Parents with children diagnosed with differences of sex development often have many questions about their child’s diagnosis and treatment. Feel free to ask your DSD team at St. Louis Children’s Hospital any questions you have. We welcome your questions!
What are Differences of Sex Development?
Differences of sexual development (or, DSDs) are variations in the external genitals, gonads (i.e., ovaries or testicles), sex chromosomes, sex hormones or internal reproductive structures (e.g., uterus). Often, atypical genitalia is the first sign you or your child’s doctor may notice. But not all people with DSDs have atypical genitalia.
DSDs used to be called “Disorders of Sex Development.” A person with this condition may be referred to as a “hermaphrodite,” among other terms that have been used in the past and are now considered inaccurate or inappropriate. These terms imply that DSDs are bad, a “disorder” — rather than simply being different than what is typical. Today, the medical and intersex communities prefer the term “intersex.”
How Common are DSDs?
Some DSDs are more common than others. Estimates of how common DSDs are vary widely between different conditions, and even among different sources.
Below are estimates provided by the Intersex Society of North America.
|Not XX and not XY||1 in 1,666 births|
|Klinefelter (XXY)||1 in 1,000 births|
|Androgen insensitivity syndrome||1 in 13,000 births|
|Partial androgen insensitivity syndrome||1 in 130,000 births|
|Classical congenital adrenal hyperplasia||1 in 13,000 births|
|Late onset adrenal hyperplasia||1 in 66 individuals|
|Vaginal agenesis||1 in 6,000 births|
|Ovotestes||1 in 83,000 births|
|Idiopathic (no discernable medical cause)||1 in 110,000 births|
|Complete gonadal dysgenesis||1 in 150,000 births|
|Hypospadias (urethral opening in perineum or along penile shaft)||1 in 2,000 births|
|Hypospadias (urethral opening between corona and tip of glans penis)||1 in 770 births|
|Total number of people whose bodies differ from standard male or female||1 in 100 births|
What Should We Tell Our Child?
Be honest but age-appropriate. What does this mean? It means start talking to your child as early as possible — in language and concepts they can understand at their stage of development.
Here are some general, age-specific guidelines:
- From infants until around 6, you can frame conversations around what is the same and different about bodies — from eye color to genitals. You can help your child feel good about his or her body the way it is: Celebrate that everyone's body is different, and that's okay!
- From around 7 to 10, you can start talking to your child about puberty and the changes their body will be going through. Your child will likely have some introduction to this topic at school. Your conversation can help them understand the “standard” explanation of puberty vs. what is specific to their body.
- From age 11 through mid-teens, your child will start to experience physical and hormonal changes from puberty. Depending on your child’s diagnosis, your child may experience these changes differently than other children. Again, talk with them about what they are going through and reassure them it is not bad or wrong — just different.
- Puberty may be when you and your child first discover they have a DSD — or when differences in their body begin to manifest more strongly. Our DSD team is available to provide counseling services to help you and your child discuss a new diagnosis or changing emotions that may come during this time of change.
- After age 15, you may wish to start talking to your child about issues they’ll want to think about moving forward, such as sexuality, fertility, hormones, etc., when they transition into becoming an independent adult. The DSD team is available to help navigate these conversations.
For all ages:
Encourage questions. Your child will likely have many more questions than what we’ve touched on here — ask what they are! If you don’t know the answers, that’s okay. We are here to help.
Talking with your child openly — throughout childhood — is an important way to show them your support and to normalize their experience.
Is There a Test or Scan to Tell Our Child's “Real” Sex?
No. Sex is more complicated than just looking at a picture of your child’s sex chromosomes (a karyotype) or checking whether they have testes or ovaries. It's a combination of their external and internal genitalia, reproductive gland tissue, karyotype, hormone levels and more.
How is Sex Assigned in a Child with Differences of Sex Development?
To determine your child’s sex, our DSD team considers the following:
- A pelvic ultrasound to check for a uterus and ovaries (or we may use direct cystoscopy or vaginoscopy)
- A genitourethrogram to look at the urethra and vagina, if present
- A chromosome study to help determine the genetic makeup of your child
- Evaluation for genes from or on the Y chromosome that start development of the testicles and penis
- Potential future fertility
- Size and potential for growth of a penis, if present
- Ability of an internal sex organ to make the sex hormones for the sex assigned to the child
- Risk of future health conditions, such as cancer, that may develop in the original sex organs later in life
- The actions of sex hormones on the fetal brain
- Your opinion
So, What's My Child's Gender? Is That Different from Sex?
Your child’s gender is different than your child’s sex. Your child’s sex refers to sexual anatomy. That’s the sex chromosomes, hormones and organs. A child with differences of sex development has one or more sexual characteristics that are not typical.
Gender is how we feel about ourselves and how we self-identify — as male, female, neither or both. We'll know your child's gender when your child tells us!
As your child grows, your child will typically begin to identify on his or her own as male or female. (Some children may identify as non-binary, meaning they identify with a gender other than male or female.)
We want to make sure that we have not performed any surgeries that make future decisions about sexual anatomy difficult — or even impossible — to reverse.
Is My Child Transgender? Are Transgender and DSD the Same?
Identifying as transgender and having a DSD are not the same thing. People who are transgender do not identify with the sex that they were assigned at birth.
For example, they may be assigned female at birth but identify as male. They may choose to pursue therapies such as hormones or surgery to affirm their gender.
People with a DSD have variations in anatomic development or hormonal production. While some children with a DSD do grow up to identify with a gender different than their assigned sex, most children identify as their assigned sex.
Will My Child Be Able to Have Children?
Whether your child can have children biologically depends on the condition your child has. Some differences of sex development make it harder to have a child biologically, while some do not affect fertility. Of course, adopting a child is an option for any person with a DSD.
We encourage you to talk about any concerns you have about fertility with our DSD team, so that we can help you better understand the implications of your child’s condition.
Did We Do (or Not Do) Something That Caused This to Happen?
No! You did not cause your child to have differences of sex development. If you are feeling concerned that you caused your child to have a DSD, we encourage you to speak with one of our therapists to talk through these emotions.
As your child grows, they will learn from you how to feel about their bodies — and about themselves. If they see that you blame yourself for their condition, this may make it more difficult for them to accept and embrace their differences.