Ghost in the Machine

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Once you label me, you negate me
Søren Kierkegaard

We know nothing of Joseph Marzo in life, so it is impossible to know what they would have ever thought of the declaration made after their death that they were “una donna” – a woman. They died after another episode of the vomiting and diarrhea that they struggled with throughout their life. Marzo lived their entire life as a man, to the extent they are reputed to have contracted what was colloquially known in 1865 as ‘the French Disease’ – Syphilis: twice.

Joseph Marzo is the earliest known, and recorded description of CAH.

In 1865 Luigi Crecchio, the anatomist who dissected poor Marzo, knew nothing of chromosomes or hormones, all they had to rely on was the physical evidence of Marzo’s internal organs. Crecchio placed their knowledge of anatomy over and above Marzo’s unambiguous social role, and so Marzo, unremarkable in life as a man was remade as someone he wouldn’t have recognised. Medical authority’s need and desire to classify and categorise everyone according to the very narrowly drawn boundaries of the binary description of men and women took no heed of Marzo’s own understanding of themselves in life. In death, they were declared a woman.

Congenital Adrenal Hyperplasia is a complex endocrine dysfunction that describes the varying capacity of a person’s adrenal glands to function properly. Additional to that some people have the added complication of not being able to regulate their hydration, to maintain an appropriate level of salt to ensures they don’t dehydrate. This is called Salt-losing CAH, and is the most complex, and potentially life threatening variation of CAH. There are now known to be other variations of CAH. All are known to be very challenging to manage. It is now known that CAH is an autosomal, genetically inherited endocrine dysfunction. It is quite possible it was the salt-losing variation of CAH that eventually killed poor Joseph Marzo.

Marzo is unusual in medical history in that we know their name. Unusual, because mostly medical history is marked by the names of the clinicians and scientists who describe their discoveries, and present their hypotheses.

Lawson Wilkins is about as big a name in medical history as many, yet you will struggle to find it so easily referenced these days. The current named Pediatric Endocrine Society used to be called the Lawson Wilkins Pediatric Society.

Why would the man credited with the synthesis of corticosteroids, the life saving drugs that enable people with CAH to live, now be anathema? Lawson Wilkins was an enthusiastic eugenicist all his life for one, and this for another:-

Lawson Wilkins, 1950: “Amputation of the clitoris at the earliest possible time” to prevent “anxiety in parents” – “already existing tradition of clitorectomies”

CAH may be a complex endocrine dysfunction, but clinicians are far more interested in the anatomical differences that some exhibit when born, and the spectrum of gender identities that people assigned female at birth often develop as they grow into adulthood. To that end CAH was classified as DsD – a Disorder of Sex Development.

Lawson Wilkins and John Money thought that surgery and hormonal intervention could, and would ensure that everyone assigned female assumed a female identity and know themselves as women, by the pre-emptive use of surgery to refashion the genitals, and a hormone milieu designed to suppress the high levels of blood testosterone that many CAH’ers exhibit. Even though it is now accepted that pre-emptive surgery does nothing to ensure a particular gender identity, the overwhelming motivation to ensure that each and every child born with CAH identifies with the sex they are assigned and adopt a gender expression appropriate to that assignment, rather than be permitted to grown up and be who they know themselves to be, remains the cornerstone of all intersex medical interventions to this day.

The Consensus Statement dedicated to CAH recommends surgery in infancy, just as Money did. A brief reference is made to the possibility of postponing intervention until such time as the individual can make their own decisions, but is dismissed for lack of evidence. That is disingenuous. There is a considerable body of evidence (sic) that those with the most anatomical variation benefit from not being pre-emptively operated on.

Clinicians are also very aware of a significant proportion of CAH’ers assigned female who live in a male or genderfluid role in adulthood, although as in all these things actual numbers are almost impossible to come by. A figure of 10% of those operated on who later reject that assignment is sometimes quoted, but there are articles that quote figures as high as 30% .

One of the means adopted in the past 20 year in an attempt to avoid surgeries is the use of pre-natal dexamethasone. Clinicians have discovered the art of reaching a foetus in utero. The experimental use of dexamethasone as a means of altering both the behaviour and anatomies of female CAH’ers is fraught with ethical questions. Papers openly discuss various clinical means of erasing tom-boyish, or lesbian behaviour in CAH females. It’s use has been described by the Endocrinological Society as “not a standard of care” , and “experimental”.

Only Sweden has banned it’s use in intersex medicine. Other countries are not so fastidious, and still continue using it. The long-term outcomes for this pre-emptive hormone intervention are entirely unknown. The use of pre-natal dexamethasone amounts to a whole life experiment on individuals, only 1:8 of whom are either female or CAH, because it is administered before the sex of a foetus can be determined.

Being intersex is to know what being marginalised and silenced means. We are spoken about but not to. Our medical histories shared amongst clinicians, yet become mysteriously untraceable when requested by the individual. To be intersex is to know about every gimcrack pet theory about gender, and identity because we are the test material for their ideas.

The protocols today differ not one whit from those enacted prior to the adoption of the 2006 Consensus Statement on the Management of Intersex Disorders. That document formalised, and made public what had been perpetrated on intersex infants, and young children for decades behind closed doors.

Intersex advocacy still has to struggle with the legacy of those decades of silence, yet powerful international organisations are now heeding activist’s work. It is slow, and immensely frustrating. Not least for the ill-starred criticisms directed at activists and advocates who have the courage to speak out against such barbarities.

That advocacy work will continue until everyone born intersex is free from the expectation that everyone else can speak for and about us, and decide our fate, in the belief that we will remain silent. Everyone born intersex is entitled to exactly the same rights of personal autonomy and bodily integrity that others take for granted. The work of raising intersex visibility, and securing legal protections will continue as long as there is a need for people to know what is being done to one of the most marginalised groups of people on the planet.

‘Excerpts from: Sandra Eder: “The Volatility of Sex: Intersexuality, Gender and Clinical Practice in the 1950s”, in: Gender & History, Vol. 22 No. 3 November 2010, pp. 692–707’

References to Joseph Marzo are drawn from translations by Alfred Bongiovanni from De Crecchio (Sopra un caso di apparenzi virili in una donna. Morgagni 7:154-188, 1865)