Bladder Exstrophy, or BE for short, is a rare birth abnormality in which the bladder and its surrounding parts form outside of the body, flat, and inside-out. As the BE Association put it, “If you can imagine a balloon that has been cut in two, opened up so that the inside of the balloon is visible, you will have a picture of what has happened.”
The condition is believed to happen around the 11th week of pregnancy when the fetus’s organs are developing and do not fuse properly. It is then that abnormalities in the forming of the bladder, urinary tract, intestines, reproductive organs, genitals, and/or pelvic bones occur.
Bladder Exstrophy is a specific diagnosis under a larger group of birth abnormalities referred to as the Bladder Exstrophy-epispadias Complex (BEEC).
Individuals with BEEC are diagnosed with one of the following conditions:
Bladder Exstrophy can typically be diagnosed before birth through a routine sonogram or MRI. If the condition is not detected then, it can be recognized after birth by the following characteristics:
The most common and effective form of treatment is surgery within five years after birth. These surgeries have three main goals according to the Mayo Clinic:
If the baby has a less severe case of Bladder Exstrophy, surgeons can perform a single operation allowing the patient to urinate on their own within weeks of birth. Individuals born with more severe cases of Bladder Exstrophy may need additional surgeries.
In some cases, the bladder fails to form all together, in which case a new bladder must be constructed entirely.
One of the symptoms many individuals with Bladder Exstrophy experience is lack of ability to drain the bladder voluntarily, even if one can drain some of the urine, the residual urine that stays in the bladder can cause infections and severe complications. Therefore many individuals with BE use a catheter to fully drain their bladder.
Some catheterize through the urethra and some through a stoma, which is an artificial opening into the bladder. It is often put into place for use before the final reconstructive surgery is completed. To use and maintain the stoma, intermittent catheters are utilized in two different ways: to urinate and to irrigate.
In order to drain the urine, an intermittent catheter is inserted through the stoma and into the bladder. It may sound complicated but it can actually be easier than using a catheter through the urethra, imagine cathing in the car, all you need to do is pull up your shirt and drain.
Bladder irrigation is the process of washing out the inside of the bladder.
In order to clean the stoma and prevent contamination, the bladder must be frequently irrigated using an intermittent catheter. It’s easy! First, empty the bladder as described above, then take a syringe full of saline solution, insert the tip of the syringe into the funnel of the catheter, and squeeze the saline into the catheter, then drain it in the regular process. The saline will irrigate and wash the empty bladder.
Bladder Exstrophy is a rare and complicated medical condition, but thanks to modern medicine, individuals born with this condition live full, thriving lives. We wanted to give a shout-out to two of CompactCath’s long-time customers, who were born with BE and continue to inspire us.
Tommy is the youngest known person with Bladder Exstrophy to have climbed to the base camp of Mount Everest at the age of 12, you can read more on his story here.
And Bridget is a teacher, and a mother, helping others with BE better navigate their condition.
The organizations and conferences below are excellent resources!
The Association for the Bladder Exstrophy Community
The Association for the Bladder Exstrophy Community (A-BE-C) is an international support network for patients and families affected by Bladder Exstrophy. Their aim is to ensure that all people affected by Bladder Exstrophy have the opportunity to realize their greatest life purpose and potential.
International Exstrophy Conference
The International Exstrophy Conference, hosted by the Children’s Hospital Los Angeles in partnership with the A-BE-C, is an annual event providing educational sessions, fun activities, and opportunities to connect the Bladder Exstrophy community. This year’s conference will take place this weekend, June 28-30th, 2019, in Los Angeles, California!
The Youth Rally is an annual, five day summer camp for kids and teens with conditions such as Bladder Exstrophy, Ulcerative Colitis and Crohn’s Disease, promoting independence, confidence, and fun! This camp services as a great place for the kids to enjoy themselves and bond through shared experiences. During their stay, they are given the opportunity to form genuine connections as well as learn ways to combat their various challenges.
John’s Hopkins Medicine
John’s Hopkins Medicine is one of the leading experts in Bladder Exstrophy, and are a great resource for more in depth information on the condition.