Browsing by Author "Frady, Heather"

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    Growing up with clitoromegaly: Experiences of North American women with congenital adrenal hyperplasia
    (Elsevier, 2022-12) Szymanski, Konrad M.; Kokorowski, Paul; Braga, Luis H.; Frady, Heather; Whittam, Benjamin; Hensel, Devon J.; Life with Congenital Adrenal Hyperplasia Study Group; Urology, School of Medicine
    Introduction To describe experiences of clitoromegaly in women with congenital adrenal hyperplasia (CAH). Methods CAH females (46XX, ≥16 years old) from the United States and Canada were eligible for a cross-sectional online survey (2019–2020) if reporting clitoromegaly (life-long: “growing up with a larger than average clitoris,” secondary: “clitoris grew over weeks or months”). A multidisciplinary team and women with CAH drafted questions assessing net effects of clitoromegaly on 10 activities and 10 life domains. Fisher's exact test was used to compare net effect (positive-negative) vs. no effect (Bonferroni p = 0.05/10 = 0.005). Results Of 97 women with CAH enrolled, 53 women (55%, median age: 36 years, advocacy group recruitment: 81%) reported recognizing clitoromegaly at median 11–13 years old, with 21% identifying it in adulthood. There was no difference in self-reported timing or clitoral shape between life-long or secondary clitoromegaly (p ≥ 0.06). There were no net positive effects of clitoromegaly. Rather, clitoromegaly had net negative effects on 7/10 activities (p ≤ 0.003) and no net effect (neutral) on 3 (Table). Women were less likely to wear tight clothing, change clothes in public locker rooms and play group sports. Women reported net negative effects for most romantic activities (dating, any sexual activity, pain-free sexual activity, having a partner see their genitalia, p=<0.003), but did not report a net effect on pleasurable sexual activity (p = 0.12). Clitoromegaly had net negative effects in 9/10 life domains (p < 0.001) and neutral on job self-perception (p = 0.25). Few women reported any positive impact (2–6%). However, 49–59% of women experienced poor self-esteem, anxiety, gender self-perception and body image, while 36% felt “down or depressed.” Also, 21–23% experienced negative self-perception as friends and parents, 42–47% reported negative effects on plans for romantic and sexual relationships. Responses did not differ with advocacy group membership (p ≥ 0.02). Discussion Our findings support qualitative and case series evidence that clitoromegaly has a negative psychological outcome on women with CAH. Clitoromegaly may add to the burden of living with a chronic endocrine disease. Women with positive and negative experiences had the same opportunity to participate. Since we could not assess objective clitoral size, baseline virilization and exact nature of any childhood clitoral procedures, these data cannot be used to estimate the impact of specific clitoral size or effectiveness of early clitoral treatments. Conclusions Clitoromegaly appears to be common among women with CAH. While experiences of clitoromegaly vary between women, the overall experience is negative in multiple social, romantic, and emotional activities and domains.
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    Validation and Preliminary Results of the Parental Assessment of Children's External Genitalia Scale for Females (PACE-F) for Girls With Congenital Adrenal Hyperplasia
    (Elsevier, 2019) Szymanski, Konrad M.; Whittam, Benjamin; Monahan, Patrick O.; Kaefer, Martin; Frady, Heather; Cain, Mark P.; Rink, Richard C.; Urology, School of Medicine
    Objective To validate a parental assessment of children's external genitalia scale for females (PACE-F) for girls with congenital adrenal hyperplasia (CAH) by adapting the validated adult female genital self-image scale. Methods PACE-F was administered to parents of girls (Tanner 1, 2 months-12 years) with and without CAH. Final questions were determined by clinical relevance and psychometric properties (scores: 0-100). A reference range was established using 95% confidence interval among controls. Age-matched controls were compared to girls with CAH (1) <4 years old before and after female genital reconstruction surgery (FGRS), and (2) 4-12-year olds after FGRS. Nonparametric statistics were used. Results Participants included 56 parents of 41 girls with CAH (median 3.9 years old, 97.6% FGRS) and 139 parents of 130 girls without CAH. Face and content validity was established by families, experts, and factor analysis. Internal consistency was high (Cronbach's alpha: 0.83). Population reference score range was 66.7-100. Ten consecutive girls had pre- and post-FGRS PACE-F scores. All scores improved at 4 months after surgery and all preoperative scores were below reference range and lower than controls (P = .0001). All postoperative scores were within reference range, no different from controls (P = .18). Scores for girls with CAH after FGRS aged 4-12 years were no different from controls (100.0 vs 88.9, P = .77) and 90.0% were in reference range, as expected (P = .99). Conclusion We present a validated instrument for parental assessment of genital appearance in girls with CAH. We demonstrate improved parent-reported appearance after FGRS, with scores similar to age-matched controls.