In a previous post I wrote about Saudi-Arabia and its raging epidemic of tribalism. Read more about it here.
The 'inbreeding' which results from tribal thinking can sometimes be devastating or even fatal to children that are born into families who practiced first cousin marriages for centuries. I've seen the most peculiar and rare genetic disorders birth defects in the pediatric patients I've taken care of in the Kingdom. The prevalence of certain genetic diseases is very high in Saudi-Arabia compared to the rest of the world because of high rates of consanguinity reaching up to 80% of all marriages in some regions.
An example of such a disorder are the children born with ambigious genitalia. "A child may be born with genitals that are not clearly male or female (ambiguous genitals, or intersex state). Most children with ambiguous genitals are pseudohermaphrodites—that is, they have ambiguous external genital organs but either ovaries or testes (not both). Pseudohermaphrodites are genetically male or female."
Read more about the condition here: http://www.merck.com/mmhe/sec23/ch265/ch265d.html
The metabolic disorder causing it is called congenital adrenal hyperplasia (CAH). Most of the cases in KSA are caused by this inherited condition. Unfortunately accurate data on the nationwide prevalence does not exist or it's very scarce. This is typical to KSA, statistics on certain "hush hush" topics are either withheld, do not exist or at least have a nice thick sugar coating on them.
In Saudi-Arabia the majority of the cases caused by CAH are type 46XX which means the child is a genetic female with male genitalia. Some are not easy to define at birth and need testing to define sex. Sometimes gender is not properly investigated and the outcome is assigning the sex as the preferred gender in Saudi: MALE.
According to this study "There was an obvious preference to assign male sex."
This, despite the evidence from studies conducted in western countries:
"It is often technically easier to treat (and therefore raise) the child as female (it is easier for a surgeon to make female genitalia than it is to make male genitalia), so in some cases this is recommended even if the child is genetically male."
"we conclude that the assignment to the female gender as a general policy for 46,XX patients with CAH appears justified, even in severely masculinized cases"
So why are so many Saudi families that have children with ambiguous genitalia assigning male sex for clearly genetical females?
I guess it's obvious what the preferred gender is in Saudi-Arabia. If you had the chance to live as a woman or as a man in KSA, I bet most would choose the latter because of all the restrictions imposed on a woman's life here. Life as a man in Saudi-Arabia is easy and privileged. For a woman it's hard with many restrictions and never earning the right to be a full citizen, rather treated as a legal child for the rest of her life.
Sometimes the patients will need gender reassignment which means they need to decide whether or not to change the official sex of the child as recommended by the doctors. Saudi parents often refuse, especially when it is suggested to change from male to female. The best for the child would be naturally to determine the sex when they are very young. It happens though that sometimes the diagnosis or decision is made late, and the child already identifies as female or male. Despite this the sex is changed which causes many emotional problems and identity crisis for the child.
So this means that Saudi patients will come in for sex change operations, which otherwise are strictly prohibited in Islam. Changing God's creation is not allowed by plastic surgery.
I tried to search regarding the Islamic ruling on this type of sex change surgery and it seems to be viewed as a deficit which therefore can be repaired.
I found this ruling for a surgical operation (removing male organs) in order to turn him into a girl:
"It is permissible to perform the operation in order to reveal what was hidden of male or female organs. Indeed, it is obligatory to do so on the grounds that it must be considered a treatment, when a trustworthy doctor advises it. It is, however, not permissible to do it at the mere wish to change sex from woman to man, or vice versa."
Interestingly, children with ambiguous genitalia are actually mentioned in old Islamic texts, they are referred to as "khunutha".
Paula Sanders, "Gendering the Ungendered Body: Hermaphrodites in Medieval Islamic Law," in Nikki R. Keddie and Beth Baron, eds., Women in Middle Eastern History (New Haven and London: Yale University Press, 1991), pp. 74-95.
"Although the marriage to a khuntha woman, for example, could not be regarded as valid as long as the sex of the khuntha was unknown, it was still considered to be mustaqim, meaning that it did not involve anything forbidden."
"The jurists reasoned that if the khuntha turned out to be a woman, this meant only that two members of the same sex had seen one another and that the marriage itself was merely a blunder. If it turned out to be a man, this constituted the gaze of a woman upon her husband, and there was no prohibition against it."
I recently had a patient that came in for sex change surgery. She had been brought up as a girl and was genetically female, but apparently had decided she wanted to become a boy after all and the surgeons were OK with performing sex change operation on her at the mature age of 19. This girl was very petite and had a short stature but she had cut her hair short and arrived at the hospital in a thobe. It was all very strange. Her medical records had to be altered because her name was to be changed to Mohammed.
After her (or his) operation in which they constructed male genitals and removed the female parts, the girl turned boy seemed quite happy and all the friends that visited him were young Saudi men that didn't seem phased by the change whatsoever.
Congenital adrenal hyperplasia could be mostly avoided by simple prenatal screening, especially for the high risk families. That way the pregnant mother could get treatments to minimize the risk of genital ambiguity. It's common for the same family to have more than one effected siblings.
Saudi-Arabia has does have a premarital screening program, in which they screen for the three most common hereditary diseases, including one metabolic disorder (but not CAH). However like I mentioned in my previous post on tribalism, the sad fact remains that even if the screened couple is deemed to be at VERY HIGH RISK for having children with the mentioned hereditary diseases, 90% of them will marry anyway and genetic diseases will continue to flourish in Saudi-Arabia.