Alice Dreger, Ellen K Feder and Anne Tamar-Mattis write in the Journal of Bioethical Inquiry, 30 July 2012, on the “use of dexamethasone in pregnant women at risk of carrying a female fetus affected by congenital adrenal hyperplasia (CAH)” in the West.
To our eyes, this is not just an intersex issue but an LGBTI issue: it is the physical and behavioural masculinisation, or virilisation, of women that is of concern to those proposing dexamethasone use. Further, preventing homosexuality and physical masculinisation is considered to be of greater benefit than the established cognitive and physical risks to treated children.
The authors focus on research on dexamethasone use by Dr Maria New, pediatric endocrinologist at the Mount Sinai School of Medicine in the US, who has received grant support from the US National Institutes of Health:
Surprisingly, results from our Freedom of Information Act (FOIA) requests … indicate that the U.S. National Institutes of Health (NIH) have funded New to see whether prenatal dexamethasone “works” to make more CAH-affected girls straight and interested in having babies.
What is CAH and dexamethasone?
The authors summarise CAH and treatment with dex as follows:
Congenital adrenal hyperplasia (CAH) is a disease of the endocrine system that can cause virilization (i.e., development of masculine traits) in female fetuses. In an attempt to prevent CAH-affected female fetuses from developing in a sexually atypical fashion, some physicians treat pregnant women “at risk” for having an affected daughter with the steroid dexamethasone. This intervention starts as soon as pregnancy is confirmed and continues throughout the pregnancy if the fetus is ultimately diagnosed as a CAH-affected female.
Treatment has become routine in many jurisdictions:
This use of dexamethasone was first described in 1984 in The Journal of Pediatrics by Michel David and Maguelone Forest, French clinician-researchers… A 2000–2001 survey of members of the European Society for Paediatric Endocrinology, representing 125 institutions, found that, “[i]n 57 % of the centres prenatal diagnosis and treatment [of CAH with dexamethasone] are routine.”
Rationales for treatment
Rationales for treatment are described as follows, from a paper in the 2010 Annals of the New York Academy of Sciences:
Without prenatal therapy, masculinization of external genitalia in females is potentially devastating. It carries the risk of wrong sex assignment at birth, difficult reconstructive surgery, and subsequent long-term effects on quality of life. Gender-related behaviors, namely childhood play, peer association, career and leisure time preferences in adolescence and adulthood, maternalism [interest in being a mother], aggression, and sexual orientation become masculinized in 46,XX girls and women with 21HOD deficiency. … Genital sensitivity impairment and difficulties in sexual function in women who underwent genitoplasty early in life have likewise been reported. We anticipate that prenatal dexamethasone therapy will reduce the well-documented behavioral masculinization and difficulties related to reconstructive surgeries (Nimkarn and New 2010a, 9).
Dreger, Feder and Tamar-Mattis note that Dr Maria New, in a meeting of the CAH-diagnosis group “CARES Foundation”, displayed a photo “of a girl with ambiguous genitalia and said”:
The challenge here is … to see what could be done to restore this baby to the normal female appearance which would be compatible with her parents presenting her as a girl, with her eventually becoming somebody’s wife, and having normal sexual development, and becoming a mother. And she has all the machinery for motherhood, and therefore nothing should stop that, if we can repair her surgically and help her psychologically to continue to grow and develop as a girl (New 2001a).
Other notable medical practitioners are also involved in this research and these justifications. Heino Meyer-Bahlburg of Columbia University is a member of the American Psychiatric Association working group on revisions to the (pathologising) Diagnostic and Statistical Manual on Mental Disorders (DSM-5) and a member of the “Standards of Care Revision Committee” of the World Professional Association for Transgender Health. In his 1999 paper, “What Causes Low Rates of Child-Bearing in Congenital Adrenal Hyperplasia?“, Meyer-Bahlburg noted:
CAH women as a group have a lower interest than controls in getting married and performing the traditional child-care/housewife role. As children, they show an unusually low interest in engaging in maternal play with baby dolls, and their interest in caring for infants, the frequency of daydreams or fantasies of pregnancy and motherhood, or the expressed wish of experiencing pregnancy and having children of their own appear to be relatively low in all age groups.
Risks of treatment
The authors discuss the outcomes of research in Sweden which has led to the discontinuation of dexamethasone use there: cognitive and behavioural issues in CAH-unaffected kids, and less masculinity in treated boys:
When compared to controls, “[i]n general, treated children were born at term and were not small for gestational age. As a group, they did not exhibit teratogenous effects/ gross malformations, although eight severe adverse events were noted in the treated group, compared with one in the control group. Three children failed to thrive during the first year of life; in addition, one had developmental delay and hypospadias; one had hydrocephalus; two girls were born small for gestational age, and one of these girls was later diagnosed with mental retardation; and one child had severe mood fluctuations that caused hospital admission. In the control group, only one child was admitted because of Down’s syndrome …
[a]n adverse effect was observed in the form of impaired verbal working memory in CAH-unaffected short-term-treated cases [i.e., the children who were not the intended targets of the intervention]. The verbal working memory capacity correlated with the children’s self- perception of difficulties in scholastic ability, another measure showing significantly lower results in CAH-unaffected, DEX-exposed children. These children also reported increased social anxiety. In the studies on gender role behavior, we found indications of more neutral behaviors in DEX-exposed boys (Hirvikoski et al. 2012, 2).
Despite these published, reported risks, dexamethasone treatment is being sold to parents of Dr Maria New in the US as “safe and effective”.
“An Ethics Canary in the Modern Medical Mine”
The authors note the similarities between the case of Dex and DES, describing use of dexamethasone as “canary in the modern medical mine”, stating that their “investigations indicate major gaps in the systems meant to protect subjects of high-risk medical research”:
It has been impossible for us not to be aware that, as we have researched this fetal intervention, the medical world has been marking the 40th anniversary of the 1971 publication of a study reporting a relatively high number of occurrences of a rare vaginal cancer in girls and young women who had been exposed in utero to diethylstilbestrol (DES)…
Three striking differences between these interventions are that: (1) this poorly studied yet widespread use of prenatal dexamethasone is happening after the lessons supposedly learned from DES; (2) while DES was never intended to alter fetal development, prenatal dexamethasone for CAH has explicitly aimed to do so; and (3) while DES was aimed at preventing fetal death, dexamethasone is directed at preventing something we would hope most people would understand to be substantially less dire, namely the development of atypical sex.
Yet rather than suggesting that the case of prenatal dexamethasone for CAH should be understood as one of the “big” stories of the history of medicine (like DES), we are suggesting something more disturbing: that this case appears to be representative of problems endemic in modern medicine, problems that threaten the health, lives, and rights of patients who continue to become unwitting subjects of (problematic) medical experimentation. Because so many systems of protection appear to have failed these women and children, we fear that prenatal dexamethasone for CAH is a canary in the modern medical mine.
To OII Australia, this is not just an issue for CAH women, or intersex people more broadly, it is a critically important LGBTI health issue.
- Journal of Bioethical Inquiry, Online First™, 30 July 2012, “Prenatal Dexamethasone for Congenital Adrenal Hyperplasia, An Ethics Canary in the Modern Medical Mine” web page
- Alice Dreger, Ellen K. Feder and Anne Tamar-Mattis, “Prenatal Dexamethasone for Congenital Adrenal Hyperplasia, An Ethics Canary in the Modern Medical Mine” – open access, full PDF
- Press release at Northwestern Unversity News Center