Sexuality and disability: Mayer-Rokitansky-Küster-Hauser Syndrome

Abstract

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, also known as Müllerian agenesis, is a rare congenital malformation of the genital tract characterised by a missing uterus and vagina in women with normal female phenotype. It is believed to arise from a multifactorial polygenic aetiology that causes inadequate fusion of the Müllerian duct during embryo development. MRKH is divided into type I, with only vaginal aplasia, and type II, associated with other malformations of the renal and skeletal systems. This anatomic anomaly results in difficulties having sexual relations, infertility, emotional burden, identity issues, confusion regarding social and sexual roles and negative self-image. The psychosocial impact of being diagnosed with this syndrome requires psychological and pharmacological support, as well as guidance systems for the woman and her family, given the repercussions on many facets of the patient’s life. We are presenting a study using the life-history method in order to understand the experience of a woman with type-II MRKH by integrating elements of her past that are deemed relevant to describe, understand or represent her current situation with regard to her expectations for the future. The biographic-narrative method was chosen as the most appropriate for this study. To draft the narrative, we conducted semi-structured interviews and analysed autobiographical stories, medical charts and school reports. The analysis used units of meaning to organise and classify the information for interpretation. The results confirm the impact on women with strong repercussions on sexual development, which implies a need to continue studying the unfinished task of sexual education with universal access to comprehensive education on sexuality.