OBJECTIVES: Describe the distribution of weight status categories and determine factors associated with overweight and obesity (OW/OB) in children and adolescents with spina bifida (SB) using the National Spina Bifida Patient Registry. METHODS: Demographic, anthropometric, and clinical data collected from 2009 through 2018 was used to describe the prevalence of OW/OB. The generalized estimating equation model (GEE) identified factors associated with OW/OB among individuals with SB. RESULTS: Participants (n = 7215) were aged 2 to 19 years (mean = 11.1; standard error, 0.06) and 51.4% female. The majority were non-Hispanic white (57.2%) followed by Hispanic or Latino (25.1%) and non-Hispanic Black (7.5%). The myelomeningocele (MMC) subgroup accounted for 76.3%. Most (60.2%) were community ambulators. The overall percentage of OW/OB was 45.2%, with 49.2% of MMC and 32.0% of nonmyelomeningocele OW/OB. Following the Centers for Disease Control Obesity Severity Classification System, 19.7% of MMC were in class 1, 6.6% in class 2, and 3.5% in class 3. Univariate analysis of MMC participants demonstrated demographic (age, sex, race/ethnicity, and clinic region) and clinical variables (functional level of lesion, ambulation, and number of shunt surgeries) were associated with OW/OB. The GEE model showed that OW/OB was independently, and significantly, associated with age, sex, race/ethnicity, lesion levels, and geographic location of the clinics. CONCLUSIONS: The demographic and clinical factors associated with OW/OB in children and adolescents with SB further our understanding of factors contributing to the higher prevalence of OW/OB in this population and may inform OW/OB prevention and treatment strategies.

BACKGROUND: Individuals with spina bifida are at increased risk for urinary tract infection (UTI), however there are few population-based investigations of the burden of UTI hospitalizations. OBJECTIVE: We assessed rates and risk factors for UTI hospitalization in individuals with and without spina bifida. METHODS: We conducted a retrospective cohort study to estimate rates of UTI hospitalization by spina bifida status. We included individuals enrolled in Tennessee Medicaid who lived in one of the Emerging Infections Program's Active Bacterial Surveillance counties between 2005 and 2013. Spina bifida was primarily defined and UTI hospitalizations were identified using International Classification of Diseases, Ninth Revision diagnoses. We also studied a subset without specific health conditions potentially associated with UTI. We used Poisson regression to calculate rate ratios (RR) of UTIs for individuals with versus without spina bifida, adjusting for race, sex and age group. RESULTS: Over the 9-years, 1,239,362 individuals were included and 2,493 met criteria for spina bifida. Individuals with spina bifida had over a four-fold increased rate of UTI hospitalization than those without spina bifida-in the overall study population and in the subset without specific, high-risk conditions (adjusted rate ratios: 4.41, 95% confidence intervals: 3.03, 6.43) and (4.87, 95% CI: 2.99, 7.92), respectively. We detected differences in rates of UTI hospitalization by race and sex in individuals without spina bifida that were not seen among individuals with spina bifida. CONCLUSIONS: Individuals with spina bifida had increased rates of UTI hospitalizations, and associated demographic patterns differed from those without spina bifida.

The multidisciplinary model (MCM) is described as one that utilizes skills and experience from practitioners belonging to various disciplines, each treating patients from a specific clinical perspective.(1) The Spina Bifida Association (SBA) supports and recommends that clinical care for people with Spina Bifida (SB) be provided in specialty clinics of which the MCM is an example; that care be coordinated; and that there be a plan for transitional care.(2) This paper explores the challenges the MCM faces with a transitioning and aging population in a care system that calls for a positive patient experience, engaged health care professionals, desired outcomes, with consideration of cost.

PURPOSE: The lifetime risk of renal damage in children with spina bifida is high but only limited baseline imaging data are available for this population. We evaluated a large prospective cohort of infants with spina bifida to define their baseline imaging characteristics. MATERIALS AND METHODS: The UMPIRE Protocol for Young Children with Spina Bifida is an iterative quality improvement protocol that follows a cohort of newborns at 9 United States centers. Using descriptive statistics, we report the initial baseline imaging characteristics, specifically regarding renal bladder ultrasound, cystogram and dimercaptosuccinic acid nuclear medicine scan. RESULTS: Data on 193 infants from 2015 to 2018 were analyzed. Renal-bladder ultrasound was normal in 55.9% of infants, while 40.4% had Society for Fetal Urology grade 1 to 2 hydronephrosis in at least 1 kidney, 3.7% had grade 3 to 4 hydronephrosis in either kidney and 21.8% had grade 1 or higher bilateral hydronephrosis. There was no vesicoureteral reflux in 84.6% of infants. A third of enrolled infants underwent dimercaptosuccinic acid nuclear medicine renal scan, of whom 92.4% had no renal defects and 93.9% had a difference in differential function of less than 15%. CONCLUSIONS: The majority of infants born with spina bifida have normal baseline imaging characteristics and normal urinary tract anatomy at birth. This proactive protocol offers careful scheduled surveillance of the urinary tract with the goal of lifelong maintenance of normal renal function and healthy genitourinary development.

OBJECTIVE: To estimate differences in the length of stay and costs for comparable hospitalizations of patients with spina bifida (SB) with and without pressure injuries. DESIGN: Retrospective, cross-sectional, observational study. SETTING: Nationwide Inpatient Sample from years 2010 to 2014. PARTICIPANTS: Hospitalized patients with SB. Hospitalizations among patients with SB and pressure injuries (n=3888) were matched to hospitalizations among patients with SB but without pressure injuries (n=3888). INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Differences in length of stay and total costs between the two groups. RESULTS: After successful matching, multivariate modelling of costs and length of stay on matched sample showed that hospitalizations with pressure injuries had an increased 1.2 inpatient days and excess average costs of $1,182. CONCLUSIONS: The estimated average cost of hospitalization increased by 10%, and the estimated average length of stay increased by 24% in the presence of pressure injuries among hospitalized patients with SB, compared with their peers without these injuries. These results highlight the substantial morbidity associated with pressure injuries, which are potentially preventable before or during hospitalizations among persons with SB.

BACKGROUND AND OBJECTIVES: Patients with spina bifida (SB) typically develop serious secondary conditions and undergo surgical procedures related to neurologic disorders, orthopedic abnormalities, bladder and bowel dysfunction, and skin breakdown. In this study, we describe the age distribution of common surgical procedures and health outcomes in patients with SB. METHODS: Using serial cross-sectional data from the National Spina Bifida Patient Registry (2009-2013; n = 4664), we examined surgical procedures (gastrointestinal, neurologic, orthopedic, skin, urologic, and other) and health outcomes (fecal continence, urinary continence, skin breakdown, and ambulation status) of patients with SB by age and SB type (myelomeningocele and nonmyelomeningocele). RESULTS: All patients who were enrolled had available health outcome data, and 81.5% (n = 3801) of patients had complete surgical procedure data, which totaled 18 891 procedures across their lifetimes. Almost all procedures (91.4%) occurred among participants with myelomeningocele SB. For both types of SB, the distribution of procedures varied by age. The most frequent procedures were neurologic, with approximately half (53%) occurring in patients <1 year of age; orthopedic and urologic procedures followed in frequency but tended to occur at older ages. The health outcomes for patients with myelomeningocele SB revealed lower frequencies of positive health outcomes than those for patients with nonmyelomeningocele SB across all age groups. Overall, the rates of fecal and urinary continence and skin breakdown increased with age whereas the ability to ambulate declined with age. CONCLUSIONS: Understanding the surgical procedures and health outcome variations by age and SB type can help clinicians and populations that are affected set expectations regarding the occurrence of these procedures and the outcomes throughout the patients' life spans.

INTRODUCTION: Most children with spina bifida (SB) now survive into adulthood, but most have neuropathic bladder with potential complications of incontinence, infection, renal damage, and diminished quality of life. We sought to 1) describe contemporary bladder management and continence outcomes of adults with SB; 2) describe differences from younger patients; and 3) assess for association with socio-economic factors. METHODS: We analyzed data on bladder management and outcomes from The National Spina Bifida Patient Registry in adults with SB. A strict definition of continence was utilized. Results were compared to young children (5-11 years) and adolescents (12-19 years). Statistical analysis compared cohorts by gender, ethnicity, SB type, lesion level, insurance status, educational attainment, employment status, and continence. RESULTS: 5250 SB patients were included; 1372 (26.1%) were adults. 45.8% of adults did not take medication, but 76.8% performed clean intermittent catheterization. Continence was lower in adults with myelomeningocele (MMC) (45.8%) versus those with non-MMC SB (63.1%) (p<0.0001). Continence rates were higher in older age cohorts for MMC patients (p<0.0001) but not non-MMC patients (p=0.1192). Bladder management and histories of urologic surgery varied among age groups. On univartiate analysis with SB-related or socio-economic variables, continence was significantly associated with educational level, but on multivariable logistic regression analysis, bladder continence was significantly associated with employment status only. CONCLUSIONS: Bladder management techniques differ between adults and younger patients with SB. Bladder continence outcomes were better in adults with nearly one-half reporting continence. Continence was significantly associated with employment status in adults 25 years or older.

The National Spina Bifida Patient Registry: Past, Present, and Future was presented at the Spina Bifida World Congress, March 17, 2017, San Diego, California. This commentary provides a summary of registry activities including the reason for development, a description of the clinic participants and their patients who are participating, analytic works and publications. Two specific efforts that are related to the work of the registry, a urologic protocol to preserve renal function for newborns and young children, and a skin breakdown prevention bundle developed and implemented in registry clinics, are highlighted.

PURPOSE: Optimal management of neurogenic bowel in patients with spina bifida (SB) remains controversial. Surgical interventions may be utilized to treat constipation and provide fecal continence, but their use may vary among SB treatment centers. METHODS: We queried the National Spina Bifida Patient Registry (NSBPR) to identify patients who underwent surgical interventions for neurogenic bowel. We abstracted demographic characteristics, SB type, functional level, concurrent bladder surgery, mobility, and NSBPR clinics to determine whether any of these factors were associated with interventions for management of neurogenic bowel. Multivariable logistic regression with adjustment for selection bias was performed. RESULTS: We identified 5,528 patients with SB enrolled in the 2009-14 NSBPR. Of these, 1,088 (19.7%) underwent procedures for neurogenic bowel, including 957 (17.3%) ACE/cecostomy tube and 155 (2.8%) ileostomy/colostomy patients. Procedures were more likely in patients who were older, white, non-ambulatory, with higher-level lesion, with myelomeningocele lesion, with private health insurance (all p< 0.001), and female (p= 0.006). On multivariable analysis, NSBPR clinic, older age (both p< 0.001), race (p= 0.002), mobility status (p= 0.011), higher lesion level (p< 0.001), private insurance (p= 0.002) and female sex (p= 0.015) were associated with increased odds of surgery. CONCLUSIONS: There is significant variation in rates of procedures to manage neurogenic bowel among NSBPR clinics. In addition to SB-related factors such as mobility status and lesion type/level, non-SB-related factors such as patient age, sex, race and treating center are also associated with the likelihood of undergoing neurogenic bowel intervention.

PURPOSE: Continence is low in individuals with spina bifida, but published prevalence varies markedly across studies. The objective of this study was to examine bladder and bowel continence among patients served by multidisciplinary clinics participating in the National Spina Bifida Patient Registry and to examine whether variation in prevalence exists across clinics. METHODS: Data were obtained from patients 5 years and older from March 2009 to December 2012. Data were gathered at clinic visits using standardized definitions. RESULTS: Data from 3252 individuals were included. Only 40.8% of participants were continent of urine; 43% were continent of stool. Bladder and bowel continence differed by spina bifida type, with those with myelomeningocele having significantly lower reported prevalence of continence than those with other forms of spina bifida. Bladder and bowel continence varied across registry sites. Adjustment based on demographic and condition-specific variables did not make substantive differences in prevalence observed. CONCLUSION: Less than half of spina bifida patients served in multidisciplinary clinics report bladder or bowel continence. Variability in prevalence was observed across clinics. Further research is needed to examine if clinic-specific variables (e.g., types of providers, types of interventions used) account for the observed variation.

PURPOSE: Achieving bladder continence among people with Spina bifida is a lifetime management goal. We investigated bladder continence status over time and factors associated with this status among people with SB. MATERIALS AND METHODS: We used National Spina Bifida Patient Registry data collected from 2009 through 2015 and applied generalized estimating equation models to analyze factors associated with bladder continence status. RESULTS: This analysis included 5,250 participants with SB in a large, multi-institutional patient registry who contributed 12,740 annual clinic visit records during the study period. At last follow-up, mean age was 16.6 years, 22.4% of the participants had undergone bladder continence surgery, 92.6% used some form of bladder management, and 45.8% reported bladder continence. In a multivariable regression model, the likelihood of bladder continence was significantly higher among those who were older, female, non-Hispanic white, had a non-myelomeningocele diagnosis, a lower level of lesion, a higher mobility level, and private insurance. Both continence surgery history and current management were also associated with continence independent of all other factors (adjusted ORs and 95% CI: 1.9, 1.7 - 2.1, and 3.8, 3.2 - 4.6, respectively). The association between bladder management and continence was stronger for those with a myelomeningocele diagnosis (adjusted OR=4.6) than with non-myelomeningocele (OR= 2.8). CONCLUSIONS: In addition to demographic, social, and clinical factors, surgical intervention and bladder management are significantly and independently associated with bladder continence status among people with SB. The association between bladder management and continence was stronger among people with myelomeningocele.

PURPOSE: Recent studies have revealed that the lack of continuity in preparing patients with spina bifida to transition into adult-centered care may have detrimental health consequences. We sought to describe current practices of transitional care services offered at spina bifida clinics in the US. METHODS: Survey design followed the validated transitional care survey by the National Cystic Fibrosis center. Survey was amended for spina bifida. Face validity was completed. Survey was distributed to registered clinics via the Spina Bifida Association. Results were analyzed via descriptive means. RESULTS: Total of 34 clinics responded. Over 90 characteristics were analyzed per clinic. The concept of transition is discussed with most patients. Most clinics discuss mobility, bowel and bladder management, weight, and education plans consistently. Most do not routinely evaluate their process or discuss insurance coverage changes with patients. Only 30% communicate with the adult providers. Sexuality, pregnancy and reproductive issues are not readily discussed in most clinics. Overall clinics self-rate themselves as a 5/10 in their ability to provide services for their patients during transition. CONCLUSIONS: Characteristics of current transitional care services and formal transitional care programs at US clinics show wide variances in what is offered to patients and families.

INTRODUCTION: Advances in care have allowed most children with spina bifida (SB) to live to adulthood. The majority have neuropathic bowel dysfunction (NBD), resulting in constipation, incontinence, and diminished quality of life. We sought to 1) describe contemporary NBD management and continence outcomes of adults with SB; 2) describe differences from younger patients; and 3) assess for association with socio-economic factors. METHODS: We analyzed data on NBD management and continence from the National Spina Bifida Patient Registry (NSBPR). Patients were segregated into young children (5-11 years), adolescents (12-19 years), and adults (20 years and older). A strict definition of continence was utilized. Statistical analysis compared cohorts by gender, ethnicity, SB type, lesion level, insurance status, educational attainment, employment status, and continence. RESULTS: A total of 5209 SB patients were included, of whom 1370 (26.3%) were adults. Management and continence varied by age and SB type. Oral medication use did not differ between groups (5.2-6.6%). Suppositories and rectal enemas were used only by 11.5% of adults, which was significantly less than among school-aged children. Antegrade enemas were used by 17.7% of adults which was significantly less than among adolescents (27.2%). Adults were more likely to use digital stimulation or disimpaction or have undergone a colostomy. Bowel continence was reported by 58.3% of overall adult cohort: 55.6% of adults with myelomeningocele and 74.9% with non-myelomeningocele. Bowel continence was significantly associated with employment (p= 0.0002), private insurance (p= 0.0098), non-myelomeningocele type of SB (p= 0.0216) and educational attainment (p= 0.0324) on univariate analysis but only with employment on multivariable logistic regression (p= 0.0027). CONCLUSIONS: Bowel management techniques differed between adults and younger patients with SB. Bowel continence was reported by over half of SB adults and was associated with socio-economic factors.

BACKGROUND: Spina bifida (SB) is monitored through birth defects surveillance across the United States and in most developed countries. Although much is known about the management of SB and its many comorbid conditions in affected individuals, there are few systematic, longitudinal studies on population-based cohorts of children or adults. The natural history of SB across the life course of persons with this condition is not well documented. Earlier identification of comorbidities and secondary conditions could allow for earlier intervention that might enhance the developmental trajectory for children with SB. OBJECTIVE: The purpose of this project was to assess the development, health, and condition progression by prospectively studying children who were born with SB in Arizona and Utah. In addition, the methodology used to collect the data would be evaluated and revised as appropriate. METHODS: Parents of children with SB aged 3-6 years were eligible to participate in the study, in English or Spanish. The actual recruitment process was closely documented. Data on medical history were collected from medical records; family functioning, child behaviors, self-care, mobility and functioning, and health and well-being from parent reports; and neuropsychological data from testing of the child. RESULTS: In total, 152 individuals with SB were identified as eligible and their parents were contacted by site personnel for enrollment in the study. Of those, 45 (29.6%) declined to participate and 6 (3.9%) consented but did not follow through. Among 101 parents willing to participate, 81 (80.2%) completed the full protocol and 20 (19.8%) completed the partial protocol. Utah enrolled 72.3% (73/101) of participants, predominately non-Hispanic (60/73, 82%) and male (47/73, 64%). Arizona enrolled 56% (28/50) of participants they had permission to contact, predominately Hispanic (18/28, 64%) and male (16/28, 57%). CONCLUSIONS: We observed variance by site for recruitment, due to differences in identification and ascertainment of eligible cases and the required institutional review board processes. Restriction in recruitment and the proportion of minorities likely impacted participation rates in Arizona more than Utah.

PURPOSE: We performed an exploratory analysis of data from the National Spina Bifida Patient Registry (NSBPR) to assess variation in the frequency of bladder reconstruction surgeries among NSBPR centers. METHODS: We queried the 2009-2014 NSBPR to identify patients who had ever undergone bladder reconstruction surgeries. We evaluated demographic characteristics, SB type, functional level, mobility, and NSBPR center to determine whether any of these factors were associated with reconstructive surgery rates. Multivariable logistic regression was used to simultaneously adjust for the impact of these factors. RESULTS: We identified 5,528 patients with SB enrolled in the NSBPR. Of these, 1,129 (20.4%) underwent bladder reconstruction (703 augmentation, 382 continent catheterizable channel, 189 bladder outlet procedure). Surgery patients were more likely to be older, female, non-Hispanic white, higher lesion level, myelomeningocele diagnosis, non-ambulators (all p<0.001) and non-privately insured (p=0.018). Bladder reconstruction surgery rates varied among NSBPR centers (range 12.1-37.9%, p<0.001). After correcting for known confounders, NSBPR center, SB type, mobility, gender and age (all p<0.001) were significant predictors of surgical intervention. Race (p=0.19) and insurance status (p=0.11) were not associated with surgical intervention. CONCLUSIONS: There is significant variation in rates of bladder reconstruction surgery among NSBPR centers. In addition to clinical factors such as mobility status, lesion type, and lesion level, non-clinical factors such as patient age, gender and treating center are also associated with the likelihood of an individual undergoing bladder reconstruction.

INTRODUCTION: Care of children with spina bifida (SB) has significantly advanced over the last half-century, resulting in gains in longevity and quality of life for affected children and caregivers. Bladder dysfunction is the norm in SB patients and may result in infection, renal scarring, and chronic kidney disease. However, the optimal urologic management for SB-related bladder dysfunction is unknown. METHODS: In 2012, Centers for Disease Control and Prevention (CDC) convened a working group composed of pediatric urologists, nephrologists, epidemiologists, methodologists, community advocates, and CDC personnel to develop a protocol to optimize urologic care of children with SB from the newborn period through 5 years of age. RESULTS: An iterative quality-improvement protocol was selected; in this model, participating institutions agree to prospectively treat all newborns with SB using a single consensus-based protocol. Over the course of the 5-year study period, study outcomes are routinely assessed and the protocol adjusted as needed in order to optimize patient and process outcomes. Primary study outcomes include urinary tract infections (UTI), renal scarring, renal function, and bladder characteristics. The protocol specifies the timing and use of testing (e.g., ultrasonography, urodynamics) and interventions (e.g., intermittent catheterization, prophylactic antibiotics, antimuscarinic medications). Starting in 2014, the CDC began funding nine study sites to implement and evaluate the protocol. CONCLUSIONS: The CDC Urologic and Renal Protocol for the Newborn and Young Child with Spina Bifida began accruing patients in 2015. Assessment in the first 5 years will focus on UTIs, renal function, renal scarring, and clinical process improvements.

PURPOSE: Clinical guidelines recommend repair of open spina bifida (SB) prenatally or within the first days of an infant's life. We examined maternal, infant, and health care system factors associated with time-to-repair among infants with postnatal repair. METHODS: This retrospective, statewide, population-based study examined infants with SB born in Florida 1998-2007, ascertained by the Florida Birth Defects Registry. We used procedure codes from hospital discharge records to identify the first recorded myelomeningocele repair (ICD-9 CM procedure code 03.52) among infants with birth hospitalizations. Using Poisson multivariable regression, we examined time-to-repair by hydrocephalus, SB type (isolated [no other coded major birth defect] versus non-isolated), and other selected factors. RESULTS: Of 199 infants with a recorded birth hospitalization and coded myelomeningocele repair, 87.9 % had hydrocephalus and 19.6 % had non-isolated SB. About 76.4 % of infants had repair by day 2 of life. In adjusted analyses, infants with hydrocephalus were more likely to have timely repair (adjusted prevalence ratio (aPR) = 1.48, 95 % confidence interval (CI) 1.02-2.14) than infants without hydrocephalus. SB type was not associated with repair timing. Infants born in lower level nursery care hospitals with were less likely to have timely repairs (aPR = 0.71, 95 % CI 0.52-0.98) than those born in higher level nursery care hospitals. CONCLUSIONS: Most infants with SB had surgical repair in the first 2 days of life. Lower level birth hospital nursery care was associated with later repairs. Prenatal diagnosis can facilitate planning for a birth hospital with higher level of nursery care, thus improving opportunities for timely repair.

OBJECTIVE: To describe factors associated with pressure ulcers in individuals with spina bifida (SB) enrolled in the National Spina Bifida Patient Registry (NSBPR). DESIGN: unbalanced longitudinal, multicenter cohort study. SETTING: Nineteen SB clinics throughout the U.S. INTERVENTIONS: Not applicable PARTICIPANTS: Individuals with SB (N=3153) enrolled in 19 clinic sites that participate in the NSBPR. MAIN OUTCOME MEASURE: Pressure ulcer status (yes/no) at annual visit between 2009 and 2012. RESULTS: Of 3153 total participants, 19% reported ulcers at their most recent annual clinic visit. Seven factors, i.e., level of lesion, wheelchair use, urinary incontinence, shunt presence, above the knee orthopedic surgery, recent surgery, and male gender were significantly associated with the presence of pressure ulcers. Of these factors, the final logistic regression model included level of lesion, urinary incontinence, recent surgery, and male gender. The three adjusting variables SB type, SB clinic, and age group were significant in all analyses (all p<.001). CONCLUSION: Adjusting for SB type, SB clinic, and age group, we found seven factors, level of lesion, wheelchair use, urinary incontinence, shunt presence, above the knee orthopedic surgery, recent surgery, and male gender were associated with pressure ulcers. Identifying key factors associated with onset of pressure ulcers can be incorporated into clinical practice in ways to prevent and enhance treatment for pressure ulcers in the SB population.

BACKGROUND: A National Spina Bifida Patient Registry (NSBPR) was begun in 2009 to help understand the natural history of spina bifida (SB) and the effects of treatments provided by SB clinics. We used the NSBPR to explore the relationship of sociodemographic characteristics with SB outcomes. METHODS: Using NSBPR data collected in 2009 to 2012, we examined the unadjusted association between demographic characteristics and 4 SB outcomes: bowel continence, bladder continence, mobility, and presence of pressure sores. We then developed multivariable logistic models to explore these relationships while controlling for SB clinic, SB type, and level of lesion. RESULTS: Data were available on 2054 patients <22 years of age from 10 SB clinics. In the multivariable models, older age groups were more likely to have continence and pressure sores and less likely to be community ambulatory. Males and patients without private insurance were less likely to be continent and community ambulatory. Non-Hispanic blacks were less likely to be continent. Level of lesion was associated with all outcomes; SB type was associated with all but pressure sores; and all outcomes except community ambulation showed significant variation across clinic sites. CONCLUSIONS: Sociodemographic attributes are associated with SB outcomes. In particular, males, non-Hispanic blacks, and patients without private insurance have less favorable outcomes, and age has an impact as well. These characteristics need to be considered by clinicians who care for this patient population and factored into case-mix adjustment when evaluating variation in clinical and functional outcomes among different SB clinics.

OBJECTIVE: To compare emergency room (ER) and inpatient hospital (IP) use rates for people with spina bifida (SB) to peers without SB, when transition from pediatric to adult health care is likely to occur, and analyze those ER and IP rates by age, race, socioeconomic status, gender, and county type. DESIGN: A retrospective cohort study SETTING: Secondary data analysis in South Carolina PARTICIPANTS: We studied individuals who were between 15-24 years old and enrolled in the State Health Plan (SHP) or state Medicaid during the 2000 - 2010 study period. METHODS: Individuals with SB were identified using ICD-9 billing codes (741.0, 741.9) in SHP, Medicaid, and hospital uniform billing (UB) data. ER and IP encounters were identified using UB data. Multivariable Generalized Estimating Equation (GEE) Poisson models were estimated to compare rates of ER and IP use among the SB group to the comparison group. MAIN OUTCOME MEASUREMENTS: Total ER rate and IP rate, in addition to cause-specific rates for ambulatory care sensitive conditions (ACSC) and other condition categories. RESULTS: We found higher rates of ER and IP use in people with SB compared to the control group. Among individuals with SB, young adults (20-24 year olds) had higher rates of ER use due to all ACSC (p=.023), other ACSC (p=.04), and urinary tract infections (UTI; p=.002) compared to adolescents (15-19 year olds). CONCLUSIONS: Young adulthood is associated with increased ER use overall, as well as in specific condition categories (most notably UTI) in 15-24 year olds with SB. This association may be indicative of changing healthcare access as people with SB move from adolescent to adult health care, and/or physiologic changes during the age range studied.

OBJECTIVE: To use data from the US National Spina Bifida Patient Registry (NSBPR) to describe variations in Contexts of Care, Processes of Care, and Health Outcomes among individuals with spina bifida (SB) receiving care in 10 clinics. STUDY DESIGN: Reported here are baseline cross-sectional data representing the first visit of 2172 participants from 10 specialized, multidisciplinary SB clinics participating in the NSBPR. We used descriptive statistics, the Fisher exact test, chi2 test, and Wilcoxon rank-sum test to examine the data. RESULTS: The mean age was 10.1 (SD 8.1) years with slightly more female subjects (52.5%). The majority was white (63.4%) and relied upon public insurance (53.5%). One-third had sacral lesions, 44.8% had mid-low lumbar lesions, and 24.9% had high lumbar and thoracic lesions. The most common surgery was ventricular shunt placement (65.7%). The most common bladder-management technique among those with bladder impairment was intermittent catheterization (69.0%). Almost 14% experienced a pressure ulcer in the last year. Of those ages 5 years or older with bowel or bladder impairments, almost 30% were continent of stool; a similar percentage was continent of urine. Most variables were associated with type of SB diagnosis. CONCLUSION: The NSBPR provides a cross section of a predominantly pediatric population of patients followed in specialized SB programs. There were wide variations in the variables studied and major differences in Context of Care, Processes of Care, and Health Outcomes by type of SB. Such wide variation and the differences by type of SB should be considered in future analyses of outcomes.

PURPOSE: We described the characteristics, treatments, and survival of persons with spina bifida (SB) who developed end stage renal disease (ESRD) from 2004 through 2008 in the United States Renal Data System (USRDS). MATERIALS AND METHODS: We used ICD-9-CM code 741.* to identify persons with SB from hospital inpatient data (1977-2010) and physician and facility claims (2004-2008). We constructed a 5:1 comparison group of ESRD patients without SB matched by age at first ESRD service, gender, and race/ethnicity. We assessed their risk of mortality and of renal transplant while on dialysis using multivariate cause-specific proportional hazards survival analyses. We also compared their survival after their first renal transplant from first ESRD service to August 2011. RESULTS: We identified 439 ESRD patients with SB, who on average developed ESRD at younger ages than patients without SB (41 vs. 62 years, P<0.001) and urological issues were the most common primary cause of their ESRD. Compared to ESRD patients without SB, patients with SB had similar mortality hazard on dialysis and after a transplant. But ESRD patients without SB were more likely to undergo renal transplantation than patients with SB (hazard ratio=1.51, 95% CI=1.13-2.03). Hospitalizations related to urinary tract infections (UTIs) were positively associated with risk of death on dialysis for ESRD patients with SB (hazard ratio=1.42, 95% CI=1.33-1.53). CONCLUSIONS: SB was not associated with elevated mortality among ESRD patients on dialysis or after a renal transplant. Proper urological and bladder management for patients with SB, particularly for adults, is imperative.

BACKGROUND: Population-based empirical information to inform health care professionals working with children with spina bifida currently is lacking. Spina bifida is a highly complex condition that not only affects mobility but many additional aspects of life. We have developed a pilot project that focuses on a broad range of domains: surgeries, development and learning, nutrition and physical growth, mobility and functioning, general health, and family demographics. Specifically, we will: (1) explore the feasibility of identifying and recruiting participants using different recruitment sources, (2) test a multidisciplinary module to collect the data, (3) determine the utility of different methods of retrieving the data, and (4) summarize descriptive information on living with spina bifida. OBJECTIVE: The overall objective of the project was to provide information for a future multistate prospective study on the natural history of spina bifida. METHODS: Families with a child 3 to 6 years of age with a diagnosis of spina bifida were eligible for enrollment. Eligible families were identified through a US population-based tracking system for birth defects and from a local spina bifida clinic. RESULTS: This is an ongoing project with first results expected in 2013. CONCLUSIONS: This project, and the planned multistate follow-up project, will provide information both to health care professionals experienced in providing care to patients with spina bifida, and to those who have yet to work with this population. The long-term purpose of this project is to increase the knowledge about growing up with spina bifida and to guide health care practices by prospectively studying a cohort of children born with this condition. (JMIR Res Protoc 2013; 2(1): e2) doi:10.2196/resprot.2209

BACKGROUND: The purpose of this study was to describe the development and early implementation of a national spina bifida (SB) patient registry, the goal of which is to monitor the health status, clinical care, and outcomes of people with SB by collecting and analyzing patient data from comprehensive SB clinics. METHODS: Using a web-based, SB-specific electronic medical record, 10 SB clinics collected health-related information for patients diagnosed with myelomeningocele, lipomyelomeningocele, fatty filum, or meningocele. This information was compiled and de-identified for transmission to the Centers for Disease Control and Prevention (CDC) for quality control and analysis. RESULTS: A total of 2070 patients were enrolled from 2009 through 2011: 84.9% were younger than 18 years of age; 1095 were women; 64.2% were non-Hispanic white; 6.5% were non-Hispanic black or African American; and 24.2% were Hispanic or Latino. Myelomeningocele was the most common diagnosis (81.5%). CONCLUSIONS: The creation of a National Spina Bifida Patient Registry partnership between the CDC and SB clinics has been feasible. Through planned longitudinal data collection and the inclusion of additional clinics, the data generated by the registry will become more robust and representative of the population of patients attending SB clinics in the United States and will allow for the investigation of patient outcomes. (Birth Defects Research (Part A), 2012. (c) 2012 Wiley Periodicals, Inc.)

AIM: Persons with spina bifida who adopt unhealthy lifestyles could be at increased risk of adverse health outcomes because the presence of spina bifida may magnify this risk. We estimated overall and age-specific prevalence of selected health risk behaviors (HRBs) in young people with spina bifida and examined the association between HRBs and depression. METHOD: We performed analyses on data obtained from individuals with spina bifida (n=130; mean age 23y SD 4y 5mo; 64 males, 66 females; 64% lumbosacral lesion; 77% with shunt) who participated in a population-based survey conducted by the Arkansas Spinal Cord Commission in 2005. RESULTS: Compared with national estimates, young people with spina bifida tend to eat less healthy diets, do less exercise, and engage in more sedentary activities. Respondents were less likely to use substances (alcohol, tobacco, illegal drugs), which peaked among 25 to 31 year olds. About 90% saw a doctor in the previous year. Nearly one half reported mild or major depressive symptoms. In the logistic regression analysis after controlling for potential confounders (age, sex, ethnic group, education, employment, marital status, living arrangement, level of lesion, presence of shunt, mobility, self-rated health and healthcare utilization), major depressive symptoms were associated with current alcohol drinking (adjusted odds ratio: 4.74; 95% CI 1.18-19.04). INTERPRETATION: Young adults with spina bifida exhibit unhealthy behaviors that continue into their late 20s. The findings highlight the need to increase awareness of their health risk profiles in the spina bifida community and show opportunities for mental health and health risk screening and counseling by healthcare providers.

OBJECTIVE: To describe the most prevalent conditions and their associated expenditures for the outpatient care of individuals with spina bifida (SB) of varying ages. DESIGN: From a large health insurance claims database of people with private insurance, we examined records on outpatient health care received during 2005-2006 for individuals with SB and a matched comparison group. Chronic conditions from the most frequently recorded 4-digit ICD-9-CM codes for individuals with SB were grouped into four categories: cardiovascular disease risk factors, SB secondary conditions, pain, and other symptoms. RESULTS: Diseases affecting the nervous, genitourinary, and musculoskeletal systems and miscellaneous symptoms (e.g., headache, fever) account for about 70% of outpatient expenditures, excluding those associated with perinatal and congenital conditions. The most common and costly conditions by age group were diseases of the nervous system for children and adults younger than age 30 years and diseases of the musculoskeletal system for adults aged 30-64 years. Individuals with SB had significantly elevated risks for essential hypertension, urinary tract infection, and constipation at young ages and headache, sleep disturbance, and fever throughout the life span. CONCLUSIONS: The frequency of health conditions associated with SB varies across the life span. These conditions should be a priority for further investigations to identify risk factors, treatment and prevention strategies for individuals with SB. 2010 IOS Press and the authors. All rights reserved.

This article outlines and summarizes the rationale and the working process that was undertaken by the National Spina Bifida Program to address the issues of transitioning throughout the life course for persons growing up with spina bifida. Their challenges include achieving independent living, vocational independence, community mobility, and participation in social activities, and health management. The creation, the underlying concepts, and the dissemination of the Life Course Model are described.

BACKGROUND: The purpose of this study was to investigate whether US female adolescents who self-reported having a physical disability or long-term health problem were more likely to report having been physically forced to have sexual intercourse than US female adolescents without a physical disability or long-term health problem. METHODS: Using data from the 2005 U.S. National Youth Risk Behavior Survey (YRBS), we employed logistic regression analyses to estimate the association between physical disability (and other variables) and the risk for sexual violence among US high school girls. RESULTS: Female high school students who reported a physical disability or long-term health problem were more likely to report having been physically forced to have sexual intercourse than those who did not (19.6% vs 9.4%;chi(2) = 14.51, p = .003). Results from our multivariate analysis reveal that this association remained significant (adjusted odds ratio [AOR], 1.57; 95% confidence interval [CI], 1.10-2.23) after adjusting for certain demographic characteristics, physical health problems, behavioral health risks, and violent conduct. CONCLUSIONS: Our findings suggest that adolescent girls in the United States with a physical disability or long-term health problem may be at increased risk for sexual violence. It is important that national efforts to reduce sexual violence consider how to address the unmet needs of children and adolescents with disabilities. As most adolescent girls spend the majority of their time in a school setting, it is of particular importance that school health professionals are aware of the current findings.