This Date in UCSF History: Intersex Dilemma

Originally published in Synapse - The UCSF student newspaper March 11, 1999.

Cheryl Chase's clitoris was surgically removed when she was 18 months old. She appears to be a clean cut woman in her forties, and as she speaks about the series of operations that were performed on her, she fills the room with a sense of her loss and anger.

Chase was born a true hermaphrodite, a condition in which the gonads have elements of both ovarian and testicular tissue.

The testosterone produced by the testicular elements in her gonads caused her clitoris to be unusually large, resembling a small penis. Like others recognized at birth to have ambiguous genitalia, or whose genitals do not match their chromosomal sex, she was classified as an intersex individual.

Initially doctors thought Chase should be reared as a boy, and she was named Charlie. But further consultation with different doctors led to the decision to raise her as a girl. She was renamed Cheryl.

Her parents decided, under medical advice, to have her clitoris removed, in order to “normalize” her appearance toward that of a girl. This clitorectomy was meant to help her develop a female gender identity.

When she was eight, doctors removed the testicular portion of her gonads, to reduce the risk they would undergo cellular changes that could lead to cancer. At no time was she truthfully told the purpose of the surgeries.

At the age of 35, Chase had a nervous breakdown. Although she had been able to access her medical records in her early 20s, support groups in which to discuss her condition did not exist.

The years of secrecy, unexplained surgeries, and sexual dysfunction caused by removal of her clitoris had taken a huge toll on her.

“Until I was 35,1 was ashamed and terrified that people would find out that I was different than a woman. Like many, supposedly happy and successful patients, I was silenced.”

Instead of retreating from the pain of her experience, she took the revolutionary step of founding the Intersex Society of North America (ISNA), a San Francisco based peer support and advocacy group.

Initially just a loose association on the internet, ISNA now has 1,400 members, holds retreats for intersex people, has produced a video, lobbies, holds demonstrations, maintains a website, and puts intersex people in touch with each other throughout North America. The latter may be their most important function.

“Every intersex person we have met with has had a common experience, in that it was immensely transformative and positive for them to meet other people like themselves,” says Chase.

Members of the group spoke to UCSF medical students last week, to make the case for changes in current medical practices.

When all causes of intersex are considered, as many as one in 2,000 babies are affected.

There are many conditions that can lead to intersex status. Chase's condition is rare, but when all causes of intersex are considered, as many as one in 2,000 babies are affected.

In the embryo, the external genital structures are originally the same in males and females. Exposure to testosterone, the male hormone, causes differentiation into a penis and scrotum.

Lack of testosterone, as in females, allows the tissue to develop along a 'default' pathway, in which a clitoris and labia are formed. Inside the body, a portion of the V chromosome, found in males, causes the primitive gonads to become testes. The testes are the source of the testosterone which causes the external genitals to differentiate in a male pattern.

Testosterone also promotes formation of the prostate, and other internal male reproductive structures.

In addition to the steroid testosterone, the testes make a peptide hormone which inhibits the formation of internal female reproductive structures such as the uterus and upper two-thirds of the vagina.

Various alterations along this pathway can lead to intersexuality. Common ones include partial or complete insensitivity of the tissue to testosterone, inadequate production of testosterone, lack of the testes determining region of the V chromosome in a male, or its presence in a female, and malformations due to exposure of the fetus in utero to certain drugs.

The cause of another type of intersex, severe hypospadia, is not understood. In this condition, seen in males, the urethral meatus (from which urine exits) is at the base of the penis, and the scrotum resembles the female labia. The incidence of this condition is increasing, for reasons that are not known.

Medical Treatment Current medical treatment for intersex individuals is in a state of flux. Recently The

The term 'functional' varies in meaning, but usually refers to the ability to use the genitals for penetrative intercourse. In cases where future fertility is possible, this too is considered.

Journal of Clinical Ethics devoted an entire issue to the subject, ultimately recommending major changes. At present, infants born with obviously ambiguous genitals undergo many tests (chromosomal, hormonal, and anatomical) to determine what sex they will be assigned.

The decision is based on criteria such as the ability to create cosmetically unambiguous and functional genitals with the tissue present. The term 'functional' varies in meaning, but usually refers to the ability to use the genitals for penetrative intercourse. In cases where future fertility is possible, this too is considered.

A multidisciplinary exam is involved in the decision. Once a determination is made, infants are named, and a birth certificate filed. The parents are instructed to treat the infant as being of the specified sex, without any ambiguity.

Surgery is performed before the age of 18 months to make the genitals match, as closely as possible, the assigned sex. It is this last step that ISNA most wants to change.

Associate Professor of Urology and Pediatrics Laurence Baskin's voice takes on a notable chill when ISNA's visit is mentioned.

“I honestly feel sorry for those people who feel mutilated by their surgeries. They need counseling to get over their loss. Their surgeries were performed years ago, and the nerve supply wasn't understood. For the three people who spoke to you, my guess is there are 97 who are happy. But they're not going to be out talking to medical students.”

Baskin admits that surgical technique in the past was not optimal.

“The surgery that was done was done by very well intended physicians, but we didn't understand the nerve supply well. We started to understand the nerve supply [to the clitoris] 10 years ago.”

Intersex individual Howard Devore, PhD, is a practicing San Francisco psychologist who works with intersex patients. He disputes the notion that things have improved much. Devore was born with severe hypospadia.

“Maybe a primary [less severe] hypospadia can be fixed in two or three surgeries. I've had 16. There's going to be fistulation over time. There's going to be scarring and stricture formation and loss of sensation. No scar tissue is as flexible as skin. There's no way they can deny that. The informed consent' they give parents to sign is totally unrealistic. One of our main issues is that parents are (old that after a few surgeries, their children will have 'normal' genitals.”

Professor of Pediatric Endocrinology Melvin Grumbach is much more optimistic. Speaking from an office cluttered with journals and papers on this subject in which

he is a renowned expert, he cites the advantages of microsurgery, unavailable when Chase's clitoris was removed.

“When I was a fellow at Hopkins, they were doing clitorectomy on girls with congenital adrenal hyperplasia (CAH) [in which the adrenals produce testosterone in utero that masculinizes the external genitalia], and I couldn't bear it. When I was at Columbia, clitoral recessions where developed. We studied four women with CAH, and three out of the four had had repairs, and they were all sexually functional and happy. Two of them had children.”

Even the terminology is a battleground in the current debate.

Chase says, “We don't see a difference between clitorectomy and clitoral recession [in which the bulk of the clitoris is reduced by removal of part of the erectile bodies of the clitoral shaft]. Kinsey showed that women masturbate by stimulating the shaft of the clitoris, not the glans. Recession removes the shaft.”

Chase isn't surprised by Grumbach's and Baskin's faith in new techniques and technology.

“They are always saying these new techniques are better, but there are no longterm outcome studies. I don't think doctors who are doing this are setting out to hurt their patients. They are confronted with parents who are upset. Surgeons are not trained to

deal with parents who are upset. They are trained to 'fix' things. When people like me grow up and say, 'this hurt me,' they don't want to hear it, because they would have to see how they had hurt patients, and they would have to admit their impotence in addressing this by surgery.”

A brief literature search shows that surgeons have persisted in using. language that suggests optimal outcomes with relatively few procedures necessary.

In a 1998 article entitled, “Feminizing genitoplasty: state of the art,” author Rinke states, 'Today's efforts achieve near normal cosmetic and functional results.” Another author, Greenfield, writing about repair of severe hypospadias, promotes a two-stage procedure in which he claims, “All children had excellent cosmetic and functional outcomes.”

This is despite a 21% incidence of diverticula formation, as well as strictures and fistulas in some patients. Perhaps more important, there is no longterm follow-up of these patients.

Average patient age in the hypospadia study was two months. Obviously, sexual function was not one of the “functional outcomes” considered. Chase is quick to point out the lack of longitudinal study of these patients.

“There is a principle in medicine — do no harm. They [surgeons] don't know what effect their drastic, invasive, irreversible interventions are having. Given that they don't have knowledge about the effect of their interventions, it's unethical to do them on unconsenting infants.”

Endocrinologist Grumbach is skeptical of this viewpoint.

“If someone is telling you, 'you shouldn't modify the external genitalia,' well the parents wouldn't stand for it. They want it repaired!” Baskin is more critical. Baskin acknowledges that it can be “very disturbing to parents” to give birth to an infant who is intersex.

“It is very disturbing,” agrees Chase. “And when people are really disturbed, it's not the time to make major, irreversible decisions.”

Instead, ISNA proposes that the parents receive counseling, and are put in contact with other parents of intersex children. They believe that the child should be assigned a sex, given a name that corresponds to the sex, and raised with age appropriate explanations of their condition.

“The child will assert their gender identity between the ages of six and ten,” notes Devore.

Chase agrees, “If that is different than the assigned sex, their name and sex assignment can be changed. This will be less frightening if the children and parents have been involved in groups where other children have changed gender identity.”

The ethical issue most prominent in this debate is that of patient autonomy — the right of patients to decide for themselves what treatments they receive. ISNA seeks to maximize patient autonomy by delaying surgery.

The traditional treatment model holds that delaying treatment would lead to psychological scarring and difficulty in accepting the assigned gender. Baskin believes that it is appropriate for parents to consent to surgery on behalf of their infants.
“Parents make decisions for their kids. That's what parents do.”

On the other side of the divide, Chase was adamant that the surgery is being done for the psychological adjustment of the parents, which strikes her and other adult intersexes as horribly wrong.

“The infant is the patient, not the parents! Gender is not so fragile that cosmetic surgery needs to be done early. Gender identity would not be undermined by asking the child about it.”

“The idea of surgery is to make the child normal, with a capital 'N',” reflects Chase. “But our experience as adult intersex people is that these kids are not going to be 'normal.' The genitals are not going to look the same as those of other kids. And when an incredible amount of money and time has been invested in making them 'normal,' and they don't feel that way, they'll feel terrible. We all wish we had the genitals we were born with back. We're not saying this would have been without emotional difficulty, but it would have been better than what they did to us.”

ISNA also takes issue with the criteria through which sex is assigned.

“In the case of congenital adrenal hyperplasia, the excuse for the surgery is that the girl who has a vagina created will be fertile as a woman. But if you had to choose between fertility and sexual function, which would you choose?” Baskin is horrified by this attitude.

“The majority of these patients [with congenital adrenal hyperplasia] have done well. They have families. They have kids. They would never be fertile as a male. They would have a small, dysfunctional penis. If you leave a big clitoris, they [patients] don't look like a girl. Most patients don't want a clitoris that looks like a penis. People want to look normal. I am trying to help kids.”

After all the debate and division, it's reassuring to hear that the two sides do share a few square yards of common ground. Everyone agrees that patients and parents need more psychosocial support.

But no one agrees on why that care has not been forthcoming. Grumbach believes that groups like ISNA have changed treatment in positive ways. For example, patients used to be subject to repeated medical photography of their genitals, a practice which made many patients feel stigmatized and uncomfortable.

“They've pointed out the deficiencies in our care of patients. Sensitivity has gone up a couple of logs.”

Like ISNA, Grumbach and Baskin bemoan the lack of psychotherapy. “A good psychological social worker would be a tremendous help. It's hard to get long-term psychiatry. We [the pediatric endocrinologists] try to provide support. That is one of the real deficiencies in care.”

Baskin agrees that mental health care is, “a critical part of their treatment,” but admits that no services are consistently available here at UCSF.

He believes this is due to HMO and insurance companies who are unwilling to pay for treatment. Grumbach thinks that another important factor is lack of psychiatrists with expertise in the area of intersex.

Back at ISNA, these attitudes prompt the type of frustration typical of patients who have been denied care.

“What are they doing about it [the lack of psychological counseling]? If you had patients dropping dead from heart attacks, you wouldn't say there's no money for cardiologists. You would say our ethical duty as care providers is to get these people the help they need. Intersexuality is actually life-threatening, because the rate of suicidality is high,” maintains Chase.

Psychologist Devore, who studied intersex individuals at Hopkins, scoffs at the idea that trained counselors aren't available.

“The parents need counseling. They need to be told that this [intersex] is a way a person can be, even though it is not what the parents expected. There are psychologists trained in this. We have developed a referral list. ISNA is also available for peer support.”

The battle of the intersexes is far from over. Each side concedes some benefits received from the other. Intersex individuals are grateful for the immense research done on the causes of intersex conditions.

Doctors acknowledge that intersex adults have shown them how discussing the patient's condition openly yields better results than the previous protocol of hiding it from them with secrets and half-truths. Some doctors are talking to adult intersex individuals openly.

Medical practice has been influenced by the debate itself, but the anger and defensiveness that have been created have left a chasm across which only a few doctors will publicly walk.

Perhaps the next step in improving treatment will be the one taken when doctors and intersex adults sit down together to discuss methods for the scientific evaluation of patient care as they exist now and as they have been proposed.

Intersex individuals demonstrating outside a recent American Academy of Pediatrics meeting.