Susan Baker, Ph.D.

What to tell an affected child and at what age, is a very complex question. The answer largely depends on the child’s sex, age, and the family situation, so has to be discussed and decided on a case-by-case basis. For example, some children are very curious and ask a lot about their medical condition, why they have to go to the doctor, get tests and so on when their siblings don’t. Such questions should always be fully and honestly answered in age appropriate ways.

A special case with respect to information is that of young girls who have had genital surgery. Most young girls won’t have the background information to ask or wonder about the early surgery, (unless they had a poor genitoplasty) to ask, for example, why they look different in their genital area than their sisters. In such cases it is usually best to wait until the child’s own questions and resulting increasing knowledge about their medical condition lead up naturally to their learning about the early surgery. If information is given at the right pace this usually occurs at or near the teen years, a time when most girls have learned discretion, and when many may need to be fully informed so as to partake in the decision whether to have or defer vaginoplasty if it wasn’t done in infancy. (If the child doesn’t ask, one can egg her [or him on non surgical issues] on to start doing so with questions like, “How come you never asked why you have to see the doctor every so many months?” and so on.)

For a young teen to understand having had the surgery (and maybe the need for a vaginoplasty, which they will need to think and make decisions about), they need to have a pretty good basic understanding of CAH. I usually do this with our patients starting at age 5 to 7, seeing the child every year or so, again varying with the child’s temperament and family. I meet with the child alone, then finish by asking the child if she would be willing for us to meet with her parents and for her to tell them what we talked about, with me to help if she gets stuck. That way everyone walks away knowing what was discussed, and is able to discuss it at home. It also gives me a check on whether the child had a misunderstanding of anything we talked about. Telling too young children who don’t have such a background about surgery can be scary for them, and leave them feeling frightened and confused. Also telling them before they have a sense of the consequences if they talk about what they have learned to other children without using discretion can cause another set of problems.

It is most important for parents to be open to informing their children as the children grow. Parents with a classical CAH affected child, particularly parents of an affected daughter with a genital birth defect often feel traumatized long after the birth and sex determination. Sometimes this leads them to not want to tell their children anything, feeling that their children will feel the same trauma. When this happens to the parents, the parents unwittingly pass on to the child the sense that there IS something wrong, but it is something too awful and scary to ask or talk about. These children later grow up to feel, among other things, that there is a failure of trust between them and their parents, and that they were lied to by parents and doctors alike.

“I’m not sure of the answer to that, let me call the doctor and find out?” even if you just need to buy a little time to think over your response to a tricky question. But you must get back and answer the question, in an age appropriate way. If the child never asks questions, parents have to ask themselves if they have been sending subtle messages to the child that she (this occurs more often with girls for the reasons mentioned above) had better not ask any questions about what she has, why she goes to the doctor, why she has blood tests frequently, why she has to take medication, and so on. If this occurs it is a signal that it is time to get experienced professional help.