These challenging situations brought new ways of thinking about behavior.
Among these ways were discussions of “identity” and “roles.” Stoller [7] coined
the term “core gender identity” to reflect a person’s “fundamental sense of
belonging to one sex [an awareness of being male or female and] an over-all
sense of identity.” He attributed this to a combination of infant—parent
relationships, the child’s perception of its external genitalia, and by a
biologic force that springs from the biologic variables of sex [7,8] Money and
colleagues [9] coined the term “gender role” to “mean all those things
[behaviors] that a person says or does to disclose him or herself having the
status of boy or man, girl or woman, respectively” [9]. Money and Ehrhardt [10]
defined “gender identity” as “the sameness, unity, and persistence of one’s
individuality as male, female, or ambivalent. . .the private experience of gender role.” This, they said, basically was
derived from rearing experiences. Gagnon and Simon [11] introduced the term
“sexual identity” to indicate the awareness of an individual as a sexual-erotic
agent within a larger “social identity” that was an appreciation of how a
person fit into society. They also introduced the concept of “sexual scripts”
that are socially imbued ways of acting in different circumstances. The basic
ideas are that sex, genes, and hormones establish one’s body and physiology,
but one’s gender is a product of learning, experience, and indoctrination.
These ideas did not go unchallenged. Several animal experiments revealed the
power of genetics and endocrines to structure males to show reproductive
sex-typical female behaviors and to induce females to display as males [12,13].
For animals, the term “sex-typical behavior” was comparable to
gender-appropriate behaviors. Reports on humans also showed that individuals
who rejected their sex of rearing and experience were not rare [14,15]. From
these studies, a distinction was made between “organizing” forces—usually
prenatal—that dictate the direction of future behaviors and “activating” events
or forces— usually postnatal—that precipitate behaviors [12,16]. Debate on
theoretic grounds also existed [4,17—19] and there were calls for a middle
ground where organizing and activating forces—built-in and learned—would
interact to mold behavior [18].
An ongoing dispute appeared among psychotherapists, biologists, educators,
and others about the forces that are involved in the development of gender and
how those forces are influenced by the environment. In contrast, a seemingly
unified medical understanding emerged. This medical consensus harkened back to
the ideas that sex-atypical gender behaviors were the product of social and
environmental forces. Most physicians believed that homosexual, cross-dressing,
and transsexual activities were deviant; the treatment for the atypical
behaviors seemed to be clear. The subject should be helped to “unlearn” and get
rid of whatever misperceptions and negative experiences had engendered these
behaviors. Often, the treatments that were applied would be considered abusive
today. They ranged from different aversion therapies to castration to
electroshock [20,21]. Such treatment was seen as justified. For example,
Bancroft [21] wrote “In the absence of unequivocal scientific criteria of
morbidity, behavior may be deemed pathological because it violates social
norms.” Intersexuality was not seen as antisocial but it was seen as something
to be hidden or disguised; often by surgical intervention [22,23]. It also was
seen as a body of conditions that resulted from some medical “error” [24].
The “middle” years:
1970s—1990s
From the l970s to the 1990s things changed. In the 1970s, the American
Psychiatric Association removed homosexuality from its list of disorders and
the American Association of Behavioral Therapy questioned the ethics of
attempting to change men’s or women’s sexual orientation. Homosexuality was
seen less frequently as a medical disease that required treatment and was seen
increasingly as a variation in orientation that only needed medical management
when it was ego-dystonic.
The Harry Benjamin International Gender Dysphoria Association (HBIGDA), named
after the physician that presented a major human face to transvestism and
transsexuality [25] was formed in 1977. This organization dedicated itself to
dealing with persons who were diagnosed as transsexuals (persons who have a
desire to change sex that persists for at least 2 years). This diagnosis was
introduced into the Diagnostic and Statistical Manual of Mental Disorders,
Third Edition as Gender Dysphoria of Adulthood. Gender Identity Disorder of
Adolescence was introduced as a separate category. In 1994, the Diagnostic
and Statistical Manual of Mental Disorders, Fourth Edition (DSM-IV) listed
Gender Identity Disorder of Childhood, Adolescence, or Adulthood. Initially,
HBIGDA clearly considered transsexualism to be a mental disorder that could
benefit from counseling, hormone therapy, and surgery. In Standards of Care that
was published in 2001, however, HBIGDA hedged its bets regarding the “disease”
status of transsexualism. In their 2001 guideline booklet, the relevant section
was entitled “Are Gender Identity Disorders Mental Disorders”? Without
answering their own question they went on to state “To qualify’ as a mental
disorder, a behavioral pattern must result in a significant adaptive
disadvantage to the person and cause personal mental suffering” [26]. Debate
occurs because many persons who are diagnosed as transsexuals consider their
behaviors to be a significant advantage to their lives. Nevertheless, many
transsexuals do manifest signs of emotional distress and recommendations were
offered to assist the individual in appropriate transformation when warranted.
In the DSM-lV the diagnosis of Transsexualism was replaced by Gender Identity
Disorder [27].
The l970s also saw the increasingly frequent use of the new term
“transgender” which was coined by Virginia Prince. The term was meant to
describe persons like Prince who were heterosexual males who wanted to live as
women, at least part
time. The more common term for such people is “transvestite.” Prince also
intended that the term include females who chose to exhibit male behaviors and
dress. In Prince’s use, the term “transgender” specifically excluded
transsexuals because transgendered persons desired to change only their
behaviors, not their sex [28].
The term has been in a constant state of
flux, and, can, at present, seemingly cover any gender-bending or
gender-blending combination of masculine and feminine [29].
Among the more significant developments of this period was the formation of
different support groups for sexual and intersex situations. These primarily
were started by parents who sought information and help in understanding
intersexuality and to press for further research and improved treatment for
their children. Previously, medical management guidelines had not fostered the
meeting of such parents or their children with others who were similarly
involved. Often, such meetings were actively discouraged and secrecy was encouraged.
The first support groups to form were those for Turner’s syndrome in Canada in
1981 and in the United States in 1987; however, others followed soon after. The
Klinefelter’s syndrome support group in the United States and the Androgen
Insensitivity Syndrome group in the United Kingdom were formed in 1989. These
groups proved to be popular. In 1993, in the United States, Cheryl Chase, an
individual who had an intersex condition, founded the Intersex Society of North
America (ISNA). This organization developed into a highly vocal and visible
association. Another intersex support group, Bodies Like Ours, has since joined
in working on behalf of intersexual persons with any diagnosis. Support groups
for lesbians and gays also formed in the l970s and 1980s.
The current years: the 1990s to the present and
intersexuality
It is probably fair to say that
intersexuality—until the last 10 years or so—was a comparatively hidden medical
condition that was far from the public’s consciousness. The general public
often had a biased view of people who were then called “hermaphrodites.” Their
view oflen was drawn from circus sideshows and their displays of women who had
beards and men who had breasts. Physicians—when they met with intersexed
patients in their practice—often recalled their uniqueness in later casual
discussions. Without asking the patient’s permission, residents and medical
students were brought in frequently to observe the most private of
examinations. Without necessarily using the words, clinicians often told these
patients that they were oddities and so rare they would never meet another
person like themselves—worse, the patients were told not to try. Over the last
several years, in addition to media exposure, several books [23,30,31] and
popular writings [32—34] have brought the phenomenon out of the closet and more
intersexed individuals to the awareness of physicians.
Intersexed persons have a biologic/medical
condition that is not uncommon. It is a diagnosis that is shared by as many as
1% of the population.1 It has been estimated that in the United States, the
incidence of intersex conditions with ambiguous genitalia is about 1 in 2000; overall, when including those who have typical looking
genitalia the incidence approximates 1 in 100 [30]. Only those conditions that
are accompanied by ambiguous genitalia are detected routinely at birth.
Increased medical attention toward intersexuality started to shift in 1997.
Until then, the attitudes regarding intersex situations and the standards of
care for the management and treatment of individuals who had the conditions
were different from those that are available today. Drawing on the theory that
psychosexual development largely was a product of upbringing and genitalia that
were typical, those who cared for infants who had ambiguous genitalia tried to
benefit those children by “normalizing” their genitalia. Surgeons reduced
enlarged clitorides in infants who were assigned as females and because of the
technical difficulty of creating a functional and cosmetically believable set
of male genitals, refashioned the genitalia as female. This practice was
standard and was sanctioned by the American Academy of Pediatrics [35].
Since 1997, many of the issues that are associated with medical concerns of
the genitalia and the treatment of intersexuals have come under review and
management techniques have been altered. It is likely that facets of intersex
management will continue to occupy the attention of health care workers for
years to come.
Significance of John/Joan: the debate
Intersexuality and its management were
brought into focus, not by a case of intersexuality, but by a circumcision accident
and its follow-up. This story is now known by the pseudonyms John/Joan [34,36].
John’s penis was burned off accidentally in a circumcision that was done by
cautery. Following the accident, the decision was made to rear the child as a
girl, Joan. The decision was based on the belief that in the absence of a
functioning and adequate penis, normal male development was impossible.
Furthermore, it was believed that an individual was psychosexually neutral at
birth and a gender would be determined by rearing [9,37,38].
Following John’s sex reassignment, it was reported that the switch to life as
a girl was successful [10]. For physicians, this report was significant. On the belief that sex
reassignment was possible for a typical child, clinicians reasoned that it
could be suitable for the numerous individuals whose genitalia were ambiguous.
Physicians were advised “.
. .an intersexed baby with female-
appearing genitals should always be assigned as female” and “in the case of a
genetic male baby born with no penis at all. . .or with major hyperplasia of the penis, the baby should be assigned as
a girl” [39]. Aside from the theoretical view that psychosexual development
would be structured by rearing, there was the practically appealing matter that
it is easy to create a vagina if one is absent, but it is not possible to
create a satisfactory penis if the ‘phallus is absent or rudimentary. “The
decision to raise the child as a male centers around the potential for the
phallus to function adequately in later sexual relations” [40]. Pronouncements
such as these essentially established that, regardless of karyotype and
prenatal endocrine exposure and particular medical diagnosis, all intersex
conditions could be managed by cosmetic attention to the genitals and gender
assignment that usually was female.
________
1
Much depends upon how intersex is defined. The nanow definition holds that
intersex is a condition that is marked by genital ambiguity. Obvious cases are
those with congenital adrenal hypetplasia (CAH) or partial androgen
insensitivity syndrome (PAIS). A broad definition includes any individual whose
biology includes an identifiable mixture of male and female characteristics,
regardless of the appearance of the genitalia at birth. Examples here are
persons who have the complete androgen insensitivity syndrome or Klinefelter’s
syndrome.
Things changed in 1997 when an article appeared that detailed a follow-up to
the John/Joan case [36]. Instead of supporting the original claims that a
typical boy could have his gender successfully reassigned to that of a girl,
the new report documented the opposite. At the age of 14 years, despite being
reared as a girl and undergoing psychiatric counseling and an estrogen regimen
to reinforce a female identity, Joan reassigned himself to live as a boy. He
never had accepted his original gender reassignment. Other cases where the sex
reassignment of intersexed children was rejected also were reported [41,42].
These new findings, with their implications for general and typical gender
development, were reported immediately on the front page of the New York
limes and in the pages of other major popular and medical media.2
This case seemed to indicate that people were psychosexually biased and
predisposed at birth. The belief that one’s sexual identity could be modified
easily by rearing and that individuals were psychosexually neutral at birth
lost its footing and a dramatic shift in thinking about the management of
intersex conditions gained momentum. New principles of management for intersex
conditions were provided [43].
The most basic recommendation was that intersexed infants should be assigned
a gender that is not based on the appearance of the genitalia and chance of
good cosmetic surgery, but on a specific diagnosis of the exact condition and
the best prediction of the child’s future choice of identity. These new
principals of management for intersex conditions also recommended that any
cosmetic, nonmedically essential surgery should be postponed and that
intersexed children and adolescents should be allowed to make their own
________
2 The case
justified such coverage because the reported success of John’s reassignment to
Joan had become one of the most cited clinical cases, not only in medicine but
also in psychology, women’s studies, political science, and other disciplines,
decisions as to
how they want to live and be treated. Other recommendations were that male
infants who had a micropenis should be reared as boys, unless evidence for
managing them otherwise was presented. This had been successful in the past
[44]
and subsequently was found to be successful The secrecy that most often
was recommended to accompany genital surgery and sex reassignment was rejected.
Honesty and information was to be provided and it also was recommended that,
whenever possible, intersexed persons are put into contact with others who have
the same condition. It also was recommended that the child and parents be given
ongoing counseling.
In 1998, at the national meeting of the American Association of Pediatrics
(AAP), evidence was offered that their standards of care for intersex
management were on shaky ground; three strong recommendations were offered
[46,47]. These recommendations are applicable to psychiatrists as well as to
pediatricians.
Recommendation
1
“There should be a general
moratorium on sex assignment cosmetic surgery when it is done without the
consent of the patient.”
This recommendation did not infer that such surgery had no application;
however, no evidence had been presented that the surgery was beneficial. The
application for such surgery was based on anecdotes and some case reports, not
evidence-based medicine. Because there was no reported evidence for the
practice, and such evidence still remains elusive, the golden rule of medicine
seemed appropriate “First do no harm, Primum non nocere.”
Recommendation
2
“This moratorium should not be
lifted unless and until complete and comprehensive retrospective studies are
done and it is found that the outcomes of past interventions have been
positive.”
Because long-term follow-up studies on thc old protocols were lacking,
evidence must be gathered to justify the practices. Because so many procedures
had been done over the years, at least the records of those physicians and
surgeons who were still active should be examined. Part of the difficulty stems
from the fact that children do not become erotically active within the 6 months
or 1 year follow-up period that might follow infant surgery; erotic sexual
activities might not occur until puberty, adolescence, or later. Research must
inquire in detail about sensuality, orgasmic thresholds, identity and the like.
Simply asking if one is sexually active or sexually experienced—whatever that
could mean—or if one is dating or married is insufficient.
Future research may find that such operations and procedures are appropriate;
however, not having the evidence lends uncertainty to life features of dramatic
importance. These can range for one opting or being forced to live as a man or woman, and
surgery can preclude males from being fertile and procreating. Such procedures
can alter future medical conditions and situations. The negative cost of
ill-advised surgeries and sex reassignments can be high. It recently was
determined, for instance, that infant clitoral and vaginal surgery is
ill-advised. Among adolescent women who were studied who had these procedures,
41% felt that the cosmetic result was poor and 98% needed further treatment to
their genitals [48]. In a separate study, women who had clitoral surgery for an
intersex condition reported associated sexual problems. These were
characterized as “difficulties with sensuality,” “communication difficulties,”
“avoidance,” and lack of orgasm. This was in significant distinction from
comparable women who did not have such surgeries [49]. Creighton et al [50]
reported that “Most vaginal surgery can be deferred. . .Repeated clitoral surgery may be more damaging to
sexual function than a single procedure.... and that Children with mild
clitoromegaly should have surgery deferred until they are old enough to be
involved in the decision.”
Recommendation
3
“Efforts should be made to undo the
effects of past physician deception and secrecy.”
Often, parents and physicians had concealed aspects of surgery and treatment
from the child and excluded maturing children from medical management
decisions. Furthermore, secrecy had kept intersexed individuals isolated from
honest contact with their families, physicians, and others who had a similar
diagnosis. Typically, patients discover their condition from an inadvertent
family slip, community gossip, personal investigation into puzzling aspects of
their lives, or mix-ups at the doctor’s office; it is better for the physician
to initiate disclosure. Without openness, the patient discovers that his or her
condition is shameful in the minds of parents and doctors. They wonder why they
were not accepted and loved as they were and on what grounds it was decided
that they could not manage the information. Also, the patient learns that s/he
has been deceived since childhood by the people who should have been the most
trustworthy—parents and physicians. All of this is damaging. To the extent that
these children are misled, as they mature to adulthood they cannot act
rationally from a realistic appraisal of their medical condition.3
Following the San Francisco meeting of the AAP, matters regarding
intersexuality moved quickly. Many physicians have changed their practices. For
________
3 At the AAP
presentation, 1 introduccd Cheryl Chase to physicians who were involved with
intersex management, Until then, she and ISNA, using the name “Hermaphrodites
with Attitude,’ had met with professional rejection. The previous year, ISNA
had applied for an opportunity to address or meet with attendees at the AAP
conference in Boston and was refused. ISNA’s response was to picket in front of
the meeting hall [51]. Henceforth, Ms. Chase was accepted as a representative
of the intersex community.
others,
skepticism of the new ideas remained and surgery still was advocated [52];
subsequently, caution and awareness of potential problems was recognized [53].
Sheldon [54] wrote “Surprisingly little has been written on the psychosocial
outcome.. .We must completely inform the parents of such children regarding not
only the physical risks of surgery, but the psychosocial risks as well. . While I strongly disagree that a moratorium on
childhood genital reconstruction is in order.. .we should present this as an
option, continue to listen carefully to our patients, make a meaningful attempt
to study psychosocial adaptation and then alter our management accordingly.”
Others quickly argued for rethinking the old protocols [55,56].
The year 1998 was important for the
study of intersexuality for other reasons. Two significant publications
appeared: Kessler’s [23] Lessons from the Intersexed and a special issue
of the Journal of Clinical Ethics organized by Alice Dreger [57].
Kessler argued that the medical community was subjugating the intersexed
child’s needs, not to evidence but to maintaining existing practices and to
their social and cultural beliefs of gender. The Journal of Clinical Ethics issue
was devoted to ethical mailers that are related to intersexuality. This issue
also contained testimonies of intersexed persons who declared that they wanted
to be allowed to develop without surgery and to participate in any medical
decisions.
A 1998 report challenged the findings of the John/Joan case. Bradley et al
[58] reported on a case in which, like David, a circumcision accident resulted
in a normal boy losing his penis; like David, this boy was raised as a girl.
When questioned as an adult, this individual claimed to see herself as a woman.
She admitted that she was a tomboy as an adolescent and presently is
predominantly gynecophilic and considers herself ambisexual [59]. Other cases,
however, have reinforced the John/Joan findings [47,60].
The Texas Conference
One rapid result following the
publication of the follow-up to the John! Joan case and the presentation to the
pediatricians was a call for a conference to consider the implications of the
findings and the subsequent three recommendations. The conference was held in
Dallas, Texas in the spring of 1999.
From the Texas conference [61], two themes were reinforced: (1) more research
with long-term studies are needed and (2) patients should be as informed as
soon as possible as to their condition. A third theme re-emerged: the brain has
to be recognized as a sexual organ [62] and “Since the human brain is sexually
dimorphic, it is not always possible to predict whether the adult will be happy
with their gender 20 or 30 years after such a critical decision has been made
in the first days of life” [63]. A moratorium on infant surgery was considered
unrealistic, however; mostly because it was hypothesized that it would not be
accepted by parents [53].
Shift in stigma: atypical versus disorder
Intersex conditions are no longer
seen universally as disorders or errors of development but are increasingly
being seen as “variations” of life. This change occurred rapidly among
intersexuals themselves but is ongoing among the medical community. It is
advocated that intersexuality be considered and labeled with a more neutral
term and seen as a condition without stigma rather than as a disorder [43].
Humiliation and shame need not accompany and taint the medical or social
circumstances; humiliation and shame often have followed from intersexed
persons being treated as bizarre and with mendacity. This led many to seek
psychiatric care. Seeing intersexuality as a typical variation—rather than as a
stigmatizing condition—is an ongoing process but one that should prove easy for
clinicians to eventually adopt and foster.
Change in medical practices: standards
Standards of care for intersex
conditions have changed markedly. In 2000, the American Academy of Pediatrics
modified their standards in recognition of the new evidence [64]. Similarly, in
2001 the British Association of Pediatric Surgeons modified their standard of
care for intersexed children [65]. In these new guidelines, some concessions
were made to the three recommendations; however, neither the US nor the British
group accepted the idea of a surgical moratorium and neither group spoke to the
recommendation for call back to those families or individuals that had previous
treatment. Both groups recognized the need for more research on the topic and
greater candor and honesty when dealing with families and patients. The
recommendations from the United States and the United Kingdom pediatric
associations are not identical; they differ in some important ways. The
following are noteworthy differences:
The identification of ambiguous
genitalia should alert the staff that this is a social emergency (US); “While
there is likely to be continuing pressure from parents for early corrective
surgery, fully informed consent for such procedures would require them to be
aware of the possibility of non-operative management with psychological support
for the child and family” (UK).
All females who are virilized
because of congenital adrenal hyperplasia (CAH) or matemal androgens should,
because of their retained fertility, be raised as girls (US) “Assignment of
gender has to be on an individual basis, and the decision may need to include
cultural considerations (UK).”
Infants who are raised as girls
“will usually require clitoral reduction” (US); “There is a strong case for no
clitoral surgery in lesser degrees of clitoromegaly” (UK).
Boys who have partial androgen
insensitivity syndrome (AIS) “in whom a very small phallus mandates a female
sex of rearing” should have their testes removed (US). The risk of malignant
testicular changes in AIS is small (UK).
Both groups
recognized that the potential role of prenatal influences on subsequent
behavior need to be taken into account. They also caution that the sex of
rearing should differ from the chromosomal sex only after careful individual
consideration. What this means in practice is not stated.
Although in some ways these guidelines might be the current recommendations,
a host of publications and events have already appeared that will modify their
application. These publications and events are within and outside of medicine.
Developments in law, for instance, are moving quickly and probably will have an
influence on future management [66,67].
Legal considerations:
recent developments
In
1998, the Constitutional Court of
Colombia, South America ruled that sex reassignment of children would no longer
be legal in that country. The Court’s purported goal was “forcing parents to
put the child’s best interest ahead of their own fears and concerns about
sexual ambiguity” [68]. The Constitution guarantees free development of one’s
own personality, which implies a right to define one’s own sexual identity.
Early in 2000, a North American Task Force on Intersex was formed. With a
broad interdisciplinary board as consultants, their goal is to gather follow-up
data from clinics and physicians about their treatments and results regarding
intersex management [69]. In 2002, a meeting that was similar to the one held
in Dallas, Texas was held in Tempe, Arizona with hopes that sufficient new data
might be reported. Some new findings were gathered and presented; however, it
was acknowledged that anecdotal reports were still the norm.4
New data and good research have been slow to develop. Cases of individuals
who change gender have continued to appear in the literature [56,70,71]. At
the Phoenix meeting, the presentation by law professor, Julia Greenberg, drew
the most attention [72]. Her talk was on the legal aspects of gender assignment
and the problems that were attendant with then current practice. She indicated
that physicians might face legal liability if they continued as before; truly
informed consent is not yet possible and the needed research has
________
4 It has been
conjectured that part of the difficulty in obtaining data from past treatments
is that physicians would have to tell their patients aspects of their treatment
that they do not want to reveal or that the patients were not told originally.
For instance, researchers cannot easily question former patients on the effects
of surgery done in infancy when the patients had never been informed of the
surgery or that there had been other options or especially, the true reasons
for it.
not been done
[66,72].
Another potential problem might occur as a result of sexual
discrimination when XX and XY children are treated differently.
Findings about intersexuality of the last several years that are of psychiatric
relevance include (in rough chronologic order):
Slijper et al [73] reported cases
of general psychopathology, excluding problems with gender, in 39% of the
intersex cases that they reviewed. They specifically found that 13% of girls
exhibited gender disorder of childhood “with intense sadness and
dissatisfaction with the assigned sex and a preference for behavior appropriate
to other sex.” They also found that there was widespread ignorance of their
condition among patients who had AIS [74]. They reported that these mental and
counseling problems need attention.
Preves [75] reported on extensive
interviews with 37 randomly gathered intersexed adults who had various
diagnoses. “At the time of interview 24% of the sample presented as a gender
different from their sex of assignment and rearing; six were transitioning or
had transitioned from male to female, and three from female to male.”
Wisniewski et al [45] found that 5 of 18 patients who had congenital micropenis had been
reared as girls; 4 of 5 were dissatisfied with their genital appearance. Of
those who were reared as boys, 50% reported satisfaction with their genitalia. All claimed to be satisfied
with their sex of rearing. It is not clear, however, if these respondents were
informed that there was an option as to sex of rearing and offered their
response without this information.
Schober [76] reported that of 10
intersexed individuals she interviewed, 8 identify as “intersexual” rather than
as male or female; 9 have a homosexual orientation. Two of the 10 originally
who were assigned as girls are undergoing transformation to male [77].
Migeon et al [78] studied a select
population of men and women who have a 46,XY karyotype but different intersex
diagnoses. Five men (24%) and four women (22%) were dissatisfied with their sex
of rearing.
Migeon et al [79] found, in an
examination of a population of 46,XY individuals, that 66% of those living as
women and 38% of those living as men were satisfied with their knowledge of
their medical and surgical history.
Colapinto [34] reviewed Money’s
original thesis [80] and found that most of the intersex patients studied
seemed, at that time, to be making an adequately normal adjustment within the
sex of rearing without any cosmetic surgery. Money had written: “Far from
manifesting psychological traumas and mental illnesses, the study showed, the
majority of patients rose above their genital handicap and not only made an
‘adequate adjustment’ to life, but lived in a way virtually indistinguishable
from people without genital difference.” Thus, genital ambiguity, without
surgery or medical treatment, did not pose a hindrance to a satisfactory life. Had this been publicized, a great deal of
surgery could have been averted.
Reiner [59] found from following a
considerable number of children and adolescents who had cloacal exstrophy, that
a significant number of males rebelled against their sex reassignment as
female, and, without knowing their histories, declared themselves to be boys.
Where are we
now?
Many physicians who used to sex
reassign males who had traumatic early loss of their penis or were born with a
micropenis, are now more likely to believe that if there were no accompanying
intersex condition, such procedures would be a thing of the past [81]. The
evidence indicates that past infant cosmetic surgery may not succeed;
procedures exist that can provide a phallus for the affected boy should he
desire one [82,83]. Surgeons also are less likely to reduce an enlarged
clitoris in a girl who has CAH; many others are examining critically how the
new and old research regarding intersex treatment should be evaluated [84].
Increasingly, attention from medical ethicists is being called upon for their
considerations [85]. This is a marked step forward.
Two other gender-related matters pertain to psychologic management and deserve
discussion. One involves dealing with homosexuality. In individuals who are
diagnosed as intersexuals, considerations of sexual orientation exist at a
different level. This factor deserves discussion with the adolescent. A
parallel issue in management is how intersexed individuals are treated when,
and if, they present for gender reassignment. Patients often complain that they
are not given credit for their own feelings. The treatment that they receive is
similar to that of transsexuals when they seek reassignment; they feel as if
they are made to prove themselves.
Clinical advice: summary and final words
A review of some of the historic
findings and controversy regarding gender- related conditions was presented
with a major focus on intersex syndromes. This area is one of intense activity
and transformation. Change is rapid among the intersex populations themselves.
Many have come out of their closets in a way that was unheard of only a few
years ago and they operate with a new degree of activism. Instead of seeing
themselves as males or females, some are identiing openly as intersexed [76,86]
or are willing to mix and match gender and sex. Be open to what your young
patients tell you and help them probe their questions and doubts about
identities and preferred behaviors.
The medical communities also are changing markedly. Physicians should be
aware of the shifting attitudes and needs of their patients and be aware that
one’s sexual profile is complex and are not made up only of what gender typical or
atypical behaviors are manifest or what type of sexual orientation is
professed. One’s sexual profile is a constellation of a host of factors that
should be explored to get a fill appreciation of the patient’s feelings and
understandings [87,88]. This is not intrusive and usually will be welcomed by
those patients who want their psychiatrist to be able to truly understand them.
References
[1] Stoller RJ. Sex and gender: on the
development of masculinity and femininity. New York:
Science House; 1968.
[2] Hamberger C. Desire for change of sex as shown by personal letters from 465
men and women. Acta Endocrinol (Copenh) 1953;14:36l—75.
[3] Money J, Hampson JG, Hampson JL. Hermaphroditism: Recommendations
concerning assignment of sex, change of sex and psychological management. Bull
Johns Hopkins l-losp
1955;97:284—300.
[4] Maccoby EE, editor. The development of sex differences. Stanford (CA):
Stanford University Press; 1966.
[5] Fagot 131. Sex differences in toddlers’ behavior and parental reaction. Dcv
Psychol 1974;10:
554—8.
[6] Kagen i. Psychology of sex differences. In: Beach FA, editor. Human
sexuality in four perspectives. Baltimore (MD): The Johns Hopkins University
Press; 1976. p.87—lI4.
[7] Stoller RJ. A contribution to the study of Gender Identity. J Psychoanal
1964;45:220—6.
[8] Stoller RJ. Gender role change in intersexed patients. JAMA 1964;188:684—5.
[9] Money J, [lampson JG, Hampson JL. An examination of some basic sexual
concepts: the evidence of human hermaphroditism. Bull Johns Hopkins Hosp
1955;97:301—19.
[10] Money J. Ehrhardt A. Man & woman, boy & girl. Baltimore (MD): John
Hopkins University Press; 1972.
[11] Gagnon JH, Simon W. Sexual conduct: the social origins of human sexuality.
Chicago:
Aldine; 1973.
[12] Phoenix CH, Goy RW, GenII AA, Young WC. Organizing action of prenatally administered
testosterone propionate on the tissues mediating mating behavior in the female
guinea pig. Endocrinology 1959;65:369—82.
[13] Barraclough CA, Gorski RA. Studies on mating behavior in thc
androgen-sterilized female rat in relation to the hypothalamic regulation of
sexual behaviour. J Endocrinol 1962;25: 175—82.
[14] Armstrong CN. Intersexuality in man. In: Armstrong CN, Marshall AJ,
editors. Intersexuality in vertebrates including man. London: Academic Press;
1964. p. 349—93.
[15] Dewhurst CJ, Gordan RR. Change of sex. Lancet 1963;309: 1213—7.
[16] Young WC. The hormones and mating behavior. In: Young WC, editor. Sex and
internal secretions, vol.2. Baltimore (MD): The Williams & Wilkins Co.;
1961. p. 1173—239.
[17] Cappon D, Ezrin C, Lynes P. Psychosexual identification (psychogender) in
the intersexed. Can Psychiatr Assoc J 1959;4:90—106.
[18] Diamond M. A critical evaluation of the ontogeny of human sexual behavior.
Q Rev Biol
1965;40:147—75.
[19] Beach FA. Human sexuality in four perspectives. Baltimore (MD): The Johns
Hopkins University Press; 1976.
[20] Bowman KM. Engle B. The problem of homosexuality. J Soc Hyg
1953;39(l):I0—1.
[21] Bancroft J. Deviant sexual behavior modification and assessment. London:
Oxford University Press; 1974.
[22] Preves SE. For the sake of the children. In: Dreger AD, editor. Intersex
in the age of ethics. Hagerstown (MD): University Publishing Group; 1999. p.
50—65.
[23] Kessler SJ.
Lessons from the intersexed. New Brunswick (NJ): Rutgers University Press;
1998.
[24] Money J. Sex errors of the body. Baltimore (MD): The Johns Hopkins Press;
1968.
[25] Benjamin H. The transsexual phenomenon. New York: The Julian Press, Inc.;
1966.
[26] Harry Benjamin International Gender Dysphoria Association’s The Standard
of Care for Gender Identity Disorders. Dusseldorf (Germany): Symposium
Publishing; 2001.
[27] Diagnostic and Statistical Manual of Mental Disorders, Revised Fourth
Edition. Washington, DC: American Psychiatric Association; 2000.
[28] Bullough B. Bullough VL, Elias J, editors. Gender blending. Amherst (NY):
Prometheus Books; 1997.
[29] Diamond M. What’s in a name? some terms used in the
discussion of sex and gender. Transgender Tapestry 2003;102:18—21.
[30] Fausto-Sterling A. Sexing the body:
gender politics and the construction of sexuality. New York: Basic Books; 2000.
[31] Dreger A, editor. Intersex in the age of ethics. Hagerstown (Mm:
University Publishing Group;
1999.
[32] Anderson C. Tests on athletes can’t
always find line between males and females (chromosome tests used in
international athletics are oflen inconclusive and give false results).
Washington Post l992;l 15(Monday, Jan 6):A3.
[33] Kiernan L. In intersex cases, gender is a complex question: pink-and-blue
world giving way to subtler shades, Chicago Tribune 1999;(Sunday, June 20);l,9.
[34] Colapinto J. As nature made him: the boy who was raised as a girl. New
York: Harper Collins; 2000.
[35] Pediatrics. Timing of elective surgery on the genitalia of male children
with particular reference to the risks, benefits, and psychological effects of
surgery and anesthesia. Pediatrics
l996;97(4):590—4.
[36] Diamond M. Sigmundson HK. Sex reassignment at birth: long term review and
clinical implications. Arch Pediatr Adolesc Med 1997;lSt:298—304.
[37] Money J. Sex hormones and other variables in human eroticism. In: Young
WC, editor. Sex and internal secretions. 3rd edition. Baltimore (MD): Williams
and Wilkins; 1961. p. 1383—400.
[38] Money J. Cytogenetic and psychosexual incongruities with a note on space
form blindness. Am J
Psychiatry 1963;1l9:820—7.
[39] Money J. Sex assignment in
anatomically intersexed infants. In: Green R, editor. Human sexuality: a health
practitioner’s text. 2nd edition. Baltimore (MD): Williams & Wilkins; 1979.
p. 136—49.
[40] Duckett JW, Baskin LS. Genitoplasty for intersex anomalies. Fur J Pediatr
1993;l52(Suppl 2):
580—4.
[41] Ghabrial F, Girgis SM. Reorientation
of sex: report of two cases. Int J Fertil l962;7(3):249—58.
[42] Reiner WG. Case study: sex reassignment in a teenage girl. JAm Acad Child
Adolesc Psychiatry 1996;35(6):799
—803.
[43] Diamond M, Sigmundson HK. Management
of intersexuality: guidelines for dealing with persons with ambiguous
genitalia. Arch Pediatr Adolesc Med l997;l5l:l046—50.
[44] Reilly JM, Woodhouse CR1. Small penis and the male sexual role. .1 Urol
1989;142:569—72.
[45] Wisniewski AB, Migeon CJ, Gearhart iF, Rock JA, Berkovitz GD, Plotnick LP,
et al. Congenital micropenis: long-term medical, surgical and psychosexual
follow-up of individuals raised male or female. Horm Res 200156:3—11.
[46] Kipnis K. Diamond M. Pediatric ethics and the surgical assignment of sex.
J Clin Ethics 1998; 9:398
—4 10.
[47] Diamond M. Pediatric management of
ambiguous and traumatized genitalia. J Urol 1999; 162:
102 1—8.
[48] Creighton S. Surgery for intersex. JR
Soc Med 2001;94:218—20.
[49] Minto CL, Liao L-M, Woodhouse CR2, Ransley PG, Creighton SM. The effect of
clitoral surgery on sexual outcome in individuals who have intersex conditions
with “ambiguous genitalia” a cross-sectional study. Lancet
2003;361(9365):1252—7.
[50]
Creighton SM, Minto CL, Steele SJ.
Objective cosmetic and anatomical outcomes at adolescence of feminising surgery
for ambiguous genitalia done in childhood. Lancet 200t;358:124—S.
[51] ISNA. Hermaphrodites with attitude. Chrysalis: The Journal of
Transgressive Gender Identities 1996;2(5):l.
[52] Glassberg KI. The intersex infant: early gender assignment and surgical
reconstmction. J Pediatr Adolesc Gynecol t998;l 1:151 —4.
[53] Glassberg KI. Gender assignment and the pediatric urologist [editorial]. J
Urol 999; 161:
t308— 10.
[54] Sheldon CA. Functional, social and psychosexual adjustment after vaginal
reconstniction [editorial]. J Urol 1999;t62:186—9.
[55] Schober JM. A surgeon’s response to the intersex controversy. J Clin
Ethics 1998;9(4):393—7.
[56] Reiner WG. Assignment of sex in neonates with ambiguous genitalia. Curr
Opin Pediatr
1999;l1(4):363—5.
[57] Howe EG, editor. The Journal of Clinical Ethics 1998;9:337—430.
[58] Bradley SJ, Oliver GD, Chernick AR, Zucker K.J. Experiment of nurture:
ablatio penis at 2 months, sex reassignment at 7 months psychosexual follow-up
in young adulthood. Pediatrics l998;102:l—5.
[59] Zucker KJ. tntersexuality and gender identity differentiation. Annu Rev
Sex Res 1999;10:l —69.
[60] Reiner WG, Gearhart JP. Discordant sexual identity in some genetic males
with cloacal exstrophy assigned to female sex at birth. N Engi J Med
2004;350(4):333—41.
[61] Zderic SA, Canning DA, Can MC, Snyder KM editors. Pediatric gender
assignment: a critical reappraisal. New York: Plenum; 2002.
[62] Diamond M. Self-testing among transsexuals: a check on sexual identity. J
Psychol Human Sex 1996;8(3):61 —82.
[63] Zderic SA. Preface, In: Zderic SA, Canning DA, Can MC, Snyder HM, editors.
Pediatric gender assignment: a critical reappraisal. New York: Plenum; 2002. p.
ix—x.
[64] American Association of Pediatrics. Evaluation of the newborn with
developmental anomalies of the external genitalia. Pediatrics 2000;t06(l):
138—42.
[65] Rangecroft L, Brain C, Creighton 5, Di Ceglie D, Ogilvy-Stuart A, Malone P,
et al. Statement of the British Association of Paediaric Surgeons Working Party
on the Surgical Management of Children Born with Ambiguous Genitalia. 2001.
[66] Beh HG, Diamond M. An emerging ethical and medical dilemma: should
physicians perform sex assignment surgery on infants with ambiguous genitalia?
Mich J Send Law 2000;
7(1)1 —63.
[67] Greenberg JA. Defining male and female: intersexuality and the collision
between law and biology. Ariz Law Rev 1999;41(2):265—328.
[68] Greenberg JA, Chase C. Columbia high court limits surgery on intersexed
infants. Volume 2003. Available at: http://www.isna.org/colombial. Accessed
February 27. 2003.
[69] Chase C, Aaronson IA. North American Task Force on Intersex formed: seeks
broad interdisciplinary consensus on treatment. Available at:
http://isna.org/pr/pr02-23-00.html. Accessed September 18, 2003.
[70] Bimbacher R, Marberger M, Weissenbacher S. Schober E, Frisch H. Gender
identity reversal in an adolescent with mixed gonadal dysgenesis. J Pediatr
Endocrinol Metab 1999;12(5 Suppl 2):
687— 90.
[71] Phornphutkul C, Fausto-Sterling A, Gmppuso PA. Gender self-reassignment in
an XY adolescent female born with ambiguous genitalia. Pediatrics
2000;106(l):135—7.
[72] Greenberg J.A. Legal aspects of gender assignment. Presented at the
Hormonal and Genetic Basis of Sexual Differentiation Disorders Conference.
Tempe, Arizona, May 17—18, 2002.
[73] Slijper FME, Drop SLS, Molenaar JC, de Muink
Keizer-Schrama MPFS. Long-term psychological evaluation of intersex children. Arch
Sex Behav 1998;27(2):125—44.
[74] Slijper FME, Frets P. Boehmer AL, Drop SLS, Niermeijer MF. Androgen
insensitivity syndrome (AIS): emotional reactions of parents and adult patients
to the clinical diagnosis of
AIS and its
confirmation by androgen receptor gene mutation analysis. Horm Res 2000;
53(l):9—15.
[75] Preves SE. Negotiating the constraints of gender binarism: intersexuals’
challenge to gender categorization. Curt Soc 2000;48(3):27—50.
[76] Schober JM. Sexual behaviors, sexual orientation and gender identity in
adult intersexuals:
a pilot study. J Urol 2001;165(6):2350—3.
[77] Schober JM, Reply to Zucker re: sexual hehaviors, sexual orientation and
gender identity in adult intersexuals: a pilot study. J Urol 2002;168:1508.
[78] Migeon CJ, Wisniewski AB, Gearhart JP, Meyer-Bahlburg HFL, Rock JA, Brown
TR, et al. Ambiguous genitalia with perineoscrotal hypospadias in 46,XY
individuals: long-term medical, surgical, and psychosexual outcome. Pediatrics
2002;lI0(3):6t6—21.
[79] Migeon CJ, Wisniewski AB, Brown TR, Rock JA, Meyer-Bahlburg HFL, Money J,
et al. 46,XY intersex individuals: phenotypic and etiologic classification,
knowledge of condition, and satisfaction with knowledge in adulthood.
Pediatrics 2002;1 10(3):32.
[80] Money 3. Heimaphroditism: an inquiry into the nature of a human paradox
[research]. Cambridge (MA): Harvard University; 1951.
[81] Bin-Abbas B, Conte FA, Grumbach MM, Kaplan SL. Congenital hypogonadotropic
hypogonadism and micropenis: Effect of testosterone treatment on adult penile
size—why sex reversal is not indicated, J Pediatr t999;t34:579—83.
[82] Ochoa 13. Trauma of the external genitalia in children: amputation of the
penis and emasculation. 3 Urol t998;160(3 Part 2):1116—9.
[83] Jordan GH. Total phallic constmction, option to gender assignment. In:
Zderic SA, Canning DA, Can MC, Snyder HM, editors. Pediatric gender assignment:
a critical reappraisal. New York:
Plenum; 2002. p. 275—82.
[84] Berenbaum SA. Management of children with intersex conditions:
pathological and methodological perspectives. Growth, Genetics & Hormones
2003;19:l —6.
[85] McCullough L. A framework for the ethically justified clinical management
of intersex conditions. In: Zderic SA, Canning DA, Cart MC, Snyder HM, editors.
Pediatric gender assignment: a critical reappraisal. New York: Kluwer
Academic/Plenum; 2002. p. 149—73.
[86] Butler 3. Groom’s intersex quandary. The West Australian Saturday 2002;5.
[87] Diamond M. Some genetic considerations in the development of sexual
orientation. In:
Haug M, Whalen RE, Aron C, Olsen KL, editors. The development of sex
differences and similarities in behaviour, vol. 73. NATO ASI Series. Dordrecht
(The Netherlands): Kluwer Academic Publishers; 1993. p. 291 —309.
[88] Diamond M. Biological aspects of sexual orientation and identity. In:
Diamant L, McAnulty R, editors. The psychology of sexual orientation, behavior
and identity: a handbook. Westport (CT):
Greenwood Press; 1995. p. 45—80.