This is an application is in response to Component B of the FOA RFA-DD-14-002: Research Approaches to Improve the Care and Outcomes of People Living with Spina Bifida. Gillette Children's Specialty Healthcare (GCSH) is a hospital and clinic system that provides care for children and adults with chronic disease with onset in childhood. All aspects of both acute and chronic care for these disorders is provided within this entity, which has been providing such care for over a century. A number of distinct programs exist at Gillette, with a particular emphasis on neuroscience disorders. The spina bifida (SB) program has a long history at Gillette, and all specialties necessary for the care of these patients are available in our program, both for the care of children and adults. These include pediatric and adult providers in PMR, orthopedics, urology and neurosurgery. We also have all forms of therapy available, with appropriate support staff. Other services are available for consultation within the community. Our physical structure includes a 60 bed hospital located in St Paul MN, with a network of specialty clinics. Our structure allows for us to see spina bifida patients in three distinct care models. The majority of patients are seen by the providers of their choosing, which reflects that we have had a large number of specialists available to care for these patients, over many years. For more than 10 years, we have had a dedicated multidisciplinary clinic for adults with SB, with all patients seen at a single event clinic. Sincethe Fall of 2013, we have started a similar model for pediatric patients, for those families who wish to consolidate their care to as few appointments as possible. In 2013, we saw nearly 500 patients in this diagnostic group, from infants to adults. Our patients come predominately from Minnesota and nearby portions of Wisconsin, Iowa and both North and South Dakota. We propose to enroll patients in the NSBPR at all sites. Our expanded research department is fully engaged in this process and we anticipate being able to capture a significant proportion of our patients, particularly given past successes in doing such work at GCSH. We are also submitting two research proposals that will be based on the use of data from the NSBPR. The first is a study of pediatric patients who have undergone intra- abdominal surgery with a cerebral spinal fluid (CSF) shunt in place. Our hypothesis is that such surgery in these shunted patients is associated with a subsequent increase in shunt morbidity, either by shunt infection (i.e., peritonitis or infectious pseudocyst) or malfunction (due to absorption failure). The second study is of children and adults, and attempts to better characterize the epidemiology of pressure ulcers across the lifespan of these patients. We are also planning on studying the risk of ulcer development as a function of ambulatory state and type of bowel/bladder management.
More than 70,000 people in the U.S. are currently living with spina bifida (SB), making it the most common permanently disabling birth defect. Because of the low prevalence and significant complexity of SB, the National Spina Bifida Patient Registry (NSBPR) was created to improve the quality of care of people living with SB by collecting a large pool of longitudinal data on children and adults with SB. Because we treat close to 500 children and adults with SB each year, and have a history of successfully participating in similar registries, we propose that Gillette Children's Specialty Healthcare serve as one of the new clinic sites in the NSBPR. As a new clinical site, Gillette will provide additional patients to the registry and also address important research questions to improve our understanding of SB and its treatment and care across the lifespan.