Melanie Cole, MS (Host): There are very few clinics in North America for adults with spina bifida. The UAB Adult Spina Bifida Clinic has almost 15 years of active experience in this area and now follows more than 200 adults with spina bifida. Welcome to UAB MedCast.

I'm Melanie Cole. And we have three UAB medicine physicians for you today highlighting the UAB Adult Spina Bifida Clinic navigating spina bifida care across a lifetime. Joining me in this thought leader panel, we have Dr. Jeffrey Blount, he's the Harvard Chair in Pediatric Neurosurgery at Children's Hospital of Alabama, and Medical Director of the Spina Bifida Program at UAB; Dr. Daniel Harmon, he's a neurosurgeon and an Assistant Professor of Neurosurgery; and Dr. Betsy Hopson, she's the Director of the STEP Program and the Administrative Director of the Spina Bifida Program at UAB Medicine.

Doctors, thank you so much for joining us today. And Dr. Hopson, I'd like to start with you. What are some of the challenges teens and young adults with spina bifida face as they transition to adult healthcare, and why is that continuum of care, that ease of transition, so important for success?

Dr. Betsy Hopson: Well, thank you for the question, Melanie. And thank you also for this opportunity. Transition has been such a challenging area for many decades. And certainly, as we've seen individuals with spina bifida living much longer well into adulthood, even being able to have a normal life expectancy now, having a place for them in adult medicine is so important. And that's what makes our program really unique and really necessary because we have a dedicated pediatric program that's an interdisciplinary clinic where patients can come in and have one-stop shopping where all of their doctors are in one place. I always say kind of the magic of spina bifida clinic is what happens in the hallways because you have a neurosurgeon who's talking to a urologist, who's talking to a Physical Medicine and Rehab Doctor. And this has historically been recognized as the most optimal care for children with spina bifida. But having a clinic like this in adult medicine really doesn't exist in most places.

And so, what we've been able to accomplish at UAB is replicating the model that has worked for so long in Pediatrics, but doing that on the adult side and having that continuity of care where there's not a gap in services from pediatric to adult medicine is so important. If you think about it, many life changes are happening right at the same time that someone goes from pediatric care to adult care, often these are times of leaving your parents' management and going off to college and many life changes, and so not losing your healthcare in the middle of all of that is so important.

Melanie Cole, MS: Well, it certainly is. So, thank you for that, Dr. Hopson. And Dr. Blount, I'd like you to speak about some of the common medical and even psychosocial complications that these patients face as they make that transition, which may make it even more challenging as Dr. Hopson was just describing. So, how do you facilitate that transition from the pediatric care to Adult Spina Bifida Clinic? I'd like you to speak about the clinic a little bit. Tell us how it works.

Dr. Jeffrey Blount: Well, thank you for that question and thank you for this opportunity. The Adult Spina bifida Clinic has been one of the epiphenomenal events of my time here at UAB, which is now approaching 25 years. And I say that with all sincerity because there are relatively few things that I've participated in that have so sort of significantly impacted the way I go about what I do. And I don't necessarily mean the technical aspects of how I do an operation or procedure, but more how we embrace the broader view of surgery, which of course is to follow the patient and address how the operations we do impact their life and how their outcomes help them and favor them.

And you are very correct in identifying that there are a specific group of problems that patients with spina bifida have. They tend to fall surgically into about three domains. One is Neurosurgery, one is Urology, and one is Orthopedics. Neurosurgery, it often has to do, it starts from the very beginning where many of these patients have a congenital anomaly from the outset. They're born with an anomaly that needs closure within the first day or two of life, and then they have problems with their spinal fluid circulation and dynamics. So, many of them need a shunt operation that we need to keep working and keep functioning. Those are the predominant ones. There's something called tethered cord, and I won't dive into all the nuances of that, but those are sort of the big three.

But really, what we've tried to do is to reach beyond just doing procedures and the technical, and we've tried to look and see how do other morbidities, how do other problems that accompany this disease weigh in on the process. And I will say that by following patients into adulthood and seeing the sort of longitudinal arc, if you will, or the long-term look of this disease, that it really does impact how we do what we do for the children, how we do what we do in the Pediatric Spina Bifida Clinic.

A big part of it is educating them about their own particular condition. Educating them as far as what are the things that they need to watch for. In fact, we were in the Adult Spina Bifida Clinic earlier today, and what Betsy and I said to many, many families, is that a big part of the magic of the Adult Spina Bifida Clinic is recognizing that there's a network of providers out there who, A, care very much about you; B, have some awareness of the condition and its many manifestations; and, C, are committed to helping you navigate the complexities of any large medical system. It's no surprise that UAB is a very large highly technically advanced, tertiary medical center, and that can be very intimidating. So, access and where to go and the sheer size of it, all of those things can be practically intimidating for people. But a big part of what we try to do is help people understand what their challenges are and navigate this system.

Melanie Cole, MS: Well, thank you so much, Dr. Blount, for that. And Dr. Harmon, as the clinic's mission is to optimize all facets of care to enable these adult patients with spina bifida to reach their full potential, which is such an amazing goal, what makes UAB Adult Spina Bifida clinic so unique? How have you seen it evolve over the past 15 years to meet the needs of these adult patients?

Dr. Daniel Harmon: I guess probably one way it's evolved is the addition of me. I am an adult neurosurgeon. I came here about five years ago, and I've been involved in the spina bifida clinic for about three years. And I guess I see my role in, one, bringing some spine expertise to this from the adult side and also helping to navigate the adult hospital and inside of the care of these patients. That's something that Dr. Blount and Betsy Hopson have created this clinic for, but I tend to be kind of the inside man. So, I can help get people ushered through the ER, into the OR if they need to be. I have them come to my clinic separately from the spina bifida clinic if need be for extra care, and trying to make sure that as they're having this transition and handoff from the pediatric side to the adult side, that there's someone there on the adult side continuing to learn, but understands their problems and continues to help them with this.

Dr. Betsy Hopson: And Melanie, just to kind of add a little bit to what Dr. Harmon is saying, we really can't overstate how adding his presence to our clinic has elevated our overall presence and overall impact that we're able to have for many, many years. Dr. Blount and I spend all of our time at Children's Hospital, and we're only coming to the Adult Spina Bifida Clinic twice a month.

But by adding Dr. Harmon to the mix now, we truly can do a warm handoff in clinic in real time and in real space, and be able to not only meet our patient's needs when everything's going well through a clinic visit, but truly meet their needs when they're kind of in an acute crisis and need us the most.

Dr. Jeffrey Blount: I could not say that better or emphasize that more. Dr. Harmon's role has been absolutely central to the growth. I feel like we're in a different place from what we were five years ago, and it's largely as a result of the role that he brings to it.

Melanie Cole, MS: Well, along those lines then, Dr. Hopson, basically, as we're talking about Children's Hospital of Alabama and UAB Medicine in that transition, can you speak a little bit about the geographic scope of the program? So, the Children's is at Children's of Alabama, right? The Pediatric Spina Bifida clinic. And then, how do you work with the families to move to a different location and get the patients comfortable going to a different place for their clinic needs, care close to home. Emphasize for us the focus on this patient-directed goals of care and quality of life as far as geography goes.

Dr. Betsy Hopson: Yeah, absolutely. So when I think about our spina bifida program, I really think about there being three arms of the program. And the first clinic, which we really haven't spent a whole lot of time talking about, but is with our Maternal-Fetal Medicine partners. And so, we actually meet these families as soon as a prenatal diagnosis of spina bifida is made, and that's where we have our first clinic and round table discussion with these families. And so, they're making that initial transition from that visit in the prenatal period into then the Children's of Alabama clinic.

Our clinic through Children's primarily serves the entire state of Alabama in some of our bordering states, but primarily the state of Alabama. For adult medicine, it's a little bit different, because we are so unique and have this clinic that doesn't exist in many other places. We are making a smooth transition for patients who have grown up receiving care at Children's of Alabama. They're able to have their last visit in the pediatric clinic when they're 21 and their first visit during that same year into the adult clinic. But we also are seeing patients from Georgia and Tennessee and Mississippi and even a family flew in from Hawaii to receive care in our adult clinic, because there just aren't very many clinics like that that exist.

And really, it's important to see each of those clinics independently, but very much under one umbrella, because we've kind of coined a phrase that we call evidence-based program building, and there's been so many times that we make an observation in our adult clinic and then we study that observation and put some science behind what we're seeing, and then we take that information and take it back into the pediatric clinic and change the way that we're doing transition readiness preparation based on that initial observation that was made in the adult clinic. And so, each of the clinics are very much working together, even though they may have an independent mission as far as the age of patients that they're serving.

Dr. Jeffrey Blount: I think is important that we don't seek with this clinic to say we're the only one and everybody needs to travel from far. Our mission is to try and help people with spina bifida and we can provide help support and service to people from geographically afar. But our primary mission is to learn how to do this and share that with potential providers in their region. Probably in an ideal world, there would be regional clinics for Adult Spina Bifida Clinic. You know, maybe a more ideal world would be every city of a significant size or every population base of a significant size would have an adult clinic. But we're a long way from there.

We are most effective when we integrate with our own system, which is UAB. And we're very pleased to provide that help, that support, that knowledge to people from outside. But we're also reaching out to colleagues from around the country, and particularly Betsy is fielding calls and going and giving talks about how we have constructed this program so that other centers can begin to build programs, maybe not exactly like ours, but learning some elements of ours and bring that to other areas.

Melanie Cole, MS: What a comprehensive program, and that's really great work that you're all doing. Dr. Blount, I'd like you to expand a little bit on the multidisciplinary management. We've mentioned briefly Urology and obviously Neurosurgery, and there's so many aspects to growing up, becoming an adult with spina bifida, as you were thinking about your team, please tell us about them, tell us about all the different people that are involved because really it is that multidisciplinary management that makes this so successful.

Dr. Jeffrey Blount: That's an absolutely central notion, and there's no single question that we're going to address today that's more important than that one. Our colleagues play critical roles in the management of these patients in the holistic offering of the clinic. Let me go through them a little bit. Physical Medicine and Rehabilitation is in a sense the ground center in that they field many of both the medical issues, general medical issues, and the specific needs that people with limited mobility, joint problems, limitations of movement, and the downstream effects from those may face.

So, Dr. Danielle Powell is one of our treasured colleagues in this. She's a physiatrist at UAB, and she and her team have spearheaded and been very engaged and committed to this patient population for many, many years. And they do things like supporting the orthotic needs, the bracing needs, the physical therapy, the occupation, as well as the general medical needs. We're all very surgically oriented, so it's absolutely crucial to have a person like that and a team like that as a base for the entire operation.

Then in Urology, that's another very important body system impacted by spina bifida. The neurologic control of the bladder and the bowel is often impacted in patients who have spina bifida, and the impact of that can cause a threat to fundamental wellbeing things like kidney function, the backup of urine, because it can't be properly expelled from the body can trash the kidneys. And those are very quickly, very acutely wellbeing and life-threatening problems, the potential for a rupture of the bladder, right?

Those are the things that are absolutely crucial as the individual matures into adulthood. By and large, the acute neurosurgical issues that predominate early oftentimes sink a little bit more to the background. And as a result, Betsy and I oftentimes find ourselves dealing more with more holistic issues of care and the downline implications of what we've done.

And then, finally, orthopedics, again, that tends to predominate early as some of the children have problems with the joints of their lower extremities. Oftentimes those issues have been resolved in childhood, so there appears to be a little bit less need for acute orthopedic intervention in adulthood. But we do have orthopedic colleagues who are available for us to reach out to and call if need be. But to your point, this is a multidisciplinary entity from the start, and this cannot be as well done in the absence of the active engagement and participation of those key team members.

Melanie Cole, MS: Wow. Thank you so much for that explanation. I'd love to give you each a chance for a final thought here. And Dr. Harmon, as you look to the future, what plans are in place to grow this program and where do you fit into that? What's your vision for the program and how do you feel that this care model is going to improve the way patients receive care for all of the conditions that go along with spina bifida?

Dr. Daniel Harmon: Well, I think as Betsy Hopson and Dr. Blount said earlier, this is a little bit of a new territory. The number of adults with spina bifida living into adulthood and the normal life expectancy is increasing all the time. And I think part of the things that Dr. Hopson has and Dr. Blount have already set into motion that I hope to kind of carry forward is making sure that we're developing programs and research how best to care for these patients.

We've mentioned that hydrocephalus is one of the threats from Neurosurgery that we have to deal with into adulthood. And I do believe there are some things about it that changes as these patients get older and their fluid dynamics, but it's hard to parse that out. There's also the tethering that Dr. Blount mentioned and as an area of interest of mine as a spine surgeon and how best to take care of those patients, whether it is what we call a direct de-tethering or whether it's what we call a shortening procedure where we actually shorten their vertebral column to take this tension off of their spinal cord that can cause severe pain and can cause them to lose neurologic function.

And so, I think one thing I would like to be grateful to be involved in, and I think that we would like to come see come out of this clinic is helping to advance that knowledge base and care for them. I guess I would also like to see us expand a little bit, the multidisciplinary approach and adding people. And I really think this transition to adulthood, as we've kind of mentioned, is difficult for these patients, going from a pediatric setting and the type of support and things that are there into an adult world. And we see that in normal people that are transitioning from children to adulthood, but you add another challenge like spina bifida, it becomes even harder for them and I think, to some degree, socially and even psychologically. And that's a thing that I would like to hopefully integrate even more into this going forward, but that's a challenge.

Melanie Cole, MS: Dr. Blount, up to you now. What do you feel that the clinic needs to do as you look forward? What would you like to see happen? What's your vision for the program? And expand a little on what Dr. Harmon was just saying.

Dr. Jeffrey Blount: Well, that's such a rich question because while we have a great program and a program that we're very proud of, every aspect of our program can be improved, right? We look at our pediatric community, there are ways we can improve that. We want to improve our interaction with Maternal-Fetal medicine. We want to improve our prenatal services.

A lot of our approach right now is very holistic. This disease is marginalizing from the beginning. People who realize they have an affected pregnancy feel isolated, alone, terrified. We want to have programs that reach out holistically, that embrace all of that, that provide interpersonal support, that provide psychological support, that provide a connection with a community of people that have lived this and they realize that they're not so alone. Move it into the pediatric community. We want to improve our capacity to answer very basic questions. We have surgical questions that we still don't know the answers to, despite years of working on it. What are the best techniques for closure? What are the best techniques for dealing with the hydrocephalus that comes along?

How can we reduce those problems, keep the wheel turning? How can we improve? Transition, is it like playing the piano where if you start at age four or five, you're a better piano player? As an adult than if you start at age 20? Well, yeah, probably. How do we start that process? That's a big one for us right now.

Let's go into the adult world. How do we get the lessons we've learned in the adult clinic, the extraordinary importance of support of marginalization, of getting these people so they're not isolated? One of the things Betsy hasn't talked about is that some of the programs she's worked on in camp and things like that. Our spina bifida, our Adult Spina Bifida Program sponsors an adult camp. It is these folks' highlight of the year. Christmas and New Year's, that's number two and number three. These folks gravitate to that because it is fulfilling. It's verifying. It's validating they're not the only person. They can reach out. They can connect with people. So, this sounds very holistic and a little bit kumbaya for a surgeon, but that's really important as far as treating the whole of the patient. And these are the areas where we feel confident that we can continue to reach out. We want to learn many things. Dr. Harmon and I have spent a lot of time talking about what's the best technique involved in an adult for untethering them. Do we do something on the spine, the bony spine? Do we do something back in the neural elements? These are unanswered, fundamental questions. So, Betsy's heard me use this, analogy a lot in our clinic. It's a little bit like the weather or like space. We know a lot, but there's so much more that we don't know that we have tremendous potential for growth. And it's exciting to think about where we can go and what we can do. Because what's happened up to this point has made an impact in hundreds of people's lives, maybe thousands. And I think the future is even brighter.

Melanie Cole, MS: That's beautifully put, Dr. Blount. And Dr. Hopson, I'm so thrilled to give you the last word here because they've been singing your praises this episode. And so, I would like you to speak to other providers around the country as you do and let them know what is so important and the key takeaways about the Adult Spina Bifida Clinic At UAB Medicine and the impact that this dedicated clinic has on patients and their families.

Dr. Betsy Hopson: I would say that fundamentally there has not been anything that has shaped the way that I think about spina bifida, the work that I do more than having a presence in the adult clinic. The adult clinic has shown me what a surgery on a five-year-old looks like when that person turns 35. It's really shown me what predicts whether someone has a high quality of life throughout their lifespan versus ones who don't. And so, the adults really give you a perspective of where the time and focus in Pediatrics should be spent to make the most impact.

But I guess the final point that I would make and the final say is truly my life has been forever enriched by knowing and working with individuals with spina bifida. They have some physical challenges, but these are remarkable human beings who just truly have a resilience that is unparalleled to anything that I've ever experienced, and everything about the time that I spend with them impacts the way that I live my life. And so, spending time in these clinics and growing these programs not only is the right thing to do for healthcare for these individuals. But what we've learned in spina bifida has now expanded into the STEP Program, which is for all patients with childhood medical complexity, it's expanded into the community, into legislation, into many, many things. And so, there's just a whole lot of good that comes out of doing this work.

Melanie Cole, MS: Well, those patients and their families are lucky to have you, and lucky to have you all because really. I can hear the compassion and the passion for what you do. So, thank you all for joining us and sharing your incredible expertise for other providers today. And for more information, please visit our website at uabmedicine.org/physician. That concludes this episode of UAB Med Cast. I'm Melanie Cole. Thanks so much for joining us today.