1 in 50,000 births)
• Klinefelter syndrome:
  Boys with an extra X chromosome have masculine
  genitals, but limited secondary sexual characteristics develop at puberty.
  Breasts may grow (estimates are as high as 1 in 500 male births).
• Partial or mixed gonadal dysgenesis:
  The genotype is usually XY, but internal
  and external anatomy may be ambiguous or mixed. Gonadal dysgenesis is a
  developmental condition with multiple causes.
• Hypospadias:
  The urethra opens below the tip of the penis or along its
  underside in boys, or inside the vagina in girls. Its cause remains unclear but
  may be genetic, hormonal or caused by drugs given during pregnancy (1 in 770 to
  1 in 2000 births).