Intersex Conditions, a Primer

Sex Difference in Christian Theology, pt. 1

I recently finished reading Megan K. DeFranza's excellent Sex Difference in Christian Theology; this marks the beginning of my short series of articles giving my rough thoughts on the book. There's a lot of thought-provoking material in this book, enough to warrant breaking this up into multiple digestible pieces.

My copy of the book, sitting on my desk.

In particular, where I want to start is by giving a short primer on intersex conditions, as I understand them. Here I feel the need to give a couple warning signposts:

1. I am not a biologist or a doctor. If anything I've said is factually incorrect, please inform me! I don't have any reason to doubt Dr. DeFranza's summary of things, but given she only talked about this for fifty pages, she cannot possibly cover all the nuances that may be important or relevant.

2. I am not intersex, nor do I personally know anyone who is intersex. Indeed, for me even the question of gender is an academic one; my personal feelings on the matter are that I'm a man, I always have been, and I've never felt uncomfortable with this fact about the world. I cannot, therefore, comment on these experiences from a first-hand perspective. So if you, dear reader, are intersex, I would love to hear about your experiences in the world.

The main body of this article will be introducing some of the main conditions that are considered "intersex", and we'll conclude with asking some philosophical questions that intersex conditions raise.¹

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Complete and Partial Androgen Insensitivity Syndrome

Androgen Insensitivity Syndrome, henceforth AIS, is an intersex condition that occurs around 1 in every 13,000 births.² It comes in two types, namely Complete (CAIS) and Partial (PAIS). It occurs in those with XY chromosomes, and its primary effect is rending the person unable to process testosterone — their cells lack the proper receptors.

CAIS is much more common than PAIS, so I will describe its effects first. At birth, someone with CAIS will outwardly appear to be female; although they will have (usually, undescended) testes, they will also have a short vagina and no cervix. This is because androgenization (male-making) of the genitals occurs via testosterone, and since someone with CAIS cannot process testosterone, the body does not form a penis. Since the external genitalia usually appear normal, it is often not discovered that someone has CAIS until they reach puberty and do not menstruate.³

Someone with CAIS will go through female puberty; testosterone is similar, chemically, to estrogen, and some of the testosterone produced during puberty will be converted to estrogen, thereby producing feminine puberty. As a result, someone with CAIS will not only be outwardly female, but will fulfill the "contemporary, Western ideal of womanhood:"⁴ that is, they will be tall, skinny and have no body hair. Generally speaking, they will also have feminine self-identity and have gone through a female upbringing.

PAIS is much rarer, occurring in around 1 in 130,000 births.⁵ Partial insensitivity comes in seven grades, with effects ranging from male genitalia with infertility (grade 1) to female genitals with pubic/underarm hair (grade 6). CAIS is often considered grade 7.⁶

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Congenital Adrenal Hyperplasia

Congenital Adrenal Hyperplasia (CAH) is an enzyme deficiency, occurring in somewhere in the range of 1 in 13,000 to 1 in 36,000 births, resulting in an overproduction of testosterone. It is generally only considered an intersex condition in individuals with XX chromosomes; in those with XY, while it can have harmful affects, including early-onset puberty, which can stunt growth (amongst other things), it is not considered an intersex condition.⁷ In 1954, Andrea Prader created the Prader scale, which classifies the degrees of masculinization CAH can cause; it ranges from a slight enlargement of the clitoris (grade I) to mild hypospadias (grade 5).⁸

Unlike many intersex conditions, CAH represents a potentially serious health emergency at birth. It can lead to severe dehydration in a newborn. Often, at least for those living as female, additional surgery is required at puberty, in order to create a vaginal opening separate from the urethra. Generally in the West, parents are encouraged to raise their child with CAH as female, since the child will have fully functioning reproductive ovaries. This does require genital surgery, preservation of the internal reproductive organs, and hormone therapy. However, in places like Saudi Arabia, parents sometimes reject this advice and instead choose to raise their child as male.⁹ This is thought to be an effect of the local culture preferring male children.

CAH can also begin later in life, which is known as the conveniently named Late Onset CAH, or LOCAH. Indeed, it is far more common than full CAH, but its occurrence rate varies very highly amongst different groups. For Hispanics, the rate is 19 out of 1000; for mixed caucasians, only .01 out of 1000.¹⁰ The extent to which LOCAH is considered an intersex condition is highly contested; often its effects are negligible. However, it can result in things like infertility and male-pattern hair growth, including facial hair, so it seems to me that at least in some cases, LOCAH is rightfully considered an intersex condition.

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Alternate Chromosome Combinations

There are several alternate chromosome combinations that result in intersex conditions; amongst the most common are Turner syndrome (1 in 2000 to 1 in 3000) and Klinefelter syndrome (1 in 1000). Within these are also conditions termed "mosaicism", wherein some of one's cells may have different sex chromosomes than other cells.

Turner syndrome is a condition primarily found in females, wherein some or all of one sex chromosome is missing. This is denoted in various ways; both XO and 45,X are common—normal female chromosomes would be XX and 46, XX, respectively (p. 36). Mosaicism in those with Turner syndrome can look like having some cells with 45, X and some cells with 46, XX. Occasionally, although it is rare, they can even have cells with 46, XY. Because this is rare, those with Turner syndrome generally appear female, with external female genitalia.

Klinefelter syndrome is the opposite issue, in a sense; it occurs when someone has 47, XXY or 48, XXYY chromosomes. Those with Klinefelter syndrome have male genitalia, and its effects include small testes, infertility, and gynecomastia.¹¹

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5-Alpha Reductase Deficiency Syndrome

The clunkily-named 5-Alpha Reductase Deficiency syndrome, henceforth 5-ARDs, affects the body's ability to convert testosterone into the stronger dihydrotestosterone.¹² The rates at which it occurs is unknown globally, and varies wildly by culture.

Occurring in those with XY chromosomes, a child with 5-ARDs will generally appear to have female genitalia at birth, but at puberty the testes descend and the genitalia develop into male genitalia. In the Dominican Republic, it is common enough that there is a colloquial term, "Guevedoche", for someone with this condition.¹³ Amongst cultures where this is more common, there is generally more space for someone with 5-ARDs to transition between gender spheres. Amongst a tribe of people in Papua New Guinea, this condition is common enough that they have a third gender role specifically for such people. Further, those who are not discovered at birth would naturally be raised as female, but once they reach puberty, they are transitioned out of being considered female to this third gender. In America, no such transitory space exists.

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Some thoughts

There are other intersex conditions that occur, of course—I have not remotely covered the entire span of possibilities. As we've seen, the extent to which conditions can be truly considered intersex is a question hotly contested. I follow DeFranza in saying "It may be best to represent the figure as a range, such as .02 percent to 1.7 percent".¹⁴ Given there are 8 billion people alive today, the lowest estimate (.02%) gives 1.6 million people, and the highest (1.7%) gives a staggering 136 million people.

If we consider, for a moment, the possibility of the highest estimate being correct, I think it's worth pausing and mourning the fact that most of us don't know about this. A quick google search tells me that roughly 20 million people are diagnosed with cancer every year. So it's entirely possible that intersex conditions are in the same ballpark as the number of people who get cancer every year. How do we not know about this? Why do we not know about these people's experiences?

There is no one, unitive "intersex experience". But, as mentioned in my article Intersex in Christ - A Review, there can be some common threads running through the lives of many with intersex conditions. One man with Klinefelter syndrome described his experience as being "caught between the sexes".¹⁵ Not everyone with an intersex condition may feel this way, but many do.

Beyond raising awareness of these conditions, I am not sure what I can do, nor what the church should do, to help intersex people. If you are intersex, or if you know anyone who is, I would love to hear about your experience—inside or outside the church. These are complicated, complex topics, and they raise questions for all Christians who want to shepherd those who are lost, who go unnoticed.

Yes, these conditions raise questions about gender—what is it, what does it mean, who determines gender—but I think it's important to never forget that, first and foremost, intersex people are people, not abstractions. I hope to avoid ever reducing intersex people to their conditions; if I ever do so, please feel free to call me out! I'm a mathematician by trade. I deal in ideas and abstractions with more ease than people. Let me know if I fall into this trap.

With that in mind, I want to frame the following questions in terms of recognizing intersex people as such, rather than as philosophically interesting edge-cases. Often, more conservative circles within Christianity consider gender to be an absolute binary—a person is either male or female, there is no in-between. This model is clearly inadequate, at least in its most common, naïve form. How can we reconstruct gender biblically in such a way that creates space for intersex people? In particular, some relevant questions are

1. What is gender? What is it characterized by? Does it have its basis in personality, in genetics, in biology?

2. To what degree should we modify the binary model? Is it as simple as thinking of gender as a bimodal distribution along a continuous male-female axis? Should we rid ourselves of gender entirely, as some liberal theologians have considered?

3. At least as it appears to me, for complementarians¹⁶ in particular, the questions of vocation, priesthood, marriage, etc., become quite complicated for intersex people. If marriage is to be only between a man and a woman, where would someone with complete androgen insensitivity fit into that? An absolutist view based on genetics seems wholly inadequate. This and other questions around vocation, I think, need to be handled especially pastorally. Compassion and gentleness are a must.

1. I feel a bit conflicted about discussing this topic in such a cold, philosophical manner. On the one hand, intersex conditions do genuinely raise important questions like “how should Christians conceive of gender?”. Those with intersex conditions often break a lot of naïve assumptions that people have about how gender works—that it’s reducible to gonads or DNA, for instance. But on the other, treating intersex people solely as a foil for gender naïveté is not fair to anyone involved. Intersex people are people, not abstractions.

2. Megan K. DeFranza, Sex Difference in Christian Theology, p. 25. Henceforth, unless otherwise noted, I will simply use “DeFranza, p. xx” to refer to this text.

3. DeFranza, p. 25.

4. DeFranza, p. 27.

5. DeFranza, p. 27.

6. DeFranza, p. 28.

7. DeFranza, p. 30.

8. DeFranza, p. 31. Hypospadias is a condition where the opening of the urethra will not be at the tip of the penis. So in this case, the degree of masculinization is sufficient that someone with grade 5 CAH will appear to have a fully functioning penis with mild hypospadias.

9. DeFranza, p. 34.

10. DeFranza, p. 32.

11. DeFranza, p. 38-39.

12. DeFranza, p. 42.

13. DeFranza, p. 43.

14. DeFranza, p. 47.

15. DeFranza, p. 38.

16. As I’ve noted elsewhere; I do not have an opinion on this question yet. My own personal intuitions lie slightly, vaguely, in the direction of egalitarianism. But I am far from having a well-informed opinion on this question, which is massive in itself.