Beyond changing diapers: stress and decision-making among parents of girls with CAH seeking consultation about feminizing genital restoration surgery

Abstract Purpose The impact of having a child with atypical genitalia due to a life-threating chronic medical condition like congenital adrenal hyperplasia (CAH) is poorly understood. Our aim was to determine parental stress and impact of CAH on parental decisions, including decisions regarding female genital restoration surgery (FGRS). Methods We surveyed consecutive parents of girls with CAH ≤3 years old presenting at a tertiary referral center for FGRS consultation (2016-2019). The survey was developed by 3 families of daughters with CAH and 6 clinicians. Nine potentially stressful past experiences were rated on a 6-point Likert scale (“not at all” to “extremely” stressful). Overall parental stress and strain (broader negative consequences) were reported using validated instruments (PSS and CGSQ-SF7, respectively). Impact of CAH on past decisions about childcare, social interactions and who changes diapers were also assessed. Non-parametric tests were used for analysis. Results Twenty-nine parents (median age: 32years) of 22 consecutive children participated (Prader 3/4/5: 59.1%/36.4%/4.5%). After the study, 20 girls (90.9%) underwent FGRS at a median 8 months old. The most stressful experiences were: having an adrenal crisis (“very much” stressful), waiting for the CAH diagnosis and making sense of the diagnosis (both “quite a bit”) (Figure1). Remaining issues were “somewhat” stressful. Deciding whether to proceed with FGRS was ranked as the least stressful issue. Overall parental stress was similar to overall stress previously reported by spousal caregivers of stoke or heart failure survivors (p≥0.15). Overall parental strain was similar to parents of adolescents receiving mental health counseling (p=0.77). CAH impacted decisions about who changed diapers, babysitting, daycare and choosing a pediatrician (p≤0.02), but did not impact parental social interactions (p≥0.11). Diapers were typically changed by parents (100.0%) and grandmothers (50.0%). Parents anticipated that some individuals currently not allowed to change diapers would be allowed after FGRS: grandfathers (+18.2%), aunts/uncles (+27.3-32.8%), cousins (+18.2%) and family friends (+45.5%). Comment We present the first assessment of parental stress with respect to different aspects of care of a daughter with CAH. Larger studies are required to determine if the parental stress associated with these experiences varies over time and how these stressors rank relative to each other through the child’s development. Conclusions Parents experience multiple stressors after having a daughter with CAH. Parental stress surrounding a decision about FGRS appears less severe than events pertaining to the diagnosis and medical management of CAH. CAH impacts multiple parental decisions.