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.

Variations in Low-Risk Cesarean Delivery Rates in the United States Using the Society for Maternal-Fetal Medicine Definition.

OBJECTIVE: To assess variations in low-risk cesarean delivery rates in the United States using the Society for Maternal-Fetal Medicine (SMFM) definition of low-risk for cesarean delivery and to identify factors associated with low-risk cesarean deliveries. METHODS: From hospital discharge data in the 2018 National Inpatient Sample and State Inpatient Databases, we identified deliveries that were low-risk for cesarean delivery using the SMFM definition based on the International Classification of Diseases, Tenth Revision, Clinical Modification codes. We estimated national low-risk cesarean delivery rates overall and by patient characteristics, clinically relevant conditions not included in the SMFM definition, and hospital characteristics based on the nationally representative sample of hospital discharges in the National Inpatient Sample. Multivariate logistic regressions were estimated for the national sample to identify factors associated with low-risk cesarean delivery. We reported low-risk cesarean delivery rates for 27 states and the District of Columbia based on the annual state data that represented the universe of hospital discharges from participating states in the State Inpatient Databases. RESULTS: Of an estimated 3,634,724 deliveries in the 2018 National Inpatient Sample, 2,484,874 low-risk deliveries met inclusion criteria. The national low-risk cesarean delivery rate in 2018 was 14.6% (95% CI 14.4-14.8%). The rates varied widely by state (range 8.9-18.6%). Nationally, maternal age older than 40 years, non-Hispanic Black or Asian race, private insurance as primary payer, admission on weekday, obesity, diabetes, or hypertension, large metropolitan residence, and hospitals of the South census region were associated with low-risk cesarean delivery. CONCLUSION: Approximately one in seven low-risk deliveries was by cesarean in 2018 in the United States using the SMFM definition and the low-risk cesarean delivery rates varied widely by state.

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.

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.

Evaluation of spina bifida transitional care practices in the United States.

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.

Healthcare expenditures for privately insured people with cystic fibrosis.

With improved survival and new therapies for people with cystic fibrosis (CF), updated information on medical care expenditures for those individuals is needed. We estimated medical care expenditures, including both insurance reimbursements and patient out-of-pocket expenses, for privately insured people with CF and investigated how those expenditures varied with certain complications of CF. From a private insurance claims database of people covered by health plans associated with large corporate employers, we identified people with CF who were currently receiving medical care for the disorder and characterized their medical expenditures during the period 2004-2006. We selected a matching group of people who did not have CF based on age, sex, and geographic area, and calculated incremental expenditures associated with CF. We also examined the effect of age and certain complications of CF on these expenditures. The annual medical care expenditure for a person with actively managed CF averaged $48,098 in 2006 dollars, which was 22 times higher than for a person without CF. This ratio is high relative to other chronic disorders. Outpatient prescription medications made up the largest component of total expenditures for people with CF (39%). Those who were recorded in claims data as having a liver or lung transplant, malnutrition, diabetes, or a chronic Pseudomonas aeruginosa pulmonary infection incurred much higher expenditures than people without these conditions. People with CF will incur high medical expenditures throughout their lifespan. These findings will assist in the development of economic evaluations of future CF screening and management initiatives.

Economic evaluation of a neural tube defect recurrence-prevention program.

BACKGROUND: Women with a pregnancy affected by a neural tube defect (NTD) are encouraged to take folic acid prior to a subsequent pregnancy, but it is unknown whether organized attempts to identify and counsel such women to prevent recurrent NTDs are cost effective. METHODS: Data from the South Carolina recurrence-prevention program for October 2001-September 2002 were analyzed between October 2002 and December 2003 to calculate costs. Cost-effectiveness modeling of the program during 1992-2006 was conducted during 2007. Results were calculated for three scenarios based on recurrence risk, supplement use, and the effectiveness of folic acid in preventing recurrences. For each scenario, quality-adjusted life years (QALYs) were calculated separately using prevented NTD-affected live births; prevented NTD-affected births (including fetal deaths); and all prevented NTD-affected pregnancies. RESULTS: The prevention program cost approximately $155,000 per year in 2003 dollars to protect 35 pregnancies and prevent approximately one NTD. The direct costs associated with an NTD depend on type and outcome, but are approximately $560,000 in 2003 dollars for a live birth with spina bifida. The base-case cost-effectiveness ratio was $39,600 per QALY gained from avoided NTD-affected live births and stillbirths, and $14,700 per QALY gained from the avoidance of all NTD-affected pregnancies. The baseline NTD recurrence risk and the use of folic acid supplements by women who are at high risk for an NTD-affected pregnancy were influential parameters. CONCLUSIONS: The South Carolina NTD recurrence-prevention program appears comparable in cost effectiveness to other preventive services. Other states might consider including NTD recurrence prevention in birth defect-prevention programs.