Bladder Exstrophy: A Multi-Institution Approach

Bladder Exstrophy: A Multi-Institution Approach

November 6, 2019 1 By Kody Olson


>>You want me to chase you?>>Yea, I do.>>Alright, I’m gonna get you.>>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.>>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?’>>We had so many unknowns – that’s when
we met Dr. Canning.>>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.>>He went over everything so that we knew
exactly what to expect medically for Bracken.>>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.>>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.>>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.>>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.>>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.>>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.>>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.>>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.>>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.>>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.>>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.>>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.>>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.>>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.>>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.