If you have gonads, chances are your DSD doctors talk about them a lot
They may discuss monitoring them. This means looking after them over a long period of time and potentially checking your hormone levels and other things in your blood.
They might discuss removing them. This means discussing with you what the risks and benefits may be and what happens afterwards.
These are big decisions. Ask your doctors for information and do some research with your parents, too. Here, we provide a ‘sample answer’ (a general answer that we think doctors might give) or offer additional background information.
Why would I move or remove my gonads? Is there a current problem? Can I wait until later?
- This depends on the condition. For some conditions, risks are low in childhood and might increase as people get older (like in androgen insensitivity syndrome.) In other conditions, risks are already high in childhood (like with Swyer’s Syndrome.)
- The location of the gonads (especially for guys) can matter—guys with gonads in their abdomen area may have higher risks than those with them outside their body or closer to the body’s surface. That’s why doctors may sometimes request to bring them down with surgery (also known as orchiopexy or gonad movement.) Doctors often can monitor the gonads better if they are near the outside of the body.
What does the most up-to-date research (for instance: articles, papers, and guidelines) say about gonads with my DSD?
- The answer here always depends on the condition. Right now, research is changing what we know about gonads in DSD and how to take care of them. Ask your doctor to show you some of the recent research. Things change quickly and it is important for you and your doctors to be up to speed on what the options are.
What are the potential drawbacks of keeping them?
- With your doctor, be prepared for discussions about tumors . Tumors (often called cancer) are when some parts of the gonads grow too quickly and may become potentially a problem to the rest of your body. Check with your doctors what the specific chances are of tumors for your DSD condition is, now and later in life. Many of the chances of a tumor also change with age, too.
- Some people might have hernias (when gonads move in the belly) that can cause pain. Doctors can help fix hernias surgically as well, without removing the gonads, as this is something that happens for lots of people.
- Be prepared for testosterone (T) effects. Keeping gonads during puberty may provide hormones for your body and help it grow. If you have PAIS, 5ARD, or 17Beta HSD, this could mean many of those T effects we talked about in the puberty section. Know your body, and don’t be afraid to ask questions.
For my condition, what is the most recent estimate of tumor risk? What studies is it based on?
- Recent estimates of tumor risk are currently varied because there is not enough research and not enough high quality studies with large groups of patients. This means that the numbers given are often not very reliable and instead a best-informed guess of what the chance of a tumor might be. Typically, though, the estimates are broken down by condition. The best studies make sure individuals actually have the same condition and confirm it with genetic testing. Make sure your doctor knows to look for those articles with genetic confirmation as the type of evidence available.
Is there any way my gonads have eggs or could make sperm?
- This may be a good question about the gonads of people with 5ARD, Ovo-testes, Mixed gonadal dysgenesis, and sometimes PAIS. Some people are able to father children or mother children when the gonads are left in place. For some people, it might be possible to freeze sperm or eggs or even parts of the gonad. But this is rare, and most people with DSD will not be able to biologically contribute eggs or sperm to make a child. Ask your doctors for more information about this.
If gonads are removed, what would I take for hormones? How often would I take it? How much does it cost?
- If gonads are removed, and sometimes if not, people take hormones like testosterone or estrogen and/or progesterone.
- In general, how often you take HRT depends on what kind you take. Pills are usually every day, while shots/injections or patches last longer.
- Costs of things like hormones (HRT) change a lot based on where you live and the type you take. There are lots of options like patches, pills, sprays, injections, and gels. Unfortunately, not all options are available in every pharmacy or country, and depending on where you live, they might be expensive.
So, what does the actual number or percentage risk mean?
- Let’s say you’ve been told that for your condition, you have a 1 out of 10 chance of developing gonad cancer by age 30. Is this a large risk? Is this a small one?
- For some people, any risk is considerable. For other people, they are okay with the risks.
- There are many other risks out there that we may not think about as much. Women have a 1 in 8 chance of having breast cancer at some point in their life. 1 man in every 7 men will have prostate cancer at some point in their life.
Why would I keep my gonads? Are they helping my body? Can I keep them?
- The answer depends on your condition. Some people (like those with CAIS) have gonads that produce hormones that can be helpful in helping your body go through puberty and maintaining health. With conditions like Swyer’s Syndrome, the gonads don’t produce those hormones and carry large risks. And for some people with 5ARD or PAIS, the gonads might cause androgen effects during puberty like body hair, lowering of the voice, growing genitals, etc.