Fact Sheet

Brief:

Patients with Androgen Insensitivity Syndrome are born with XY chromosomes, produce androgens, but their bodies have trouble understanding androgens. Patients with AIS have no internal female reproductive organs and have internal or descended testes that are unable to reproduce.

Complete Androgen Insensitivity Syndrome (CAIS): Phenotypically female, and completely unable to process androgens like testosterone. Patients with CAIS have no uterus or ovaries, have a blind ended vaginal canal, and have testes in inguinal canal. They can also get hernias from the testes.

Partial Androgen Insensitivity Syndrome (PAIS): Phenotypically female, phenotypically ambiguous, or phenotypically male and partially able to process androgens like testosterone. May be hard to distinguish between penis and clitoris and scrotum and vulva. If they can pee, let them be - they do NOT need surgery to correct genitals, especially as infants. Patients with PAIS deserve to grow up and express their gender identity before making an informed choice if they want to have gender assignment surgery; gender assignment surgery should never be performed on an infant.

Mild Androgen Insensitivity Syndrome (MAIS): Phenotypically male and mostly able to process androgens like testosterone. Has penis, scrotum and testes, but is unable to have children without intervention.

What does a patient with AIS’s care team look like?

Primary Care

  • Bone Density Monitoring

  • Normal Blood Work & Care

OBGYN (LGBTQ+ Friendly)

  • Hormone Replacement Therapy (Estrogen / Testosterone)

  • Gonadectomy (If patient elects to have a gonadectomy)

  • May need vaginal dilation (example)

  • Does not have Cervix or Uterus or Ovaries

  • May have breast tissue, so age appropriate breast cancer screening.

Endocrinologist (LGBTQ+ Friendly)

  • Hormone Replacement Therapy (Estrogen)

  • Gonad Monitoring - Ultrasound, CT Scans (If patient elects to not have a gonadectomy)

  • Bone Density Monitoring

Physical Therapist (Intersex / Trans Friendly)

Dermatologist

  • May or may not have eczema from dry skin.

Therapist

  • Pharmacotherapy

  • Behavioral Therapy

  • Cognitive Behavioral Therapy

Sensitivity Training

Patients with AIS have been through some trauma. Below are words that can be found in medical journals that are best to be avoided.

Offending Term: Pseudohermaphrodite / hermaphrodite

Why It’s Wrong: This is an old term from the 1950s and is very painful for patients with AIS. It is perceived in a derogatory way. Please do not use this term.

Offending Term: Testicular Feminization Syndrome

Why It’s Wrong: This is an old term for AIS that is also very painful, and factually incorrect. Patients with AIS have bodies with varying degrees of understanding androgens, and a term focusing on one aspect of the anatomy kind of misses the point.

Offending Term: undermasculinization

Why it’s Wrong: Terms like these gender the patient one way or another; this can be painful for patients that identify as the opposing gender. If your patient identifies as a Female, and hears terms like “undermasculinization,” these terms can be very confusing and traumatic.

Offending Terms: Intersex, Disorders of Sexual Development, & Differences of Sexual Development

Why it’s Wrong: These three terms are the three current and approved terms for patients who are on the sex spectrum between what is classically through of as male and female. Personally, I am ok with the term “Intersex” so I use it on this website, but many people are not. I personally dislike DSD and do not use it on this website. Ask your patients which terms they prefer to use.

Need More Data?

Read Affirming Primary Care for Intersex People.