What Do Patients and Parents Say About Parents' Ability to Choose Early Genital Surgery for Girls with Congenital Adrenal Hyperplasia?

PURPOSE: We assessed opinions of females with congenital adrenal hyperplasia and their parents about the parent's ability to choose early genital surgery for these patients.

MATERIALS AND

METHODS

We conducted an online survey of females with congenital adrenal hyperplasia (46XX,16+ years old) and independently recruited parents (2019-2020) diagnosed in first year of life in the United States. A multidisciplinary medical team, women with congenital adrenal hyperplasia and parents drafted the survey. Fisher exact test was used.

RESULTS

Of 57 females with congenital adrenal hyperplasia (median age 39 years), 93.0% underwent genital surgery (median 1-2 years old). Most females (79.0%) believed legislation prohibiting surgery in childhood would cause harm. Most (64.9%) believed a ban "would have been harmful to me" (24.6% not harmful, 10.5% neutral). Most females (70.2%) believed a ban undermined parental rights to make medical decisions in their child's best interest. While 75.4% did not believe a ban was in the best interest of females with congenital adrenal hyperplasia, 14.0% did (10.5% neutral). For 132 parents of females with congenital adrenal hyperplasia (parent/child median age 40/11 years), 78.8% of children underwent surgery (median <1 year old). Most parents (93.9%) believed legislation prohibiting surgery in childhood would cause harm. Most (77.3%) believed a ban "would have harmed my daughter" (12.1% no harm, 5.3% neutral, 5.3% no answer). Parents were more likely than females with congenital adrenal hyperplasia to oppose a ban (p ≤0.02). Most parents (90.9%) believed a ban undermined parental rights. While 93.9% did not believe a ban was in the best interest of females with congenital adrenal hyperplasia, 3.8% did (2.3% neutral).

CONCLUSIONS

The majority of females with congenital adrenal hyperplasia and their parents support the parents' ability to decide about potential genital surgery, opposing moratoria on surgery in childhood.