Intersex health issues – a brief summary

Please note that this paper is not current. For a detailed, more up-to-date analysis, please read our 2016 submission to the UN Committee against Torture.

OII Australia was recently asked to write a quick summary of intersex health issues, and we thought we’d share the paper publicly, as it might interest a range of people and organisations.

The paper is intended to be read in conjunction with the OII Australia/OII Aotearoa submission on the draft Diagnostic and Statistical Manual of Mental Disorders 5th edition (DSM-5) and the World Professional Association for Transgender Health 7th Standards of Care (WPATH SOC). That paper contains information on additional health issues, and issues only summarised or not discussed here, as well as additional references and personal stories.

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Summary of intersex health issues

Introduction

This document is a brief summary of intersex health issues by Morgan, board member, OII Australia. It aims to give a very brief overview of the health concerns of intersex people.

It is not intended as an introduction to intersex; it is intended to be read in conjunction with the OII Australia/OII Aotearoa submission on the draft Diagnostic and Statistical Manual of Mental Disorders 5th edition (DSM-5) and the World Professional Association for Transgender Health 7th Standards of Care (WPATH SOC) [1].

Gender non-conformity and healthcare

We draw attention to the attached OII Australia/OII Aotearoa submission, which reports on healthcare approaches towards gender variance, and their impact on intersex people. The submission presents a range of evidence and references on harm caused by the pathologisation of gender variance [1].

Emily Grabham gives further evidence of the harm and loss of potentialities caused by non-consensual childhood surgeries [2].

Alice Dreger, Ellen Feder and Anne Tamar-Mattis, in a 2012 paper in the Journal of Bioethical Inquiry on the “use of dexamethasone in pregnant women at risk of carrying a female fetus affected by congenital adrenal hyperplasia (CAH)” [8]. They found that dexamethasone, a steroid, is being used, off label, to prevent homosexuality and physical masculinization, and that is considered to be of greater benefit than established cognitive and physical risks to treated children. They found that:

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. [8]

Dreger, Feder and Mattis note that Maria New, a paedriatic endocrinologist who prescribes dexamethasone, in a meeting of the US 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). [8]

Other notable medical practitioners are also involved in this research and these justifications. In a 1999 paper in the Journal of Clinical Endocrinology & Metabolism, titled “What Causes Low Rates of Child-Bearing in Congenital Adrenal Hyperplasia?”, Heino Meyer-Bahlburg wrote:

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. [9]

Heino Meyer-Bahlburg of Columbia University, the author, is a member of the American Psychiatric Association (APA) working group on revisions to the DSM-5 [16] and a member of the “Standards of Care Revision Committee” (SOC Committee) of the World Professional Association for Transgender Health [17]. The DSM and SOC are the standard protocols in use here in Australia for the medical treatment of intersex people, and other people who are gender nonconforming.

Treatment with dexamethasone does not affect salt wasting associated with CAH, something that is easily treated but which can otherwise be life threatening, it just affects psychological and physical “virilisation”. Treatment with dexamethasone targets the “virilisation” of girl fetuses with CAH but, because it is delivered to “at risk” mothers before amniocentesis testing for CAH, it also affects boys and other girl fetuses, with risks that mean that doctors in Sweden have discontinued treatment [18].

The rationale for treatment with dexamethasone is shocking, given that the APA no longer pathologises homosexuality. We would like to be confident that prenatal dexamethasone is not used in Australia. CAH is one of a number of intersex variations which can be discovered through amniocentesis in Australia, and we would like to see data on terminations arising from diagnosis. The AIS Support Group Australia has obtained evidence of terminations for a range of intersex variations dating back to 1989 [10].

We believe that fear and rejection of naturally-occurring sex differences, and their designation as “disorders of sex development” provide a rationale for surgery and “gender confirming” treatments which also remove the potential for alternative life paths. Having removed that potential, intersex people who are uncomfortable with their designated gender role are further disordered, as having Gender Identity Disorder or Gender Dysphoria with treatment protocols from the DSM and SOC.

Current surgical practice in Australia

Garry Warne and Jacqueline Hewitt, in a 2009 paper in the Medical Journal of Australia demonstrate that genital surgeries, including gonadectomies remain standard procedure in Australia, on the basis that they are life preserving. On gonadectomies, they write:

They were asked to respond to a proposal — advanced by an advisory committee representing the interests of the gay, lesbian, bisexual, transsexual and intersex communities — that doctors wanting to perform surgery to treat ambiguous genitalia in children too young to consent on their own behalf should have to seek approval from the Family Court of Australia on a case-by-case basis…

What has largely been missing from the debate is recognition of the fact that surgery forms a necessary part of the risk management strategy for preventing gonadal malignancy. In any DSD associated with a Y chromosome, there is an increased risk of germ cell cancer,7 especially when the testes are intra-abdominal (the risk of seminoma in partial androgen insensitivity is 50% for an intra-abdominal testis) or when there is gonadal dysgenesis. [3]

Warne and Hewitt’s assertion regarding the percentage risk of malignancy in internal gonads imply a general, across the board, risk of 50%. This is considerably different from research elsewhere, suggesting either sampling bias, or a hitherto unknown cancer hot spot. It’s a generalised statement that has significant adverse consequences.

Alternative views are stated by Karkazis (reference 32 in the accompanying paper) or, in the case of AIS specifically, by Quigley et al [5], Batch et al [6], Crouch [4]. The AISSG UK summarise some of the research in this field, showing sampling bias in many studies, and far lower risks for most intersex people with internal gonads, albeit risks that increase with age:

An early (1963) study (Morris et al) … estimated a risk of 22% but this is most likely an overestimate, since many of the cases were referred primarily because of the malignancy. A 1992 Danish study reported tumours in 4 of 21 patients but a 1976 study had found no tumours in 23 patients of their own and only 7 tumours in 82 cases gleaned from the literature (8.5%). The risk of such tumours increases with age, the 1976 study (Manuel et al) suggesting an age-related risk of 3.6% at age 25 but approaching 33% at age 50. Two reports from one group (1981 and 1991) estimate the overall risk to be 6 to 9%.[4]

Pleskacova gives a good current (2010) overview, with properly nuanced data, compared to that of Warne and Hewitt, stating:

The most numerous are patients with androgen insensitivity syndrome. The overall prevalence of CIS and invasive type II GCT (seminoma and nonseminoma) in this group is estimated to 5.5%. There is, however, an important difference between patients with complete and partial androgen insensitivity syndrome in whom malignancies occur in 0.8% and 15%.

Patients with gonadal dysgenesis (with either a 46,XY or 45,X/46,XY karyotype) seem to be the most endangered subgroup, although the prevalence in different series is rather incoherent, being reported in 15–100% of all cases [Slowikowska-Hilczer et al., 2001; Cools et al., 2006a]. After the rational interpretation of available data, Cools et al. [2006a] rated the total occurrence at 12% and possibly at more than 30% if gonadectomy had not been performed [7].

The AISSG UK report:

At the 2009 AISSG UK group meeting Dr. Naomi Crouch (gynaecology registrar at the University College Hospital London multi-disciplinary intersex clinic) talked about the cancer/gonadectomy issue, as follows:

Guest speaker Naomi Crouch said that the risk of cancerous changes in CAIS testes is thought to be about 5% by early adulthood and that gonadectomy at age 18 is recommended. The advantage of not doing it in childhood is that the intact testes will facilitate a natural puberty (testosterone from the testes gets converted in the body to oestrogen which promotes breast growth etc.). [4]

Note that Crouch gives the recommended age of surgery in the UK as 18, the age of majority, while Warne and Hewitt, in an Australian paper, were referring to the same surgeries in “children too young to consent on their own behalf”. The AISSG UK continue:

A group member asked why then aren’t breasts removed from young XX women, when the risk of breast cancer in the general population of women is about 8%? Naomi confirmed that the risk of breast cancer in general is about 1 in 12, but it was easier to monitor breast tissue for changes than it is to monitor intra-abdominal testes in AIS women. Ultrasound technicians, for example, do not have so much expertise in terms of knowing what to look for, since these conditions are rare. [4]

There are also, however, considerable lifetime risks associated with lifelong hormone replacement therapy, which is a consequence of gonadectomy. In addressing the issues raised by Crouch, we believe that technician familiarity should be addressed, in preference to gonadectomy and lifelong HRT.

We believe that gonadectomies are taking place on minors in Australia, where they are assessed as life preserving and not requiring judicial oversight. This is on the basis of inadequate data or unjustified generalisations. Such surgeries in Australia take place before the age of majority, while a child is not able to consent, while good practice in the UK is that surgery takes place at age 18. We believe that gonadectomies in such cases arise, in large part, from a simple belief that women should not possess testicles. They arise because of the pathologisation of sex differences.

Healthcare on the basis of need, not gender

In many situations, it’s our belief that Medicare provides funding for procedures on the basis of patient gender, not need.

For example, a board member of OII Australia has commented:

When I first came to Australia, I was an international student. My healthcare was covered by Medibank Private. For medical reasons, I needed a mammogram. The young woman at the Medibank Private service counter giggled when I passed her the receipt. The company initially refused to cover this examination. They couldn’t even enter it into their system, as that showed my sex as “male”. I called over the supervisor and protested her colleague’s behaviour and the lack of funding for a necessary examination. She apologised, and partly because of that situation, managed to override the system, by providing some kind of ad hoc payment equivalent to one which a woman would’ve received for a mammogram. Now that I’m covered by Medicare, I’m hoping that this situation won’t re-occur.[8]

Many intersex people, including those who have undergone gonadectomies, will have extant or remnant body parts not associated with their recorded gender. These include prostates in women with AIS, remnant streak gonads and other parts of the sex anatomy. Other areas of concern include Prostate Specific Antigens, and, in CAH, complete adrenalectomy.

OII Australia has one member who received an “appendectomy” that was actually a misdiagnosed ovarian cyst. It was thought our member, who indicated ‘intersex’ on their admission papers, was male and that males could not have ovaries.

We believe that Medicare, the Therapeutic Goods Administration and other health bodies, should address the health needs of individuals based on their actual body parts, rather than recorded gender.

Removing or adding testosterone

While not of the same order of concern as preceding healthcare issues, situations where an intersex person needs to add or remove testosterone from their bodies are fraught with unnecessary difficulties and indignities.

Intersex people, who are recorded as male but who need anti-androgen medication find that medication is not recognized as a treatments for their specific diagnosis, so the only path to that medication is to register the intersex person in question as a potential sex offender at the Therapeutic Goods Administration in Canberra. That register also contains the names of transsexual individuals who can only gain access to anti-androgens because of this inappropriate medication protocol.

From the Australian Human Rights Commission (AHRC) paper Sex and gender diversity, Report of initial consultation:

Other responses noted that the health system is not inclusive of people who are sex and gender diverse. Several responses mentioned that in order to receive specific hormone treatment a person must be labeled a sexual deviant.
Androcur is prescribed to block and reduce production of testosterone in men with testicular cancer, prostate cancer or other androgen-aggravated cancer or to convicted sex offenders to reduce sex drive and chemically castrate if taken in high doses. Because one of these reasons must be given to prescribe Androcur, and the patient is not a male with cancer, the one left is ‘reduction of drive’ – and this means, that to have this medication the patient will then have the letters “SD” on their medical record for Sexual Deviant. [12]

Comments on the AHRC’s Sex Files: Sex and Gender Diversity forum, which is no longer available, includes the following:

B., you might be interested to know that one of my friends has been denied a “Blue Card” to work with kids, as she has been on Androcur and is listed as a Sexual Deviant, and I understand that stays on your records at HIC central for all time. K.A.N. [12]

Most intersex people are hypogonadal, due to gonadectomies or biological reasons. In contrast to people seeking to reduce testosterone, intersex people who need testosterone, as their lifetime hormone replacement regimen are subjected to a combination of federal and state restrictions that make compliance with their healthcare regimen difficult, especially if they have a job that requires travel. Morgan, OII Australia board member writes:

The federal Schedule of Pharmaceutical Benefits restricts testosterone prescriptions to 6 months. Private scripts are the same (in contrast, most prescriptions are valid for 12 months).

PBS requirements stipulate a minimum repeat interval of 20 days. It’s not possible to get a couple of repeats in one trip, except with special authorization, which according to my endocrinologist is closely monitored.

In New South Wales, pharmacies are required to retain the original prescription on presentation [13]. It’s not possible to get a repeat prescription filled from any other pharmacy, so travelling or relocating intrastate, let alone interstate or overseas, is restricted by a need to return to an original pharmacy every minimum 20 days for 6 months.

This restriction applies, effectively, lifelong, and it makes drug compliance very difficult. I use testosterone gel, which comes in 30 day packs, and I’ve had problems maintaining supply. When I’ve lived in Europe, in countries that are signatories to the same international conventions targeting drug misuse, these restrictions don’t apply. I can get a 12 month prescription for testosterone gel, which I can fill at any pharmacy in 27 countries.

Also unlike in those European countries, imports, including re-imports of Australian-prescribed, PBS-funded medication taken on a trip overseas, are subject to an application for a permit on each occasion. Import permit applications oblige a traveller to detail “planned arrival and departure dates in Australia and the country/s you are travelling from and returning to” [14].

Common sense rules while travelling in Europe and elsewhere suggest carrying original packaging and the original prescription. Unfortunately, NSW prescription retention rules make this impossible to comply with easily. [15].

Summary

We seek dignity, and freely given, fully informed consent for all treatment, at every age.
We seek patient-centred care that focuses on our actual health needs, rather than assumptions based on the gender noted in our health records.

We seek a review of surgeries on intersex infants that are assumed to be life preserving. We believe that the justifications for such surgeries are often inadequate and based on unsubstantiated or inaccurate information.

We are concerned still about a history of non-disclosure of intersex status to intersex people (see [1] p. 19), about a lack of adequate care and information provision to people before they commence life changing treatment (see [1] pp. 24-27).

We are concerned about the pathologising nature of standard protocols for the treatment of intersex and other gender non-conforming people, and would wish to see the Australian government repudiate such pathologisation.

We would like to be sure that dexamethasone is not being used as an experimental treatment for aspects of CAH in Australia. We’d like to be sure that terminations are not occurring for intersex variations, traits that have no impact on persons’ ability to contribute fully to society, despite evidence that these do occur.

We would wish for a review of treatment protocols for intersex people who need to add or remove testosterone, so that they can live active, fulfilling lives.

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References

[1] OII Australia and OII Aotearoa, 2012, Submission on the DSM-5 and SOC-7, https://oii.org.au/20360/submission-release-on-the-dsm-5-and-soc-7/, accessed 7 June 2012.
[2] Emily Grabham, 2012, Bodily Integrity and the Surgical Management of Intersex, Body & Society 2012 18: 1, doi: 10.1177/1357034X12440825, http://bod.sagepub.com/content/18/2/1, accessed 1 August 2012.
[3] Garry L Warne and Jacqueline K Hewitt, 2009, Disorders of sex development: current understanding and continuing controversy, Med J Aust 2009; 190 (11): 612-613. https://www.mja.com.au/journal/2009/190/11/disorders-sex-development-current-understanding-and-continuing-controversy, accessed 23 August 2012.
[4] AISSG UK, 8 June 2012, “Orchidectomy (Gonadectomy)”, http://www.aissg.org/32_gdctomy.htm, accessed 23 August 2012.
[5] Quigley C.A. et al, 1995, Androgen Receptor Defects: Historical, Clinical and Molecular Perspectives. Endocrine Reviews, Vol. 16, No. 3, pp271-321, see [4].
[6] Batch J.A. et al: Androgen Insensitivity Syndrome. Reproductive Medicine Review 1992, 1: 131-150, see [4].
[7] Pleskacova J. et al, 17 June 2010, Tumor Risk in Disorders of Sex Development. Sexual Development Sex Dev
DOI: 10.1159/000314536, http://www.aissg.org/PDFs/Pleskacova-et-al-Tumour-Risk-2010.pdf, accessed 23 August 2012.
[8] Alice Dreger, Ellen K Feder and Anne Tamar-Mattis, 2012, Prenatal Dexamethasone for Congenital Adrenal Hyperplasia, An Ethics Canary in the Modern Medical Mine in Journal of Bioethical Inquiry, 2012, Volume 9, Number 3, Pages 277-294, http://www.springerlink.
com/content/m1523l7615744552/?MUD=MP
, accessed 23 August 2012.
[9] Heino Meyer-Bahlburg, 2009, What Causes Low Rates of Child-Bearing in Congenital Adrenal Hyperplasia?, doi: 10.1210/jc.84.6.1844, The Journal of Clinical Endocrinology & Metabolism June 1, 1999 vol. 84 no. 6 1844-1847, http://jcem.endojournals.org/content/84/6/1844.full, accessed 4 August 2012.
[10] AIS Support Group Australia, 15 February 2003, Discrimination against People affected by Intersex Conditions, report to the ACT Chief Minister and Department of Justice and Community Safety, http://home.vicnet.net.au/%7Eaissg/Intersex Submission ACT AISSGA Feb03.pdf, accessed 10 May 2012.
[11] Personal communication, 2012.
[12] AHRC, 2008, Sex and Gender Diversity, Report of Initial Consultation, http://www.hreoc.
gov.au/genderdiversity/consultation_report2008.html
, see also https://oii.org.au/4117/
intersex-australians-sex-offenders-register/
, accessed 23 August 2012.
[13] New South Wales Consolidated Regulations, 2008, Poisons And Therapeutic Goods Regulation 2008 – Reg 42, http://www.austlii.edu.au/au/legis/nsw/consol_reg/patgr2008398/s42.html, accessed 23 August 2012.
[14] Department of Health and Ageing, 13 July 2011, General Guidance for travellers bringing medicines to and from Australia – Advice for travellers bringing medications into Australia or taking medications out of Australia, http://www.health.gov.au/internet/main/publishing.nsf/Content/general-guidance-for-travellers-bringing-medicines-to-and-from-australia, accessed 23 August 2012.
[15] Personal communication, and OII Australia, 20 August 2009, Testosterone: suffering the system, not the medication, https://oii.org.au/3231/hrt-system/, accessed 23 August 2012.
[16] Heino Meyer-Bahlburg, 2012, APA DSM5 Work Group Member Disclosure Report, http://www.dsm5.org/MeetUs/Documents/Sex%20and%20Gender%202011/Meyer-Bahlburg%20-%20Disclosure%20-%204-28-11.pdf, accessed 23 August 2012.
[17] WPATH, 2012, Standards of Care, 7th Version, http://www.wpath.org/documents/SOC%20V7%2003-17-12.pdf, accessed 23 August 2012.
[18] Hirvikoski, T., A. Nordenström, A. Wedell, M. Ritzén, and S. Lajic. 2012. Prenatal dexamethasone treatment of children at risk for congenital adrenal hyperplasia: The Swedish experience and standpoint. The Journal of Clinical Endo- crinology and Metabolism 97(6). doi:10.1210/jc.2012- 1222, http://jcem.endojournals.org/content/97/6/1881, accessed 24 August 2012.