The Age newspaper reports:
Two little boys will have their testes removed after a court allowed their parents to authorise the procedure to prevent them developing potentially fatal cancers.
The boys, aged 18 months and three years, have been diagnosed with Denys-Drash syndrome, which is so rare they are thought to be the only two people in Australia who have it.
According to Medscape, “The frequency of Denys-Drash syndrome is unknown. Worldwide, [little] more than 200 cases of Denys-Drash syndrome have been reported since 1967 when Denys et al originally described a child with nephropathy, ambiguous genitalia, and Wilms tumor.”
Kids First, the website maintained by London’s Great Ormond Street Hospital, has a section devoted to the syndrome and, in lay terms, describes these features:
A person with this condition will have:
- kidney disease, which they will have from birth
- a Wilms tumour, which is a cancer of the kidney
- a strange appearance of the sexual organs which makes the penis or the vagina look abnormal
There are different types of this condition and in some cases only one or two of these three things are present.
The presence of so-called “ambiguous genitalia” and the use of terminology such as “pseudohermaphroditism” demands comment.
WebMD on Drash syndrome:
This disorder usually appears early in life. In its complete form, it is characterized by the combination of abnormal kidney function, genital abnormalities (pseudohermaphroditism), and a cancerous tumor of the kidney called a Wilms’ tumor. Some affected individuals may have the incomplete form of Drash Syndrome, which consists of abnormal kidney function with either genital abnormalities (pseudohermaphroditism) or Wilms’ tumor. This disorder predominantly affects males but a few female cases have been reported.
Fortunately, demeaning psychomedical language like male or female pseudohermaphroditism and “true hermaphroditism” has largely fallen into disuse.
“True hermaphroditism” was a misnomer in any event. There are no known occurrences of a human being, or, for that matter, any other mammalian life-form, being capable of self fertilisation.
In this case court orders were sought allowing the removal of the boy’s testes on the basis of possible future carcinoma. Milton Diamond and Keith Sigmundsen offer this contribution to such rationales, firstly with regard to AIS, then to other situations where so-called “streak gonads” may be present:
From Management of Intersexuality, by Milton Diamond, Ph.D. and H. Keith Sigmundson, M.D.:
Advice regarding gonad removal from true hermaphrodites, individuals with streak gonads and others where malignancies can potentially occur is not so clear…
From Medscape at medscape.com/viewarticle/499501_5:
… mixed gonadal dysgenesis is the second most common cause of ambiguous genitalia, after congenital adrenal hyperplasia. There is an estimated incidence of gonadal tumor … of approximately 15% to 20%…
By way of a comparison, in 2009 the estimated lifetime risk of males developing testicular cancer is 1 in 210 in the UK. See info.cancerresearchuk.org/cancerstats/types/testis/incidence/
And the lifetime risk of UK females developing ovarian cancer was estimated at 1 in 50. See info.cancerresearchuk.org/cancerstats/types/ovary/incidence/
Finally, “… nearly a third of all new cancers in women are breast cancers. It has been estimated that the lifetime risk of developing breast cancer is… 1 in 9 for women in the UK.” See info.cancerresearchuk.org/cancerstats/types/breast/incidence/
Individual readers can draw their own conclusions from these comparisons. But, from OII Australia’s perspective, and bearing in mind that is most unusual for cancers to occur in gonadal tissue before puberty, the overwhelming need to castrate these two boys seems more likely motivated by medicine’s dislike of streak gonads than by any real medical urgency.
OII urges parents to exercise a great deal of caution before allowing surgeries such as these. The vast majority of these decisions can be delayed until the children are old enough to approve or to veto them.
The Judgement can be found at http://www.familycourt.gov.au/wps/wcm/resources/file/eb46cd42d5d82ba/2010_FamCA_948.pdf
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