Psychosocial well-being in Dutch adults with disorders of sex development

https://doi.org/10.1016/j.jpsychores.2016.03.005Get rights and content

Highlights

  • Dutch patients with DSD reported good psychosocial well-being and self-esteem

  • Men and women were coping well with DSD

  • Women born with ambiguous external genitalia reported more self-isolation

  • These women scored in masculine ranges on antisocial personality problems

  • All women experienced fatigue and concentration problems

Abstract

Objective

Atypical sex development is associated with psychosocial vulnerability. We investigated psychosocial well-being in individuals with disorders of sex development (DSD) and hypothesized that psychosocial well-being was related to degree of genital atypicality at birth.

Methods

120 male (n = 16) and female (n = 104) persons with DSD, aged 14–60 years, participated in a follow-up audit on psychosocial well-being. They were stratified in: women with 1) 46,XY and female genitalia, 2) 46,XY or 46,XX and atypical genitalia, and 3) men with 46,XY and atypical genitalia. We used the Illness Cognition Questionnaire (ICQ), Checklist Individual Strength (CIS8R), TNO-AZL Quality of Life questionnaire (TAAQOL), Adult Self-Report (ASR), and the Rosenberg Self-Esteem Scale (RSES).

Results

Data were compared to reference groups. Participants generally were coping well with DSD (ICQ). Women with DSD reported elevated levels of fatigue (CIS8R) and slightly more attention and memory problems (TAAQOL, ASR). Women with atypical genitalia reported more emotional and behavioral problems. On the ASR Rule-breaking Behavior and Antisocial Personality scales, these women had similar scores as reference men. Women with DSD reported a higher self-esteem (RSES). No differences in psychosocial well-being were found between men with DSD and reference men.

Conclusion

Individuals with DSD across all diagnostic groups generally reported a good psychosocial well-being. The results further suggest involvement of prenatal androgens in the development of personality traits related to assertiveness and egocentricity. We recommend that individuals with a DSD and their families are involved in decision-making processes and have access to multidisciplinary care.

Introduction

In individuals with disorders of sex development (DSD) the development of chromosomal, gonadal, and/or anatomic sex is atypical [1]. It is assumed that this incongruence puts them in a vulnerable position in society [2]. Current clinical management strategies therefore will include advice for early gender assignment, genital corrections, and hormonal treatments [1]. Lately, these early interventions have raised debate: it has been argued that they reflect society's intolerance to variance in sex and gender and major decisions are made without consent of children themselves [3], [4], [5], [6]. It has been suggested that postponement of gender assignment and genital surgery until the child is old enough to decide him/herself will benefit the child's well-being [7]. Randomized controlled comparison of the current treatment policy and the policy of delayed interventions is highly valued [8] but is difficult to conduct. The majority of parents living in Western countries choose gender assignment and genital surgery in early childhood [9], [10], [11].

Outcome studies on psychosocial well-being have been conducted. Due to differences in applied methodology and measures, findings are difficult to compare and show inconsistencies. These studies have mainly been carried out in females and focused on gender identity [12], [13], [14], [15], [16], [17], [18], sexual quality of life [19], [20], [21], [22], [23], and (psycho)sexual functioning [24], [25], [26], [27], [28], [29], [30], [31], [32], while studies on quality of life [33], social participation, self-esteem, and emotional problems are scarce. Studies addressing health related quality of life (HRQoL), emotional distress, and psychopathology in women with 46,XX congenital adrenal hyperplasia (CAH) revealed inconclusive outcomes, from reduced to a better HRQoL [20], [34], [35], [36], and from no substantial emotional distress to increases in emotional problems [28], [37], [38], [39], [40]. Women with complete androgen insensitivity syndrome (CAIS) reported to function psychologically well or even better than reference groups [20], [41]. In individuals with partial androgen insensitivity syndrome (PAIS), disorders in the biosynthesis of androgens, or gonadal dysgenesis, mental health problems have been reported [42], but findings had not been replicated in another study [16].

A few studies have been conducted in men and focused on sexual functioning [29]. Men with 46,XY CAH suffer from adrenal problems and testicular adrenal rest tumors and its consequences [43], [44]. These men reported more negative emotions [45], anxiety and depression [40], and psychiatric morbidity [46], [47]. Impairments in subjective health status have been reported [40], [48], but also a favorable health status compared to the general population [36].

In DSD there is a great variety in genital development between and within different diagnostic groups. In the current study we evaluated psychosocial well-being in relation to gender of rearing and degree of genital atypicality in Dutch individuals with DSD. In their prenatal development, persons with DSD have been exposed to atypical levels of androgens. We hypothesized that persons with DSD who underwent an atypical prenatal development leading to physical atypicality are more vulnerable to experiences that negatively affect their psychosocial well-being compared to persons with typical female or male genitalia [2].

Section snippets

Study design

The present study was embedded in a national follow-up audit on sexual well-being, gender identity development, and psychosocial well-being in persons with DSD [24], [25], [29], [30]. The study protocol was in line with the World Medical Association declaration of Helsinki and was approved by the boards of the ethical committees of the three medical centers that joined the study [49]. Data collection was carried out between 2007 and 2012. The Dutch patient support groups were involved in the

Statistical analysis

Before statistical analyses we evaluated the internal consistencies of the abovementioned measures for our sample using Cronbach's alpha. For most questionnaires, internal reliability values were good (Cronbach's α above .70). For three ASR-scales, internal reliability values were acceptable (Rule-breaking Behavior, Thought Problems and Antisocial Personality Problems; Cronbach α's respectively .64, .66, and .66).

For categorical variables, between-group differences were tested using Chi-square

Participant characteristics

Table 2 summarizes socio-demographic characteristics. Groups did not differ in median age (p = .49), ancestry (p = .09), marital status (p = .27), educational level (p = .52) or vocational status (p = .65).

Reasons for non-response remained unknown; most no-participation forms were not returned or were not filled out completely. Responders and non-responders did not differ with respect to diagnosis, medical treatment, age, living in urban or rural areas, or medical center [25], [29], [30].

Illness cognitions

Details on

Discussion

This study aimed to investigate psychosocial well-being in Dutch individuals with DSD. Overall, we conclude that participants reported good psychosocial well-being; they generally reported a good HRQoL, no serious emotional problems, a high self-esteem, and seemed to cope well with DSD compared to reference groups. In a recently published review [67] on this subject, seven out of 10 studies reported a (mildly) affected psychosocial well-being, in three studies such changes were not reported.

Conflicts of interest

The authors declare that there are no conflicts of interest.

Acknowledgements

We thank all participants who volunteered in this study for their carefulness and efforts. We thank Jan van der Ende and Erwin Birnie for statistical advices. We thank Ingrid van Slobbe, Joke Dunk, Karen Kwak, Jacqueline Knol, and Hanneke Kempes for their assistance in carrying out the study.

This study has been supported by the Edli Fund (Arianne B. Dessens), FWO Flanders Fund for scientific research (Nina Callens), and Fonds Swart – van Essen (Nita G.M. de Neve –Enthoven).

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