Psychological Aspects of the Treatment of Patients With Disorders of Sex Development

David E. Sandberg, Ph.D.; Melissa Gardner, M.A.; Peggy T. Cohen-Kettenis, Ph.D.

Disclosures

Semin Reprod Med. 2012;30(5):443-452. 

In This Article

Adolescence

In adolescence, questions may emerge or re-emerge about gender identity, gender re-assignment, and surgical intervention (e.g., some may need to know why they have to dilate the vagina or have to make decisions about genital surgery). The sharing of more detailed information with the child is an imperative during this stage which leads to transition to adult care and the need for the DSD-affected person to take responsibility for their own healthcare. Sexuality, sexual orientation and fertility are important and sensitive topics that emerge during this phase.

Gender Reassignment

In transgender (i.e., physically typical at birth) adolescents and adults experiencing gender dysphoria, the gender reassignment decision is taken in phases. In the first, diagnostic phase, it has to be established that the person fulfills DSM or ICD criteria for the diagnoses "gender identity disorder" or "transsexualism" and that the person is physically and psychologically ready to undergo gender reassignment. The next phase usually consists of receiving hormones of the desired gender and social transitioning. Undergoing surgery to change the genitals and sometimes other somatic sex characteristics is typically the last phase of the treatment. In some cases, however, the sequence may be changed, depending on the needs of the individual (e.g., large breasts of a person who is going to live in the male role). In case of gender dysphoric individuals with DSD, specific attention has to be paid to limitations of the treatment. In cases of female-reared individuals who choose to live as males, the hormones may not be completely virilizing and previous operations may limit surgical options. If the person is confused about their gender or seeks gender reassignment for reasons other than a gender identity that does not match their assigned gender, psychological interventions in lieu of hormonal or surgical gender reassignment are indicated. In either case, persons with DSD will benefit from the opportunity to engage in psychological counseling to address commonly experienced apprehensiveness over the ability to form romantic and satisfying sexual relationships.

A very useful part of the gender reassignment procedure is what was previously called the "real-life experience" phase. In this phase, one lives full-time in the desired gender role to tryout, on a daily basis, what it is like to live in the desired gender before one makes irreversible change.[64] In this phase the person's feelings about the social transition, including coping with the responses of others, is a major focus of the discussions and counseling.

In gender dysphoric adolescents without DSD, one option is to suppress puberty by using gonadotropin-releasing hormone analogues. This reversible treatment is meant to provide the adolescent time to consider gender reassignment without experiencing development of secondary sex characteristics (see[64] for a review). In contrast to these adolescents, agonadal adolescents with DSD do not need suppression of puberty. If they are gender dysphoric but not yet certain on whether want to pursue a gender reassignment, they may profit from a delay in the start of hormone replacement therapy.

Feminizing/masculinizing hormone therapy (estrogens/anti-androgens/progestins for male-to-females, androgens for female-to-males) is considered only partially reversible, as some of the changes persist even if hormone therapy is discontinued. Some changes (breast growth in natal males, low pitched voice and facial hair growth in natal females) require surgery or other treatment to "reverse." Feminizing/masculinizing hormone therapy typically does not begin until a gender-dysphoric adolescent without DSD is 16 years or older.[64] Because of the differences between DSD and non-DSD conditions, and the wide variation in DSD conditions, general age requirements for initiating these hormones do not exist for adolescents with DSD who seek to change gender.

Gender reassignment surgery is rarely performed prior to adulthood in non-DSD children and adolescents. There is international clinical consensus that the risks of early surgical intervention outweigh the potential benefits in most cases. Surgical feminization/masculinization procedures are explained in detail in a document, "Care of the Patient Undergoing Sex Reassignment Surgery."[64] Again, considering the differences between the conditions, this age restriction is not necessarily directly applicable to adolescents with DSD.

Sexuality

When entering puberty, some adolescents with DSD may develop anxieties. Repeated genital exams and medical photography, treatment by clinicians experienced as disrespectful, or an atypical genital appearance are particularly anxiety provoking. After entering puberty, some feel increasingly uncertain about their masculinity/femininity, sexual adequacy, or sexual orientation. They often postpone initiating intimate relationships because of such insecurities and fear of rejection. Sexual problems indeed occur more often in DSD than non-DSD groups.[64,71,72] For instance, the sexual lives of women with CAH differ from control groups in terms of timing of psychosexual milestones (delayed), sexual experiences (less), sexual activity and imagery (less), sexual motivation (less), partnership and marriage (less), and sexual self-image (less favorable)[73–75] and the sexual orientation of women with CAH is more often homosexual as compared with the general population.[75,76] Various sexual problems and an elevated percentage of non-heterosexuality were reported in women with enzyme deficiencies,[72] but systematic studies in larger groups are lacking. In one study, sexual problems, primarily low sexual desire and inability to become sexually aroused and experience orgasm, were reported by women with CAIS, whereas most women with PAIS feared to have sexual contact and had experienced dyspareunia or fear of becoming hurt through sexual contact. In the latter group, an elevation of non-heterosexuality was also found.[72]

Comprehensive sex education together with timely preparation for romantic and sexual relationships can contribute to a positive HRQoL. Adolescents should have the opportunity to discuss their concerns repeatedly, and in private, with a mental health clinician.

processing....