An integrative literature review of menstruation patterns in people with congenital adrenal hyperplasia

Abstract

Congenital adrenal hyperplasia (CAH) encompasses a range of inherited autosomal recessive enzyme conditions that impact steroid hormone production. This lifelong condition has associated health risks for some individuals, including adrenal crisis, and menstruation and fertility issues. Health literacy materials focused on the physical and pharmacological aspects of managing menstruation (i.e., treatment to initiate or maintain menses for those with CAH) are scarce. This article addresses this gap by exploring menses patterns in people with CAH. An integrative literature review searched articles published between 2005 and 2020, and 30 articles met inclusion criteria. Study findings indicated that while menarche seems to occur at an average age for people with CAH, menstruation is commonly disrupted and irregular. Several studies reported individuals achieving regular menses and pregnancy, while others noted individuals consistently showing poor control, irregular menses, and infertility despite ongoing treatment. A few studies reported such individuals as “non-adherent” or worse “non-compliant” with their medication regimens when health outcomes were lower than expected. We emphasize that such fault finding by health service providers around adherence acts as a barrier to communication with people with CAH. We argue that a sustained and coordinated person-centred approach focusing on communication and education across situational, developmental, and organizational transitions, can promote health literacy, foregrounding individuals’ health and bodily autonomy across their life span. Future research needs to address gaps in knowledge translation that supports menstrual education for individuals with CAH. Studies should also address how medication management is assessed and adherence negotiated with individuals with CAH.