Utilization of a shared decision-making tool in a female infant with congenital adrenal hyperplasia and genital ambiguity

Abstract

Background Shared decision-making (SDM) is the process by which patients/families and providers make healthcare decisions together. Our team of multidisciplinary disorders of sex development (DSD) has developed an SDM tool for parents and female patients with congenital adrenal hyperplasia (CAH) and associated genital atypia. What is new Elective genital surgery is considered controversial. SDM in a patient with genital atypia will allow patient/family to make an informed decision regarding surgical intervention. Case presentation Our patient is a 2.5-year-old female with CAH and genital atypia. Initially, her parents had intended to proceed with surgery; however, after utilizing the SDM checklist, they made an informed decision to defer urogenital sinus surgery for their daughter. Conclusions We successfully utilized an SDM tool with parents of a female infant with CAH and genital atypia, which allowed them to make an informed decision regarding surgery for their daughter. Future directions include a prospective enrolling study to determine the generalizability and applicability of SDM with families of children diagnosed with CAH.

Dealing With Uncertainty and Lack of Knowledge in Diverse Sex Development: Controversies on Early Surgery and Questions of Consent

“… 60 , 62 , 63 Shared decision-making tools have also been developed for dsd conditions such as CAIS and CAH, and their generalizability and applicability need to be researched. 64 , 65 …”

“…60,62,63 Shared decision-making tools have also been developed for dsd conditions such as CAIS and CAH, and their generalizability and applicability need to be researched. 64,65 As we have to deal with controversies and knowledge gaps, matters of handling risk and uncertainty 66 and ways of dealing with specific dilemmas 41,67 play a crucial role. Thus, awareness of these issues should be raised.…”

Multidisciplinary teams caring for people with variations of sex characteristics: a scoping review

“…Out of the final 12 articles, 6 were theoretical, [11][12][13][14][15][16] 5 were empirical [17][18][19][20][21] and 1 was a mixed-method study. 9 Onethird (4) of the articles were published in the UK, 9 11 12 18 the other third in the USA 13 15 17 20 and the remaining third came from Switzerland and Germany, 21 Sweden and UK, 19 Australia 16 and Germany 14 (see tables 2 and 3).…”

“…In most studies, [11][12][13][14][15][16][17][18][19][20][21] the model of collaboration-multidisciplinary, interdisciplinary or transdisciplinarywas not explicitly mentioned. Still most of the papers seemed to indicate a multidisciplinary approach in MDTs described as the simultaneous but independent contribution of two or more team members.…”

“…In most empirical [17][18][19][20][21] and theoretical [11][12][13][14][15][16] articles the model of collaboration was not explicitly mentioned but most of the papers seem to indicate that MDTs take a multidisciplinary approach insofar the teamwork was described as the simultaneous and independent contribution of two or more team members. Only the mixedmethods study of Sanders and colleagues 9 included an interprofessional team approach where patients, parents and members of the MDT actively cooperate in the treatment process in order to cocreate knowledge and improve the care of people with VSC and help parents cope with their child's condition.…”