91av

CHERYL CHASE has a mission. She’s fighting one of society’s strictest
conventions—that there are men and there are women, and that’s all. In a
way it’s 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’t leave her that way.
Because it was easier to make her look female rather than male on the outside,
that’s 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’t think someone should have been allowed to make that decision for
her.

But this is not just a personal complaint. It’s a battle she’s 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’t fall neatly
into the categories of male and female. That’s more babies than are born each
year with Down’s 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
“normalising” babies born with intersex conditions or ambiguous looking
genitalia. “There is no demonstrated benefit to the patient,” she says. “And
there are plenty of instances of people speaking out to say that this has harmed
their sexual function or has harmful emotional consequences.”

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’s 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’s the distraught new parents, grief-stricken and worried about
their child’s future, that the physicians have to deal with. This has led to
accusations that surgery is done purely for the parents’ benefit, to make them
more comfortable with the child’s 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’t.
“What seems to be happening is that society’s so-called standards are colluding
with medicine to make sure that people fall into one of two bipolar positions,”
he says. “You 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.”

In a world where the most common first question asked of new parents is “boy
or girl?”, it’s amazing how many variations there are
(see “Common intersex conditions and genital anomalies”).
Problems include anomalies in the genital structures, often an enlarged clitoris
or a very small penis. Sometimes the penis or vagina don’t form at all, or the
urethra may open in the wrong place. Some conditions are called intersex, where
the chromosomes and phenotype don’t match, often leading to a blurring of the
male and female anatomies.

Currently, if a girl’s 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’s penis
is underdeveloped, and doesn’t 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’s 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’s birth certificate must still state male or female.

But whether it’s 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’s options open in case you
choose wrong.

“One 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’s a boy,”
says Anne Fausto-Sterling, Professor of Biology and Women’s studies at Brown
University, and author of Sexing the Body, about our biological and
cultural views on gender. “That could have been prevented if he’d been allowed
to be raised with some level of ambiguity for some period.”

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’s a real medical necessity, believes Fausto-Sterling. “And by medical
necessity, I mean life or death,” she says.

“We shouldn’t be doing surgery just to make the genitalia look normal,”
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. “Who is going to look after
these patients when they get stone formation in the vagina, recurrent urinary
infection and are incontinent of urine?” 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. “If you do nothing, you will undoubtedly create a disaster,”
says Ransley. Moreover, he points out, the gonads in some intersex patients seem
to be at a higher risk of becoming cancerous. “There are risks that you may lose
the patient, that they may disappear and therefore carry a substantial cancer
risk with them perhaps unknowingly,” 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’d been given a
choice, but it’s not at all clear how many would have been happier if they’d
been left alone. Ransley believes that the psychological anguish of leaving a
child is “outstandingly awful”. And he thinks that the effects of parents’
anguish should not be underestimated. “If the parents are unhappy then they
rapidly transmit that feeling to the child.”

“The way things have been managed is a response to a real anguish of parents
and children dealing with feeling different,” says Polly Carmichael, a clinical
psychologist at the Great Ormond Street Hospital. “I’ve been surprised about how
disturbed they are in the difference in appearance from their peers. It’s
something that really affects their sense of themselves and their self-esteem.”
She gives an example of a boy with a very small penis with the urethra opening
on its shaft. “People at school are calling him a girl,” 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—by surgically reassigning the boys as
girls—was 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. “These are not
all happy people either,” says Creighton. “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 these things out.” 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: “It’s
going to take us years to sort out the questions. We don’t 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.”

Sue Elford, who chairs the CAH support group, has a son with CAH and now
spends a lot of time talking to new parents. “If it had been my daughter I would
willingly have had everything made ‘normal’, as they call it, as soon as
possible. But now, you know, it frightens me because it often isn’t the best
thing.” 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’t
everything. “They should be given all the facts, which I feel isn’t always
done,” says Elford.

And too often, doctors push parents to decide too soon, say intersex
advocates. “They speak to the parents while they’re still in emotional crisis
and the parents OK the surgery at that point,” says Chase—even 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. “The thing that causes the most trauma is not
finding out the truth but rather finding that they have been lied to,” says
Alice Dreger, an ethicist at Michigan State University who serves on the board
of ISNA. “What it says is that your condition is so shameful we can’t even speak
of it. It’s like the way cancer used to be treated.”

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’t “cure” 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. “Part of the solution is by
people just speaking out about their lives,” says Chase. “It’s been shocking to
parents because they haven’t 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.”

• 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’t 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).