Transitional Urology

And we're back. So, uh, I hope that you all availed the chance to go and, uh, empty your bladders and, uh, do the bio break and get some hydration. Uh, we're back for the next session, which is, uh, devoted to a very, very important topic in pediatric urology, and that is how do we take care of our aging patients. And it's spawned the, uh, generation of a new field. Within pediatric urology and adult urology that kind of straddles and that's a transitional urology. Uh, we're so fortunate to have one of the thought leaders in this field joining us today, uh, Doctor Rosalia Masri. She is the chief of pediatric urology at, uh, Riley Children's Hospital in Indiana and a close friend of ours, and, uh, she is one of the leaders in this entire field, uh, uh, you know, as I mentioned, brand new field of transitional urology. So we're gonna have Doctor Maceri present to us and then after that we have a one hour case discussion where we're gonna be talking about some uh adults with congenital congenitalism and uh how we have to manage their um ongoing care. So big, big issue and Rosalia is gonna teach us how we can do a better job of this. Thank you so much for joining us today. Thank you so much for inviting me to speak today. It's, it's such a pleasure and an honor, uh, to be part of this, um, really a long-standing successful program that you've had, um, and I think so much to learn, um. Every year. Um, so I, uh, as a disclosure, I do not have any financial disclosures, but I am a pediatric urologist and I became interested in this field because As I was training, I realized that uh I was doing all these great operations and taking care of these kids and watching them flourish, but I really didn't know what was happening as they got older. Um, I was asked to write a question about transitional urology and it's quite difficult to write a question about transitional urology, especially after being, uh, On the American Board of uh Urologist Exam Committee where you have to think very hard about those questions and what the answers are. So I have a very simple question here and as we go along, we'll see what the answers um are to these questions. Um, I don't know when you vote or if you vote, but, uh, this is the question. And of course, it's not meant to be a scientific question. So, uh, Rose, we're not seeing a pulse, so if you wanna just go through the choices and tell us. Oh, sure. Um, so, our adults with congenital urologic disease are just bigger kids. They're a heterogeneous group. They're always a challenge, they're never a challenge, and they are in need of lifelong care. And I don't think the answers are really important to this, these questions, so, um, I'm just gonna move on from here. Um, why is transitional urology important? Um, there is a necessity. We are taking care of children that don't have a static, uh, disease. This is a disease that continues in this lifelong, and essentially I had curiosity. What happens? What can we do better in children so that they have better lives as adults? Um, when I look at kind of who needs our help the most, it's certainly the spina bifida patients cause that's the biggest group of patients that we see, but it does include the valve patients and the atrophy patients, the epispadius, um, The CAH patients but also hypospadius. So I'm not gonna talk about hypospadius today because I think that deserves uh several hours of talking about the issues uh with that. So I'm gonna focus primarily on patients with spina bifida um because that's where most of my uh knowledge is gleaned from. So if we look at pediatric urology and adult urology, there's certainly things that each group of, of us do better. Um, we probably are better at patient-centered relationship with the family and integrated care, but the adults certainly are better at the adult care, um, adult urologic screening. How many times does a pediatric neurologist think about things like PSA or fertility or sexual function? And so, In doing this transitional urology, I've actually learned to start thinking about those things maybe a little bit earlier um in life. We know that the needs of these patients are always changing. Um, the natural history of the disease varies from patient to patient based on what they've had done as children. Um, they have intellectual disabilities, so every patient gets to be treated differently based on what they are capable of, um, intellectually. Um, some have had complex reconstructions that need follow-up and their social structures changed. They have parents who are caring for them, uh, Meticulously and so devoted to them early on in life, and they get older and things change for them. When we talk about transitional care, there are probably a few different models, and these models really are based on what is available in your community. Um, some places you're forced to continue to care for these patients because there's not an adult urologist who can. They might not have an office that's, that's capable of having a patient in there who can't transfer from a chair to a bed. There's transfer of care where someone just says, OK, tomorrow you're gonna see doctor so and so. At their office, um, you send them along with a little brief summary of their, of, uh, their history, and on they go. Um, you can have transition where it's true transition means that the patient has seen both an adult and pediatric provider simultaneously, and that care is transferred in a very slow, subtle, and deliberate manner. And then, of course, there are those people who disappear from pediatrics and show up when things are really hitting the fan. So we tried to figure out how we were delivering care to patients with spina bifida as they got older and we found that 50% of pediatric neurologists continued to care for them and only about 20% co-managed care with eventual transition. Now the study is a few years old, but I don't feel like there's been very much that has changed. And in clinics that had um Urologists as part of them. These are adult multidisciplinary spina bifida clinics. Over half of them were actually staffed by pediatric urologists. So that shows you how sometimes this Continued task goes with the pediatric urologist. We also surveyed to see who thought Who should be taking care of the patients. So, um, uh, adult urologists who were part of reconstructive groups like Sufu and GRS felt that they were sufficiently trained to take care of these patients, as they got older and, uh, uh, a little over half of pediatric urologists felt that they were sufficiently, um, Trained. Um, it was kind of unanimous that general urologists weren't sufficiently trained in both the adult and pediatric group. Um, and of course pediatric neurologists thought they could do better than adult urologists and adult urologists thought they could do better than pediatric urologists. So again, the answers are all over the board and For that reason, I think that we need to, to really focus on what we have available in, in our um communities. Um, multidisciplinary clinics I think are very, very important. When I first started, I had a pedia I had an Adult spina bifida urology clinic and I didn't have all the other disciplines in there. And what I found was that I spent most of my time coordinating care for other things, you know, they had headaches or they needed a new wheelchair and things like that. So I found that over time, having multidisciplinary clinics uh for adults is also very helpful. Um, this is just a little schematic and some of you may have seen this before and some of you have been at Riley know the layout. So the spina bifida clinic is across the hall from the urology clinic, so I thought this would be a good place. Um, for us to house the adult spina bifida clinic. Now, you have to understand one other thing is that nowhere on campus would they give me any other space to have this clinic. So, um, as we have control over our office space, that's where, that's where it started and that's where it remains. We keep looking for better space, but this to date is still the best space we have. So again, that local um environment is really, really important. Um, We look to see how successful we were at transitioning patients and you can see this bar graph kinda looks a little dismal and a lot of what I'm gonna talk about today sounds dismal, but I have to tell you that what we do is very, very important and we are very, very successful. But I think we know all the good. I think we need to just be aware of what is important to follow as time goes on. So this is prior to having a multidisciplinary clinic where we just had urology care and you can see that over time the number of patients lost to follow-up got a little bit better and the number of patients that we saw in clinic transitions a little bit better aside from that little lapse in 2012. Why didn't they come? It's really hard. Is it that we prepared them poorly for transition? Was that they were newly independent, now that they're 18, they're not doing what their parents tell them to do. Did they just get tired of, of seeing the, uh, the doctors? It's probably a drag growing up and having to see a doctor on a reg uh on a regular basis. Um, so you can make it as easy as you want, but the patients need to wanna come. But the most important thing in these clinics is that the care is uninterrupted and it's developmentally appropriate. We can't treat every patient with spina bifida the same because their needs are very, very different and their abilities are very, very different. And I think it's very important to pay attention to unique quality of life concerns and life goals in these patients because What's good for me or a patient who's an ambulatory, uh, man with spina bifida may be very different from a, a woman who has spina bifida and has lived her life, uh. Using a wheelchair for mobility. So we have to keep that in mind, and there are also incredible obstacles and threats to multidisciplinary care. Um, I already touched on the space. You have to have staff that's willing to do this. So, not every nurse in my clinic likes caring for adults, and not every, um, MA in my clinic knows what to do with them. Um, you have to have the hospital's support and make them understand why you need to do it where it is. And some hospitals have a, a drop-dead date of 21 years and You can't come through the doors if you're over 21, and, and these are all things that need to be mitigated. There's certainly finances, uh, uh, associated with this. This is not a moneymaker or cash cow for the hospital. Um, these are multidisciplinary clinics, so they take more time to see the patients, and there's, uh, reimbursement for multidisciplinary care has always been, uh, an issue and something that's discussed on a, on a continual basis. When or why we're doing this? Do we know if we're improving follow-up or improving outcomes? I think that needs to be determined. I don't think anyone in the world, whether it's in the United States or abroad, has actually proved that if you continue to follow these patients in a very organized and uh Meticulous way that you're changing their outcomes. Um, and also remember that if this is something that you want to embark on and you're an American pediatric urologist, that your practice has to be at least 75% pediatric urology. So that might mean you might not be able to dedicate all the time you want to, to this, um, To this endeavor. Now, I've learned a lot of lessons and uh a lot of these lessons were learned through looking through these giant enormous charts that fortunately have been transferred into electronic medical records which also makes it a little bit more difficult to look through. Um, and these things are, are just lessons looking at patients and learning these things, reading about them, um. We know that independence varies on age. That happens with able-bodied patients and, uh, and patients with disabilities, but shunts and the use of mobility equipment decrease independence in these patients. Um, things like seizures, socioeconomics, and ethnicity. So in some ethnicities, if you have a child who has a disability, you treat them as they are. In in need of your help at all times, whereas in other ethnicities and other families, they're given the same responsibilities, chores. And expectations that their other children have and there's certainly familial stress associated with this. It's not uncommon for us to see families torn apart because a patient has A disability that one or the other uh parent can't cope with. These patients, their interest change as they become adults, and they're gonna do the same thing any other teenager or young adult does, you know, they smoke, they drink, they have sex, they want jobs, they want relationships, and most of all, just like all of us wanna be accepted. Once they graduate from school, things really do change for these patients, uh, especially those whose only social interaction is going to school. And if their mobility is, is limited or their executive function is limited, um, they're gonna integrate very poorly with society once school is done. And it's not uncommon for me to hear from The parents of patients that they have so few friends that they become their only friend and so then they're, their parent, their friend, and their caretaker, so that becomes a little bit difficult for them as well. Um, the other thing that's very striking to me is how parents get old and they become less and less avail available and able to care for their, for their adult children. And so these patients end up uh going to their siblings or group homes or nursing facilities, and these are demanding, uh, disabilities that these patients have. So, uh, not only are their physical disabilities but their disabilities about, well, how do you catheterize a patient or um how do I interact with the patient who has an, an intellectual disability. Um, money is an issue, you know, many of them need to be on Social Security because they're unable to work. So lots and lots of challenges for these um for these individuals as they're, they grow up. And we all know, uh, especially once you pass the fifty-year mark that sometimes it's like the wheels fall off the bus and, um, and it gets hard to get old and your medical complexity increases. Uh, adults with spina bifida use Many more healthcare resources than kids with spina bifida do and um they're admitted 12 times more to the hospital than uh healthy adults. Um, it's hard for them to find primary care doctors. And there are also issues with guardianship and decision-making that are important, and these are things that we need to start thinking about as they're approaching the age of, uh, transitioning to adulthood because someone needs to make, um, decisions for them. Somebody needs to be able to sign consents for them, make sure that their, um, bills are paid, that their banking is in order, so, uh, lots of, uh, burdens for families and, um, and caretakers. Um, as you get older, you tend to pack on a few pounds, um, but in this patient population, it really, uh, does change many things, um, because it affects their mobility and, uh, it's harder to move around. Um, obesity also will interfere with simple things like being able to catheterize. It might decrease their willingness to, uh, to catheterize on a schedule. Um, as, uh, Doctor Stevenson, uh, spoke about, there are some changes that are neuromuscular as well. Um, And decreases in upper body strength are, are very important. Imagine a patient who's able to transfer from their wheelchair now not being able to because their upper body strength has diminished or they have rotator cuff injuries from that constant transfer. Um, we know that there are urologic consequences. We talked about the difficulties catheterizing, uh, because of body changes, but it's also very difficult to examine a patient. You know, sometimes it takes 10 minutes to get a patient transferred from their chair to the bed so that you can examine them and uh certainly this becomes an issue when you have a busy clinic or you're trying to be as efficient as possible. And certainly for those of us who, who operate on adults, uh, operating on them is, uh, no chore. So every time I am about to start something, I always say this is why we operate on patients when they're When they're young. Um, everything becomes harder, the complications go up, the positioning becomes difficult, more difficult. Um, I think, um, Doctor Vanderbrink and lots of people on the call have done a lot of work trying to figure out what, um, and how to evaluate renal function in these patients, and it's primarily because of their body habitus and their muscle mass which is low. So, um, we do know that there is renal impairment. And up to 40% of adults with spina bifida and that end-stage renal disease, although we feel like we've done a really good job of decreasing the risk of, uh, people dying from kidney disease, still does occur in this patient population. Um, why do the kidneys seem to, um, Change over time. So, uh, I've looked at this really closely. It seems like, you know, you, you look at an ultrasound over and over again, looks great, looks great, looks great, and then somewhere along the line, you start to see a kidney that doesn't look quite as good as it did when they, when they were 10 or 11. And I think that we have to think about adherence to um To catheterization schedules, we have to look at adherence to um medication schedules. So, you know, you for if you forget to take your antimuscarinic once, twice, 3 times, it might not be a big deal, but if you forget about it for a month or so, that might cause injury to your bladder. But we have to continuously think about reflux, abnormal voiding function. And then in addition to all the things we think about in kids, we have to think about obesity and hypertension, right? These are major causes for uh renal injury in, in able-bodied adults as well. Uh, UTIs in this patient population are probably the, uh, most challenging and troublesome things, you know, um, and I think part of it may be related to Not being very strict about what urinary tract infections get treated in childhood. Um, so I continuously spend time repeating and telling patients do not have your primary care doctor check your urine looking for a UTI if you don't have any symptoms, um, because we know that up to 85% of them will have chronic bacteruria. Um, And that this makes up a lot of hospital admissions, so I can't stress enough to patients and their families that these UTIs should be treated or this bacteruria should be treated only when there are symptoms. And interestingly, these patients may not have those typical symptoms that we think about the burning with urination or the suprapubic pain. It may be malaise, it may be headaches. Um, so always really listening to the patient and understanding what they know as their symptoms of UTIs. If suddenly a patient starts getting UTIs, you need to start thinking about why is this happening. Um, do they have a new stone? Is it how they're catheterizing? Is their bladder changed, the bowel movement changed? And are there things in the surgical, uh, realm that, that occur? Do they have neo hydronephrosis or diverticula or these post-surgical changes? And I'll tell you that more often than not, it's catheterization technique and people become sloppy. Um, don't wash their hands, don't use lubricant, um, and then start having troubles with UTIs. Um, so really kind of remembering that there are some cognitive difficulties and that we need to pay attention to those. Um, we know that in continence affects quality of life in a negative fashion, and it also leads to, um, skin breakdown and infections, um, as, as well as underemployment, so that's a rampant problem in the spina bifida population is underemployment and being incontinent makes it even worse. Um, John Wiener has done some great work looking at fecal continence, uh, based on the National Spina bifida Patient Registry, um, and has noted that there are there are differences based on age, um, and that about, uh, just a little over half of patients are continent. Um, it seems that older people rely more on less, uh, Um surgical, uh, ways uh to address continence versus the younger people who more likely have ACEs. Colostomies are used in adults and I do have several patients who have, who have failed MACEs, have failed, um, Medical management of their constipation and have moved on to colostomy and are very, very happy with that choice. So don't, uh, dismiss that. Of course, you know, in kids, we, we find it a little bit unsavory to have ostomy appliances, but it, it sure seems to make lots of adults happy and that they're not worried about it. Um, And I do think that colonic transit time changes with uh with age, um, and I do think that the bowel itself changes, um. And the amount that it could hold and the like, and I do think that this, uh, adds to the worsening, uh, fecal um incontinence that can occur in adulthood. Um, now I'm gonna talk a little bit about surgical considerations, and I hate that everything that I'm gonna show is a complication of our surgical, um, outcomes, but again, I wanna get back to what is it that we need to think about to avoid in childhood so that we could have the best success in adulthood. So, um, along with the group in Cincinnati, we looked at, uh, risk of reaugmentation and we did find, find that if you detubularized and augment and you reconfigured it, that, that risk went down significantly. Now, fortunately, I think even before the, uh, paper, Came out and we looked at the data, um, we did, um, Hypothesize that this was the case and then these patients, we found that they had intractable incontinence, small capacity bladder, and upper tract changes and you can see in the image on the screen how you see almost a waste between the um Between the native bladder and the augmented bladder and this patient certainly uh had improvements with, with reconfiguration of their um Um, Augment. I'm sorry, a little technical difficulty there for a second. Um, we've also looked at perforation and if there's one thing that I'm worried about in a patient that I'm planning on augmenting and it, it's perforation. And this is a difficult thing to tackle, right? Uh, when, when we're when we're doing augments, the kids are usually under the care of their parents and, and there's somebody who's watching them. They go to school and they get catheterized there, but once they become adults, This may not be uh the case at all times and with a decrease, um, In executive function, things like keeping time or knowing that if I don't catheterize, I may perforate, may become an issue. And so similarly in this case, we found that if you detubularize the bowel and you reconfigured it, that that risk went down to about 9.9% over 10 years and that the risk of reperforation also was lower. Now, this is pretty scary because once you've had one perforation, even if you detubularize and reconfigure The bowel, that risk is at about 30% and um And this is, uh, this is a major concern to me, and, uh, at what point do you, um, uh, well the residents and fellows make fun of me for saying lose your bladder privileges and talk a little bit about that. Um, so yes, there are patients that lose their bladder privileges and these patients have gotten diverted and in our, in our series, it's about 3% of the patients and most of, uh, most of them were either because of intractable incontinence or um or perforation, um, with perforation now leading the pack, um, as a, as a reason. We are always concerned about malignancy. I'll spend just a quick second talking about it because I do think that this is important and I think that there's controversy associated with it. So, um, uh, what I use is the criteria that, uh, Doug Husman put forth, and I think he put a lot of thought into this along with, uh, uh, Tai Higuchi and so, um, I, I think that patients who are smokers immunosuppressed have gastric augments, um, Probably need to be Seen more, more frequently and whether or not you wanna do cystoscopy on them, I'm not quite sure. I, I would leave that up to you and how much you think the patient uh will communicate with you, but I can tell you that the gastric augments and, um, fortunately, I made it 15 years before having one patient have uh a malignancy. Um, that, that malignancy was in a, in a patient that was scoped once a year, um, by me. I found a small tumor when I did her surveillance cystoscopy and within 6 months, she was, um, She had expired um because the, the tumor was just so aggressive. Um, I use um Doctor um Husman and Higuchi's criteria for endoscopy, uh, certainly if they have gross hematuria or new pelvic pain, I think they need to be seen, uh, and scoped, um, and, um, If they've had new or uh more recurrent UTIs. Uh, before scoping them, if they've had more UTIs, I always get imaging, making sure that they don't have any stones as the cause before we just do a surveillance, um, endoscopy. Um, the other thing I worry about immensely in, uh, in this patient population is bladder neck procedures, and I think that there is a role for them, particularly if the patient's having skin breakdown, poor hygiene, um, if it's affecting their quality of life, but I think you have to be very, very, very selective about who gets a bladder neck procedure. The patient needs to be motivated. The patient has to have an excellent way to empty. So, um, either they have Some sort of incontinent diversion, an ileal chimney or the like, or uh an appendicovsicostomy or a Monty that has been um tried and true, has never been a, a problem, but they also need a good person to support them. Uh, somebody's gonna troubleshoot catheterizing their channel at home and a team in your clinic that's gonna be able to troubleshoot it. If you're on an island by yourself, this is not a good operation for a patient. Um, Many groups, um, have looked at the long-term fate of the bladder after bladder neck procedures. Um, Doctor Vanderbrink ourselves, um, have looked at it and what we found was about 45% of the patients who seem to have a safe bladder prior to bladder neck. Procedures actually went on to require an augment and the image that's shown here is an image of a patient who had a very normal appearing bladder preoperatively. Who postoperatively within 6 months had a bladder that had decompensated and became quite unsafe. Um, Doug Husman again looked at these patients and reported his uh experience and he found that Almost 70% were not compliant with catheterizations and 40% of them developed renal scarring. Afterwards. So this is sobering data. So really patient selection is key and patient follow-up is key. And I think before we embark on any surgery in a child, we need to start thinking about what is gonna happen 1020, 30 years from now when they're adults, when they're on their own, when their siblings are, are caring for them, or when they're in a group home somewhere. I think catheterizable channels have revolutionized what we can do in urology. Uh, I think, um, learning about catheterizable channels is the thing that made me want to become a urologist, believe it or not, because I thought it was just the coolest thing ever. Um, and I still think that they're amazing and, and really change people's lives. Um, imagine having a child who needs to be catheterized when you're at The supermarket, you know, what do you do? Do you put them on the dirty floor? Um, do you expose their genitalia in a public place? Um, these are all considerations and sometimes the surgeries that we do for kids are really to help the parents, um, To, um, better care for them. Um, channels are fraught with some problems, about 20-30% of them will have problems. Many of them, however, can be, um, treated without surgical intervention, and many of them will get better just by putting a catheter in and, um, letting things settle down for a little bit. But you always have to be ready to scope these patients. Um, don't delay that and, um, having a scope ready and available in your clinic at all times for when they show up is quite helpful. Uh, fortunately, we now have, uh, lots of disposable scopes on the markets that are helpful. Um, if the channel is incontinent, always think about What's going on with the bladder, but what did, what happened? During surgery that made this happen later on. Um, many of these channels will need subfascial revisions, and these are usually because there's a stenotic area along the channel or polyps, uh, may have grown within them. Um, redundancy and prolapse seems to happen more with rapid, uh, weight changes, whether up or down. Um, these tunnels do get longer with time, and I think they telescope on themselves. I think catheters catch them in certain areas and make it harder to catheterize and less rarely fistulas and hernias do occur, um, and I've seen. At least one fistula where, you know, this was a a a catheterrizable channel that had been created 15 years prior but then subsequently developed a fistula. Where you put things really really is important because the stomas can become hidden. It makes it harder for the patient to catheterize and then next thing you know is they stop catheterizing because it's too hard, um, but also they can kink. Um, remember where your mason and your, um, appendicovsicostomy or your monty may be, if they're too close, poop is gonna get on them and they're gonna get infected. Um, Rick Rink has taught us all short, straight, and supple for your channels, and I think that this really is, um, key to success with these channels. And I also think it's helpful to fix the channel to the anterior abdominal wall. So what I do is hitch some stitches alongside the channel on the bladder to the anterior abdominal wall. And that seems to help as well, um, to avoid any kinking that can occur with recurrent, um, catheterization. Um, A few years ago, we looked at um where to put channels and it, and what kind of channel was most successful and an appendicle vesicostomy to the right lower quadrant seems to be the most successful with a spiral monte to the umbilicus being the least successful, and when I say least successful, I mean more prone to needing um Of revision. Um, again, going back to Houston because I think he's thought very, um, carefully about a lot of these transitional problems. He found that the lowest Revisions were in tapered ileum and reinforced ileocecal valves, um, or the hemi indiana. This is not something typically that we use in kids. Um, perhaps we should consider this, but we're also, um, so worried about their bowels that I think that has kept us from, um, from using the Hemi Indiana pouch in kids. Um, I'm gonna change, uh, tunes a little bit now and go to something else that I really did not think about when I started, um, on this, uh, journey of taking care of adults with spina bifida. Um, they think about sex and they wanna hear about sex. So, uh, 92% of them wanted to hear about sex from us, from the physician, and about half of them. Couldn't recall ever hearing about sex, um, from us as physicians. Um, so a lot of them think that they are unable to have sex or they're unable to become pregnant and we probably should, um, should remember to talk about this with them. Um, Activity and sexuality are affected by several things, um, One of them being lack of privacy because they spend so much time with their families. Um. And, um, and they are sexually active. What we found in our study was that 91% of men masturbated, um, and 62% of the patients who answered, uh, had vaginal intercourse. Um, when we asked about the quality of their erections, about 40% had erections that were firm enough for sexual intercourse, um, and the ambulators were more likely to have normal erections than the non-ambulators. Now, what we don't know, um, very well from the, the study and how we, um, how we did the study was, um, what their levels Where we did separate between ambulators and non-ambulators and those with VP shunts and no VP shunts and we found that those without VP shunts um had similar erectile function as those with VP shunts. Uh, fortunately, we know that there are some therapies, um, PDE5 inhibitors were useful in these patients. Interestingly, anytime I ask in clinic, no one ever asked for a PDE5 inhibitor, uh, and they shortly after clinic will call and ask for a prescription to be called in. Um, the Tomax procedure seems to show some promise, uh, however, the, uh, patients need to have a pretty intact, uh, nerves in order for this to be successful. Uh, paternity does occur, um, and it's up to 73% in those actively attempting. However, we have to think again how many, who is actively attempting, um, is it the patients who have lower lesions or is it those who have higher lesions? My suspicion is that these are all patients who have L5 or sacral lesions that are trying. Um, sexuality and, uh, and sexual function in women is also affected by spina bifida, um, and it is level dependent. Um, it's rare to have orgasm, uh, if your lesion is above L2. Um, these are some, uh, photographs of children. They are, um, random children, but nonetheless, I have been involved in several, um, births, um. When we talk to patients about sex and sexuality, we should also talk about pregnancy, particularly in, in women. Um, about 4% Uh, there's a 4% risk to offspring of patients with spina bifida, and folic acid is the most important thing that we can offer them. These patients are high-risk patients, so the minute you find out one is pregnant, you should probably refer them to an MFM. Um, they have urological complications. Uh, all of them, uh, that I've been involved with have had worsening incontinence and hydronephrosis, um, but they're also at risk for infection and injury to the reconstruction. In fact, one of my patients has had, um, 4 children. She had uh singletons and then twins, and the way she knew that she was pregnant each time was because she had a urinary tract infection which she typically doesn't have. Um, uh, Courtney Stewart, um, and the folks at the University of Michigan have done a lot of work with women, uh, with spina bifida, and they found that women with spina bifida are much more likely to have Um To have C-sections, and this was disproportionate with the normal population. Now, many times, uh, I get calls from former residents who, who say, I have a patient with spina bifida who's pregnant and they want me to participate. Um, so it's probably not the most fun thing to do because it's very stressful. Now you have, you know, typically we're responsible for one life, now you're responsible for, for two or at least um partially. Our goal primarily when we help with C-sections is to protect the reconstruction. Um, the obstetricians that I've worked with are very, very happy to have us there, um, to help guide their decision-making during the, the deliveries. And I think in many cases I've found that it's very helpful um for me to be there to avoid injury. So when you look at the um at the, the augmented bladder, it typically sits in front of the uterus. As the uterus grows, it kind of pushes the mesentery to the augmented bladder off to the side. Uh, many times the augmented bladder is just plastered to the, um, to the uterus, um, but sometimes you get just a small enough window that you can start working, um, toward getting that, that baby delivered. Um, there are landmines everywhere. Um, these patients have VP shunts. Typically those are not seen during the procedures. Artificial sphincters and that's, that fine tubing is, um, is something that's, uh, quite tricky for the, uh, gyne the obstetricians to get around. Um, if they've had slings or bladder neck procedures, I do think that a C-section is warranted. If they are, uh, ambulatory, they have good pelvic floor strength, they haven't had anything done to their, um, bladder neck. I do think that, uh, a vaginal delivery may be attempted. Um, during these cases, the vascular pedicles to the augment to the channels really need to be protected because they are, um, they're stretched out, they're tenuous, and they're right in the way of where the obstetrician wants to get to deliver that baby. Um Looking at uh reconstructions and pregnancies. They, they are difficult pregnancies, um, about, uh 45% of our patients needed nephrostomy tubes during the pregnancy. Um, Another patient who's had two children, um, as soon as she becomes pregnant, gets flank pain and needs nephrostomy tubes. Um, most recently, she, um, had flank pain, found out she was pregnant, got nephrostomy tubes, but unfortunately had a miscarriage. Um, so these, these things, uh, do occur and you need to be out on the lookout for them. Um, catheterizing becomes more difficult as the channels get kinked or the urethra becomes less accessible. Um, Over 2, about 2/3 of our patients needed emergent delivery. Um, so we try to schedule them, however, um, sometimes it's, it's happens at inopportune times in the middle of the night. And the other thing that 2/3 of our patients had were classical incisions in the uterus. So typically the uterus is in size. Horizontally and the classical incision requires a vertical um incision, and these vertical incisions put the patients at increased risk for uterine rupture later on. Um, even with us involved, uh, there were cystotomies and bowel dissocializations that occurred. Um, Moving on from the kids, um, and really looking at what makes adults with spina bifida sick, um, There are um Lots of reasons, uh, the primary, uh, Some of the primary reasons are infectious and pulmonary, um, This is um Uh, from one of my patients funerals, and, um, I kinda keep this up in my office because I, it's It's a terrible image to me that this life was so bad that being out of a wheelchair and, and being free and, and being dead is, is a better choice, um, but, uh, I, we can only, um, I think empathize with, with these patients and what they go through. Um, so, We have to keep thinking about how, what are we gonna do as urologists to keep them alive and I think our goals really are to, to keep those kidneys as healthy as we can. Uh, Brad DeSiano who is a physiatrist in, uh, Pittsburgh has done a lot of work looking at causes of death in these patients. We can see that acute renal failure is one of the causes, but septicemia and pneumonia are quite high. Uh, recently, this is a multi-institutional study, um, that Conrad Syansky, uh, Kind of, uh, spearheaded and you can see that uh our causes, the causes of death in the institutions that uh looked at this were um Unknown in, in many cases, but um, Neurologic and pulmonary in, in many cases as well and this was um uh Kind of what we thought we might find, uh, but certainly, um, But certainly, um, a bit worrisome, uh. For us because it, it just reiterates the need for this multidisciplinary approach to the, to the adult with spina bifida. Um, similarly, we found that, uh, infectious death was the most common cause of death for patients with bladder augmentations. So I'm getting to the end here and um Here's another question and I hope you can see the choices this time. Um, And it's not a difficult question I meant to tax anybody, but the care and treatment of adults with congenital urologic, uh, conditions requires knowledge of, uh, prior surgeries, their support system, their reproductive goals, their continence goals, and their comorbidities. Um, I would like to um Summarize, um, I think kids are very easy compared to adults. They're cute. Their parents do things for them, um, and this is always the case, um, in adults, and we have to really, really, really focus on protecting those kidneys long term and keeping them as healthy as we can. And that's the thing that we can do as urologists. We can encourage them to see other specialists. We can encourage them to, um, To catheterize and take their medications, um, but I do think that, um, our role is very, very important. Um, Additionally, we have to protect our reconstruction, um, that sex is almost on everybody's mind, that people get sick and die, and that we can't abandon the patients. So if in your community there's not somebody who you can count on to help, Uh, you may need to do it yourself, and, and it is what it is. Those of us who are fortunate enough to have adult colleagues who are willing to help, um, We are quite lucky, um. In that, in that regard. Um, so, Finally, I, I just wanna thank all the urologists on the call, um, all the people that have helped train me who have contributed to the, um, to the literature, um, And who keep uh working every day to make these, uh, patients have better lives, um, because at the end of the day, I think what we want is to see healthy adults. Um, And I do have some things that I want for the future. Um, it's a, it's a great list. Hopefully, we'll get to them at some point. Thanks all for your attention and I'm happy to take questions. Doctor Maceri, Bob Defore, how are you? That was a fantastic talk and um you do such a, a great job kind of uh summarizing so many issues that these patients deal with as they transition into the adult world. Can you um talk a little bit about um how do you have sort of a set process where you have a, an adult colleague that always sees your patients, or do you have like a transition clinic where you both see patients together kind of with the Frenberger model that we've heard before? So, um, Essentially what happens is they, um, they graduate from uh pediatric multidisciplinary spina bifida clinic to adult multidisciplinary spina bifida clinic. Now, because this is something that I'm, I'm very passionate about, I think it's, it's done. So much to help me care for um kids with spina bifida in a more thoughtful way that I continue to do it. Um, one of our newest colleagues, Josh Roth, did a year of um adult uh reconstruction up in Minnesota where he was able to train with Sean Elliott who has a, a pretty, uh, Large group of uh patients with neurogenic bladders that he follows, so he's come back and uh has helped and Certainly, um, has taken some of the burden off of me. Now, patients who transitioned from other pediatric urologists to me are, now we're trying to convince them that they need to transition to Doctor Roth, but, um, but nonetheless, it's something that I still feel passionate about and, and I'm willing to dedicate part of my time to doing. So, uh, we do not have uh Um, An adult urologist, we have someone who's trained in adult and pediatric reconstructive urology who helps out similar to um uh perhaps the situation in um Chicago with um Diana Bowens. Do. Missouri, um, Amman Mehdi, the, uh, uh, adult neurologist here at the University of Cincinnati. Thank you very much, very, very educational, uh, talk that covered a huge problem in a short time, relatively short time. I just have a couple of comments and a couple of questions if you don't mind me. Um, So the, uh, I mean, many of the issues that you brought up actually gonna come up in, uh, this case discussion to follow this session. Um, but, uh, speaking of the body habitus and the difficulty examining those patients that add to the burden of our clinical efficient clinic efficiency and staffing issues, I tended to do the exam during either urodynamics or cystoscopy just because they gotta get positioned and undressed anyhow for those procedures, just a, a point I'm trying to make. Uh, and also you highlighted, uh, briefly on the, uh, modified Indiana pouch. It tends to be my, uh, most favorite divergent procedure because I can also use a CCA as a bladder augment at the same time. Um, interestingly, I've heard a lot about the issue with the diarrhea in the pediatric population with this procedure, but interestingly, in our adult population, they, they tend to have constipation that resolves after this surgery, uh, which kind of bonus they, they get oftentimes. Um, Regarding the cancer screening in um gastric augmentation patients, um, between myself and our pediatric compound group, uh, we caught 4 cancers. Uh, 3 of them actually were metastatic at the time of diagnosis. The only one that we were able to save was a young man who had a dysplasia of the, uh, gastric portion of the augment. And interestingly enough, those are the patients that got, got the gastric segment because they have a baseline end-stage renal disease. So then they proceed to transplant, and now they start taking immunosuppression therapy, and that makes the risk kind of increase for cancer development. So the, we're able to save only one of those 4, unfortunately, and the rest of them just get kind of salvage, um, cystectomy divergent. Now, um, the screening itself, there is no standard way of screening. Doctor Pramodridi, uh, uh, led a symposium a couple of years ago on this very topic, just to see if you come up with a, with a protocol. Uh, there are a lot of difficulties to screen those patients, you know, many of them have, uh, bladder nuclear reconstruction or closure, so our only access to the bladder is actually the stoma. Which barely allows a large enough caliber scope that we'll be able to irrigate the mucus out and have a better visualization. In addition, they have the symptoms of hematuria, dysuria to start with, which kind of mask the picture of cancer development, which added to the difficulty of screening them. And if we're able to see a lesion, the biopsy becomes another. Uh, challenge, uh, just given the, the small caliber scope. It's just some practical point that myself, I faced during the practice. Now, the two questions I have for you, uh, number one, it seems to me, we, the providers, the patients and their families are the only sufferers in, in silence. I don't see this issue is brought up to a higher level, to the government level, legislative level, even, even half the administrative level, and I can see from your talk, you mentioned that you were trying to find a space to get to a transition clinic space, which is a very basic request, but you don't pretty much get that support from the hospital leadership and reimbursement and finances are a major part of it. How do you think can we make our voices heard at a government and legislative level to kind of help us kind of solve this problem as a As a, as a global uh level other than just providers and patients. Uh, you know, you bring up, you bring up quite, quite a, a number of points that I agree with you on completely, you know, the issue. Um, just going back a minute to your comments, the issue with, uh, scoping these patients and doing it effectively. I, I think it's, it is quite a challenge. Um, I think, uh, being creative in the scopes that we use helps a little bit, but I, you can never scope, a bladder. Through a channel, the way you can scope a bladder through a native urethra. There's, there's absolutely no way to get into every nook and cranny. Um, but now going on to your question, the, um, I, I think we really need to get patients fired up about this because if, as, and we can support them as physicians, but if the message comes from us, To hospital administrators, to legislators, um, I think we're just the doctors. We're not living it, but if you can If you can eloquently state your case and if enough people eloquently state their case, perhaps we can, uh, we can, um, Make a change, but we have to get, we have to get the patients fired up about it. And so many times I've asked patients to write a letter to the congressman, uh, call, call your insurance company, and it's, it's something that you have to really be motivated to do. Um, so I look to organizations like the spina bifida Association. Um, for their help and their, their ability to lobby, um, the government, um, but I really haven't seen that much change, uh, at least in prior administrations, and, and I'm not sure that financially we can afford it in, in the current administration, but I, I think really getting the, getting the person, getting The person with the disease to make the first move and then having all the stakeholders contribute might be, uh, might be the best way to do it. Thank you very much. Rosalie, I, I really enjoyed your talk and um one of the lessons I've learned from transition is, uh, It takes, uh, several years before you can come to some conclusion about, uh, say, transition readiness, right? That this is a process of, as you said, I already know there are some patients that will never, ever leave their parents' house, and you and I probably take care of some children that are getting their bachelor's degree and quite Um, very, very cool is that one of our spina bifida patients has gone on to medical school and has asked to shadow our pediatric urology clinic. So, as you said, it's such a huge spectrum. At what age would you recommend starting that conversation with your pediatric? Patients and their families to kind of say, what does the future look like? You may not be going to the Riley campus, or, well, I guess that's where this is, or those that may not have it, uh. Um, words of advice for where maybe you're gonna have a hard stop and that patient can't come in your facility after age, I'll pick 21. What would you recommend to those that don't have the ability to have the perpetual care model? Uh, excuse me, I, I think 14 is probably a good age to start. Um, if, if you're gonna go to 18 or maybe 16 if you're going to 21, um, I, I think, you know, and you know this as well as I do. This is the most heterogeneous of heterogeneous groups, right? It's, it's all kinds of education backgrounds, all kinds of family and financial support. Um, but I think, I think it's probably at that 14 to 16 years. So you want them to be stable enough that they're not having more surgeries, right? And you don't want them to be too old to where you're springing it on them the time the, the minute before they're, you're gonna send them off to somebody else. So, um, so I think probably 14 is, is that age. Um, I think at 14, The patients who are gonna understand what it means. Um, might also be invested a little bit in it and start to see themselves as more independent beings and the like. Um, so I, I, I think that would be probably the best age for, for everyone involved. Yeah, that, that's kind of the, my practice pattern has been around that age. Uh, as, as you know, somebody threw out the number to say, it takes usually on average around 7 years for you to Build up that uh skill set and education of the patient in those that had successfully transitioned. Um, I've always found it amazing that when you do ask, say, the, the 1213, 14 year old, uh, do you know why you use a catheter, they'll look at you like, I have no idea. And, and did you know you have scars on your body? And, uh, they kind of look at you. First of all, probably for a decade straight, nobody maybe reliably interacted with them, depending upon the provider. So they're used to their parents answering all these questions, but um, I've continuing and repeatedly been um Uh, not dismayed, uh, but just shocked at how little information the patients themselves have of their body and the surgeries, the doctors they see, um, the scars in their body and what they may represent. Uh, so, sometimes, I, I agree with you. Starting really early and just educating what kind of was your medical history. That can be a several year long process, let alone skill sets for what medicines do you take? Where do you get medicines? Where do you get catheters? How do you get catheters? These, like you said, the executive functioning, uh, abilities in the, specifically the spina bifida population, which represents the largest, is, um, Never to be dismissed. And I think many providers like to shy away from the patient because it's easier to talk to a non-executive functioning disabled parent, but that may not be the best long-term solution. Uh, I, I actually have converted to, once you turn 12, I turn my chair and look at you, at the patient rather than the family. And I'll tell the, the family quietly, you know, I know you're listening, um, because I feel like at least they start to, they may start investing some, some attention or some care to things, um, and, and And those kids I've seen kind of them be more engaged, asking more questions, wanting to know a little bit more about different things. Rose Lee, uh, thank you so much for a fantastic lecture on a very important topic that I think we all struggle with on how to do a better job. And, uh, one of the things that we've done here at Cincinnati is that we actually made it a quality improvement project to say that, you know, we were successfully transitioning our patients. But, um, you know, um, if we just ask families to do that without giving them guidance and where to go, right? Because as you mentioned, there's, uh, a lot of adult urologists are not comfortable or familiar with taking care of these patient populations. So we're also creating, um, it's an ongoing project. We're creating an atlas of providers who have, that we've reached out to and vetted to say, would you be willing to take care of these patients and also what insurance plans do they accept because That's another big issue is that, you know, they'll, they'll say, well, I wanna go to my, my mom and dad's urologist, but then they find out that they don't accept their Medicaid. And so, um, challenges that are certainly barriers to these individuals transitioning into that adult world. Uh, we've, um, actually, it's such an important thing for us that we've got a nurse practitioner whose job is to oversee this whole QI project and she personally makes sure all of our records, once we identify a patient. At age 16 for transitioning, she'll actually get all the records ready and made um made available for them to just take over to the adult doctors that they've chosen. Yeah, and that, that's a fantastic thing. Um, again, you're very fortunate in that you have the, um, the resources to do that where not every institution has the financial support for that, um, and, and you are doing what's, what's best in your In your, when I say community, in, in your healthcare system, and I think we um I think we have to adapt and I, I, you know, is that a, is that a fantasy that all kids with developmental, with congenital diseases have that, absolutely. Um, but until we start paying primary care doctors better for taking care of these complex patients, uh, or at least training. The, the specialists, um, take care for them. We're, we're gonna, we're gonna find lots of roadblocks and, you know, the, the bigger the city, the bigger the community, the, the, the better off you, you are, but I, I, it, it is a continual challenge. Definitely. Um, I, I do have two quick follow-up questions, uh, with the, you know, Brian's point about the executive functioning of these individuals sometimes because they do have cognitive impairment. Um, how do you counsel your patients about alcohol use? Because to me, that's always a big issue, you know, they go, they go out with their friends, they drink, they get drunk, and you know, now they're diuresing and they figure decaf, and that's like a recipe for bladder perforation. Uh, so I actually have them put out, I say, you should get a medical alert bracelet made that says, I use this size of catheter and I have to empty my bladder every 3 or 4 hours in case something happens to them. So, um, and some of them I've had them change the home screen on their, on their, uh, cellphones, um, to say that, um, I usually bring it around in like a joking way, you know, what are you doing this weekend, and, um, and, and we start, we start the conversation that way, um, but it, it is an issue and, um, I think Doug Husman has spent a lot of time talking about drug and alcohol abuse in these patients. And you know, part of, part of the, uh, naivety of being a pediatric subspecialist is that you think these are just kids, right? They're just big kids and that's, that's part of the reason why that was one of the choices in the questions is you're, you know, they're just big kids, but they're not just big kids. They, they wanna do the things that all other kids wanna do as they grow up or Things that they see on television and the like. Right. And definitely, as you mentioned, you know, that uh everyone's thinking about sex, so we also talked to them about the, uh, you know, the condoms and that most of them are latex containing and they have to seek non-latex condoms because of their exposure to, uh, you know, chronic hospitalizations and their increased risk of having a latex allergy. Yeah, very good point that you bring up. Um, yeah, I, and I think that that discussion about sex sometimes, if you start it And, and it's a patient whose parent thinks that they're not capable, is very off-putting to the family. They kind of give you the eyes, like, what the heck are you doing? And, and in fact, it's, it's important information, and I, and the, the risk. Of sexual abuse in this, in patients, and people with developmental disability and um physical disability is quite higher than the, than the general population and so I, I make a big, big deal about, you know, you're at the doctor's, it's OK for me to look even if it's the millionth time they've seen me. um I still think it's an important message and that you need to, you know, you need to be in agreement with, uh, with whatever is going on around you. Sure. I have one last question before we get to the case presentations, and that is, you know, this whole issue of the, uh, neoplasms in augmented bladders, um, a lot of times those decisions are made by the parents and that discussion about the risk happens when the children are much younger. And even though we do. We still do surveillance cystoscopies. I know a lot of programs have gone away from them. So when we are talking about surveillance cystoscopies, we're telling them that this is why we're looking is because of the risk of the cancer. So we bring that up so it's still fresh in the family's mind. But how do you bring up that risk in the adult patient now because they were not part of that decision making that resulted in them having this, um, surgically augmented bladder that puts them at risk for cancer. And have you ever had a patient that you've told them that, listen, you know, I, I need to follow you. You're, you're now 20 years out, you're at the risk of having this. Uh, have you ever had somebody say, well, I, I don't wanna live with that risk, so take it out? I haven't had that, I haven't had that happen. Um, I, uh, but that's certainly something that I can see down the pike. It sounds like you're speaking from experience. Um, what, what I do is I go over the list and I actually have a, a little sheet printed out with what the risks are for, um, You know, I get Huseman's risks and I put them on a sheet. If you have this, this or this, call immediately. You'll need to, and when I give them that sheet, I tell them it's because you have a bladder augment and you're at increased risk for having, um, for having cancer. And whether it hasn't registered at that moment or um it doesn't seem real to them. I, I, I haven't had that response, but uh but I do send them home with a list. Our, uh the, you know, the few patients that Doctor Matty mentioned to you that we have uh unfortunately had to uh take care of together where they did develop neoplasms in their augmented bladders. The families were very angry and they had forgotten that that risk had been discussed with them. And it was a rather painful, uh, discussion for us to have with them saying, you know, this was something that was brought up at the time that you had your surgery 15 years ago with the parents, not the individual, with the parents, cause the parents felt very guilty and angry that maybe had they known, they wouldn't have done it. I'm like, well, you knew and, uh, you know, even though I wasn't the surgeon who had that discussion with you cause I inherited these patients from my senior colleagues, um, there is that. That, um, that distance from decision to actual complication happening is so long now that they sometimes have that guilt that I wish I hadn't done this, and they forget about the 15 years of good quality of life that the child actually had, uh, as a result of that surgery, but, um, again, I don't know how to, you know, it's not our place to tell the parents how they feel. It's not our place to ever tell anybody how they feel. Their, their emotions are their own, and they should own them, but, um, it is hard for us as the clinicians who are picking up the baton, such as yourself when you weren't there with the original surgery, but now you're having to deal with the complications 1520 years later and having to deal with the parents' angst and anger and guilt. It, it's, it's certainly challenging and, um, and for parents, all the decisions that, that are made for their children, right, and what the ramifications are long term. Um, I'm not a parent, but I can empathize and, and, and understand that, wow, this, this, you know, what school you send them to or you know. You know, what extracurriculars, how much that affects just everything down the road, so it's, it's a challenge. Taking care of kids is certainly challenging. Andrew,