Bladder exstrophy is a rare and complex birth defect that involves the urinary, reproductive and intestinal tracts as well as pelvic bones and muscles. Formed in 2010, the Multi-Institutional Bladder Exstrophy Consortium is a collaboration between Children's Hospital of Philadelphia, Boston Children's Hospital and Children's Hospital of Wisconsin dedicated to improving care and outcomes of children with bladder exstrophy. MIBEC's long-term goal is to standardize the surgical approach across each institution, to rigorously study and report outcomes as a national team, and to ultimately make improvements to care based on these findings in an effort to better understand the factors that result in urinary continence.
Bladder Exstrophy: A Multi-Institution Approach
Bridget: You want me to chase you?
Bracken: Yea, I do.
Bridget: Alright, I'm gonna get you.
Bridget, Bracken’s mother: Bracken was born one day late. He was so big. He was like a nine pound baby he was a big baby. He was absolutely a healthy baby except his bladder was on the outside. The day we got to CHOP we went to the fetal surgery center and we had the ultrasound and the MRI and the genetic counseling.
Jason, Bracken’s dad: We kinda came to grips with what was going on and the doctors came back in and we knew then that ‘OK, well what’s next? What do we have to do to give Bracken as normal a life as possible?’
Bridget: We had so many unknowns - that’s when we met Dr. Canning.
Jason: He basically took control of the situation. He explained what was gonna happen from the time Bracken was born all the way through surgeries and even into his future adulthood.
Bridget: He went over everything so that we knew exactly what to expect medically for Bracken.
Brynne Bonitatibus, MSN, CPNP, Nurse Practitioner, Division of Urology: MIBEC is the Multi-Institutional Bladder Exstrophy Consortium and that is the collaboration between the three hospitals which is Children’s Hospital of Philadelphia, Boston Children’s Hospital, and Wisconsin Children’s Hospital. It’s basically how we work together to treat these bladder exstrophy patients.
Bladder exstrophy is such a rare diagnosis and we see it so infrequently that in order to gain kind of the collective experience we decided that it would be a good idea to have surgeons from each of the three hospitals be present for each surgery because these hospitals that were seeing one to two cases per year previously, if they combined their experience they would see, you know, up to 10 cases a year.
Joseph G. Borer, MD, Director, Bladder Exstrophy Program, Boston Children’s Hospital: One of the major benefits of having a group of pediatric urologists with a specific interest in these rare patients who require complex reconstructive surgery is that we can work together, we can refine this operation and we can improve the outcomes for these patients.
John V. Kryger, MD, Chief, Pediatric Urology, Children’s Hospital at Wisconsin: Putting our three experiences together we realized that if we all participated in each other’s surgery as a team, we would more than triple our experience in all likelihood and get to see this procedure much more frequently and add to the volume of our experience.
Michael E. Mitchell, MD, Professor Emeritus, Children’s Hospital at Wisconsin: We can study the disease and we can study the repair of the disease and maximize everyone’s experience and the patients benefited. The greatest success would be when we get to the point where the patient never felt they were abnormal.
Douglas A. Canning, MD, Chief, Division of Urology, Children’s Hospital of Philadelphia: This is a very predictable problem with very little variation but without pooling our resources it’s hard to gain the leverage and the years of experience to really further the field without putting a group together. Like many things - the more experienced we are, the better our results are and that’s one of the greatest challenges.
Jason: I think the thing that – at least the thing that got me through it, you know – was knowing the team that was working on Bracken. It wasn’t, you know, it wasn't just CHOP it was the doctors from Boston, it was the doctors from Wisconsin as well, that collaborative unit. And knowing we have probably the world’s best here working on our son so you know, this is gonna be OK.
John V. Kryger: When I’m explaining it to a patient what I do is I tell them it’s essentially a weakness in the abdominal wall that didn’t fuse together and through that weakness, that’s how you end up with a bladder that’s outside the surface of the abdomen and exposed. And then the other pelvic organs, the pubic bones have come apart, the sphincter muscle has come apart and the reproductive track in a female can prolapse anterior slightly. Atomically, the parts are there and this is a deformity of the anatomy but yet the pieces are there and if we can put them together accurately and precisely and consistently we can hopefully restore the anatomy, optimize and restore the function, optimize the cosmetic appearance and really make an impact on quality of life that hopefully is better than ever achieved.
Douglas A. Canning: These children are otherwise absolutely healthy. They have a normal brain. They can run and jump and sing and do mathematics beautifully, but they have this rather devastating plumbing problem and it’s also very rare. It’s a rare disorder. It’s a surgical problem that can be corrected, completely corrected, with the right operation.
Brynne Bonitatibus: We do the one-stage repair which involves repairing both the bladder, the epispadias and the pelvic floor – the pelvic bones – and then we also taper the bladder neck to make it more anatomically correct and those are all things that help to provide these children with continence later on in life.
Joseph G. Borer: We see and we’ve talked about how devastating it is to an individual and their life and what they can do in their life when they don’t have the ability to stay dry. We want to see these children in two, three, four years of life develop some urinary continence and hopefully normal, dry intervals of two hours, three hours.
Michael E. Mitchell: One of the benefits of doing it in one-stage is we enable the functioning parts – the bladder, the urethra, the neck of the bladder – to work. Urine comes into the bladder, the bladder is cycling and our objective is to have a normal voiding patient that functions just like anybody who never had exstrophy.
John V. Kryger: We decrease the number of surgeries these children have to go through and that’s really important to lower their anesthetic exposure and their time spent in the hospital and hopefully if we achieve a better outcome we’re gonna be also lowering their incidence of infections, their time spent in the hospital with illness, improving their continence eventually.
Joseph G. Borer: Parents come to us because of our expertise and the institutions that we work at. They come to us under tremendous stress. And I think they want us to accept responsibility for the care of their child and they wanna know that we have a passion for that and that we’re willing to do that. And so I think in our consortium, one of the major benefits that we have is when I tell a parent or parents what I’m going to do for their son or daughter, I also tell them that they’re gonna get the benefit of several other experts in this field who have trained extensively in complex reconstruction and this is what they think about night and day and this is their passion.
Douglas A. Canning: It’s rewarding to know you’re doing even better than your best job cause you have the other people in the room with you. I haven’t been involved in my 30 years of surgical experience in a more rewarding program.
Jason: It’s gonna be nerve wracking. You’re gonna be introduced to a lot of things that people just don’t know about. Speaking with doctor Canning he said ‘Look, there’s senators that have bladder exstrophy, there’s National Hockey League players that have had bladder exstrophy. And to realize that, it’s definitely uplifting that bladder exstrophy is not a limitation by any means. It’s definitely something that you deal with in the beginning and it gets easier as you go along. It becomes routine, it becomes normal and it’s definitely not a limiting factor for him at all. He’s a normal kid, and he’s absolutely a blessing. He’s just perfect.