Living with - Androgen insensitivity syndrome

Children with androgen insensitivity syndrome (AIS) and their parents will be supported by a team of specialists who can offer ongoing information and care.

Various options are available to someone with AIS. Some are available in childhood, but most are offered in adolescence and adulthood.

Some people with AIS may want to discuss surgery and hormone therapy with their specialist team.

However, psychological support and advice is probably the most important part of care. This can help you understand your or your child's body and deal with any issues that arise as a result of AIS.

People with AIS lead full and satisfying lives and can maintain good health.

Gender identity

Gender identity is something that a person becomes aware of as they grow up. It's how they feel and how they show themselves to others when living as male, female or non-binary.

One of the aims of the specialist healthcare team involved with a baby with AIS is to advise parents about raising their child and their gender.

This is based on the team's estimation of how the child's physical sex development will continue with and without their help.

Parents play a big part in these discussions about babies. Older children and adults can take over discussions with doctors later.

Specialist healthcare teams advise that children with complete androgen insensitivity syndrome (CAIS) are raised as girls because they have female genitals, no testosterone response and almost always identify as female gender when they're older.

The decision is more difficult if a child has partial androgen insensitivity syndrome (PAIS).

A child's genitals may seem underdeveloped for a girl (short vagina). A boy may have a small penis with a hole at the base, not the tip.

You're entitled to specialist advice about your child's future development and any gender identity issues that could arise later.

Most children with PAIS stay with the gender they've been raised as. However, some people feel this does not represent who they are and decide to change gender in later life.

Talking to a child about AIS

Parents often wonder when and what they should say to their child about their AIS.

Many people feel it's best to help their child learn about how their body works as they grow up.

When any child learns about themselves and their bodies, it can be helpful for them to know from the earliest stage that people vary and difference is common.

Knowing this makes it easier to explain the basic facts about AIS as soon as possible.

They can then be given more detailed information as they get older, as their ability to understand increases.

It's recommended that a child fully understands their AIS before they reach puberty.

This can be a stressful time anyway, but just as adults try to prepare any child for changes that happen at puberty, a person with AIS needs to know what to expect their body to do.

It could be traumatic for a child to start puberty without knowing about the possible changes they may experience, or the differences between them and their friends.

When a girl with CAIS finds out about her AIS because her puberty is unusual, the healthcare professionals can explain and help her understand her body.

Support for parents

If a child is diagnosed with AIS, parents should be offered specialist counselling to help them understand their emotions.

A diagnosis of AIS can come as a surprise, and feelings of shame, guilt, anger and anxiety are common.

Talking to other families with AIS may also help. There are organisations that can put you in touch with other families, such as:

• DSD Families
• Contact helpline on 0808 808 3555

The DSD Families website also provides information and support for families affected by differences in sex development.

Support for children

Many young children with AIS do not need special psychological help because their condition is completely natural to them.

It can be good for children to know other children with AIS or similar conditions so that they do not grow up feeling alone or different from all other children.

However, as a child gets older, they may need support from someone with lots of understanding and experience in AIS to help them prepare for life with AIS.

A long-term relationship between the child and their specialist team is ideal so any new issues can be discussed as the child gets older.

Surgery

Children with PAIS will sometimes be offered surgery to help change their body and maintain their health.

People with CAIS will not be offered surgery until adolescence or adulthood.

Some procedures may be offered while a child is still young, but others can be delayed until they're older.

Hernia repair 

Children with AIS sometimes develop a hernia (where an internal part of the body pushes through a weakness in the surrounding tissue) soon after birth. This is a result of their testicles not moving from the tummy to the scrotum.

This can be repaired by closing and strengthening the gap created in the surrounding tissues. The testicles may also be removed during another operation.

Removing the testicles

Adults with CAIS will usually have their internal testicles removed. This is because there's a very small risk they could become cancerous if left in place throughout adulthood.

This procedure happens after puberty because the testicles produce hormones that help girls with CAIS develop a normal female body shape without hormone treatment.

Also, the risk of the testicles becoming cancerous before adulthood is extremely low.

If, for any reason, a child's testicles are removed before puberty, hormone treatment will be needed to keep them healthy and help them develop a more female body shape.

Surgery for boys with PAIS

Boys with PAIS may be born with fully or partially undescended testicles. If this happens, an operation can be carried out to move the testicles into the scrotum.

Read more about treating undescended testicles.

Surgery can also be used to straighten the penis and move the hole that carries urine out of the body to the end of the penis, rather than the underside.

Vaginal surgery

Girls with AIS often have a shorter vagina than most other girls and women, which can make penetration during sex difficult.

Treatment for this is usually delayed until after puberty, so that natural changes can take place.

Also, the young person can decide if she would like to know more about the treatment options.

The vagina can often be lengthened using a method called dilation. This involves inserting small plastic shapes that gradually widen and deepen the vagina.

Sometimes, a procedure to remove skin and tissue from the genital area and use it to surgically alter the vagina may be offered.

Some women with PAIS may want to discuss surgery to reduce the size of their clitoris. This may make it less sensitive and affect the ability to have an orgasm.

Male breast reduction

Boys with PAIS will sometimes have some breast development around puberty.

If this happens, talk to the specialist healthcare team, as breast reduction surgery may be offered. However, it's not usually available on the NHS.

Read more about male breast reductions.

Hormone therapy

Women with CAIS who have had their testicles removed will need to take oestrogen to prevent the menopause and osteoporosis.

Children with PAIS may need to take hormone supplements. Girls with PAIS who have their testicles removed may need oestrogen to encourage puberty.

This won't cause periods to start, as people with CAIS do not have a womb, but it will help them develop a more female body shape.

Hormone therapy will also be continued after puberty to stop people developing menopausal symptoms and weak bones (osteoporosis).

Boys with PAIS may be offered androgens (testosterone) to encourage certain male characteristics, such as facial hair and penis growth.