What is intersex?

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  • How many people do you know have an intersex condition?
  • How many people do you know have a child/children with an intersex condition?
  • How would you feel if you had an intersex condition?
  • How would you feel if your friend/child was intersexed?

Shrouded in secrecy
Terms like transexuality or homosexuality may come to mind, but how many people understand what intersex really is? One of the reasons for this lack of knowledge is because intersex is shrouded in secrecy. Medical professionals, when facing a diagnosis for the first time, may be taken unawares, refer a patient to a specialist endocrinologist. The GP, gynaecologist, obstetrician, etc. gain very little knowledge about intersexuals and how to treat them on a medical or psychological level.

One of the reasons I am writing this article, is to increase awareness of intersex conditions, with a view to ultimately improving care for intersexed individuals.

Intersex defined
An intersexed person may be described as: one whose biology includes an identifiable mixture of male and female characteristics, regardless of the appearance of their genitalia at birth (Diamond 2004). There are a number of intersex conditions. In some cases, the genitalia may appear female. There are other cases where the genitalia appear male at birth. However, where there is genital ambiguity at birth, things get complicated.

In a binary world, one expects to raise their child as either boy or girl. Rushed decisions are made when such a baby is born, sometimes surgically rearranging its genital equipment to appear normal. The incorrect decision for the child can result in disastrous emotional, physical and psychosexual consequences in the long term.

Complete Androgen Insensitivity Syndrome (CAIS)
For the time being, I am going to describe an intersex condition where there is no physical ambiguity. This particular condition is called: Complete Androgen Insensitivity Syndrome (CAIS). The child appears, feels and behaves like a little girl. However, their chromosomes are genetically male: XY. Inheritance is X-linked recessive. CAIS is only expressed in individuals who have an XY Karyoptype. The affected individuals lack reproductive organs, so it is the XX women who pass on the condition from one generation to the next.

After conception, a human neonate develops along a female path unless testosterone from the testes prevents masculinisation. Testosterone is one of a series of male hormones, collectively called “Androgens”. People with CAIS cannot respond to androgens. Their bodies do not recognise their own testosterone and they cannot develop into males. They are like other females, but lack ovaries, and a uterus. The vagina is a blind-ending sac and is shorter than normal.

CAIS often undetected
When a baby with CAIS is born, there is no question that it is female. Unless the karyotype is known before birth (e.g. from amniocentesis), there is no reason to suspect anything amiss. The condition may be detected in infancy when a hernia/hernias appear e.g. after crying. The hernias are caused by testes descending through the inguinal canal.

The condition is often undetected often into adulthood when there is failure to menstruate or develop secondary (pubic or underarm) hair. The girl’s and her parent’s world is turned upside down overnight and the diagnosis can be extremely traumatic.

For this reason, the complete truth regarding the diagnosis is not always revealed, in the hope that they can continue life as a normal female. This is rarely the case. It becomes quite apparent to the sufferer in time that they do not have exactly the same credentials as other girls. Many begin to feel like a freak. They cannot communicate their feelings with others because the diagnosis is shrouded in secrecy. They become alienated, introverted and depressed.

Honest diagnosis required
It is indeed a crisis for a young girl or woman who does not feel in the slightest bit unfeminine that she is genetically male. The honesty of a diagnosis, however, is usually helpful emotionally because it provides an explanation for their “feeling different”.

Part of the crisis, is due to the lack of psychological preparation with regard to the patient’s condition. This is further complicated by medical advice encouraging affected patients to keep their condition to themselves. It is unfair to expect CAIS woman to go home and forget all about it. This does not happen.

Contact with others necessary
If intersexed people are prohibited from making contact with others like themselves, feelings of alienation, depression and introversion can only increase. It is unlikely that a patient wants to communicate their misfortune to everyone, but it is always helpful to have emotional support from people who truly understand your welfare. Secrecy is not only unhealthy from an emotional point of view, but also from the perspective of general health and medical management.

Extended family may be at risk
There may be a risk to other girls/women in the extended family which goes unappreciated. It is important to be aware of the inheritance of such conditions because there is a high risk of malignancy of the gonadal tissue of intersexed people. With CAIS, the risk is low until adulthood. However with other intersex conditions, the risk may be extremely high, even at quite a young age.

Where there is partial insensitivity to androgens (Partial Androgen Insensitivity Syndrome, PAIS), it may be a better decision to consider pre-pubertal removal of testicular tissue. It is easy to misdiagnose some cases of PAIS, or Gonadal Dysgenesis, as CAIS, unless proper biochemical and genetic techniques are carried out. A thorough and proper diagnosis is extremely important for the medical management of these conditions.

Puberty options
If the gonads (testes) have been removed in infancy, the issue of malignancy is avoided. However, because testosterone behaves as oestrogen for patients with CAIS, it is the gonads (testes) that allow them to develop their femininity during puberty. It they are removed, pubertal development has to be artificially induced. If there is a low risk of malignancy (as is the case for CAIS) it is an option to allow for a more natural transition into puberty and remove the gonads later in the teenage years.

Not enough specialists
When managing a patient, the above has to be taken into account. In S.A. there are few endocrinologists with the expertise to properly manage intersex conditions, plus they are extremely busy people. They lack time, access to medical equipment and support teams (Consultant Endocrinologist + Psychologist + geneticist, etc). The patient may in turn, lack the knowledge and funding for appropriate testing.

In the past, decisions based on the opinion of the medical practitioner have often resulted in some dissatisfaction. Patients may have had their gonads removed during childhood without their consent, whereas they might have preferred to choose when they would like to have a godadectomy themselves. The doctor ultimately gets the blame, resulting in a less than satisfactory patient/doctor relationship. I am sure most doctors make what they perceive to be their best decision, but it may/may not always be what is best for the patient.

I believe with an improved medical knowledge of intersex conditions and the associated risks, taking into account the emotional and physiological experiences reported by sufferers from existing support groups (e.g. AISSG or ISNA), a better understanding can be reached on how to medically manage intersexed patients.

Improvement over the last decade
Things have improved during the last decade. Practitioners are tending towards becoming more honest with their patients. Patients are more informed, according to what they can understand for their age/stage of development. Most patients prefer to make their own decisions regarding what will ultimately affect their well being. The less they know, the tougher it is for them to come to terms with their condition.

One only has to read a couple of published letters to their group website to get an indication of how helpful it is to put the affected individuals in touch with one other: e.g. “…your site was one of the most helpful in reassuring me that what I was discovering (about myself) really did happen to people. Thank for all you do and have done…” I urge all endocrinologists, gynaecologists, obstetricians, paediatricians, etc. who come across CAIS or any other intersex condition, to make the time to look at some of the websites listed below, before they make judgements on behalf of their patients.

Condition not as rare as previously believed
The importance of intersexuality is often dismissed as it is a so-called “rare” condition. However, just because a condition is rare, does not mean that it does not affect a significant proportion of the population. When a support group was started in the U.K. in 1995, only 16 people attended their first gathering. Four years later attendance was 78 people. It may not be as rare as it is believed to be if there was less secrecy amongst patients and medical colleagues.

Some important addresses:
Androgen Insensitivity Syndrome Support Group (AISSG), U.K: http://www.aissg.org/
South African Androgen Insensitivity Support Group (SAAIS): saais@iafrica.com
Androgen Insensitivity Support Group Australia:
aissg@iprimus.com.auIntersex society of North America: http://www.isna.org/

These groups also provide support for people with related conditions such as: Partial and Complete Gonadal Dysgenesis, MRKH (Vaginal Agenesis), 5 Alpha-Reductase Deficiency, 3 Beta-Hydroxysteroid Dehydrogenase Deficiency, 17-Ketosteroid Reductase Deficiency, 17-Beta Hydroxysteroid deficiency. These conditions may present with similar emotional and physiological and medical problems e.g. HRT, Osteoporosis, Infertility, problems with relationships, etc.

- (Angela, South African Androgen Insensitivity Support Group)

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