Abstract
This
report is a long term follow-up to a "classic" case in the pediatric,
psychiatry, and sexology literature. In this case an XY individual had
his penis accidentally ablated and was subsequently raised as a female.
The initial reports were that this individual was developing into a
normally functioning female. The present findings show the individual
did not accept this sex of rearing. At puberty this individual switched
to living as a male and has successfully lived as such from that time
to the present. The significant factors in this switch are presented.
In instances of extensive penile damage to infants it is standard to
recommend rearing the male as a female. Subsequent cases should,
however, be managed in light of this new evidence.
Text
Among
the more difficult decisions physicians have to make involve cases of
ambiguous genitalia or significantly traumatized genitalia. The
decision as to how to proceed typically follows the following
contemporary advice: "The decision to raise the child as a male centers
around the potential for the phallus to function adequately in later
sexual relations (pp. 580)."1 and "Because it
is simpler to construct a vagina than a satisfactory penis, only the
infant with a phallus of adequate size should be considered for a male
gender assignment (pp. 1955)."2 These
management proposals depend upon a theory which basically says: "It is
easier to make a good vagina than a good penis and since the identity
of the child will reflect upbringing, and the absence of an adequate
penis would be psychosexually devastating, fashion the perineum into a
normal looking vulva and vagina and raise the individual as a girl."
Such clinical advice, concerned primarily with surgical potentials, is
relatively standard in medical texts 3, 4, 5, 6 and reflects the current thinking of many pediatricians7 .
This
management philosophy is based on two pediatric beliefs held strongly
enough that they might be considered postulates: 1) individuals are
psychosexually neutral at birth, and 2) healthy psychosexual
development is dependent upon the appearance of the genitals. These
ideas arise most strongly from the original work of Money and
colleagues.8, 9, 10, 11, 12
The following are typical pronouncements from that research: "...
erotic outlook and orientation is an autonomous psychological
phenomenon independent of genes and hormones, and moreover, a permanent
and ineradicable one as well (pp. 1397)."9; "It
is more reasonable to suppose simply that, like hermaphrodites, all the
human race follow the same pattern, namely, of psychological
undifferentiation at birth."10 The first
postulate was derived, not from normal individuals but from
hermaphrodites and pseudohermaphrodites and the second postulate had
only anecdotal support. Money no longer holds such extreme views13
but his involvement in one particular case was significant enough that
it became a totem in the lay press and a classic for the academic and
medical community. And, as quoted above, the textbooks have not kept
abreast of the new thinking.
Report of a Patient:
The
case involved a set of normal XY twins, one of whom, John (pseudonym),
at seven months of age had his penis accidentally burned to ablation
during phimosis repair by cautery.11 After a
great deal of debate, the child was seen for consultation at The Johns
Hopkins Hospital and, following the two postulates mentioned above, the
recommendation was made to unequivocally raise the child as a girl,
Joan (pseudonym). Orchiectomy and preliminary surgery to fashion a
vagina followed within the year to facilitate feminization. Further
surgery was to wait until Joan was older. This management was monitored
and reinforced with yearly visits to Hopkins. The treatment was
reported as developing successfully and that John was accepting life as
Joan11
"Although this
girl is not yet a woman, her record to date offers convincing evidence
that the gender identity gate is open at birth for a normal child no
less than for one born with unfinished sex organs or one who was
prenataly over-or underexposed to androgen, and that it stays open at
least for something over a year after birth (pp. 98)."12
"The
girl's subsequent history proves how well all three of them [parents
and child] succeeded in adjusting to that decision (pp. 95)."12
The
effect of such reports were widespread for theory and practice.
Sociology, psychology and women's study texts were rewritten to argue
that, as Time magazine (Jan 8, 1973) reported: "This dramatic case . .
. provides strong support . . . that conventional patterns of masculine
and feminine behavior can be altered. It also casts doubt on the theory
that major sex differences, psychological as well as anatomical, are
immutably set by the genes at conception." Lay and social science
writings still echo this case and so do medical texts.3, 4, 5, 6, 14.
The following quote is typical: "The choice of gender should be based
on the infant's anatomy...Often it is wiser to rear a genetic male as a
female. It is relatively easy to create a vagina if one is absent, but
it is not possible to create a really satisfactory penis if the phallus
is absent or rudimentary. Only those males with a phallus of adequate
size which will respond to testosterone at adolescence should be
considered for male rearing. Otherwise, the baby should be reared as a
female."15
Our current
report is in contrast to those reports and advice. It is based on a
review of the medical clinical notes and impressions of therapists
originally involved with the case and on contemporary interviews. One
of us (H.K.S.) was the Head of the Psychiatry Department to which the
case was referred in the patients home area. While the patient was
assigned to the immediate care of female psychiatrists to foster female
identification and role modeling, H.K.S. maintained direct supervisory
control of the case. The unique character of this case attracted the
attention of the British Broadcasting Co. and they invited M.D. as a
consultant.16 In 1994 and 1995, we
collaboratively reinterviewed and recorded John, his mother and his
wife to provide updated accounts of his progress. Findings are listed
in general chronological order under the more appropriate of the two
postulates for pediatric sexual assignment. John himself, while
desiring to remain anonymous, strongly desires his case history be made
available to the medical community to reduce the likelihood of others
having his psychic trauma.
Postulate 1: Individuals are psychosexually neutral at birth.
Mother
recalls: "As soon as he had the surgery, the doctor said I should now
start treating him as a girl, doing girl things and putting him in
girl's clothes. But that was a disaster. I put this beautiful little
dress on him, . . . and he [immediately tried] to rip it off. . . I
think he knew it was a dress and that it was for girls and he wasn't a
girl."
On the other hand, Joan could act quite
feminine when she wanted to, and was reported as doing so, e.g. mother
was quoted to have said: "One thing that really amazes me is that she
is so feminine. I've never seen a little girl so neat and tidy as she
can be when she wants to be. . . (pp. 119)."11
However, she most often, would prefer to reject such behavior. It was
also more common that she, much more than the twin brother, would mimic
Father. One incident Mother related was typical: When the twins were
about 4 or 5 they were watching their parents. Father was shaving and
Mother applying makeup. Joan applied shaving cream and pretended to
shave. When Joan was corrected and told to put on lipstick and makeup
like Mother, Joan said: "No, I don't want no makeup, I want to shave."
Girl's
toys, clothes and activities were repeatedly proffered to Joan and most
often rejected. Throughout childhood Joan preferred boy's activities
and games to those of girl's; she had little interest in dolls, sewing
or girl's activities. Ignoring the toys she was given, she would play
with her brother's toys. She preferred to tinker with gadgets and tools
and dress up in men's clothing; take things apart to see what makes
them tick. She was regarded as a tomboy with an interest in playing
soldier. Joan did not shun rough and tumble sports nor avoid fights.
John recalls of Joan at the age of 12 or 13 wanting an umbrella:
"I
had a couple of bucks and went to the store to take a look at the
umbrellas, and right beside the umbrellas was the toy section. I
started to eyeball a machine gun. I said to myself 'Do I have enough
money for that?'. . . I put the gun on the counter and asked the clerk
if I had enough money. She had that look like 'You don't have enough
but we'll let you go anyway.' I used it to play army with my brother".
Brother often refused to let Joan play with his toys, so she also saved
her allowance money and bought a truck of her own.
Joan's realization that she was not a girl jelled between ages 9 and 11 years. John relates:
"There
were little things from early on. I began to see how different I felt
and was, from what I was supposed to be. But I didn't know what it
meant. I thought I was a freak or something; . . . I looked at myself
and said I don't like this type of clothing, I don't like the types of
toys I was always being given, I like hanging around with the guys and
climbing trees and stuff like that and girls don't like any of that
stuff. I looked in the mirror and sees my shoulders are so wide, I mean
there is nothing feminine about me. I'm skinny, but other than that,
nothing. But that is how I figured it out. [I figured I was a guy] but
I didn't want to admit it, I figured I didn't want to wind up opening a
can of worms."
Joan
knew she already had thoughts of suicide brought on by this sort of
cognitive dissonance and didn't want additional stress. Joan fought
both the boys as well as the girls who were always "razzing" her about
her boy looks and her girl clothes. She had no friends; no one would
play with her. "Every day I was picked on, every day I was teased,
every day I was threatened. I said enough is enough . . ." Mother
relates that Joan was good looking as a girl. But it was "When he
started moving or talking, that gave him away and the awkwardness and
incongruities became apparent."
The other
girls teased Joan so aggressively that she felt forceful retaliation
was called for. One girl sat behind Joan and continued to hit her.
"[John demonstrating] I grabbed her like that, by the shirt, and rammed
her round the wall like this, threw her on the ground. . . until the
teacher grabbed me." This resulted in Joan being expelled from school.
Despite
the absence of a penis, Joan often tried to stand to urinate. This made
a mess as it was difficult to direct the urine stream. While she
learned to sit and void she would nevertheless continue to occasionally
stand and urinate. Despite admonitions against the behavior and the
untidiness, Joan persisted to such an extent that, at school, she was
caught standing to urinate in the girls' bathroom sufficiently often
that the other girls refused to allow her entrance. Mother recalls the
other girls threatening to "kill" her if she persisted. Joan would also
go to the boy's lavatory to urinate.
Joan was
put on an estrogen regimen from the age of 12 years but rebelled
against taking the hormones. They made her "feel funny" and she didn't
want to feminize. She would often dispose of her daily dose. She
unhappily developed breasts but wouldn't wear a bra. Things came to a
head at the age of 14. In discussing her breast development with her
endocrinologist she confessed "I suspected I was a boy since the second
grade". The physician, who personally believed Joan should continue to
take her estrogens and proceed as a girl, used that opening to explore
in a nonjudgmental manner, the possible male or female paths available
and what either one would mean. Since the local management team had
already noticed Joan's preference for boys activities and refusal to
accept female status and they had discussed among themselves the
possibility of accepting Joan's change back to male, the
endocrinologist explored Joan's options with her. Shortly thereafter,
at age 14 years, Joan decided to switch to living as a male.
Joan
was the daily butt of her peer's jibes and the local therapists, having
knowledge of her previous suicidal thoughts, went along with the idea
of sex re-reassignment. In a tearful episode following John's prodding,
his father told him of the history of what had transpired and why. John
recalls: "All of a sudden everything clicked. For the first time things
made sense and I understood who and what I was."
John
requested "male hormone" shots and gladly took these. He requested a
mastectomy and phalloplasty The mastectomy was completed at the age of
14 years; surgeries for phallus construction were at age 15 and 16
years. After the surgical procedures, John adjusted well. As a boy he
was relatively well accepted and popular with both boys and girls. At
16 years, to attract girls, John obtained a windowless van with a bed
and bar. Girls, who as a group, had been teasing Joan, now began to
have a crush on John. When occasions for sexual encounters arose,
however, he was reluctant to move erotically. When he told one girl
friend why he was hesitant --that he was insecure with his penis-- she
gossiped at school and this hurt John very much. Nevertheless, his
peers quickly rallied round and he was accepted and the girl rejected.
John's
life subsequently was not unlike other boys with an occult physical
handicap. Subsequent to his return to male living he felt his
attitudes, behaviors and body were in concert; they weren't when living
as a girl. At the age of 25 he married a women several years his senior
and adopted her children.
Postulate 2: Healthy psychosexual development is intimately related to the appearance of the genitals.
First
in Baltimore, and then with the local therapists prior to the sex
reassignment, Joan's expressed feelings of not being a girl would draw
ridicule and she would be told something like: "All girls think such
things when they are growing up". He recalls thinking: "You can't argue
with a bunch of doctors in white coats; you're just a little kid and
their minds are already made up. They didn't want to listen." To ease
pressures to act as a girl, Joan would often not argue or fight the
assignment and would "go along".
Starting at
age 7 years, Joan began to rebel at going for the consultations at the
Johns Hopkins Hospital. Her reason was discomfort and embarrassment
with forced exposure of her genitals and constant attempts,
particularly after the age of 8 years, to convince her to behave more
as a girl and accept further vaginal repair. This was always strongly
resisted and led to recurrent confrontations. To encourage the visits
and temper Joan's reluctance to travel to the consultants, they were
combined with vacation trips.
In Baltimore,
the consultants enlisted male-to-female transsexuals to convince Joan
of the advantages of being female and having a vagina constructed. She
was so disturbing by this that in one instance Joan, at age 13 years,
ran away from the hospital. She was found hiding on the roof of a
nearby building. After age 14 years, Joan adamantly refused to return
to the hospital. Joan then came instead fully under the care of local
clinicians. This group consisted of several pediatricians, 2 pediatric
surgeons, an endocrinologist, and a team of psychiatrists.
John
recalls thinking, from preschool through elementary school, physicians
were more concerned with the appearance of Joan's genitals than was
Joan. Her genitals were inspected at each visit to The Johns Hopkins
Hospital. She thought they were making a big issue out of nothing and
they gave no reason to think otherwise. John recalls thinking: "Leave
me be and then I'll be fine . . . Its bizarre. My genitals are not
bothering me, I don't know why its bothering you guys so much."
When
asked what Joan thought of her genitals as a youngster, John replied "I
didn't really have anything to compare myself against other than my
brother when we were taking a bath." Mother confirmed that as a devout
family in a very conservative religious community there would be few
opportunities for the twins to have seen anyone else's genitals. Nudity
was never acceptable. At their yearly visit to The JohnsHopkins
Hospital the twins were made to stand naked for inspection by groups of
clinicians and to inspect each other's genitalia. This experience, in
itself, was recalled with strong negative emotions. John's brother,
decades later, recalls the experience with tears.
John
recalls frustration, which remains, at not having his feelings and
desires recognized. Without consideration of genitals, with the obvious
absence of a penis, he nevertheless knew he was not a girl. When he
tried to express such thoughts: the doctors "would change the subject
whenever I tried to tell [doctor] how I felt. [He] didn't want to hear
what I had to say but wanted to tell me how I should feel." Clinical
notes from the time report he felt "like a trapped animal".
In
middle school Joan had a very difficult time making friends. Her
clothes and demeanor, to her peers, did not jibe. Because of her
behaviors they teased her with names like: "caveman" and "gorilla". Few
children would play with her. None of Joan's peers knew anything of her
genitals.
At first the local physicians, as
did Joan's parents, continued to reinforce her feminine identity as
suggested by the consultants and continued to treat Joan as a girl
preparing her for vaginal reconstructive surgery and life as a female.
Psychotherapy, primarily by female therapists, aimed to reinforce her
female identity and redirect her male ideation. This became
increasingly difficult due to Joan's growing insistence she did not see
herself as a girl and anger at being treated as one. John recalls:
"They kept making me feel as if I was a freak."
John
knew what the clinicians wanted and recognized it wasn't what he
wanted. Starting at age 14, against the recommendations of the
clinicians and family, and without yet knowing of the original XY
status, Joan, as much as possible, refused to live as a girl. Jeans and
shirt, due to their gender neutral status, were her usual preferred
clothes; boys games and pursuits her usual activities. Joan's daytime
fantasies and night dreams during elementary school involved seeing
herself "as this big guy, lots of muscles and a slick car and have all
kinds of friends. . ." She aspired to be a mechanic. She rejected
requests to look at pictures of nude females he was supposed to
emulate. Rorschach and Thematic Apperception Tests at the time elicited
responses more typical of a boy than a girl. Her adamant rejection of
female living and her improved demeanor and disposition when acting as
a boy, convinced the local therapists of the correctness of sexual
re-reassignment.
Following the surgery for
penile construction there was difficulty with urethral closure and
despite repeated attempts at repair, that problem was never rectified.
John now urinates through a fistula at the base of his penis while
sitting down. Much of the penis is without sensation as are the areas
of scaring from where the grafts were taken.
John's
first sexual partner was a girl. He was 18 years old. While living as a
girl and afterward as a boy, John was approached sexually by males. He
claims never to have been attracted to any and his responses to such
questions were matter-of-fact and not homophobic. John thinks his first
recognizable sexual interest occurred about the age of 17 or 18 years
although he does recall wanting to go see the "sexy" Rockettes in New
York on one of his trips to see the consultants.
Coitus
is "occasional" with his wife. This frequency is sufficient for his
needs but is less than his wife would desire. They mostly pleasure each
other with a great deal of physical affection and mutual masturbation.
John can have coital orgasm with ejaculation.
John
recalls thinking it was small minded of others to think all his
personality was summed up in the presence or absence of a penis. He
expressed it thus: "Doctor . . . said, it's gonna be tough, you're
going to be picked on, you're gonna be very alone, you're not gonna
find anybody unless you have vaginal surgery and live as a female. And
I thought to myself, you know I wasn't very old at the time but it
dawned on me that these people gotta be pretty shallow if that's the
only thing they think I've got going for me; that the only reason why
people get married and have children and have a productive life is
because of what they have between their legs." ... "If that's all they
think of me, that they justify my worth by what I have between my legs,
then I gotta be a complete loser."
General comments:
As
an adult, John was asked "Why not accept being a female rather than
fighting it?" His answer was simple. Basically he wanted to please his
parents and placate the doctors so he often went along. But doing so
didn't feel right and the confusion between his feelings and theirs he
saw was mentally devastating and would lead to suicide if he were
forced to continue. The most often voiced and deeply felt emotion
expressed by Joan was always feeling different from what was expected
or desired by others. At first, as a toddler, the feeling of being
different was relatively amorphous. Then, even as a preschooler, it
shifted to clearly being different from girls. And later, in elementary
school, he began to not only feel different from girls but similar to
the group called "boys". Certainly having a twin might have made this
comparison much easier. Such a progression in thinking is common for
atypical individuals such as homosexual males and females17 and hermaphroditic individuals or those with ambiguous genitalia18.
The
transition was gradual. When Joan thought she might really be a boy,
instead of the girl her parents and the doctors told her she was, the
psychic discord greatly frightened her even though she had suspected
such was true since second grade. When finally told the truth, she was
relieved since her feelings now made sense. John's anger at not being
told the truth from the beginning persists.
Following
John's sex re-reassignment, the family decided to disregard the
clinical recommendation to move from their family home. Instead they
stayed and were open about the change. Aside from the financial
concerns, the parents decided the word would get out anyway. This
strategy seemed to work and John was accepted in a way that Joan never
was.
John was given testosterone following his
return to male status. As is typical of many teen-age boys, John began
to work out with weights. He blossomed into an attractive muscular
young man.
According to John's wife: "Before
he came along I was a lot tougher on the kids because I had to be.
[Now] John is the real hard one and I am the soft one. ... There is no
doubt who wears the pants in this family."
John
is a mature and forward looking man with a keen sense of humor and
balance. While still bitter over his experience, he philosophically
accepts what happened and is trying to make the most of it with support
from his wife, parents and family. He has job satisfaction and is
generally self-assured.
COMMENT
Long
term follow-ups of case reports are unusual but often crucial. This
up-date to a case originally accepted as a "classic" in fields ranging
from medicine to the humanities completely reverses the conclusions and
theory behind the original reports. Cases of infant sex reassignment
require inspection and review after puberty; 5 and even 10 year post
sex reassignment follow-ups are still insufficient.
Possibly the initial impressions of the consultants 11, 12
were appropriate at the time and Joan's behavior shifted with
development. However, it appears from clinical notes and impressions of
the local physician's at the time, and John's contemporary
recollections, that at no time was he fully accepting the sex
reassignment as a girl. Indeed, the local physicians expressed their
reservations early on16. When it became
obvious, however, that the original management program of maintaining
this male as a girl was no longer tenable and proving psychologically
damaging --even life threatening-- they revised their thinking.
It
is also possible that interpretations from the early years were
mistaken since it is often difficult to see results not in keeping with
one's hypotheses and management plans. Behavioral manifestations of
typical boy behaviors would thus repeatedly be interpreted as
tomboyish. This was true regardless if this was in terms of preferred
activities, games, toys or clothing. Significantly, the conclusions
that hermaphrodites and pseudohermaphrodites offer a model for normal
development had been challenged before.19-24.
The implications of such challenges does not seem to have been accepted
or integrated by the majority of pediatricians or surgeons.7
John was repeatedly admonished for behaving like a boy. Such management is in keeping with the belief25-28,
that any acquiescence to doubt expressed by the patient would decrease
the likelihood of an eventual successful outcome. It is circular
reasoning to contend that Joan did not accept the imposed sex of
rearing because of ambiguity in treatment. There is no evidence of such
and the initial reports held that the rearing was appropriate11, 12.
It is known, particularly from transsexuals, that casting doubt as to
sexual identity usually forces greater introspection and security
regarding a preferred life's direction even though counter to
upbringing, parent's wishes, social and cultural norms and may result
in less than adequate genitalia.29, 30
In
the case under consideration, the initial management protocol was
predicated on postulates which saw successful treatment involving
attention to a male's self-image supposedly dependent upon the adequacy
of a functional penis. While it can be accepted that such adequacy is
important there is no body of data establishing its centrality.
Other
considerations are in order. Gender reassignment as proposed for John,
and the postulates upon which they are based, assume the individual
will learn to accept rearing-appropriate sex-typical behaviors,
particularly when the genitals are at issue. These situations range
from the urinary to erotic to narcissistic. Such behaviors, while
important, are only one aspect of an individual's total sexuality. An
individual's sexual profile is comprised of at least five levels:
gender Patterns, Reproduction, sexual Identity, Mechanisms and sexual
Orientation (PRIMO).31, 32
The
sex reassignment of John to Joan only attended to the gender patterns,
and gender roles to which he would be subject with the expectations his
identity and other levels would follow. Joan did indeed become aware of
the social expectations concomitant with the female gender but these
were not in keeping with those with which he felt comfortable. Standing
to urinate, despite its housekeeping and social consequences is a
dramatic display of preference. The sex reassignment thus obviously
failed in the area in which it was most designed to succeed.
But
it failed in the other four levels as well. The contrast between the
female gender-typical behaviors the child was being asked to accept and
his inner directed behavior preferences presented a discordance that
demanded resolution. Joan's analysis of the situation was that she best
fit in, not as a girl but as a boy. Thus, despite her upbringing,
Joan's sexual identity developed as a male. Sex reassignment also
obviously went against Joan's or John's reproductive character.
Castration removed any reproductive capacity. Certainly unaware of this
as a child, John very much resents this now and decries this loss.
Castration also removed the androgen source for sex-typical mechanisms
of sexual arousal and other physiological processes. His ability to
ejaculate returned with androgen treatment. The castration and surgical
scaring, however, has dramatically reduced erotic sensitivity to the
perineum and subsequently reduced this option. And significantly, as
many studies strongly indicate, sexual orientation is prenataly
organized or at least predisposed.33-40 The sex
reassignment did nothing to effect sexual orientation. Joan remained
totally gynecophilic despite being reared as a girl.
Comments
from John's parents reveals another important consideration. With a sex
reassignment they are asked to make a dramatic psychological adjustment
in how to rear an otherwise normal child. Mother herself required
psychiatric treatment to help manage her feelings in this regard. The
penile ablation, not withstanding, they were more comfortable dealing
with their child's original sex and the accident than with a reassigned
sex. Although they had definitely tried to make a success of the
original sex reassignment, they were very supportive --while guilt
ridden-- of Joan's reversion to John.
This
last decade has offered much support for a biological substrate for
sexual behavior. In addition to the genetic research mentioned above
there are many neurological and other reports which point in this
direction.31, 32, 41-56
The evidence seems overwhelming that normal humans are not
psychosexually neutral at birth but are, in keeping with their
mammalian heritage, predisposed and biased to interact with the
environment, familial and social forces, in a male or female mode. This
"classic" case demonstrates this dramatically. And the fact that this
was particularly expressed at puberty --a critical period-- is logical
and has been predicted.20, 44.
Although
this report is of the classic case of sex reassignment so often cited
in the literature, follow-ups to other related cases are available.
Reilly and Woodhouse57 , described20
patients with micropenis reared as boys and none having any doubt as to
the correctness of assignment as males. And many reports exist where,
despite the absence of a normal penis19, 32, 58-62,
males were originally reassigned as girls, that switched back, and
successfully lived as males. Several of these cases offer the same
sorts of findings presented by us; with great similarity at the ages at
which various milestones were passed, feelings developed and their
reassignment challenged59, 60 A most recent case illustrates this.
Reiner63
reported on an adolescent Hmong immigrant who precipitously dropped out
of school at 14 years of age. Upon subsequent interview she declared,
although having been unequivocally raised as a girl from birth "I am
not a girl, I am a boy". Indeed, physical examination revealed a 46
chromosome, XY male with mixed gonadal dysgenesis with a female
appearing pelvis with clitoral hypertrophy. All her school-age friends
had been boys. She enjoyed rough and tumble play, avoided dolls and
girls activities and would dress in a gender-neutral or boys way. Her
feelings of being different --being a boy-- developed from about the
age of 8 and came to a head at 14 years. Treatment involved surgery and
endocrine therapy. This individual, after a period of some depression,
progressively developed into a gynecophilic sexually active male.
These
cases of successful gender change, as well as the present one, also
challenge the belief that such a switch after the age of 2 years will
be devastating. Indeed, in these cases it was salutary.
It must be acknowledged that cases of males accepting life as females after the descruction of their penises has been reported64 These reports, however, do not detail the individuals' sexual or personal lives.
Conclusions
Considering
this case follow-up, and as far as an extensive literature review can
attest, there is no known case where a 46 chromosome, XY male,
unequivocally so at birth, has ever easily and fully accepted an
imposed life as an androphilic female regardless of the physical and
medical intervention. True, surgical reconstruction of traumatized male
or ambiguous genitalia to that of a female, and attendant sex
reassignment of males is mechanically easier than constructing a penis
but there might be an unacceptable psychic price to pay. Concomitantly
there is no support for the postulates that individuals are
psychosexually neutral at birth or that healthy psychosexual
development is dependent upon the appearance of the genitals. Certainly
long term follow up on other cases is needed.
In
the interim, however, new guidelines are offered. We believe that any
46-chromosome, XY individual born with a normal nervous system, in
keeping with the psychosexual bias thus prenataly imposed, should be
raised up as a male. Surgery to repair any genital problem, while
difficult, should be conducted in keeping with this paradigm. This
decision is not simple7, 13, 18, 63, 65-67. and analysis should continue.
As
parents will still want their children to be and look normal as soon
after birth as possible, physicians will have to provide the best
advice and care, consistent with present knowledge. We suggest this
means referring the parents and child to appropriate and periodic long
term counseling rather than immediate surgery and sex reassignment just
because that seems a simpler immediate solution to a complicated
problem. With this management, a male's predisposition to act as a boy
and his actual behaviors will be reinforced in daily interactions on
all sexual levels and his fertility preserved. Social difficulties may
reveal themselves as puberty is experienced, however, there is no
evidence that with proper counseling and surgical repair when best
indicated, an adjustment will not be managed as teen-agers manage other
severe handicaps. Future reports will determine if we are correct.
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