Bladder exstrophy is a rare condition that affects males and females. Only 1 out of every 20,000 babies born will have bladder exstrophy, and there is no known cause. This guide is intended as an overview for teen girls and young women who have been diagnosed with this condition.
What is bladder exstrophy?
Bladder exstrophy (classic exstrophy) happens while a baby is developing in the mother’s womb. The bladder develops inside out and is visible when the baby is born. Since the bladder is exposed to the outside, urine constantly trickles onto the skin, causing irritation.
This condition affects the bladder and may affect other parts of the body, such as the:
- Entire urinary tract
- Digestive system
- External genitalia (private parts)
Epispadias and cloacal exstrophy are conditions that are closely related to bladder exstrophy.
What is epispadias?
Epispadias is almost always seen with bladder exstrophy. Some babies are born with epispadias without bladder exstrophy. Epispadias occurs when the urethra (tube that drains urine from the bladder to the outside of the body) is not fully closed. Because the tube is open where it should be closed, urine can leak from wherever the opening occurs. In girls, the opening may be higher up in the urethra and may be bigger and longer than normal. Often, the opening extends to the bladder.
What is cloacal exstrophy?
Cloacal exstrophy happens when a baby is born with a portion of their intestines and bladder on the outside of their abdomen (belly). The bladder is split in half with the two sides attached to the intestines.
What causes bladder exstrophy/epispadias?
Some studies have found that this condition may be inherited but the gene has not been identified. A baby born to a parent who had exstrophy has a 1/77 chance of having exstrophy.
The urinary system:
Knowing the basics of how the urinary system normally works can help you understand the treatments for bladder exstrophy.
Here are the parts of the urinary system and what they do:
Kidneys: A pair of purplish-brown organs located below the ribs toward the middle of the back. Their function is to:
- Remove liquid waste from the blood in the form of urine
- Keep a balance of salts, water, and other substances in the blood
- Make erythropoietin, a hormone that helps make red blood cells form
Ureters: Two narrow tubes that carry urine from the kidneys to the bladder. If urine backs up, or is allowed to stand still, a kidney infection can develop.
Bladder: A balloon-shaped organ in the lower abdomen (belly) that stores urine. The bladder muscle squeezes to empty urine when a person pees.
Sphincter muscles: Two circular muscles that help keep urine from leaking by closing tightly like a rubber band around the opening of the bladder. The sphincter muscles relax when a person urinates or pees.
Nerves in the bladder: Signal the body when it’s time to urinate or empty the bladder.
Urethra: A tube that is attached to the bladder and allows urine to exit the body
What are the treatments for bladder exstrophy?
Surgery: The first treatment for bladder exstrophy is surgery to place the bladder inside the body. The surgery is almost always done early in life, soon after a baby is born.
After surgery, patients require ongoing care, which may include:
- Catheterization (passing a tube to empty urine from the bladder)
- More surgery is sometimes needed
The goals of surgical treatment for bladder exstrophy are:
- Normal kidney function
- Good bladder function
- Urinary continence, meaning no leakage of urine
- Making sure that the patient and their parents are satisfied with the appearance and function of their genitalia (private parts)
Medicine: Many people with bladder exstrophy need to take medicine to help the bladder work correctly. A person may also need medicine to treat or prevent bladder and kidney infections.
If you have bladder exstrophy it’s important to take your medications as prescribed by your health care provider, even if you’re feeling fine. This is because many of the medications prevent problems from happening.
Two types of medications used for teens and young adults with bladder exstrophy are:
Anticholinergics: These medicines relax the bladder, help the bladder hold urine longer, and take pressure off of the kidneys.
Antibiotics: These medicines treat and prevent infections of the urinary system.
Catheterization: Many patients with bladder exstrophy have trouble emptying their bladders completely and require help using catheterization.
Catheterization involves passing a tube through the urethra or a surgically created opening in the abdomen so that urine can be emptied from the bladder. It is usually done on a set schedule, multiple times per day (similar to if you were to urinate on your own). It is important to stay on the schedule that your health care provider gives you, even if your bladder doesn’t feel full.
Appearance of the external genitalia (private parts) can look slightly different following exstrophy surgery:
Mons (rounded mass of fatty tissue that covers the pubic bone – after puberty it is covered with hair): The mons is sometimes thicker
Clitoris: The clitoris can be split in two with exstrophy; this can be corrected with surgery
Most women with exstrophy are able to have a satisfying sexual life, and are able to have children. If you choose to become sexually active, it is very important that you use birth control and condoms to protect yourself from pregnancy and sexually transmitted infections.
For patients with exstrophy, pregnancy requires specialized care – talk to your health care provider right away if you think you are pregnant or thinking of becoming pregnant.
Infection prevention – It’s important to empty your bladder before and after sexual activity to lower your risk of infection. If you have questions about sex, pregnancy, birth control or how to prevent STI’s, talk to your health care provider.
Carrying your catheter: Many teens and young women fold their catheters and put them in a sealable plastic bag in their purse or make-up bags.
Beach: Some teens say having a mons area that appears more prominent can be a challenge at the beach.
- Bathing suit styles that seem to work well include swim minis and tankini with a slightly longer top.
- Scars are sensitive to the sun. Make sure to wear sunscreen with an SPF of at least 30, and reapply often.
Jeans: Some teens and young women are concerned by the appearance of the mons area in jeans.
Styles that tend to be more flattering include:
- Looser fitting
- Fabrics with some stretch to them
- Longer shirts/sweaters over more fitted jeans
Bladder Exstrophy Health Tips
Following your treatment plan will:
- Lessen your chances of getting urinary infections and keep your kidneys working well
- Prevent unscheduled clinic or ER visits, and overnight hospital stays
- Avoid having to take extra medicines
- Lower the risk of bladder rupture
- Help you feel well so you can participate in fun activities
Ways to keep your bladder healthy:
- Drink plenty of fluids
- Catheterize and/or urinate at least 5 times per day
- Empty your bladder before playing sports or going on a long car ride
- Take medications as instructed, even if you feel fine
- Flush your bladder or antibiotic wash (only if your health care provider tells you to)
Call your health care provider right away if you have any of the following symptoms:
- Fever greater than 101.5°F
- You can’t empty your bladder by urinating or catheterizing
- Severe pain in your abdomen (belly) or back
- Nausea or vomiting that doesn’t go away
- Unable to have a bowel movement (poop) for more than 2 days