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February 23, 2007

Commentary on the Article by Scott Rivkees, Journal of Pediatric Endocrinology, November 2006
Kelly R. Leight
CARES Foundation, Union, NJ, USA

Dr. Scott Rivkees correctly points out in his editorial, "It is (Sort of) a Boy and (Sort of) a Girl. .." (Nov. 2006), that it is now time to re-frame the debate over reconstructive genital surgery in disorders of sexual development, such as congenital adrenal hyperplasia. The debate has, for the most of last decade, been defined by a vocal few, and the voices of the quiet many have gone unheard. While the vocal "intersex" advocates have brought about many needed changes in the realm of care of those with genital anomalies, Dr. Rivkees asks whether it is now time to treat genital structural anomalies as we do other congenital birth defects. Rather than insisting that societal acceptance without surgical intervention is best for the child as has been argued by some "intersex" advocates, perhaps we should be looking at improving outcomes.

Overall, women with congenital adrenal hyperplasia (CAH), who make up more than 95% of individual with disorders of sexual development, and their parents do not necessarily agree that unreconstructed societal acceptance is best for girls with CAH and would support Dr. Rivkees argument. CARES Foundation, Inc, a support and advocacy organization for those affected by congenital adrenal hyperplasia, is now giving a voice to those with CAH and their families, and allowing this community to define its own needs and desires around the issues of surgical reconstruction of genital anomalies in CAH.

The issues involved are multifaceted and complex. Dr. Rivkees points out that little research has been done to study surgical techniques that improve outcomes. Moreover, those that oppose surgery have not shown that those individuals who have grown up without surgery have better outcomes. In fact, the definition of "better outcome" is itself ill defined. Is it functional outcome? What is functional outcome? Is it the ability to engage in intercourse or achieve orgasm? Sexual dissatisfaction is common among those without reconstructed genitals, so this is a complicated measure to use. There are many barriers to doing outcome research but nevertheless, if we want answers, this research must be done.

So much has changed thanks, in part, to the attention placed on genital surgery by the "intersex" advocates. Now, most surgeons and pediatric endocrinologists will agree that minimally virilized girls with CAH should not have surgical intervention. Clitoral reduction should be done conservatively, if at all. Many physicians are learning the importance parental education about all options, meaningful informed consent and referrals to surgeons with substantial experience with genital reconstructive surgery. However, significant problems still exist. The American Urological Association and The American Academy of Pediatrics, Urology Division must set standards and define centers of excellence for reconstructive genital surgery for each individual disorder of sexual development including specifics for training of young surgeons in this specialty. Designating centers of excellence will allow families to get coverage for these complex surgeries out-of-network from managed healthcare and Medicaid. All children deserve high quality surgical care, no matter their financial circumstances. Right now, it is not always available to those without financial means and the best insurance plans.

Parents of girls with CAH make decisions about surgery based upon a variety of factors. While cultural background may make some more likely to seek early surgical intervention, degree of virilization seems to be the most critical determinant. Despite the best efforts of the vocal "intersex" advocates over the last decade to change parental decision-making, very few parents of significantly virilized CAH girls, 4's and 5's on the Prader scale, have made the choice to defer surgery beyond early childhood. This speaks to an inherent desire on the part of these parents to help their girls live a normal life, minimizing the physical manifestations of their disease. Some adults with CAH whose parents waited to allow their children to undergo surgery until they were teen/pre-teens have expressed the sentiment that they wished their parents had done it earlier and saved them their perceived shame of being "different". They longed to be "like other girls". This is a common refrain.

As Dr. Rivkees postulates, discomfort with genital anomalies will not fade-not among the parents, those affected or society. With that said, we owe it to the children to help them adapt to the life they will live-today- and that may include surgical reconstruction. We owe them the best quality surgical reconstruction available, and we must not be overly distracted by the pull from the far-left. Pursuing outcome research and designation of centers of excellence will help us move closer to that goal.