Comparing binary & ordinal definitions of urinary & stool continence outcomes: Data from the National Spina Bifida Patient Registry.

INTRODUCTION: The National Spina Bifida Patient Registry (NSBPR) assesses bladder and bowel incontinence using ordinal categories, but prior NSBPR analyses employed binary classification. Our aims were to 1) perform the first NSBPR analysis of bladder and bowel incontinence as ordinal outcomes to compare to the binary definition and subject variables; 2) explore the correlation of incontinence with undergarment usage, and 3) assess incontinence status following continence surgeries. METHODS: Data from NSBPR participants' most recent clinic visit from 2013 to 2020 were analyzed. Ordinal categories of incontinence were compared to previously used binary definitions. Incontinence surgical outcomes were analyzed for those with data at least three months post-operatively. Chi-square tests evaluated associations among categorical variables. Univariate and ordinal logistic regression models were used to test associations of ordinal incontinence status with patient and condition factors. Statistical tests were 2-sided; p values < 0.05 were considered significant. RESULTS: Analysis of 7217 individuals using ordinal incontinence outcomes showed little difference from previously used binary outcomes. The final multivariable logistic regression models with ordinal multinomial outcomes showed that associations of incontinence with age, sex, race/ethnicity, health insurance, level of lesion, and continence management technique were similar to prior studies. Among those reporting never being incontinent of both bladder and bowel, 14% reported using protective undergarments. Of the 500 individuals who had bladder outlet surgery, 38% reported never being incontinent of urine. Of 1416 individuals who had appendicostomy (ACE) bowel surgery, 48% reported never being incontinent of stool. DISCUSSION: Our current analysis showed that ordinal continence outcome classification had similar continence findings as previous studies using the binary definition of continence. Expanding the binary definition of continence to include monthly episodes of incontinence did not greatly increase the proportion of continent individuals and, therefore, would have not likely made meaningful differences in continence outcomes in prior NSBPR analyses. However, it is known that even mild incontinence can affect quality of life, therefore, capturing any level of incontiennce is of clinical importance. Confirmation of the association of continence outcomes with sociodemographic, condition-related, and interventional factors with both approaches further validates previous analyses using the binary definition of continence. CONCLUSION: The previously used binary definition of bladder and bowel continence appears robust. Undergarment choice was a poor surrogate for reported incontinence. After bladder and bowel continence surgeries, 38% and 48%, respectively, reported never being incontinent.

Age-Specific Probability of 4 Major Health Outcomes in Children with Spina Bifida.

OBJECTIVE: This study aimed to estimate the age-specific probability of 4 health outcomes in a large registry of individuals with spina bifida (SB). METHODS: The association between age and 4 health outcomes was examined in individuals with myelomeningocele (MMC, n = 5627) and non-myelomeningocele (NMMC, n = 1442) from the National Spina Bifida Patient Registry. Sixteen age categories were created, 1 for each year between the ages of 5 and 19 years and 1 for those aged 20 years or older. Generalized linear models were used to calculate the adjusted probability and 95% prediction intervals of each outcome for each age category, adjusting for sex and race/ethnicity. RESULTS: For the MMC and NMMC groups, the adjusted coefficients for the correlation between age and the probability of each outcome were -0.933 and -0.657 for bladder incontinence, -0.922 and -0.773 for bowel incontinence, 0.942 and 0.382 for skin breakdown, and 0.809 and 0.619 for lack of ambulation, respectively. CONCLUSION: In individuals with SB, age is inversely associated with the probability of bladder and bowel incontinence and directly associated with the probability of skin breakdown and lack of ambulation. The estimated age-specific probabilities of each outcome can help SB clinicians estimate the expected proportion of patients with the outcome at specific ages and explain the probability of the occurrence of these outcomes to patients and their families.

Education and employment as young adults living with spina bifida transition to adulthood in the USA: A study of the National Spina Bifida Patient Registry.

AIM: To describe the education and employment transition experience of young adults with spina bifida (YASB) and investigate factors associated with employment. METHOD: We queried education and employment data from the US National Spina Bifida Patient Registry from 2009 to 2019. We applied generalized estimating equations models to analyze sociodemographic and disease-related factors associated with employment. RESULTS: A total of 1909 participants (850 males, 1059 females) aged 18 to 26 years contributed 4379 annual visits. Nearly 84% had myelomeningocele and, at last visit, the median age was 21 years (mean 21 years 5 months, SD 2 years 10 months). A total of 41.8% had at least some post-high school education, and 23.9% were employed. In a multivariable regression model, employment was significantly associated with education level, lower extremity functional level, bowel continence, insurance, and history of non-shunt surgery. This large, national sample of YASB demonstrated low rates of post-secondary education attainment and employment and several potentially modifiable factors associated with employment. INTERPRETATION: Specific sociodemographic, medical, and functional factors associated with employment are important for clinicians to consider when facilitating transition for YASB into adulthood. Additional research is needed to understand the impact of cognitive functioning and social determinants of health on transition success in YASB. WHAT THIS PAPER ADDS: There were low education attainment and employment rates in a large sample of young adults with spina bifida. Specific sociodemographic, medical, and functional factors are associated with employment. Some employment-associated factors, such as continence and self-management skills, are modifiable.

Weight Status of Children Participating in the National Spina Bifida Patient Registry.

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.

Association between age of starting clean intermittent catheterization and current urinary continence in individuals with myelomeningocele.

INTRODUCTION: Patients with myelomeningocele often use clean intermittent catheterization (CIC) for renal preservation and to promote urinary continence. While starting CIC at an early age is associated with better renal outcomes, the impact of age of CIC initiation on continence outcomes has not been examined. OBJECTIVE: To examine whether earlier CIC initiation is associated with higher likelihood of current urinary continence for patients with myelomeningocele. STUDY DESIGN: Data of patients aged ≥5 years at last visit were obtained from 35 spina bifida clinics participating in the National Spina Bifida Patient Registry from 2013 to 2018. Sociodemographic characteristics, disease characteristics, and current bladder management strategies were collected. Via univariate and multiple logistic regression models, the latter conducted controlling for all variables associated with current continent status, associations between continence and sociodemographic factors, condition characteristics, and age CIC began (<3 years of age, 3-5 years, 6-11 years, ≥12 years) were analyzed. RESULTS: Data from 3510 individuals were included (mean age at last visit = 17.0 years, range 5.0-88.7). The sample was evenly distributed by sex (52% female); most individuals were non-Hispanic White (62.6%). The majority of patients (55.2%) started CIC before age 3 years. Continence varied markedly across those who never started CIC (0.6% of patients were continent) and those who started at any age (range 35.3-38.5%). Among those who started CIC, the magnitude of the association was not proportional to age CIC was started. Compared with those who started CIC at age 12 or older, estimated adjusted odds ratio of being continent ranged from 1.04 (6-11 years, 95% CI, 0.72-1.52) to 1.25 (<3 years, 95% CI, 0.89-1.76). DISCUSSION: Although CIC may be positively associated with achieving urinary continence in individuals with myelomeningocele, we could not demonstrate that younger age at CIC initiation increased the likelihood of achieving this goal. Limitations include lack of data on reason for starting CIC, urodynamic data, and the observational nature of data collection. CONCLUSIONS: Further study is needed addressing limitations of the current investigation to determine if urinary continence outcomes are influenced by the age of starting CIC among patients with myelomeningocele.

Time to shunt failure in children with myelomeningocele: an analysis of the National Spina Bifida Patient Registry.

OBJECTIVE: Hydrocephalus is common among children with myelomeningocele and is most frequently treated with a ventriculoperitoneal shunt (VPS). Although much is known about factors related to first shunt failure, relatively less data are available about shunt failures after the first one. The purpose of this study was to use a large data set to explore time from initial VPS placement to first shunt failure in children with myelomeningocele and to explore factors related to multiple shunt failures. METHODS: Data were obtained from the National Spina Bifida Patient Registry. Children with myelomeningocele who were enrolled within the first 5 years of life and had all lifetime shunt operations recorded in the registry were included. Kaplan-Meier survival curves were constructed to evaluate time from initial shunt placement to first shunt failure. The total number of children who experienced at least 2 shunt failures was calculated. A proportional means model was performed to calculate adjusted hazard ratios (HRs) for shunt failure on the basis of sex, race/ethnicity, lesion level, and insurance status. RESULTS: In total, 1691 children met the inclusion criteria. The median length of follow-up was 5.0 years. Fifty-five percent of patients (938 of 1691) experienced at least 1 shunt failure. The estimated median time from initial shunt placement to first failure was 2.34 years (95% confidence interval [CI] 1.91-3.08 years). Twenty-six percent of patients had at least 2 shunt failures, and 14% of patients had at least 3. Male children had higher likelihood of shunt revision (HR 1.25, 95% CI 1.09-1.44). Children of minority race/ethnicity had a lower likelihood of all shunt revisions (non-Hispanic Black children HR 0.74, 95% CI 0.55-0.98; Hispanic children HR 0.74, 95% CI 0.62-0.88; children of other ethnicities HR 0.80, 95% CI 0.62-1.03). CONCLUSIONS: Among the children with myelomeningocele, the estimated median time to shunt failure was 2.34 years. Forty-five percent of children never had shunt failure. The observed higher likelihood of shunt revisions among males and lower likelihood among children of minority race/ethnicity illustrate a possible disparity in hydrocephalus care that warrants additional study. Overall, these results provide important information that can be used to counsel parents of children with myelomeningocele about the expected course of shunted hydrocephalus.

Change in unemployment by social vulnerability among United States counties with rapid increases in COVID-19 incidence-July 1-October 31, 2020.

OBJECTIVE: During the COVID-19 pandemic, the unemployment rate in the United States peaked at 14.8% in April 2020. We examined patterns in unemployment following this peak in counties with rapid increases in COVID-19 incidence. METHOD: We used CDC aggregate county data to identify counties with rapid increases in COVID-19 incidence (rapid riser counties) during July 1-October 31, 2020. We used a linear regression model with fixed effect to calculate the change of unemployment rate difference in these counties, stratified by the county's social vulnerability (an indicator compiled by CDC) in the two months before the rapid riser index month compared to the index month plus one month after the index month. RESULTS: Among the 585 (19% of U.S. counties) rapid riser counties identified, the unemployment rate gap between the most and least socially vulnerable counties widened by 0.40 percentage point (p<0.01) after experiencing a rapid rise in COVID-19 incidence. Driving the gap were counties with lower socioeconomic status, with a higher percentage of people in racial and ethnic minority groups, and with limited English proficiency. CONCLUSION: The widened unemployment gap after COVID-19 incidence rapid rise between the most and least socially vulnerable counties suggests that it may take longer for socially and economically disadvantaged communities to recover. Loss of income and benefits due to unemployment could hinder behaviors that prevent spread of COVID-19 (e.g., seeking healthcare) and could impede response efforts including testing and vaccination. Addressing the social needs within these vulnerable communities could help support public health response measures.

Differences in rapid increases in county-level COVID-19 incidence by implementation of statewide closures and mask mandates - United States, June 1-September 30, 2020.

BACKGROUND AND OBJECTIVE: Community mitigation strategies could help reduce COVID-19 incidence, but there are few studies that explore associations nationally and by urbanicity. In a national county-level analysis, we examined the probability of being identified as a county with rapidly increasing COVID-19 incidence (rapid riser identification) during the summer of 2020 by implementation of mitigation policies prior to the summer, overall and by urbanicity. METHODS: We analyzed county-level data on rapid riser identification during June 1-September 30, 2020 and statewide closures and statewide mask mandates starting March 19 (obtained from state government websites). Poisson regression models with robust standard error estimation were used to examine differences in the probability of rapid riser identification by implementation of mitigation policies (P-value< .05); associations were adjusted for county population size. RESULTS: Counties in states that closed for 0-59 days were more likely to become a rapid riser county than those that closed for >59 days, particularly in nonmetropolitan areas. The probability of becoming a rapid riser county was 43% lower among counties that had statewide mask mandates at reopening (adjusted prevalence ratio = 0.57; 95% confidence intervals = 0.51-0.63); when stratified by urbanicity, associations were more pronounced in nonmetropolitan areas. CONCLUSIONS: These results underscore the potential value of community mitigation strategies in limiting the COVID-19 spread, especially in nonmetropolitan areas.

Neurosurgical procedures for children with myelomeningocele after fetal or postnatal surgery: a comparative effectiveness study.

AIM: To compare the frequencies of neurosurgical procedures to treat comorbid conditions of myelomeningocele in patients who underwent fetal surgery versus postnatal surgery for closure of the placode. METHOD: By utilizing the National Spina Bifida Patient Registry in a comparative effectiveness study, 298 fetal surgery patients were matched by birthdate (±3mo) and spina bifida clinic site with one to three postnatal surgery patients (n=648). Histories were obtained by record review on enrollment and yearly subsequently. Multivariable Poisson regression was used to compare frequencies of procedures between cohorts, with adjustments for sex, ethnicity, insurance status, spinal segmental level of motor function, age at last visit recorded in the Registry, and, for shunt revision in shunted patients, age at cerebrospinal fluid (CSF) diversion. RESULTS: The median age at last visit was 4 years. In fully adjusted analyses in patients aged at least 12 months old, fetal surgery was associated with decreased frequency of CSF diversion for hydrocephalus by ventriculoperitoneal shunt insertion or endoscopic third ventriculostomy compared with postnatal surgery (46% vs 79%; incidence rate ratio=0.61; 95% confidence interval [CI] 0.53-0.71; p<0.01). Over all ages, fetal surgery was associated with decreased frequency of Chiari decompression for brainstem dysfunction (3% vs 7%; incidence rate ratio=0.41; 95% CI 0.19-0.88; p=0.02). Also over all ages, differences were not significant in frequencies of shunt revision in shunted patients (53% vs 55%; incidence rate ratio=0.87; 95% CI 0.69-1.11; p=0.27), nor tethered cord release for acquired spinal cord dysfunction (18% vs 16%; incidence rate ratio=1.11; 95% CI 0.84-1.47; p=0.46). INTERPRETATION: Even with the variations inherent in clinical practice, fetal surgery was associated with lower frequencies of CSF diversion and of Chiari decompression, independent of covariates. What this paper adds Fetal surgery was associated with lower frequencies of cerebrospinal fluid diversion and decompression of Chiari II malformation than postnatal surgery. Frequencies of ventriculoperitoneal shunt revision and tethered cord release were not significantly different between cohorts.

Neurogenic bowel treatments and continence outcomes in children and adults with myelomeningocele.

PURPOSE: Neurogenic bowel dysfunction (NBD) is a common comorbidity of myelomeningocele (MMC), the most common and severe form of spina bifida. The National Spina Bifida Patient Registry (NSBPR) is a research collaboration between the CDC and Spina Bifida Clinics. Fecal continence (continence) outcomes for common treatment modalities for NBD have not been described in a large sample of individuals with MMC. NSBPR patients with MMC and NBD were studied to determine variation in continence status and their ability to perform their treatment independently according to treatment modality and individual characteristics. METHODS: Continence was defined as < 1 episode of incontinence per month. Eleven common treatments were evaluated. Inclusion criteria were established diagnoses of both MMC and NBD, as well as age ⩾ 5 years (n= 3670). Chi-square or exact statistical tests were used for bivariate analyses. Logistic regression models were used to estimate the odds of continence outcomes by age, sex, race/ethnicity, level of motor function, and insurance status. RESULTS: At total of 3670 members of the NSBPR met inclusion criteria between November 2013 and December 2017. Overall prevalence of continence was 45%. Prevalence ranged from 40-69% across different treatments. Among continent individuals, 60% achieved continence without surgery. Antegrade enemas were the most commonly used treatment and had the highest associated continence rate. Ability to carry out a treatment independently increased with age. Multivariable logistic regression showed significantly higher odds of continence among individuals aged ⩾ 12 years, female, non-Hispanic white, and with private insurance.

Kidney Function Surveillance in the National Spina Bifida Patient Registry: A Retrospective Cohort Study.

PURPOSE: Chronic kidney disease affects 25% to 50% of patients with spina bifida. Guidelines recommend kidney function surveillance in these patients but practice patterns are unknown. Variations in kidney function surveillance were assessed in patients with spina bifida based on the hypothesis that the treating clinic and spina bifida type would be associated with kidney function surveillance. MATERIALS AND METHODS: A retrospective cohort study was conducted of U.S. patients in the National Spina Bifida Patient Registry from 2013 to 2018. Followup was anchored at the 2013 visit. Participants with either an outcome event within 2 years of followup or more than 2 years of followup without an outcome event were included. Primary outcome was kidney function surveillance, defined as at least 1 renal ultrasound and serum creatinine within 2 years of followup. Primary exposures were clinic and spina bifida type, which were analyzed with covariates including sociodemographic and clinical characteristics in logistic regression models for their association with the outcome. Sensitivity analyses were performed using different kidney function surveillance definitions. RESULTS: Of 8,351 patients 5,445 were included with a median followup of 3.0 years. Across 23 treating clinics kidney function surveillance rates averaged 62% (range 6% to 100%). In multivariable models kidney function surveillance was associated with treating clinic, younger patient age, functional lesion level, nonambulatory status and prior bladder augmentation. Treating clinic remained a significant predictor of kidney function surveillance in all sensitivity analyses. CONCLUSIONS: Within the National Spina Bifida Patient Registry wide variation exists in practice of kidney function surveillance across treating clinics despite adjustment for key patient characteristics.

Differences in continence rates in individuals with spina bifida based on ethnicity.

BACKGROUND: Ethnic disparities in continence rates in spina bifida (SB) have been studied regionally but not nationally. National SB Patient Registry (NSBPR) data were analyzed to explore differences in prevalence of bowel and bladder continence and interventions between Hispanics/Latinos and others. METHODS: Participants 5 to 21 years were categorized into Hispanic/Latino and non-Hispanic/non-Latino. Bladder/bowel continence was defined as dry/no involuntary stool leakage during the day or none/⩽ monthly incontinence. Chi-square test, Wilcoxon Two Sample Test, and generalized estimating equation (GEE) were used for statistical analysis. RESULTS: Twenty-five percent of the 4,364 patients were Hispanic/Latino. At their most recent clinic visit, Hispanics/Latinos demonstrated lower rates of urinary continence (38.6% vs. 44.9%; p= 0.0003), bowel continence (43.9% vs. 55.8%, p< 0.0001), private insurance (p< 0.0001), bowel (p< 0.0001) or bladder surgeries (p= 0.0054), and more vesicostomies (p= 0.0427) compared to others. In multiple GEE models, Hispanic/Latino participants demonstrated lower odds of bowel continence as compared to non-Hispanic/non-Latino participants (estimated odds ratio, 0.82, 95% CI, 0.72-0.94, p= 0.0032). CONCLUSIONS: After controlling for covariates, Hispanics/Latinos with SB are less likely to report bowel continence. Clinicians are encouraged to consider the risk of negative health disparities for Hispanic patients with SB and work to mitigate this risk.

Bladder Management and Continence Outcomes in Adults with Spina Bifida: Results from the National Spina Bifida Patient Registry, 2009 to 2015.

PURPOSE: Most children with spina bifida now survive into adulthood, although most have neuropathic bladder with potential complications of incontinence, infection, renal damage and diminished quality of life. In this study we sought to 1) describe contemporary bladder management and continence outcomes of adults with spina bifida, 2) describe differences from younger individuals and 3) assess for association with socioeconomic factors. MATERIALS AND METHODS: We analyzed data on bladder management and outcomes in adults with spina bifida from the National Spina Bifida Patient Registry. A strict definition of continence was used. Results were compared to young children (age 5 to 11 years) and adolescents (12 to 19). Statistical analysis compared cohorts by gender, ethnicity, spina bifida type, lesion level, insurance status, educational attainment, employment status and continence. RESULTS: A total of 5,250 patients with spina bifida were included, of whom 1,372 (26.1%) were adults. Of the adult patients 45.8% did not take medication, but 76.8% performed clean intermittent catheterization. Continence was decreased in adults with myelomeningocele (45.8%) vs those with nonmyelomeningocele spina bifida (63.1%, p <0.0001). Continence rates were higher in the older cohorts with myelomeningocele (p <0.0001) but not in those with nonmyelomeningocele spina bifida (p = 0.1192). Bladder management and history of urological surgery varied among age groups. On univariate analysis with spina bifida related or socioeconomic 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 children with spina bifida. Bladder continence outcomes were better in adults, with nearly half reporting continence. Continence was significantly associated with employment status in patients age 25 years or older.

The contribution of family history to the burden of diagnosed diabetes, undiagnosed diabetes, and prediabetes in the United States: analysis of the National Health and Nutrition Examination Survey, 2009-2014.

PURPOSE: Given the importance of family history in the early detection and prevention of type 2 diabetes, we quantified the public health impact of reported family health history on diagnosed diabetes (DD), undiagnosed diabetes (UD), and prediabetes (PD) in the United States. METHODS: We used population data from the National Health and Nutrition Examination Survey 2009-2014 to measure the association of reported family history of diabetes with DD, UD, and PD. RESULTS: Using polytomous logistic regression and multivariable adjustment, family history prevalence ratios were 4.27 (confidence interval (CI): 3.57, 5.12) for DD, 2.03 (CI: 1.56, 2.63) for UD, and 1.26 (CI: 1.09, 1.44) for PD. In the United States, we estimate that 10.1 million DD cases, 1.4 million UD cases, and 3.9 million PD cases can be attributed to having a family history of diabetes. CONCLUSION: These findings confirm that family history of diabetes has a major public health impact on diabetes in the United States. In spite of the recent interest and focus on genomics and precision medicine, family health history continues to be an integral component of public health campaigns to identify persons at high risk for developing type 2 diabetes and early detection of diabetes to prevent or delay complications.

Changes in care coordination and health insurance in the population of US children with muscular dystrophy, 2005-2006 and 2009-2010.

INTRODUCTION: We aimed to assess changes in care coordination and health insurance coverage among US children with muscular dystrophy. METHODS: We used 2005-2006 and 2009-2010 data from the National Survey of Children with Special Health Care Needs. We examined the distribution of sociodemographic and health characteristics of children with muscular dystrophy by survey cycle. Multivariable regression was used to calculate odds of not receiving effective care coordination, not having adequate health insurance coverage, receiving no help coordinating care, and having problems obtaining referrals in each survey cycle. RESULTS: In the 2005-2006 and 2009-2010 survey cycles, there were 135 and 117 children with muscular dystrophy (representing 34,672 and 31,169 US children with muscular dystrophy), respectively. The percentage of children with muscular dystrophy who did not receive effective care coordination changed from 59.2% (95% confidence interval (CI), 45.6%-72.7%) in 2005-2006 to 53.4% (95% CI, 38.3%-68.6%) in 2009-2010. The odds of not receiving effective care coordination (adjusted odds ratio (aOR) = 0.77; 95% CI, 0.32-1.89) or having problems obtaining referrals (aOR = 0.52; 95% CI, 0.17-1.59) did not change significantly between the two periods, whereas odds of having inadequate insurance coverage decreased significantly (aOR = 0.41, 95% CI, 0.18-0.93) and odds of not receiving help coordinating care increased significantly (aOR = 4.22, 95% CI, 1.24-14.29) between the two periods. CONCLUSION: Our results suggest key health care needs for many families with children with muscular dystrophy have remained unmet for a prolonged period. Although there were significant improvements in health insurance coverage, nearly one-third of children with muscular dystrophy still had inadequate health insurance coverage in 2009-2010; it is likely that this situation has not changed much since then.

Bladder Reconstruction Rates Differ among Centers Participating in National Spina Bifida Patient Registry.

PURPOSE: We performed an exploratory analysis of data from the NSBPR (National Spina Bifida Patient Registry) to assess variation in the frequency of bladder reconstruction surgeries among NSBPR centers. MATERIALS AND METHODS: We queried the 2009-2014 NSBPR to identify patients who had ever undergone bladder reconstruction surgeries. We evaluated demographic characteristics, spina bifida 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 spina bifida enrolled in the NSBPR. Of these patients 1,129 (20.4%) underwent bladder reconstruction (703 augmentation, 382 continent catheterizable channel, 189 bladder outlet procedure). Surgical patients were more likely older, female, nonHispanic white, with a higher lesion level, myelomeningocele diagnosis, nonambulators (all p <0.001) and nonprivately insured (p=0.018). Bladder reconstruction surgery rates varied among NSBPR centers (range 12.1% to 37.9%, p <0.001). After correcting for known confounders NSBPR center, spina bifida 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, nonclinical factors such as patient age, gender and treating center are also associated with the likelihood of an individual undergoing bladder reconstruction.

Longitudinal Study of Bladder Continence in Patients with Spina Bifida in the National Spina Bifida Patient Registry.

PURPOSE: Achieving bladder continence in individuals with spina bifida is a lifetime management goal. We investigated bladder continence status through time and factors associated with this status in patients with spina bifida. 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 spina bifida in a large, multi-institutional patient registry who accounted for 12,740 annual clinic visit records during the study period. At last followup mean age was 16.6 years, 22.4% of 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 greater in those who were older, were female, were nonHispanic white, had a nonmyelomeningocele diagnosis, had a lower level of lesion, had a higher mobility level and had private insurance. Continence surgery history and current management were also associated with continence independent of all other factors (adjusted OR 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 nonmyelomeningocele (adjusted 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 in individuals with spina bifida. The association between bladder management and continence is stronger in those with myelomeningocele.

Variation in bowel and bladder continence across US spina bifida programs: A descriptive study.

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.

Variation in surgical management of neurogenic bowel among centers participating in National Spina Bifida Patient Registry.

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.

Bowel management and continence in adults with spina bifida: Results from the National Spina Bifida Patient Registry 2009-15.

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.