Psychosexual development and quality of life outcomes in females with congenital adrenal hyperplasia

Abstract

Background/AimsOutcome information regarding females with classical congenital adrenal hyperplasia (CAH) have generally suggested poor quality of life (QoL), general maladjustment, problems regarding sexuality, and decreased fertility. The aim of this study was to assess QoL, psychosocial adaptation, and psychosexual characteristics, includingchildhood gender role behavior, gender identity, and sexual orientation in females with CAH.MethodsFemale patients with 21-hydroxylase deficiency CAH were evaluated using a questionnaire with items relating to knowledge of their condition and its therapy; consistency of medical, surgical, and psychological care; childhood friends and play behavior; and genital, pubertal, and sexual development. The subjects’ perception of outcome was compared with family support and adolescent and adult QoL perspectives, including social relationships, self and body image, and gender and sexual issues.ResultsChildhood play and gender characteristics, childhood and adult genital perception, and sexual identity and orientation varied as previously reported. However, most patients indicated good family support, understanding of their condition, good quality medical care, positive self-satisfaction, indices of happiness and body image perception, and satisfaction with their sex lives.ConclusionThe data reported here suggest that overall outcome can be very good for females with CAH and that good outcome appears to relate to quality of care and positive social support.Electronic supplementary materialThe online version of this article (doi:10.1186/s13633-015-0017-z) contains supplementary material, which is available to authorized users.

Experiences of people living with disorders of sex development and sex reassignment: Meta‐ethnography of qualitative studies

“…Many patients experience poor intimate relationships due to the fear of rejection, stigmatization, and bullying (Cohen‐Kettenis, ). The difficulty in sexual intercourse was due to physical or psychological barriers (Cohen‐Kettenis, ; Kanhere et al., ).…”

Cognitive abilities in women with complete androgen insensitivity syndrome and women with gonadal dysgenesis

“…For adults, the outcome results are mixed. Some reports show unaffected psychological adjustment measured by self-assessment instruments, (158,159) other report impaired QoL and wellbeing (133) for patients with CAH. Health related QoL outcome domains that are most often affected is general health and vitality (160).…”

Mental health of a large group of adults with disorders of sex development in six European countries

“…The lowered satisfaction was mostly attributed to low breast volume. In one study, women with CAH expressed complaints about lack of breast development (Kulshreshtha et al, 2012), albeit in a sample with significant delay of adequate treatment, and in another study 35% of women reported they wanted larger breasts (which was more frequently reported than dissatisfaction with genital appearance; 26%) (Kanhere et al, 2015). One-third of the women surveyed with CAH and 46,XY karyotype reported breast dissatisfaction, which was more prevalent than in the matched controlled sample (Ediati et al, 2015).…”

“…With regard to breast satisfaction, even fewer studies have been published (Migeon et al, 2002;Bannink et al, 2006;Bartolin et al, 2012;Kulshreshtha et al, 2012;Ediati et al, 2015;Kanhere et al, 2015;van de Grift et al, 2018). In both males and females with DSD, breast satisfaction has been observed to be lower than control values (Ediati et al, 2015).…”