Patient discussions
Patients taking hydrocortisone should continue treatment with hydrocortisone. If adverse effects or questions arise, consultation with a physician is vital before stopping or changing medication. Withdrawal effects may be severe. Magic Foundation: congenital adrenal hyperplasia Opens in new window Congenital Adrenal Hyperplasia Research Education & Support (CARES) Foundation Opens in new window
Patients should be instructed on how and when to increase glucocorticoid dose in times of stress.
Genetic counseling should be provided by medical professionals familiar with congenital adrenal hyperplasia.[14] Patients should be encouraged to discuss pregnancy plans with their endocrinologists, since medication changes may be necessary.[14]
Feminizing genitoplasty
Surgical feminization is usually performed early in life. It involves correction of the urogenital to provide a normal vaginal orifice that functions adequately for menstruation, intromission, and delivery and/or clitoroplasty. However, this is an area of controversy and active debate given the young child’s inability to consent or express desire for such procedures. Some centers will not perform the elective procedures until the age of consent. Recent review of practices shows an increase in conservative approach.[40] Clinical guidelines recommend discussing the pros and cons of surgery and time of the procedure with parents. Patients who had genitoplasty in early childhood will need a repeat urology evaluation in late adolescence to determine if additional procedures, such as vaginal dilations, are needed.
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