intersex

Last month, activists from Intersexuelle Menschen e.V., an international organization representing several intersex advocacy groups, protested outside of the Children’s Hospital in Zurich. It looks a lot like the protests ISNA organized in 1996 outside the annual meeting of the American Academy of Pediatrics. Protestors in Zurich carried signs that read ”Human rights for zwitters too.” Apparently “zwitter” is pejorative term for hermaphrodite that activists have reclaimed. (They also held a protest in Cologne in December 2007.)

On July 20th, this group, along with XY Frauen, presented a lengthy report to the UN CEDAW committee (Convention on the Elimination of All Forms of Discrimination Against Women) outlining human rights violations stemming from the medical treatment of intersex conditions. More information is here. The day before, Intersexuelle Menschen e.V. also submitted a list of demands to the the German Parliament.

Dexamethasone, or DEX as it is called, is a powerful steroid that has been administered to pregnant women who have previously given birth to a child with congenital adrenal hyperplasia (CAH). The aim is to minimize (or altogether prevent) the virilization of the female fetus’s genitals that happens in utero. It’s a practice that has been going on for over two decades.

Genital development occurs early in pregnancy, so DEX is typically given as soon as the woman knows she is pregnant, which is often before anyone knows the sex of the child. When what is known as CVS testing is possible at about 11-12 weeks gestation, DEX will be discontinued if it is found the fetus is either male or an unaffected girl. Because of the gap between when genital development occurs and when fetal sex is typically known, there is a great deal of overtreatment. Some estimates are that as many as 7 out of 8 women given DEX have healthy fetuses. New methods that enable sex determination earlier in gestation will help to reduce this overtreatment, but these methods may not be widely used and this still doesn’t address a larger problem: we have very little information about the long term safety of DEX on the fetus and mother.

DEX, which has been used for years in adults and has not been approved for use in pregnant women, has a bunch of nasty side effects for pregnant women. Most of these, it is believed, disappear after discontinuation. A recent article, however, reviews worrying evidence from animal and human studies that suggests possible effects on cognition (such as working memory) as well as future health of the child (e.g., type II diabetes and high blood pressure). Based on the evidence, the authors conclude there is an "urgent need for in-depth studies of long term outcome of prenatal treatment of CAH regarding both maternal side effects and possible negative metabolic as well as cognitive and behavioral effects in the growing fetus and the child in her development into adulthood."

After 15 years the Intersex Society of North America has closed it doors. Cheryl Chase, who now goes by her legal name, Bo Laurent, started ISNA after some very frustrating conversations with doctors about the harm she felt as a result of her medical care. Some doctors responded to her suffering with indifference, but, more often, her experience of harm didn’t make sense to them because they gave what they believed to be excellent care. Rather than retreat, she decided to do something. As is often the case with outsiders in a limited position to effect change, members of ISNA protested to try to get doctors to listen and, ultimately, to improve care.

It’s not been easy. Some of the difficulties have come from trying to work with doctors and parents who often saw things very differently. Other difficulties came from within the intersex movement, where people had different ideas about what the goals of intersex activism should be. The most recent example is the heated controversyover the change in medical nomenclature (from intersex and variations of hermaphroditism to disorders of sex development). As a result of these diverse views over what ISNA’s aims should be—and ISNA having had the privilege and burden for many years of being the only group out there—many of the people who were instrumental in ISNA’s success distanced themselves from or left ISNA over the years. ISNA’s closer ties with doctors were too accommodating for some. Others felt betrayed by the new nomenclature, which they felt classified them, not their conditions, as disorders. This reaction is understandable and something and I have been thinking and writing about.

I’ve already seen some “good riddance” responses to ISNA’s closure, arguing the group did more harm than good, especially of late. But I disagree. I have been talking to doctors since 1997 about how they understand and deliver care and there is a world of difference between then and now. Back then, the idea that you would not do early genital surgery was largely unthinkable. Most surgeons also felt their results were great, or at least said as much publicly. Today, they are far more circumspect. I have talked to many clinicians who often do not recommend surgery and have cases to back this up. (Now whether parents accept this recommendation or seek out another surgeon who is willing to perform surgery is another issue.)  I was just at a meeting where surgeons talked openly about how they often did not know what to do and weren’t sure whether surgery was helpful. They didn’t just say this, this doubt had changed their clinical practice. Whether they are this honest with parents isn’t clear, but I don’t think you can be questioning your work this deeply with colleagues and not let it permeate conversations with parents sooner or later.  This would not have happened without ISNA.

In 1997 most assumed that so-called correct gender assignment, genital and gonadal surgery, and hormonal interventions were all that mattered for a child’s quality of life. Not so today. At the meeting I was at everyone was very clear we know almost nothing about what determines quality of life, but that it surely isn’t as narrow as getting gender assignment right.

And in 1997 it was still common to think homosexuality or gender change signaled a bad outcome, and that patients and parents were better off not being told certain things about their bodies, diagnoses, or treatment, and not meeting others. I haven’t heard clinicians say this in quite some time.

I could describe many other shifts. My point is that I think it’s easy to get caught up in rhetoric—and certainly there is room for differing views about the goals of intersex activism—but for as long as I could tell ISNA has always been primarily concerned with changing medical practice in the here and now in an effort to improve lives in the here and now. This has meant working closely with doctors—often uncomfortably. I have been at quite a few meetings with clinicians with Bo over the years. And, believe me, some of these meetings have been really depressing. Imagine bearing your deepest pain only to be told you are unlucky or just have a bad attitude. Now imagine experiencing that over and over, again and again, over a decade. I think a lot of people would have given up by now finding the pace of change frustratingly slow. What struck me is that by the next day she would be back at it, scheduling more meetings, and doing what she could to improve care. I imagine that what makes her continued commitment possible after years of frustrating experiences is her deeply held conviction that kids with intersex conditions are entitled to a life that allows them to flourish, and a deep well of hope and optimism that her efforts could help to make that happen.

Yet another study has examined gender outcomes in women with CAH. This time the focus is sexual orientation. This makes me think again of a paper psychologist Tom Mazur presented at the 2008 meeting of the Lawson Wilkins Pediatric Endocrine Society. Tom and his colleagues did a literature review of all of the studies they could find on outcomes across DSD diagnoses. They found that less than 1% of all outcome studies assess quality of life; the rest largely deal with gender change later in life, degrees of gender-atypical behavior, sexual orientation etc.

This is unfortunate and also not surprising. Scientific interest in intersexuality stemmed from an interest in human psychosexual development in general, which is tied to long-standing debates over the relative roles of nature and nurture in determining physical and behavioral differences between men and women.

Researchers have looked for ways to assess the relative role of biology and socialization in making us men and women. Girls with CAH have been of particular interest because they provide an “experiment in nature” due to their atypical exposure to androgens in utero, which not only masculinize the genitals, but, researchers hypothesize, the brain, leading to more male-typical behavioral traits, including what is understood as male-typical sexual desire (i.e., desire for women, or homosexuality). These theories are united by the claim that sex hormones play an important role in the production of sex differences, though researchers and theorists differ in their opinions about which behaviors are affected.

The unfortunate consequence of this overwhelming focus on gender outcomes is that after fifty years we know surprisingly little about the factors that affect quality of life for children and adults with DSD—a fact that too few researchers are seeking to rectify.

I had the opportunity to share this case report suggesting vaginoplasty may be easier at puberty with several pedaitric urologists. Brad Kropp, of the University of Oklahoma, had the following to say:

Interesting case report.  The images are very interesting.  When I look at the vaginogram at 3 months you see a very small vagina. The MRI, years later, then demonstrates a large fluid (most likely urine) filled vagina and uterus.  I would propose that the vagina is closer to the perineum because the chronic vaginal distention that occurred from the retained urine in both the vagina and uterus.  I have seen this same picture on several of the cloacal kids prior to my reconstruction that develop this type of distention.  There is no doubt that a large vagina is easier to work with but I would be interested to know how many UTI’s and problems this child had because of the chronic distention and retention of urine.

I have been talking a lot with Bo Laurent of late about how health care teams function. I find it fascinating and it’s about a close as I can get to understanding organizational development without going back to school. Bo recently did a study of the craniofacial team at University of Washington—a team that from what I can tell does a pretty marvelous job of being a team. One of the reasons Bo felt that team worked is that it had a high degree of what people in OD have come to call psychological safety. This concept was introduced in a 1999 article and basically it argues that for teams to function well and learn from mistakes, members need to believe the team is a safe environment for interpersonal risk taking.

What does this mean? That irrespective of status or position on the team (i.e., whether nurse, social worker, or surgeon), all members feel safe to raise concerns, offer opinions, challenge views, and disagree even about a patient that isn’t “theirs.” Not only should team members be encouraged to voice their views and feel safe about doing so, but that they will not suffer professionally for their views by not getting raises or promotions and the like. Psychological safety doesn’t just emerge in a team; it has to be  learned and built upon so that it becomes part of the team’s culture and way of being. Seems to me that without this in place, it would be really hard for doctors and teams to learn from mistakes. With all of the focus on clinician-patient interaction in medical education, how to be part of a team may be even more important and would likely improve clinician-patient interactions as well.

Going against conventional wisdom that vaginoplasty in infants is technically easier, a new case study suggests that surgery at puberty may not be all that more difficult due to anatomical changes in the pelvis as the child grows.  The surgeons report the the case of a girl with CAH on whom they chose not to do vaginoplasty in the neonatal period because of what they described as her “short vagina and long urogenital sinus,” which would have made an already difficult surgery even more complex. When she began puberty at age 9 they did a magnetic resonance scan of her pelvis and found that her vaginal size had increased and her vagina and urogenital sinus had descended toward the perineum—changes that would make surgery less difficult than if it had been performed earlier in her life. This is despite the greater difficulty “mobilizing” tissue in an older child. They conclude:

We therefore presently recommend that, since the vagina is not an organ that is essential in early life, for most children with CAH there is no urgency for vaginal reconstruction. Indeed the natural vaginal growth and development that occurs at puberty may have significant advantages in reducing the extent of surgery, which can then be done with fully vascularized, innervated, and lubricated local vaginal tissue.

I don’t think it will be surprising to anyone working in the field of intersex, or DSD, that few families or individuals receive psychosocial support. Most of the clinicians I’ve spoken to say it’s because it is hard to find qualified people to provide the care and harder still to get reimbursement for this care. There’s a new study showing that few families get psychosocial support, which is not a big surprise, but what’s surprising is the gap between how many programs said they offer support and the number of families receiving support. The abstract is below.

ABSTRACT: The birth of a child with an intersex condition is often an emotionally stressful event for parents. Preparation and ongoing support systems could be beneficial to both parents and children and would alleviate some of the shame and isolation associated with intersex conditions. To assess the extent to whichpsychological support is available, a short e-mail survey on this topic was sent to the directors of 50 pediatric endocrinology fellowship training programs (PEFTPs), who are most likely to evaluate and treat intersex children and their parents. Of the 29 PEFTPs that responded, 69% offer psychological support and 58% have a mental health specialist on staff. However, only 19% of patients or families receive emotional support during diagnosis and only 15% receive support after diagnosis. We found two barriers that prevent patients and families from receiving psychological help from their intersex care team. First, there is a lack of training for mental health professionals regarding the needs of intersex patients and families. Second, some families refuse help even though it is offered. This study reveals that further research is needed to overcome these two barriers regarding mental health treatment of intersex patients and families.