Living with congenital adrenal hyperplasia (CAH) means living with adrenal glands that don’t produce certain hormones, including some sex hormones, in the typical amounts. While the symptoms of CAH can vary, the most dangerous ones can potentially trigger an adrenal crisis, which can be life-threatening.

However, even if you don’t ever have an adrenal crisis, CAH can affect your life. Among other considerations, it can change the way genitalia look on certain children. This can cause emotional anguish and confusion over gender and sexual identity. Some people who experience this refer to themselves as “intersex.” If you have been diagnosed with CAH and you’re wondering if that makes you intersex, here’s what you need to know.

What Does It Mean To Be Intersex?

Being intersex means that something about your body doesn’t fit easily into the medical binary of male or female anatomy. Being intersex can mean that you have differences in what hormones you make, how your body uses hormones, your genes, your reproductive organs, your sex organs, or other physical characteristics usually associated with your sex.

The bodies of people who are intersex can look very different from the bodies of people who are endosex (fit more easily into the male-female binary). Intersex people’s bodies can also vary from one person to another. Some people may have reproductive organs that don’t match their external genitalia — for example, they might have the tissue of both testes and ovaries. Others may have reproductive organs and external genitalia that align, but these organs may not function or appear in a way that is commonly expected or medically typical. They may also have chromosomes (part of the DNA associated with biological sex) that don’t line up with XX for female anatomy or XY for male anatomy.

This might sound like a rare occurrence, but researchers estimate that nearly 2 percent of the global population has traits that could make them intersex.

People talk about being intersex in different ways. For some, it’s a medical designation. It refers to their body and how it works, and it doesn’t have anything to do with their gender identity. Others use intersex as their gender identity, rather than considering themselves male, female, nonbinary, etc.

How Is CAH Related to Being Intersex?

Congenital adrenal hyperplasia makes it more difficult for your adrenal glands to make a hormone called cortisol. Since your body needs cortisol, your adrenal glands work really hard to try to make enough of it. In the process of doing that, they produce larger amounts of other hormones. This can result in a number of changes to the external genitalia of people born with two X chromosomes.

These changes can vary quite a bit. Some babies will only have a large clitoris, while others will have a clitoris that looks like a penis, or labia that look like a scrotum. This can give the baby ambiguous genitalia.

The effects of these hormones can continue after a child is born, too. Babies born with two X chromosomes may continue to develop characteristics that are usually associated with being male. These include having a deep voice, more defined muscles, more body hair, more facial hair, and a hairline that recedes as they age.

Note that CAH can also change the genitals of those with XY chromosomes, but this does not result in characteristics that might make them intersex. Males diagnosed with CAH can struggle with infertility when they reach reproductive age. They may also develop testicular masses called adrenal rests, and although these are usually not harmful, regular testicular ultrasounds are recommended to all males diagnosed with this condition.

Should You Think of Yourself as Intersex?

Some people with CAH do choose to embrace being intersex, while others do not. In fact, one study published in the Journal of Pediatric Urology in 2021 showed that most females with CAH and their parents did not consider CAH to make them intersex.

If you don’t think you want to be intersex, note that your doctor or endocrinologist (hormone specialist) may designate you medically that way. If you’re ever in an emergency situation or hospitalized, it may be important for medical staff to know about the unique traits of your body, so they have to note it in your records in some way. If you feel strongly that you’re not intersex, you may want to talk to your healthcare provider and request that they remove this designation from your records and note your body’s characteristics in another way.

Reasons Why a Person With CAH Might Choose To Identify as Intersex

Some people living with CAH consider themselves to be intersex because they experience differences in their bodies when compared to those without that diagnosis.

In addition, some people find that describing themselves as intersex fits their experience of living in a body that is different from an endosex body. Choosing to identify as intersex may also help people find community. For instance, it may give them access to online communities with others who have had similar experiences so that they can discuss what it’s like to live in a body that doesn’t fit the norm.

Some people with CAH experience a lot of shame and other negative emotions over the ways their bodies are different from what other people expect. When people discover intersexuality and learn that others also have bodies that don’t match societal expectations, this can free them from those negative feelings.

Reasons Why a Person With CAH Might Not Identify as Intersex

For the most part, people living with CAH who choose not to identify as intersex do so because they don’t feel like the term accurately describes their experience. Some point out that they have all the female body parts, even if some of them look different than they do on an endosex person.

Others focus on the fact that people with CAH do not have genetic or chromosomal differences that indicate they’re intersex. They want to be considered women despite the way their external genitalia might look. Many of these people have lived as women and feel like the term “woman” fits their experience better than the term “intersex.”

Some people with CAH don’t want to call themselves intersex because they would like to be categorized within another, separate group, at least in the eyes of the law.

What Works Best for You?

In the end, whether or not you consider yourself intersex is up to you. If doing so helps you feel whole, raises your quality of life, and improves your well-being, then you can choose that. If, on the other hand, it feels like it invalidates your experiences, you don’t have to choose that designation. You can also choose to be medically designated as intersex, but to not use it as your gender identity.

Talk With Others Who Understand

On CAHteam, the site for people with congenital adrenal hyperplasia and their loved ones, people come together to gain a new understanding of CAH and share their stories with others who understand life with the condition.

Has congenital adrenal hyperplasia affected how you think about your gender or sexual identity? Do you consider yourself intersex? Share your insights in the comments below.