Medical Mistrust Mediates the Relationship Between Nonconsensual Intersex Surgery and Healthcare Avoidance Among Intersex Adults.

BACKGROUND: Intersex individuals experience poor health due, in part, to healthcare avoidance. Nonconsensual intersex surgery may contribute to medical mistrust and avoidance among intersex populations. PURPOSE: The purpose of this study was to explore the relationship between nonconsensual surgery and healthcare avoidance among intersex populations and to examine if medical mistrust mediates this relationship. METHODS: Data for this cross-sectional study were collected in 2018 and analyzed in 2022. Participants completed a survey collecting information on demographics, medical mistrust, history of nonconsensual surgery, and history of postponing healthcare. One hundred nine participants with valid responses to all regression model variables were included in the study. Multivariable logistic regression models controlling for age, race, and income, examined the relationship between nonconsensual surgery and postponing preventive and emergency healthcare. Mediation analyses of cross-sectional data examined whether medical mistrust mediated the relationship between nonconsensual surgery and postponing preventive and emergency healthcare. RESULTS: Mean medical mistrust score was 2.8 (range = 1-4; standard deviation = 0.8), 49.7% of participants had nonconsensual surgery in their lifetime, 45.9% postponed emergency healthcare, and 61.5% postponed preventive healthcare in their lifetime. Nonconsensual surgery was associated with increased odds of delaying preventive (adjusted odds ratio [AOR] = 4.17; confidence interval [CI] = 1.76-9.88; p = .016) and emergency healthcare (AOR = 4.26; CI = 1.71-10.59; p = .002). Medical mistrust mediated the relationship between nonconsensual surgery and delaying preventive (indirect effect = 1.78; CI = 1.16-3.67) and emergency healthcare (indirect effect = 1.66; CI = 1.04-3.30). CONCLUSIONS: Nonconsensual surgery contributed to healthcare avoidance in this intersex population by increasing medical mistrust. To decrease healthcare avoidance, intersex health promotion interventions should restrict nonconsensual surgery and build trust through trauma-informed care.

Many intersex people experience nonconsensual surgery during childhood to alter their genitalia and other anatomy. Some intersex people who have experienced nonconsensual surgery develop subsequent mistrust in medical providers and avoidance of healthcare. The purpose of this study was to understand the relationship between nonconsensual surgery and delay in emergency and preventive healthcare among intersex adults. Additionally, this study aimed to understand whether mistrust in medical providers mediates the relationship between nonconsensual surgery and delaying emergency and preventive healthcare. This study found that ever having nonconsensual surgery was positively associated with delaying both emergency and preventive healthcare among intersex adults. Additionally, this study found that increased mistrust in medical providers mediated the relationship between nonconsensual surgery and delaying emergency and preventive healthcare. Interventions aimed at improving the healthcare engagement of intersex adults may focus on building trust between intersex patients and healthcare providers and restricting nonconsensual intersex surgeries.

A national study on the physical and mental health of intersex adults in the U.S.

OBJECTIVES: To describe the health of intersex adults (people with differences of sex development) in the U.S. using community-based research methods. METHODS: In July-September 2018, we conducted a national health study of intersex adults aged 18 and older in the U.S., using a survey hosted on Qualtrics. The study describes the physical and mental health experiences of intersex adults, including differences by age (18 to 39 vs. 40 and older). Questions were derived from national (Behavioral Risk Factor Surveillance System) and intersex-related health studies. RESULTS: A non-probability sample of 198 intersex adults completed the survey over three months. Over 43% of participants rated their physical health as fair/poor and 53% reported fair/poor mental health. Prevalent health diagnoses included depression, anxiety, arthritis, and hypertension, with significant differences by age. Nearly a third reported difficulty with everyday tasks and over half reported serious difficulties with cognitive tasks. CONCLUSIONS: To our knowledge, this is the first national study of intersex adults in the U.S. Greater understanding of intersex health over the life course is essential. Findings highlight the need for longitudinal studies and further examination of potential health disparities experienced by intersex populations.

A Management Protocol for Gonad Preservation in Patients with Androgen Insensitivity Syndrome.

Historically, individuals with androgen insensitivity syndrome (AIS) were managed with removal of gonadal tissue at various ages to avert the risk of gonadal malignancy. Recently, clinical practice changed, with gonadectomy being postponed until late adolescence. Adolescents and adults with complete AIS have questioned this approach. Additionally, testicular germ cell tumors are increasingly believed to be quite rare with rates as low as 0% in molecularly confirmed individuals with AIS. Gonadectomy deprives patients of the benefits of their endogenous hormones and potential fertility. Furthermore, human rights organizations advocate for deferring irreversible surgery in conditions known as differences of sex development, which includes AIS, to allow patient autonomy in decision-making. Recent literature supports an approach that uses risk stratification to manage gonads in AIS. Herein we review what is known about malignancy risk in the different subtypes of AIS and propose a management protocol for gonad retention.

Fertility Preservation for a Teenager with Differences (Disorders) of Sex Development: An Ethics Case Study.

Fertility preservation has become more common for various populations, including oncology patients, transgender individuals, and women who are concerned about age-related infertility. Little attention has been paid to fertility preservation for patients with differences/disorders of sex development (DSD). Our goal in this article is to address specific ethical considerations that are unique to this patient population. To this end, we present a hypothetical DSD case. We then explore ethical considerations related to patient's age, risk of cancer, concern about genetic transmission of a DSD condition to children, co-occurring gender dysphoria, and access to experimental fertility preservation procedures. Given the limitations of current technologies, we recommend offering fertility preservation to individuals living with DSD using an informed decision-making approach that instills realistic expectations and minimizes the potential for false hope. Finally, we conclude with practical recommendations for this case based on the ethical considerations.

The Ethics of Fertility Preservation for Pediatric Patients With Differences (Disorders) of Sex Development.

Differences (disorders) of sex development are diverse conditions with variations in chromosomal, gonadal, and/or genital development. Fertility potential in this population is variable. Recent investigations into fertility potential in those previously thought to be infertile suggest that the majority may have fertility potential through experimental protocols. Fertility preservation may be more successful if pursued in childhood. As fertility research and techniques advance, it is important to carefully consider pediatric ethical issues specific to this population, including gonadectomy, consent/assent, experimental treatment and false hope, cost and insurance coverage, genetic transmission to offspring, and gender dysphoria.

Ethical Controversy About Hysterectomy for a Minor.

One of the most complicated ethical issues that arises in children's hospitals today is the issue of whether it is ever permissible to perform a procedure for a minor that will result in permanent sterilization. In most cases, the answer is no. The availability of good, safe, long-acting contraception allows surgical options to be postponed when the primary goal of such surgical options is to prevent pregnancy. But what if a minor has congenital urogenital anomalies or other medical conditions for which the best treatment is a hysterectomy? In those cases, the primary goal of therapy is not to prevent pregnancy. Instead, sterility is an unfortunate side effect of a medically indicated treatment. Should that side effect preclude the provision of a therapy that is otherwise medically appropriate? We present a case that raises these issues, and asked experts in law, bioethics, community advocacy, and gynecology to respond. They discuss whether the best option is to proceed with the surgery or to cautiously delay making a decision to give the teenager more time to carefully consider all of the options.

Attitudes towards "disorders of sex development" nomenclature among affected individuals.

INTRODUCTION: Although now commonly used in medicine, the updated "disorders of sex development" (DSD) nomenclature formally introduced in 2006 has never been universally accepted by members of the affected community, particularly advocacy groups. Use of this nomenclature by medical professionals may unintentionally negatively affect access to healthcare and research for individuals with DSD conditions. OBJECTIVE: Among individuals affected by various DSD diagnoses, this study sought to (1) evaluate attitudes towards potentially controversial DSD terminology, (2) determine potential impact of terminology on how affected individuals access healthcare, and (3) explore alternate terms. STUDY DESIGN: A web-based survey was developed in collaboration with the AIS-DSDSG (Androgen Insensitivity Syndrome-DSD Support Group) leadership. AIS-DSDSG members (caregivers and affected individuals) were surveyed about attitudes towards DSD, potential impact on healthcare utilization, and alternate terms. A qualitative analysis of reasons for using/avoiding specific terms was performed. RESULTS: Surveys were completed by 202 out of 580 (35%) AIS-DSDSG members (61% affected, 39% caregivers; 16% non-gender binary; age range of affected individuals 0-86 years). Only 24% use disorder of sex development to describe themselves/their child. A majority (69%) had a negative emotional experience because of clinical use of nomenclature; 81% changed their care because of it. Preferred and non-preferred terms for clinical care and research are illustrated in the figure. Preferred diagnostic terms were intersex, variation in sex development, and difference of sex development (55%, 52%, and 50% liked/strongly liked, respectively). Disorder of sex development was not preferred (17% liked/strongly liked). About one-third reported that they would not attend a clinic named the Disorder of Sex Development Clinic. Overall, 81% provided qualitative comments; flexible terminology use was a key theme. DISCUSSION: These study findings are consistent with previous studies that demonstrated negative perceptions of DSD nomenclature. This study adds to previous findings by surveying a large group of affected individuals with a range of diagnoses, and by exploring emotional impact and healthcare utilization. Several possible alternative terms were also defined. The study was limited by inclusion of only members of AIS-DSDSG, a convenience sample where complete AIS is over-represented, and whose views may not represent the opinion of all individuals with DSD conditions. CONCLUSIONS: A group of affected individuals and parents have negative views about the DSD terminology commonly used by medical professionals. Use of certain terms may affect the choice of healthcare provider/institution. Evaluation of DSD terminology in other affected individuals, and re-evaluation of current nomenclature, in collaboration with advocates, is needed.

Disorders of sex development peer support.

The 2006 Consensus Statement on Management of Intersex Disorders describes peer support as integral to a comprehensive model of care for disorders of sex development (DSD). Affected adults and families look to peer support groups (PSG) for informational, emotional and social support to strengthen coping and assist with the process of shared and informed decision making. Peer support for DSD is relatively new and much can potentially be learned from studies examining the relationship between PSG characteristics and their benefits in other medical conditions. Healthcare providers' awareness of and attitudes toward PSG can influence the degree to which families value such support. This chapter begins with a brief history of peer support for DSD, followed by a summary of the evidence-based literature on PSG across varied medical conditions. We then summarize findings from a recently conducted poll of key DSD peer support and advocacy organizations. The chapter concludes with recommendations for further development of DSD-specific PSG, opportunities for more complete integration of peer support in the model of healthcare and the advantages of input of patient stakeholders in establishing clinical research priorities.

Guidelines for the Development of Comprehensive Care Centers for Congenital Adrenal Hyperplasia: Guidance from the CARES Foundation Initiative.

Patients with rare and complex diseases such as congenital adrenal hyperplasia (CAH) often receive fragmented and inadequate care unless efforts are coordinated among providers. Translating the concepts of the medical home and comprehensive health care for individuals with CAH offers many benefits for the affected individuals and their families. This manuscript represents the recommendations of a 1.5 day meeting held in September 2009 to discuss the ideal goals for comprehensive care centers for newborns, infants, children, adolescents, and adults with CAH. Participants included pediatric endocrinologists, internal medicine and reproductive endocrinologists, pediatric urologists, pediatric surgeons, psychologists, and pediatric endocrine nurse educators. One unique aspect of this meeting was the active participation of individuals personally affected by CAH as patients or parents of patients. Representatives of Health Research and Services Administration (HRSA), New York-Mid-Atlantic Consortium for Genetics and Newborn Screening Services (NYMAC), and National Newborn Screening and Genetics Resource Center (NNSGRC) also participated. Thus, this document should serve as a "roadmap" for the development phases of comprehensive care centers (CCC) for individuals and families affected by CAH.