Differences of Sex Development (DSD), or Intersex Conditions

The I in LGBTQIA+ stands for intersex, which describes when a person's biological sex doesn't fit in the categories of "male" or "female." Intersex is an older term for differences of sex development (DSD).

The DSD categories may be based on a person's chromosomes, genital presentation, reproductive tissue, or some combination of the three. The estimated percentage range of people who are intersex is 1.7 to 4%, but the percentages may be higher. Here's what else you need to know about intersex conditions, or DSD.

Categories

Females have two X chromosomes, and males have one X and one Y chromosome. For people with DSD, that may not be the case.

In addition, DSD was initially called intersex and hermaphroditism, but the latter term is outdated and no longer used. Intersex conditions are instead referred to as DSD. There are four DSD categories:

• 46, XX DSD
• 46, XY DSD
• Ovotesticular DSD
• Complex or undetermined DSD
  

46, XX DSD

In general, an individual who has DSD may have female chromosomes but ambiguous or male-appearing genitals. This is called 46, XX DSD which can be caused by:

• A deficiency of aromatase, an enzyme that converts hormones responsible for male characteristics to hormones responsible for female characteristics
• Exposure to or taking hormones responsible for male characteristics during pregnancy
• Tumors produced by hormones responsible for male characteristics in the pregnant person
  

46, XY DSD

The individual alternatively may have male chromosomes but ambiguous or female-appearing genitals. This is called 46, XY DSD which can be caused by:

• Androgen insensitivity syndrome—when the receptors to male hormones don't function properly
• Problems with the testes
• Problems with testosterone formation
• Problems using testosterone
  

Ovotesticular DSD

A person who is intersex could have what's called ovotesticular DSD, having both ovarian and testicular tissue. The cause of ovotesticular DSD is unknown, although there has been research done in animals that links true gonadal intersex to exposure to common agricultural pesticides.

Complex or Undetermined DSD

The last category of DSD is a complex or undetermined disorder of sex development that doesn't fit into any of the other categories. This involves chromosome configurations other than 46, XX or 46, XY that result in sex development disorders.

DSD Characteristics

Being intersex is not about gender identity or sexual orientation; it's about physical sex traits and genes. Depending on the cause, characteristics related to having DSD could include:

• Abnormalities with electrolyte levels
• Delayed or absent puberty
• Having external genitalia that isn't clearly male or female at birth
• Hypospadias, where the penile opening is not at the tip or of the urethra is not separated from the vagina
• A larger-than-expected clitoris
• Masses in the labia or groin
• A smaller-than-expected penis
  
• Unexpected puberty changes

While the qualities of an intersex condition are typically discovered at birth, it is possible that it may not be diagnosed until puberty. Georgieann Davis, PhD, an associate professor of sociology at the University of New Mexico and author of "Contesting Intersex: The Dubious Diagnosis," was born with complete androgen insensitivity syndrome.

"My parents, in my case and many others, didn't know I was intersex until I was in my teenage years and I didn't menstruate," said Davis. "That's when they discovered what they didn't expect to find inside."

DSD and Gender Assignment Surgery

In the past, if a baby was born with DSD traits, healthcare providers and caregivers may have chosen a sex assignment for the child. They may have elected for surgery so the child would have a penis or vagina. Caregivers were often urged to make a quick decision and to have surgery as soon as possible.

"If something is framed as a medical problem or emergency, parents are likely to move forward with medical intervention because that's the option they're presented," explained Davis. "Parents may later express decisional regret because they didn't have the information presented to them, that [intersex] is a natural variation of one's body that does not have to dictate one's gender identity."

Possible Risks

There are some risks associated with gender assignment surgery. They include:

• Chronic pain
• Inaccurate sex or gender assignment
• Infertility
• Mental health conditions
• Patient dissatisfaction
• Sexual dysfunction
• Surgical complications

The Surgery's Effects at a Young Age

While healthcare providers and caregivers still often choose gender assignment surgery for children with DSD, this issue is not without contention. These children cannot decide for themselves; their caregivers choose for them. For children with DSD, that decision may result in the following:

• Decreased sexual function
• Increased substance use disorders
• Suicide

Caregivers can forgo surgery and let the child decide at a mature age if surgery or treatment is desired. The Intersex Society of North America (ISNA) has advocated for this shift.

The organization recommended caregivers wait on genital surgery until a child is mature enough to make an informed decision for themself. Before making a surgical decision, the ISNA also recommended that the child be fully informed. Introductions to people who have and have not had the surgery are another part of the recommendation.

How To Provide Support

There are different ways to provide support for someone with DSD. The best thing is to advocate for people to have the ability to make their own choices about their bodies rather than a caregiver or someone else.

Social Support

Davis suggested that caregivers of children with intersex conditions reach out to other caregivers of children with DSD. They can then rely on those caregivers' experience and support so their children can make informed decisions about their bodies.

"I haven't met an intersex person or activist who's been against surgery," said Davis. "We're against having these surgeries done on those who don't have a say in what's done to their bodies."

Psychosocial Support

Davis also emphasized the roles of psychosocial support. This support may be in the form of psychiatric counseling or support groups to help individuals who are intersex navigate any challenges.

One study found that 53.6% of the participants who were intersex self-reported that their mental health was fair or poor. Many of the participants noted that they were experiencing:

• Anxiety
• Depression
  
• Post-traumatic stress disorder (PTSD)

"We, as advocates, would like to see the psychosocial part of care be the predominant thing," said Davis. "While surgeons and endocrinologists are key, psychological counseling is of utmost importance in handling our unique identities and traits in day-to-day life. There's no proof that growing up with a body that looks different is inherently harmful."

A Quick Review

Intersex, known as DSD, is a complex genetic condition. Four categories exist, and due to gene-level changes, they differ based on various physical characteristics.

It should be on their terms if a person chooses to pursue treatment for DSD. Being an ally to intersex individuals means ensuring that those individuals feel they have a sense of agency and autonomy.