Medical Management of Intersex Infants and Children

The medical management of Intersex infants and children was nearly a set in stone set of protocols for the last half of the 20th Century. The birth of a child with ambiguous genitalia was considered a medical and social emergency. Within a very short period of time a sex was assigned to the infant and plans were being made to perform surgery to normalize the genitals and match them to the assigned gender.

Once an assignment had been made and surgery performed, appropriate hormone replacement therapies would be planned and instigated. Parents were advised to accept their child as assigned and not to speak about the circumstances of the birth.

Thus was a population of unhappy Intersex born and raised in a veil of shame and secrecy that is only recently coming to the public's attention. These people have come forward to speak out for the rights of those youngsters now facing the fate they've lived through and deplored. An overwhelming call for immediate change in the medical management of Intersex people is a controversial and emotional issue which needs to be resolved quickly. The fates of roughly 1000 babies a year hang in the balance.

Public awareness and education will be vital in the future management of these children. Surgical intervention should be postponed and counseling provided for families facing the many issues involved in cases such as these. To better understand what's at stake, some history on Intersex follows.

Intersex conditions have been acknowledged for centuries. Ancient Greek and Roman sculptures depicted the offspring of the God Hermes and the Goddess Aphrodite,Hermaphroditus, as a young male with well developed breasts. Although the term "hermaphrodite" was taken from this mythological character, true hermaphrodites cannot be easily identified by appearance alone. In fact, there are really too many different forms of Intersex conditions to have them classified by a single term.

However, the medical community has for decades divided the forms into three sub-sections, first classified by German pathologist Edwin Klebs in 1876: female psuedohermaphroditism, male psuedohermaphroditism and true hermaphroditism. Although this classification system is still in use, it is important to note that Klebs formulated the system in an era long before the discovery of sex chromosomes and genes, and their influence on various conditions. (Money, pg 4)

Female psuedohermaphroditism is recognized as a genetic female (XX chromosomes) with masculinized genitalia. Internal organs include all or variations of the presence of ovaries, uterus and fallopian tubes, but due to high levels of androgens (male hormones) during gestation, the external organs present as a penis and scrotum, or some variation of genitalia which appears to be more male than female. An enlarged clitoris is not uncommon. In rare cases the external appearance of the genitals is such that the condition is not recognized until puberty, when the onset of menstruation in the presumed male adolescent may occur. More often the genitals appear ambiguous at birth and trigger a response reaction from the medical staff involved.

Male psuedohermaphroditism is recognized as a genetic male (XY chromosomes) with feminized genitalia. Testicular tissue is present, but the external organs have failed to adequately respond to the male hormones which should have promoted penile growth, scrotal closure and the decent of testicles into the scrotal sac. In rare cases the external sex organs are such that the condition goes undiagnosed until puberty, when the presumed female adolescent fails to develop breasts or menstruate. More often, as with female psuedohermaphroditism, the ambiguity of the genitalia prompts a response from medical staff present at birth.

True hermaphroditism is recognized by the presence of both male and female internal structures, ovarian and testicular tissue, regardless of the appearance of external sex organs. Although the mythological idea of a being with complete anatomies of both sexes is in fact never the case, it is possible that one may have testes on one side and an ovary on the other. Diagnosis in such a case can be delayed by the assumption that the second testicle has failed to descend, which is not uncommon in normal newborn boys. Since this condition often resolves on its own, unless surgical intervention is needed, ovarian tissue remains undetected until such time as other issues, such as pubertal breast development or an onset of menses, occurs. However, as with psuedohermaphroditism,more often the appearance of the external genitalia is such that medical staff present at birth reacts. (Money, 1994)

Clearly, these general sub-classifications of Intersex conditions are vague at best. Since the time when Krebs first introduced this system, a great deal more has been learned about so-called hermaphrodites and the many possible causes and manifestations of their conditions. Genes, chromosomes and fetal environment play significant parts in physical sexual development. This paper is not devoted to identifying or describing all the various conditions or the possible factors contributing to their development. Instead, let us address the ways in which the medical community has chosen to manage and treat the Intersex child over the last several decades, and the factors that influenced the protocols which were followed for so long.

As we have established, Intersex conditions have been acknowledged for centuries. However, it was only in the latter half of the 20th Century that the medical community began to feel that it was necessary to devote itself to "fixing" those individuals who presented with ambiguous genitalia.

In order to understand the need for change in the treatment of Intersex, it will be necessary to understand some of the history behind established protocols.

Much of the criteria developed for the management of Intersex children was influenced by clinical information published by Dr. John Money, Ph.D., a sex researcher at Johns Hopkins.

Interestingly, while still a student at Harvard in 1952, Money had written aDoctoral dissertation entitled "Hermaphroditism: An Inquiry into the Nature of a Human Paradox", which investigated the psychological affects of growing up with ambiguous genitals. His findings: the majority had adjusted to the situation and lived lives "virtually Indistinguishable from people without genital difference". It is unclear why, when he'd established that lack of medical and surgical intervention did not preclude a normal life for the patient, he chose to devote so much of his time to advocating the surgical assignment of gender to infants and young children born with ambiguous genitalia. (Colapinto, 2000; Diamond, 2004)

Money and his colleagues John and Joan Hampton were the researchers assigned to the Psychohormonal Research Unit at Johns Hopkins in the mid 1950's and much of their research focused on intersexuality. In 1955 Money postulated that, based on their studies of hermaphrodites and their flexibility in adjusting to their assigned genders, that all humans are born sexually undifferentiated at birth, and the development of gender identity as male or female was dependent on learning experienced in the environment of rearing.

Publications by Money and his team led to a widely accepted idea that genes and hormones led to the physical appearance of male or female sex (nature), but that gender is produced by indoctrination (nurture). (Diamond, 2004)

This idea was publicly questioned by a younger, lesser known researcher by the name of Milton Diamond. Animal studies done by his own team of researchers had shown that exposures to certain hormones during gestation not only altered the exterior genitalia and internal sex organs of mice and other small mammals, but also the sexual behaviors of the affected animals. Surgically altering the appearance of the genitals would not alter the behaviors. These studies suggested that humans, the most developed of mammals, might be similarly affected. While willing to concede that Intersex children exhibited a high level of flexibility, Diamond pointed out that as there was no proof that sexual reassignment of a developmentally normal child had ever been attempted, it was therefore irresponsible to speculate that such a child would accept or adjust to such reassignment. (Colapinto, 2000)

In the meantime, Money had begun to advocate a more aggressive approach to the treatment of Intersex infants, including early gender assignment and surgical "repair" of atypical external genitalia and internal organs. During this same time frame, he was actively campaigning for Johns Hopkins to establish a psychiatric and surgical team for the gender reassignment of adults. Both ideas were initially met with reluctance by Johns Hopkins administration, but eventually these treatment facilities were established there, and the Psychohormonal Research Unit continued to grow.

A great deal of research during this era was focused on non-traditional sexual issues. Why, researchers wondered, would anyone want to deviate from their natural sex and undergo painful surgical procedures to become the opposite sex? Was it ethical to imprison homosexuals and deny them employment? What would make an otherwise normal heterosexual male want to dress up in his wife's clothes? In this environment of increased interest in all things sexual, it seems only natural that Intersex conditions would intrigue the medical community.

In the midst of it all, a child was born. One of identical twins, he was destined to become the unwitting participant in an experiment which would alter treatment and management of Intersex conditions for decades. Bruce Reimer and his twin brother Brian were born to Ron and Janet August 22, 1965.

When the twins were eight months old, they were scheduled to have routine circumcision done after developing pain and difficulty while urinating, a condition known as phimosis. The performing physician elected to use a cautery machine rather than a scalpel to remove foreskin. Normally, this is a routine procedure with little risk, and the cautery machine simply closes blood vessels while cutting away skin, decreasing blood loss. However, on this occasion, April 27, 1966, this was not to be the case. For unknown reasons, the machine malfunctioned and Bruce's penis was literally burned off. Daunted, the medical staff elected not to circumcise his twin.

Naturally, his parents were devastated. Although they lived in a relatively large metropolitan Canadian city, the case was markedly unusual, and they were soon referred to the Mayo clinic in the United States, where doctors recommended adopting a wait and see approach, as phalloplasty, the surgical construction of a penis, was not a satisfactory procedure at the time. The couple returned home discouraged with their child's prognosis; physicians had told them that Bruce would never be a normal boy, would never be capable of normal sexual relations and would have to accept that he was destined to "live apart."

Some months passed before the Reimers happened to see Dr. John Money on television. On the program, This Hour has Seven Days, he introduced a young lady who had once been a man. She claimed to be completely happy with the gender
transformation. Intrigued, the Reimers discussed the issue and decided to write to Dr. Money.

Presented with a developmentally normal child who could serve as his experimental subject, Dr. Money wasted no time in inviting the family to Johns Hopkins to discuss reassigning the child as a girl. The fact that this child came with a built-in control model, his twin brother, was not lost on Money. If successful, the case would prove his theories regarding the development of gender identity.

The Reimers traveled to Baltimore to meet with Dr. Money and his staff. After much thought and discussion, Ron and Janet Reimer elected to go through with sexual reassignment for their child.

On July 3, 1967, Bruce Reimer went into surgery a male with testes and no penis and emerged castrated, his testes removed and scrotal sac refashioned to resemble female labia and genital folds. The plan was that he would later undergo further surgery to create a vagina and more feminine appearing genitalia. His name was changed to Brenda, and he went home with his parents, who had been instructed to treat him as a girl - give her dolls to play with, dress her in dresses only and instruct her in all things feminine.

The years passed. The Reimers struggled to raise their children as a normal boy and girl. They appeared in Baltimore for annual visits to John Money for follow up.

In December of 1972, John Money announced to the medical world that a genetic male had been successfully reassigned as female. His book, Man & Woman, Boy & Girl, was published at the same time of the announcement. In this book, Money described Reimer as "tomboyish", but emphasized that he and his twin brother were as different as night and day. Brenda, he claimed, was tidy and feminine, interested in dolls and kitchen work, while Brian played with cars and guns, and showed no interest in keeping his room neat. Brian provided the ultimate match for pair comparisons, Money claimed, as he and Brenda were identical in every other way. Although acknowledged as the dominant twin, Brenda's treatment of her brother is describes as that of a "mother hen".

In reality, Brenda was more likely to beat on Brian when he didn't go along with what she wanted. She was also known to beat on other boys at school in response to ridicule and was virtually a social outcast. Although she appeared at school in dresses and wore her hair long, Brenda was in no way feminine, and her peers recognized her differences in spite of having no knowledge as to her real condition.

Money continued throughout the 1970s to publish articles and publicly speak about the great success in the Reimer case, although the child was never identified by her real name. He also alluded to Milton Diamond's challenge of his theories, citing the case as proof that he did not deal only in atypical cases.

Diamond was not the only researcher to question Money's assertion that human beings are born psychosexually neutral, and that gender identity is formed through environmental interaction. Other researchers had found plenty of evidence that other factors were involved, including hormone exposure during gestation, chromosomes and genes. They published results of studies which contradicted the views of Money and his followers. Unfortunately, Money's views were met with more acceptances by the medical community and the general public . (Colapinto, 2000)

In particular, those who were faced with the management of Intersex patients were relieved to have evidence that gender assignment and surgical intervention was recommended and that choosing to assign particularly difficult cases to a female gender was acceptable. Believing that a child would conform to the assigned gender, physicians were more comfortable in assigning ambiguous cases as female. Creating a vagina was preferable to trying to create a satisfactory phallus, so choosing to raise a child as male depended on the size and probable function of the phallus present at birth, regardless of genetic and chromosomal sex. (Diamond, 2004)

Over the next several years, protocols in the management and surgical intervention of Intersex conditions emerged primarily based on the evidence presented in John Money's famous twin's case. Protocols followed until recently include the following recommendations.

1.The birth of a child with ambiguous genitalia was to be considered a social and medical emergency. Medical staffs were advised not to identify the child as male or female to the parents, pending formal gender assignment. In this way there would be no question as to the validity of the sex stated and the parents would not be ambivalent regarding acceptance of their child as a boy or girl.

2.Intersex babies with female appearing genitals should always be assigned as female.

3.Genetic males with ablatio penis (absence of penis) or severe micropenis should be assigned female.

In short, it was generally established that all Intersex conditions could be managed by cosmetic surgery, regardless of genetic or chromosomal sex or prenatal hormonal exposure. Typically, the assigned gender has been female, since such surgical alteration has been aesthetically more acceptable to both physicians and parents.

Since it has largely been held that surgical intervention early in life and treatment with hormones at puberty would cement the assignment of gender, a majority of cases have been lost to follow up, and long term implications were generally unstudied until more recently.

In March 1997 Milton Diamond and his colleague Keith Sigmundson published a long over-due follow-up to the infamous twins case in the Archives of Adolescent and Pediatric Medicine. It revealed for the first time that the case which had been reported as an unqualified success was in fact a dismal failure.

Keith Sigmundson had first met Brenda Reimer in 1976, when she was 11 years old. Although he had reservations, he decided at the time to support John Money's proposed gender assignment, and over the next few years worked with a procession of doctors, social workers and councilors in attempts to help her accept herself as a girl.

Communications with Dr. Money were discouraging for Sigmundson. Money was pushing for the child to undergo vaginal surgery, which Brenda vehemently resisted even talking about. Sigmundson was encouraged to talk her into it. He had no success.

When Brenda began hormone replacement, the medical team hoped that it would help her acceptance of herself as female, but the estrogens she was ingesting conflicted with her own male endocrine system. Although she was developing breasts, her voice had deepened to a baritone register similar to her brother's. She began to overeat in an attempt to gain weight and disguise her breasts. She continued to refuse vaginal construction and when asked if she wanted to be a girl she flatly declared, "No!"

It was at this point that her doctors decided it was time for her to be told the truth. Her father, Ron Reimer, took her out for ice cream and explained about the accident which had changed the lives of the entire family.

The revelation was a great relief to Brenda, who finally understood the reason behind her feelings of confusion regarding her gender identity. She changed her name to David, after the Biblical figure who battled a giant and won. At the age of 14, she began to live as a boy.

It wasn't easy. He had a mastectomy to remove the breasts grown while taking estrogen. He went through painful procedures to fashion a rudimentary penis. He hid in the basement of his parent's house, watching television and waiting to emerge publicly as a male. Further surgeries led to the construction of a more satisfactory penis.

David eventually married a young woman who had three children from previous relationships, whom he adopted and raised as his own. He found work in a slaughterhouse and supported his family as any "real" man should.

In the early 1990s Milton Diamond identified Keith Sigmundson as one of the teammembers who had worked on Money's famous twins case. He had attempted to discover the ultimate outcomes of the case for years. Although initially reluctant to get
involved in follow-up issues, Sigmundson soon joined Diamond in an attempt to interview David Reimer.

Reimer was also reluctant to get involved until he learned that John Money had published reports that the case was successful and that children all over the world were being surgically reassigned sexually as a result of that information. (Colapinto, 1997)

Since the revelation that the landmark case which so many practitioners relied on for justification in their treatment of Intersex patients was in fact a failure, several publications have run various stories about the case, raising public awareness of conditions which until now have largely been kept secret. More physicians have come forward to offer their views on the matter.

Even before the 1997 publication of Diamond and Sigmundson's article, there were physicians who questioned the standard practice of surgical reassignment. One is Psychologist William Reiner, director Johns Hopkins's Gender Identity and Psychosexual Disorders Clinic. A former urologist, Reiner re-trained in psychology after beginning to question the protocols and outcomes of sexual reassignment. Although he'd been actively involved in cases of gender assignment, and advocated the old protocols throughout the 1970s and 80s, he'd become increasingly concerned about the poor outcomes he was
seeing.

His studies have led him to conclude that children are born either boys or girls, and regardless of surgery or rearing, they are what they are.

Reiner has made a clean break from his former beliefs and now advocates adopting a wait and see attitude in regard to genital surgery. Most Intersex conditions are not life threatening, and the decision to wait can be made without causing the child physical harm. (Hendricks, 2000)

More recent studies have shown that gender identity is a complex interaction between chromosomal, genetic, prenatal and postnatal endocrine influences and postnatal environmental influences. While humans are now more commonly perceived to be predisposed to a specific gender identity, a conscious development is still thought to take place during the formative years, just as cognition and social development progress in stages in young children.

In normal development, children are able to distinguish between the sexes by the end of their first year of life. Between the ages of 2 and 3 they correctly label themselves and others as male or female. By the age of 3, children will readily define themselves as male or female.

While there are few studies to substantiate the claim, it may be that genital appearance may not be a crucial determining factor in developing gender identity. (Ahmed, et al, 2004)

If this is so, the rush to surgery is unfounded. Much of what led physicians to embrace the theory that cosmetic genital surgery should be done before a child was old enough to remember anything was the belief that said child's psyche would be damaged by viewing their own atypical genitals.

But if new evidence is true, there would be less psychological harm in waitinguntil a child is old enough to declare their gender, rather than assigning one and making irreversible surgical alterations which may prove contradictory to the gender identity.

According to genetics and sexual medicine expert Dr. Eric Vilain of the David Geffen School of Medicine at UCLA, surgical sex assignment of newborns should never been done for cosmetic reasons. There are too many factors, including brain development and genetics, which too little is known about, and until these things are fully understood, making legal and surgical decisions that cannot be reversed is not advisable. (HealthDay, 2005)

Yet for decades these surgeries have been done repeatedly, and for this very reason: cosmetics. The presentation of an infant who cannot be easily identified as male or female creates an atmosphere of great tension. The medical staff is faced with explaining to anxious parents that their child is not "normal", not "typical", not "classifiable". Parents want to know if they have a son or a daughter; they want to know what to tell their families and friends. An event that should be a cause for celebration has become one of uncertainty and fear.

Enter the quick fix. Pick a sex and get the scalpel. The sooner the better.

But things are changing. In the past decade many intersex people who underwent surgical reassignment of sex have come of age and are speaking out.

One of the first to speak out was Cheryl Chase, a true hermaphrodite who was assigned male at birth and named Charlie. After discovering that she had a uterus, she was reassigned as female. Her clitoris, which was enlarged enough to have the
appearance of a penis, was removed. Her family was told to move to a new city and never breathe a word of what had happened.

Ms. Chase founded the Intersex Society of North America in 1993 and has worked tirelessly to promote her beliefs that children should not have surgical reassignment of sex until they are old enough to declare themselves male or female and take an active part in their own health care.

Her efforts were not well met at first, but as her organization and others like them grew, the Intersex community has gained a voice among the medical community. (Torossa, 2002)

On April 28, 2005 the Human Rights Commission of the City and County of San Francisco held a public meeting to discuss the medical management of Intersex people, and most particularly what they termed the "normalization" of these people. Cheryl Chase and Alice Dreger wrote an essay that was presented as part of this meeting. (Arana, 2005, pp 10-15) In it they state that "ISNA maintains as its fundamental principal that the fate of a child's sexual anatomy should not primarily rest on the needs of others, but should be left to that individual to decide, barring true medical emergency."

Testimony by many Intersex people at this meeting left little doubt that patients felt traumatized by the medical management chosen for them as children. Betsy Driver, born with congenital adrenal hyperplasia, is the founder of "Bodies Like Ours", a support group for those with her syndrome. She testified that having undergone a clitorectomy (surgical removal of the clitoris) and frequent genital examinations during childhood left her feeling that she had been sexually abused. (Arana, 2005 pg 31)

Michael Borriello of the New York Association of Gender Rights Advocacy offered the opinion that the birth of an intersex child should be addressed as an opportunity for education, not operation. "Taking the wings off a butterfly does not turn the butterfly into a beetle," he said. "Removing the parts of our anatomy that made our parents or doctors uncomfortable did not make us comfortable - in our bodies, or in a society barely able to admit that we exist." (Arana, 2000 pp 36, 37)

Doctors are speaking out as well. Dr. Robin Hoburg states, "Intersex people, like people with disabilities, will gain social acceptance and diminish their stigma through visibility - not through being hidden or 'normalized'."

Dr. Alice Dreger states that surgical sex assignment is experimental. (Arana, 2005, pg 67)

Cheryl Chase contends that "These surgeries are plainly experimental.

1. The are not medically necessary to alleviate pain or any physiological dysfunction

2. There is no medical consensus that these procedures are advisable or beneficial…on the contrary, there is growing concern over the efficacy and the ethics of these procedures among medical experts in many fields

3. There are no outcome studies to support the hypothesis that these painful, invasive and irreversible surgical procedures result in any psychosocial benefit to the child or enhance the child's well being in any way."

The American Civil Liberties Union stated that genital modification surgery is unlawful under California and Federal Statutes. Several of the surgical techniques used on Intersex infants are prohibited under laws that prohibit genital mutilation. They also
declared that parents have "no legal authority to consent on behalf of their children to unnecessary, invasive and potentially harmful procedures".

Finally, A.C. Lareau of the Georgetown Law Journal Association pointed out that we do not "normalize" other children who might be at a social disadvantage due to race, intelligence or other physical features that deviate from societal norms. (Arana, 2005)

It was also noted at this meeting that David Reimer, in infamous "boy who was raised as a girl", had committed suicide on May 5, 2004. Milton Diamond stated "He was forced to live a life that was not his own, was not of his making, not of his choice, in which every time he tried to assert himself, he was thwarted by the two forces which are supposed to be the most helpful in our lives - our parents and our physicians."

Milton Diamond, having at last unveiled the truth about a case that led to the alteration of so many lives, offers us this view: Intersexuality should be seen as a condition without stigma, rather than a disorder.

While this controversial subject remains under review, these recommendations are being considered. First, since there has been no evidence presented that surgery is beneficial, remember the medical golden rule: "First, do no harm." Moratoriums need to be in place regarding any surgery done without patient consent. Second, until comprehensive follow up studies of surgical outcomes are done, moratoriums must remain in place.

In spite of all the new evidence being presented, change is a slow process. Many physicians still believe that what they've been doing all these years is in the best interests of the patients. In spite of having been exposed for withholding the truth about his famous twins case, John Money has never publicly altered his opinion that surgical reassignment is the best option for Intersex infants.

Dr. Peter Furness, Associate Professor of Surgery and Pediatrics and Associate Chief, Pediatric Urology at The Children's Hospital in Denver, Colorado was kind enough to respond to questions regarding management of Intersex patients. "Gender assignment is a very controversial, emotional and multifaceted subject," he wrote. "With that said, gender assignment for the most part is assigned based on the genotype (genetic male or female) and not necessarily on the phenotype (genitals)." Although he has not been involved in this type of surgery - he states that it is extremely rare - his input is encouraging. In the past, assignment was often based on phenotype more so than genotype, and this distinction is a sign of change for the better. (Furness, 2005)

As studies continue, one hopes that the words of Dr. William Reimer will be remembered. "The sense of who one is - boy or girl - is a crucial existential aspect of humanity. It is powerful and inborn. The most important sex organ is the brain." (Hendricks, 2000)