Standards of Care for Congenital Adrenal Hyperplasia: a Call for Change in the Caribbean Region

Abstract

The prevalent policy is that 46, XX children born with congenital adrenal hyperplasia (CAH) should be assigned to the female sex given their potential reproductive capacity. The parsimonious argument is that the scarcity of long-term outcome studies with regard to gender identity and overall sexual health among these individuals does not warrant a moratorium to current medical practices. Nevertheless, new propositions suggest that clinical sex assignment at birth does not necessarily need to follow traditional biological criteria to define sex, such as karyotype, gonads, hormonal profile, and genital appearance. In agreement with a number of international organizations, the Caracas Consensus Group (2012) has taken the position that gender non-conformity among individuals born with genital variance can be an iatrogenic artifact. We present data on ten adolescent and adult 46, XX individuals with confirmed CAH diagnosis who were born, raised, and managed in Cuba or Puerto Rico. Given the lack of international agreement and based on long-term outcome data on gender expressions among 46, XX CAH individuals, we advocate for a Caribbean social policy that prioritizes the right to live gender as chosen by individuals born with genital variance.