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This paper describes the development and characteristics of a multi-disciplinary spina bifida clinic in Qatar considering the recently revised and globally available Guidelines for the Care of People with Spina Bifida (GCPSB).A retrospective chart review was performed on individuals in Sidra’s multidisciplinary spina bifida clinic database from January 2019 to June 2020. Their electronic health records were reviewed for demographics, as well as neurosurgical, urologic, rehabilitation, and orthopedic interventions.There were 127 patients in the database; 117 met inclusion criteria for diagnoses of myelomeningocele, meningocele, sacral agenesis/caudal regression, and/or spinal lipoma. Generally, Qatar is following GCPSB recommendations for multidisciplinary care. Consanguineous relationships, difficulties with access to urological and rehabilitation supplies and equipment, school access, and variable timing of neurosurgical closure were areas that demonstrated differences from GCPSB recommendations due to barriers in implementation.The GCPSB recommendations are applicable in an international setting such as Qatar. Despite a few barriers in implementing some of the recommendations, this new multi-disciplinary spina bifida clinic demonstrates alignment with many of the GCPSB guidelines. [ABSTRACT FROM AUTHOR]

BACKGROUND: Social isolation during the COVID-19 pandemic had a harmful impact on the psychological and physical health of children and teenagers. It is known that interruptions in rehabilitation can cause soft tissue contractures, bone deformities and a decline in motor functions among other complications. OBJECTIVE: The aim of this study was to compare the quality of life and physical activity levels of physically disabled children who continued and did not continue rehabilitation during the COVID-19 pandemic. METHOD: The gross motor levels of 18 children who continued special education and rehabilitation during the COVID-19 pandemic and 18 children who did not continue were determined with the Gross Motor Function Classification System (GMFCS). The International Physical Activity Questionnaire Short Form (IPAQ) and Children's Quality of Life Scale (PedsQL) questionnaires were administered. RESULTS: The study participants comprised 54.1% females and 45.9% males with a mean age of 9.02 years. No significant differences were detected between the two groups in respect of demographic, clinical and functional characteristics (p > 0.05). The walking parameters of PedsQL (p = 0.02) and IPAQ-SF scores (p = 0.03) were determined to be statistically significantly better in the group that continued rehabilitation. CONCLUSION: The results of this study demonstrated that the quality of life and walking capacity of children who continued rehabilitation during the COVID-19 pandemic were better. Methods should be developed to ensure that rehabilitation is not interrupted during isolation periods of any future pandemic. [ABSTRACT FROM AUTHOR]

PURPOSE: Mothers who have had a pregnancy affected by spina bifida are advised to take 4-5 mg/day folic acid pills to prevent recurrence. The folic acid prescription pattern was examined for high-risk mothers whose children received spina bifida surgery in an urban Ethiopian hospital. METHODS: Data were analyzed from a large Ethiopian urban tertiary care hospital that provided spina bifida care. General practitioners recorded 5 mg/day folic acid prescriptions administered to mothers of infants with spina bifida born between January 2019 and June 2022. RESULTS: Among 500 baby-mother pairs, 340 (68%) received a 120-day prescription for 5 mg/day folic acid pills. Of these 340 mothers, 331 (97%) received their folic acid prescription at their child's first or second patient encounter. Almost all mothers (94%) had documentation of only one prescription for the study duration. The percentage of mothers receiving at least one prescription varied by the baby's year of birth (2019:75%; 2020:92%; 2021:46%; Jan 2022 –June 2022:79%). CONCLUSION: This prioritization of spina bifida recurrence prevention demonstrates feasibility for other healthcare centers in low-income countries. Sustained funds to provide women with free folic acid pills can favor high compliance and uptake of this prevention intervention. [ABSTRACT FROM AUTHOR]

PURPOSE: This article provides an update to the 2018 Spina Bifida Association's Transition to Adult Care Guidelines. METHODS: A workgroup of topic experts was convened including authors from the initial guideline workgroup. The workgroup reviewed and updated the primary, secondary, and tertiary outcome goals, clinical questions, and guideline recommendations based on a literature review. RESULTS: Twenty-two additional articles were identified from the literature search. Updated references included observational studies describing transition to adult care outcomes, transition care model initiatives, and a validated self-management assessment tool. CONCLUSION: Structured transition initiatives increase the likelihood of establishing with adult care, decrease acute care use for young adults with spina bifida, and have the potential to improve quality of life and optimize chronic condition management. However, there is still a need to implement structure transition practices more broadly for this population using these recommended guidelines. [ABSTRACT FROM AUTHOR]

PURPOSE: This project aimed to launch an international learning community to guide the development of a spina bifida (SB) curriculum for global health trainees. METHODS: Using a descriptive study design, a convenience sample of SB curricula were identified in 2022–23 by members of the Spina Bifida World Congress Outreach Committee and evaluated during a series of monthly Zoom calls to discuss SB education in a global health context. Participants included (1) leadership from the ReachAnother Foundation, (2) invited panelists from the Spina Bifida World Congress Global Health Symposium, and (3) global health students and preceptors. Education initiatives in Ethiopia, Sweden, Argentina, Ecuador, and the United States were evaluated vis-à-vis format and content. RESULTS: All of the education initiatives referenced the framework of the World Health Organization International Classification of Functioning, Disability and Health. Formats varied and included both virtual and interactive workshops, print materials, videos, and guides for small group discussion. Content addressed four domains: Folate Prevention, Neurosurgical Training, After Care, and Data Collection. A multidisciplinary approach, partnerships with families, and workforce pipeline training were identified as guiding themes for educating the next generation of SB researchers and clinicians in global health settings. CONCLUSION: The Spina Bifida Global Learning Collaborative is a transnational group of advocates, clinicians, and investigators whose mission is the advancement of SB-related global health education. Lessons learned from the collaborative are being leveraged to develop a global health curriculum for learners, which may improve services for individuals with SB around the globe. [ABSTRACT FROM AUTHOR]

PURPOSE: The purpose of the study was to determine if the use of continuous temperature monitoring smart socks is feasible in adolescents with spina bifida (SB) by obtaining user feedback on comfort. METHODS: Participants were provided temperature monitoring socks and a 4 G hub. Follow-up phone calls were performed to answer questions or discuss barriers. Sock temperatures were monitored throughout four weeks. Following sock wear, participants were asked to complete a satisfaction survey. RESULTS: Seventeen of the 33 participants enrolled (eight males, nine females) with a mean age of 14.8 years completed the study. Average sock wear was 8.0 hours per day for four weeks. The mean temperature was 83.4°F with a mean temperature differential between feet of –0.74°F (left–right). The duration of sock wear varied from 14.6 to 595.9 hours over the four-week period. Lastly, eleven participants (84.6%) reported that the socks were comfortable and very easy to put on. CONCLUSION: Continuous temperature monitoring smart socks are an option for monitoring risk for developing pressure injuries in adolescents with SB. Future directions would include increasing sample size, obtaining normative data for temperature ranges in this population, and correlating to clinical outcomes. [ABSTRACT FROM AUTHOR]

PURPOSE: This pilot study aimed to determine the parent/caregiver's role in nutrition/eating habits, physical activity behaviors, and food access among children diagnosed with spina bifida (SB). METHODS: Parents/caregivers of children with SB were asked to participate at a single, outpatient SB clinic. Demographic, biomedical data, parent/caregiver nutrition knowledge, family nutrition and physical activity (FNPA), and food security survey scores were compared. Descriptive, regression, and correlational statistics were conducted for analysis via SPSS 29. RESULTS: Of the 117 parents/caregivers surveyed, completed data suggested most were overweight/obese (average body mass index [BMI] of 30.63 kg/m2±8.40; n = 99) with an average nutrition knowledge score of 71% (17.83±3.33). As FNPA scores decreased, the patient/child's maximum BMI z scores increased (β= –0.043; confidence interval –0.079, –0.007; p = 0.020), suggesting the less active and/or less healthy eating habits, the higher body mass was noted for the child. Forty four percent of children (n = 99) were in the overweight/obese weight range based on maximum BMI z score. CONCLUSION: These findings suggest there is a need for parental/caregiver nutrition education to assist children with SB with meal and activity planning to achieve optimal health. [ABSTRACT FROM AUTHOR]

PURPOSE: This study aimed to describe outcomes of motor function with a special focus on ambulation ability at 36 months among children with open prenatal repair of spina bifida aperta (SB). METHODS: A prospective cohort study was conducted including 87 patients with open prenatal repair of SB at the investigating center born between 2010 and 2018. Anatomic lesion level and motor function level in the neonatal period, as well as motor function level, ambulation status, and use of orthotics and assistive devices at 36 months were assessed. RESULTS: At 36 months, ambulation was assessed in 86 children; of those, 86% (n = 74) were ambulating. Independent of ambulation, orthotics were worn in 81.6% (71/87) and assistive devices in 47.1% (41/87). Children with a lower lumbar or sacral motor function level were the first to reach independent ambulation and were more likely to ambulate at 36 months than children with higher motor function levels (p = <.001). The anatomic lesion level determined on the neonatal MRI correlated with ambulation status at 36 months (p = < 0.001). CONCLUSION: At 36 months, most children with open prenatal repair for SB showed favourable ambulation status. However, most still used assistive devices or orthotics. Anatomic lesion level on neonatal MRI, motor function level during the neonatal period, and motor function level at 36 months were associated with ambulation status at 36 months. [ABSTRACT FROM AUTHOR]

PURPOSE: Pediatric patients with spina bifida often experience neurogenic bowel dysfunction. Although cecostomy tubes could improve bowel continence, their effectiveness is not well established in this population. The aims of this study were to better understand the effectiveness of cecostomy tubes relative to other management strategies (between-subject) and to explore their effectiveness among patients who received these placements (within-subject). METHODS: Retrospective analysis of data from pediatric patients enrolled in a national spina bifida patient registry (n = 297) at a single multidisciplinary clinic was performed, covering visits between January 2014 –December 2021. Linear and ordinal mixed effect models (fixed and random effects) tested the influence of cecostomy status (no placement vs placement) and time (visits) on bowel continence while controlling for demographic and condition-specific covariates. RESULTS: Patients with cecostomy tubes had higher bowel continence compared to patients without placements (B = 0.695, 95% CI [0.333, 1.050]; AOR = 2.043, p =.007). Patients with cecostomy tubes had higher bowel continence after their placements compared to before (B = 0.834, 95% CI [0.142, 1.540]; AOR = 3.259, p = 0.011). CONCLUSION: Results indicate cecostomy tubes are effective for improving bowel continence in this pediatric population. Future research is needed to conduct risk analyses and determine the clinical significance of these effects. [ABSTRACT FROM AUTHOR]

PURPOSE: The purpose of this project was to establish a pathway for electronic medical record (EMR) customization, utilizing quality improvement methodology, to both identify and address adverse social determinants of health (SDOH) among a diverse spina bifida (SB) population. METHODS: Starting in September 2020, the four fundamental steps were to (1) facilitate an advisory committee to safeguard the standard clinical protocols, (2) characterize barriers to implementation, (3) evaluate workflow to sustain data entry capture, and (4) manage the technology platform for seamless integration. The SB clinic was the first clinic within the enterprise to rollout the use of an adverse SDOH mitigation activity. A Spanish-speaking interpreter was scheduled for all clinics, as many families were limited in English proficiency. RESULTS: The customization of the EMR to support an efficient workflow to address SDOH was feasible in a large and diverse urban medical center. Of the 758 patients served in the clinic, a myelomeningocele diagnosis was present in 86% of individuals. While 52% of participants were female, ethnically 52% of individuals served were Latino. Many of these individuals disclosed being recent immigrants to the United States. Often immigration and asylum related issues were at the forefront of the SDOH issues addressed. CONCLUSION: Given the occurrence of adverse SDOH among individuals with SB, many of whom are new Latin-American immigrants, meaningful clinical efforts are needed to both identify and address the causes of the observed disparities. EMR customization is feasible and can identify and, through social prescriptions, address SDOH to support the provision of safe, high quality, and equitable care for vulnerable and medically complex populations at home and potentially abroad. [ABSTRACT FROM AUTHOR]

PURPOSE: This study aimed to analyze organ system-based causes and non-organ system-based mechanisms of death (COD, MOD) in people with myelomeningocele (MMC), comparing urological to other COD. METHODS: A retrospective review was performed of 16 institutions in Canada/United States of non-random convenience sample of people with MMC (born > = 1972) using non-parametric statistics. RESULTS: Of 293 deaths (89% shunted hydrocephalus), 12% occurred in infancy, 35% in childhood, and 53% in adulthood (documented COD: 74%). For 261 shunted individuals, leading COD were neurological (21%) and pulmonary (17%), and leading MOD were infections (34%, including shunt infections: 4%) and non-infectious shunt malfunctions (14%). For 32 unshunted individuals, leading COD were pulmonary (34%) and cardiovascular (13%), and leading MOD were infections (38%) and non-infectious pulmonary (16%). COD and MOD varied by shunt status and age (p < = 0.04), not ambulation or birthyear (p > = 0.16). Urology-related deaths (urosepsis, renal failure, hematuria, bladder perforation/cancer: 10%) were more likely in females (p = 0.01), independent of age, shunt, or ambulatory status (p > = 0.40). COD/MOD were independent of bladder augmentation (p = >0.11). Unexplained deaths while asleep (4%) were independent of age, shunt status, and epilepsy (p >= 0.47). CONCLUSION: COD varied by shunt status. Leading MOD were infectious. Urology-related deaths (10%) were independent of shunt status; 26% of COD were unknown. Life-long multidisciplinary care and accurate mortality documentation are needed. [ABSTRACT FROM AUTHOR]

PURPOSE: The purpose of this study was to evaluate associations of ethnicity and adaptive function with health-related quality of life (HRQOL) in youths with spina bifida myelomeningocele (SBM). METHODS: Participants included Hispanic (n = 75) and non-Hispanic White (n = 86) children and adolescents with SBM. Participants completed ratings of adaptive function and SBM-specific HRQOL. A series of linear multiple regression models was computed to investigate whether ethnicity moderates the relation between adaptive function and HRQOL. RESULTS: Results showed that greater adaptive function was associated with higher HRQOL. While no relations were found between ethnicity and HRQOL, a significant interaction was observed between adaptive function and ethnicity over and above other terms. Although groups did not differ on HRQOL at lower levels of adaptive function, Hispanic participants rated higher HRQOL relative to non-Hispanic White participants at higher levels of adaptive function. Further analysis showed this was true of both nonmotor and motor aspects of adaptive function. CONCLUSION: Results suggested complex relations between ethnicity and adaptive function with HRQOL, highlighting the importance of optimizing adaptive functioning in youth with SBM. [ABSTRACT FROM AUTHOR]

PURPOSE: This study aimed to conduct a national survey of individuals with spina bifida (SB) and their care partners to assess COVID-19 vaccination behaviors and vaccine uptake. METHODS: A survey instrument was designed to assess current vaccination status, general perceptions towards vaccinations, and barriers to vaccination within the SB community. Surveys were administered to individuals with SB or their representing care partner. Chi-squared and independent-samples t-tests were used to analyze the relationship between vaccine uptake and demographics. Multivariable logistic regression modeling was used to test which predictors impacted the odds that a participant received a COVID vaccine. RESULTS: A total of 1,412 participants completed the questionnaire, and 1,145 participants reported their COVID-19 vaccine status. The most common reason for not getting vaccinated was a concern about vaccine safety and efficacy. Overall, healthcare professional recommendations played a significant (OR 2.77 p < 0.001) role in whether to get vaccinated. CONCLUSION: About one in five individuals with SB have not received any COVID-19 vaccine. Actionable and modifiable factors were identified which may help increase vaccine uptake. Importantly, health providers play a critical role in COVID-19 vaccination messaging and should emphasize vaccine safety and efficacy. [ABSTRACT FROM AUTHOR]

PURPOSE: Functional level of lesion (FLOL) is a grading of the level of neurological function in patients with myelomeningocele and other forms of spina bifida. It has been widely used as an independent variable in studies of spina bifida, but its inter-rater reliability has not previously been tested. The purpose of this study was to measure inter-rater reliability of FLOL testing and compare testing performed by a non-medically trained research associate to testing performed by a pediatric rehabilitation medicine specialist. METHODS: Children in a multi-disciplinary spina bifida clinic underwent FLOL grading by a non-medically trained research associate. On the same day, these children were also graded by a pediatric rehabilitation medicine specialist. Cohen's weighted kappa statistic was used to compare grading, with the rehabilitation medicine specialist considered the gold standard. RESULTS: A total of 71 patients participated. FLOL was graded for left and right leg for each participant, resulting in 142 measurements. Cohen's weighted kappa was κ= 0.809, with a standard error of 0.034 and 95% confidence interval 0.723–0.875, indicating substantial agreement. CONCLUSION: FLOL as measured according to the instructions of the National Spina Bifida Patient Registry by a non-medically trained researcher is a reliable method to grade lower extremity function in spina bifida. [ABSTRACT FROM AUTHOR]

PURPOSE: Previous studies have found motor function to correlate with spinal motor level and, accordingly, individuals with spina bifida are frequently categorized clinically in this manner. The aim of the current study was to describe how lower extremity functions including strength, selective motor control, and mirror movements vary by motor level in children and young adults with spina bifida. METHODS: A single center, retrospective, cross-sectional, descriptive study using data collected in the National Spina Bifida Patient Registry and by a gait laboratory was performed. RESULTS: Seventy-seven individuals with spina bifida were included with the majority having myelomeningocele (59 lumbar, 18 sacral motor level). Lower extremity strength and selective motor control varied to a certain extent with motor level. However, 90% of individuals showed strength or weakness in at least one muscle group that was unexpected based on their motor level. Mirror movements did not clearly vary with motor level. CONCLUSION: Lower extremity strength, selective motor control, and mirror movements in individuals with spina bifida were not entirely predicted by motor level. This highlights the possible need for an improved spina bifida classification system that describes not only spinal motor level but more clearly defines a particular individual's functional motor abilities. [ABSTRACT FROM AUTHOR]

PURPOSE: This study aimed to describe health care use by type of health providers and care settings visited by children with spina bifida (SB) and to compare this use between children with and without a shunt. METHODS: Health care use data were extracted from a larger study on the health and functioning of children with SB aged 3–6 years. The present study focused on the medical information subsection of a parent-reported survey related to SB care, general care, specialty care (e.g., neurosurgery), emergency care, and complications related to SB and shunts. RESULTS: Parents of 101 children with SB participated. Most of the children were male with myelomeningocele and had a shunt. They visited a health care provider for SB care an average of 7.4 times and a specialist an average of 11.9 times in the previous 12 months. Most visited a multidisciplinary clinic for SB-related care and a private physician's office for general care. Children with a shunt had more SB-related medical visits, more visits to a specialist, and a greater number of different types of specialists than those without it. Frequency of emergency room visits did not differ between the two groups. Health providers informed parents about headaches, vomiting, and fever as signs of complications, and some parents did report shunt-related complications. CONCLUSION: SB is a complex medical condition requiring that children receive medical care from various medical specialists, especially for children with a shunt. Findings on health care use suggest high levels of monitoring and care coordination that parents navigate to care for their child. [ABSTRACT FROM AUTHOR]

PURPOSE: Sleep related breathing disorders (SRBD) are seen at disproportionately higher rates in children with spina bifida compared with their same aged peers. SRBD such as obstructive sleep apnea (OSA) or central apnea are associated with developmental and cognitive consequences, and sudden death. METHODS: Participants aged 1 to 20 years with a diagnosis of spina bifida were recruited from a pediatric academic hospital spinal cord specialty clinic to evaluate the prevalence and impact of sleep disorders in the spina bifida population; 76 polysomnogram (PSG) reports spanning a 3-year period were reviewed in this retrospective cohort study. RESULTS: Of the PSGs reviewed, 37 (49%) indicated the presence of SRBD, and 28 (76%) of those children required an escalation of management (surgical intervention or additional respiratory support). These results are consistent with previous studies and further emphasize the clinical impact of SRBD on children with spina bifida by describing the interventions that followed an abnormal PSG. CONCLUSION: The high prevalence of SRBD in the spina bifida population supports the need for additional research to develop sleep questionnaires specific to spina bifida that can predict abnormal PSG clinically and to determine the standard of care following an abnormal PSG, chiefly in OSA and central apnea. [ABSTRACT FROM AUTHOR]

PURPOSE: The purpose of this exploratory study was to investigate the types of academic and health-related accommodations provided to adolescents and emerging adults with spina bifida aged 9-20 years. METHODS: Data were extracted from the paper and electronic records of transition-age youth enrolled in the study. Four open ended items involved content analysis. RESULTS: The most frequently identified accommodation was enrollment in special education classes in 47.7% of the charts. Other academic accommodations that were most often reported were adaptive physical education (n = 71, 39.9%), tutoring (n = 28; 15.7%), and home schooling (n = 21; 11.8%). Clean intermittent catheterization was the most frequently identified health-related accommodation provided by the school nurse/aide (n = 57; 32%).The largest percentage of requests for additional accommodations were made during the middle school grades (15; 54.8%) followed by high school (10; 32.2%). CONCLUSION: Findings demonstrated that persistent issues were identified by parents/adolescents regarding the provision of school-related accommodations. This is a relevant area for clinical practice to ensure students with special health care needs and those with spina bifida receive the academic and health-related accommodations in their Individualized Education Program/504 plans. [ABSTRACT FROM AUTHOR]

PURPOSE: This study aimed to describe brace use, progression of scoliosis, and surgery in children with cerebral palsy (CP) and spina bifida (SB) who were initially treated with a brace. METHODS: Medical records were retrospectively analysed for brace compliance, treatment complications, curve size measurements with and without the brace at both start and at follow-up, and number of patients undergoing spine surgery. RESULTS: Sixty-eight children were included (CP 47, SB 21), 37 of whom were girls, with a mean age at start of treatment of 11.1 (CP) and 8.2 (SB) years. Most had severe motor problems; only four children with CP and five with SB were able to walk. Thirty-five in the group with CP and 11 in the group with SB had a curve size over 40°. Forty used the brace full-time, 19 half-time and nine for a varying proportion of time. Transient complications of brace treatment were seen in 28%. The yearly progression of curve-size was 4.2° in CP and 2.3° in SB. Twenty-eight patients underwent surgery and complications were present in 75% of these patients. Twenty-seven out of 46 patients with severe scoliosis did not undergo surgery. CONCLUSION: Brace treatment was possible, even in patients with severe scoliosis. Bracing can delay the progression of scoliosis. [ABSTRACT FROM AUTHOR]

PURPOSE: The purpose of this study was to assess if the incidence of lower extremity (LE) pressure injuries resulting in amputations was more frequent in ambulatory versus non-ambulatory patients with spina bifida. METHODS: The medical records of all patients at a large pediatric rehabilitation hospital who had spina bifida and a history of a pressure injury between 1997–2018 were retrospectively reviewed. RESULTS: In this study, 112 patients with spina bifida developed LE pressure injuries, 53 patients walked with braces and/or an assistive device, and 59 patients used a wheelchair for mobility. Of the 11 patients who had amputations, 10 walked and 1 did not walk prior to their amputation. Ambulatory patients with LE pressure injuries had a 19% chance (10 out of 53 patients) of requiring an amputation. Non-ambulatory patients with a LE pressure injury had only a 2% chance (1 out of 59 patients) of requiring an amputation. Ambulatory patients with LE pressure injuries were at a much higher risk of requiring an amputation than patients who did not walk (p-value of < 0.005). CONCLUSION: Patients with spina bifida who are ambulatory and develop a pressure injury have a high risk of ultimately needing an amputation and should be monitored and treated aggressively. [ABSTRACT FROM AUTHOR]

Selective dorsal rhizotomy (SDR) was used to treat lower extremity spasticity in an ambulatory 7-year-old girl with a history of a T10 spinal lipoma. The spasticity was the result of an AIS D spinal cord injury (SCI) suffered during untethering surgery at age 2 years. After SDR and a course of intensive inpatient rehabilitation, the patient's gait improved markedly. To the authors' knowledge, this is the first published case of SDR as a treatment for spasticity in a patient with a spinal lipomatous malformation. [ABSTRACT FROM AUTHOR]

Whereas legislation mandates for folic acid fortification have been implemented throughout many nations, divergent neural tube defects (NTDs) prevalence rates still remain among the world's populations. In North America, the prevalence estimate is 39 infants per 100,000 live births. Open spina bifida (SB), also known as myelomeningocele, remains the most complex congenital abnormality of the central nervous system compatible with long term survival; this recognized complexity gives rise to emerging comorbidities and interventions. For example, increasing autism spectrum disorder rates have been reported among individuals with SB utilizing a 31,220 subject population-based birth cohort. Along with new clinical observations, telecommunication platforms such as Zoom® have evolved as clinical and investigational tools. Historically, society meetings, research conferences, and journals have provided opportunities for professional development and dissemination of up-to-date materials. The Journal of Pediatric Rehabilitation Medicine (JPRM) has arisen as an open-access global platform for the dissemination of SB-related inquiry. The journal has also highlighted the research presented at the Spina Bifida Association's previous Spina Bifida World Congresses. At the last congress, which was held in 2017, twenty-three countries were represented; this number is expected to grow by the next convocation in 2023. This congress will provide an opportunity for health care professionals from around the globe to present a broad array of research topics and build collaborations. Concurrently, the JPRM will continue as an open-access platform for SB advocacy, care, education, and investigation, across our fast changing world for the international SB community well into the future. [ABSTRACT FROM AUTHOR]

PURPOSE: The purpose of this longitudinal study was to evaluate the internal consistency reliability and construct validity of the Adolescent/Young Adult Self-Management and Independence Scale-II (AMIS-II), an interview-based measure of self-management for youth with chronic health conditions. METHODS: A diverse sample of adolescents and young adults (AYA) with spina bifida (SB) (n = 64 AYA; mean 20.88; age range 18–25 years) completed an AMIS-II interview. Six years earlier, parents from 55 families completed questionnaires that assessed children's responsibility for SB-related care (Sharing of Spina Bifida Management Responsibilities) and their ability to perform skills across condition-related tasks (The Spina Bifida Independence Survey). Parents also reported on child's communication skills, adaptive behaviors, and independent management of finances (Adaptive Behavior Assessment System-Second Edition; Scales of Independent Behavior-Revised). Descriptive and correlational analyses were conducted to assess the construct validity and the internal consistency reliability of the AMIS-II. RESULTS: The AMIS-II demonstrated excellent internal consistency reliability (AMIS-II total scale α= 0.95; subscales α= 0.90 –0.91). Evidence in support of construct validity was found in associations between the AMIS-II and measures of child responsibility for SB-related care, ability to perform condition-related skills, and adaptive behaviors (r's = 0.378 –0.777; p's < 0.05). CONCLUSION: This study provides further evidence of strong reliability and validity for the AMIS-II. Additional research with this measure will facilitate a better understanding of factors related to self-management behaviors in adolescents and young adults with spina bifida. [ABSTRACT FROM AUTHOR]

The COVID-19 pandemic has posed distinctive challenges to adolescents and young adults living with spina bifida, especially those from ethic minority populations. With this public health challenge in mind, developing a customized electronic health record to leverage registry data to promote and quantify COVID-19 vaccination uptake among this population is feasible. We provide a brief description of our activities in customizing an electronic health record to track vaccination uptake among adolescents and young adults with spina bifida (AYASB); and the lessons learned, in hopeful support of those scaling-up vaccination delivery across the globe for AYASB as they transition to adult-centered care. Thus, as providers think globally and act locally, COVID-19 immunization efforts can be implemented while providing culturally appropriate transition policies and services for individuals with neurodevelopmental disabilities. [ABSTRACT FROM AUTHOR]

PURPOSE: Post-operative complication rates may vary among racial and/or ethnic groups and have not been previously described in individuals with spina bifida (SB) undergoing urologic surgery. The aim of this study was to compare in-hospital complication frequencies of individuals with SB following urologic surgery by race/ethnicity. METHODS: The Nationwide Inpatient Sample was used to identify pediatric patients with SB who underwent inpatient urologic procedures. A pediatric cohort (<18 years old) with SB that underwent urologic surgery were assessed. All analyses report weighted descriptive statistics, outcomes, and race/ethnicity was the primary predictor variable. The primary outcome of interest was post-operative complications which were defined using NSQIP ICD-9 code definitions. Secondary analysis included length of stay (LOS), and encounter cost was estimated using the cost-to-charge ratio files provided by the Healthcare Cost and Utilization Project. RESULTS: The unadjusted model showed no differences in complications, LOS, and cost. In the adjusted model there were no differences in complications, LOS, and cost between Black and White encounters. However, Hispanic ethnicity was associated with a 20%(95%CI: 4–40%) increase in LOS and 18%(95%CI: 2–35%, p = 0.02) increase in cost compared to White encounters. CONCLUSION: There was no evidence of variation for in-hospital complication rates among racial/ethnic groups undergoing urologic surgery. Hispanic ethnicity was associated with higher costs and longer LOS in pediatric SB encounters. [ABSTRACT FROM AUTHOR]

PURPOSE: To identify the accuracy of Body Mass Index (BMI) to categorize body weight in a sample of children with spina bifida and Down syndrome as compared to typically developing peers. METHODS: A secondary analysis of 32 children with spina bifida, Down syndrome or no chronic illness. A calculated BMI was plotted on the Centers for Disease Control and Prevention age- and sex-specific BMI growth charts to determine each child's weight status. Percentage of body fat, obtained by labeled water, was plotted on two different body fat percentile reference curves, one derived from a whole body measure (DXA) of body fat and one by skin-fold measure. Differences in weight categories between calculated BMI and body fat percentile curves were reported. RESULTS: The calculated BMI for children with a disability had significant misclassifications as a screening tool for body fat when compared to children without a disability. Misclassifications were increased with the body fat percentile reference curve derived from skin-fold measures and for children who primarily used a wheelchair. CONCLUSION: The current recommendation to use BMI to categorize weight status is not useful for many children with disabilities. Further research to identify an alternative pragmatic strategy is necessary. [ABSTRACT FROM AUTHOR]

PURPOSE: The objective of this study was to describe the knowledge, attitudes, and practices related to caring for youth with SB within a statewide pediatric primary care medical home network. METHODS: Health care professionals who were members of the Pennsylvania Medical Home Program (PAMHP) statewide network were surveyed on sociological demographics, confidence in and knowledge of topics that impact the spina bifida (SB) population, education on SB, designating responsibility of care, tasks completed in the office, and transition from pediatric to adult care from October 2016 through November 2016. RESULTS: Among 67 respondents, 78%believed counseling families/caregivers about the impact of SB on everyday life was a shared responsibility between primary care physicians (PCP) and SB clinics. Eighty five percent of participants indicated they had exposure to SB through clinical courses or didactics in training but only 54%of participants felt knowledgeable on the impact of SB on everyday life. CONCLUSION: Findings suggest a need for increased educational efforts, improved awareness of SB, and access to evidence-based guidelines for PCPs to promote accessible and coordinated care to individuals with SB. [ABSTRACT FROM AUTHOR]

PURPOSE: Children with myelomeningocele are known to be consumers of substantial healthcare resources, with many early hospital encounters. The purpose of this study was to survey the extent of medical and surgical care that patients with myelomeningocele receive during the first four years of life. METHODS: Clinical and demographic data were collected on newborn infants with open myelomeningocele from the Children's of Alabama Spina Bifida Web Tracker, a prospective, comprehensive spina bifida database. Additional data pertaining to all hospital admissions, surgical procedures, and clinic visits were collected from the medical record. RESULTS: One hundred and fourteen subjects with a primary diagnosis of myelomeningocele between 2004 and 2015 were included. Males slightly predominated (55%), 61.4% were Caucasian, 11% Hispanic/Latino; 28% had a mid-lumbar functional lesion level. Over the first four years of life, 688 total surgical procedures were performed (an average of 86 per child): 438 in year 1, 100 in year 2, 84 in year 3, and 66 in year 4. The mean number of hospital visits was 40.5. Total average drive time per patient over 4 years being 103.8 hours. Average number of nights spent in the hospital was 51. CONCLUSION: Children with myelomeningocele need multiple hospitalizations, surgeries, and medical encounters in the first 4 years of life. These data will be valuable when counselling new parents and prospective parents of children with this condition. [ABSTRACT FROM AUTHOR]

PURPOSE: Neurogenic bowel dysfunction (NBD) affects 80% of individuals with spina bifida. Performing and disseminating research on NBD to reach the appropriate audience is difficult given the variability among medical specialties managing NBD. This study aimed to identify which medical specialties and types of providers are currently managing NBD in the United States. METHODS: A survey was developed and sent to 75 spina bifida clinics. Surveys queried which specialty was primarily responsible for medical and surgical management of NBD and any others that assist in NBD care. The license and certification level of the providers were collected. Descriptive statistics were performed to describe the results. RESULTS: Response rate was 68%. Urology was the leading specialty primarily responsible for NBD management (39%) followed by rehabilitation medicine and developmental pediatrics (22% and 20%, respectively). Physicians were the primary providers of care followed by nurse practitioners (54% vs 31%). Urology performs 65% of NBD surgeries. CONCLUSION: Multiple specialties and providers are involved in NBD management with variation among clinics. Development of improved NBD care should include a spectrum of specialties and providers. Dissemination of research should be aimed at multiple specialty groups. [ABSTRACT FROM AUTHOR]

PURPOSE: The objective of this study was to analyze the effects on patient access by decreasing missed appointments after hiring a clinic coordinator using medical informatics. METHODS: A single-center retrospective analysis of the rates of missed appointments before and after hiring a clinic coordinator in a multidisciplinary spinal differences clinic were analyzed using a commercially available business software system (SAP® Business Objects). The total number of clinic visits was collected for each month to determine the access available for patients. RESULTS: The median number of missed appointments per clinic by month before employing the clinic coordinator was higher than in the two years following implementation (p < 0.0005). No differences were seen in the number of available appointment slots per month indicating no new clinics were needed to improve patient access (p = 0.551). Projected billing amounts prior to hiring the clinic coordinator indicated that $91,520 was lost in the 2 years prior to hiring this coordinator compared to $30,160 lost during the 2 years following the creation of this position (p = 0.0009). CONCLUSION: Hiring a clinic coordinator decreased the rate of missed appointments and was a cost-efficient intervention to improve patient access and provide effective patient care in a multidisciplinary setting. [ABSTRACT FROM AUTHOR]

PURPOSE: To determine characteristics associated with self-management independence and quality of life (QOL) among adolescents and young adults with spina bifida (AYASB) engaged in a spina bifida (SB) health care transition clinic. METHODS: During SB transition visits, Eighty-eight AYASB ages 14-20 completed the Adolescent/Young Adult Self-management and Independence Scale II Self-Report/SB (AMIS II-SR/SB), scores ranging from 1–7 with 7 indicating full independence in activities, and the QUAlity of Life Assessment in Spina bifida for Teens (QUALAS-T), which has two subscales, family/independence (QFI) and bowel/bladder (QBB), with scores ranging from 0-100 with 100 indicating maximal QOL score. Demographic and clinical variables were collected from the electronic medical record. RESULTS: The baseline AMIS II-SR/SB score was 3.3 (SD 1.0). Baseline scores for QUALAS-T QFI and QBB subscales were 73.8 (SD 19.9) and 63.8 (SD 25.8). Older age was associated with a higher baseline AMIS II-SR/SB score (p = 0.017). Over time, AMIS II-SR/SB total significantly improved (p < 0.001), but QFI and QBB did not. AYASB not on chronic intermittent catheterization (CIC) and those using urethral CIC significantly improved in AMIS II-SR/SB total scores (p = 0.001), but those using abdominal channel CIC did not. CONCLUSION: Baseline bladder management method was associated with self-management improvement for AYASB engaged in a SB-specific transition clinic. [ABSTRACT FROM AUTHOR]

PURPOSE: To characterize common clinical indications for urodynamic, a bladder function test, in adults with spina bifida. METHODS: A retrospective chart review was performed for 215 patients seen in an adult multidisciplinary spina bifida clinic who were registered with the National Spina Bifida Patient Registry from October 2011 to October 2018. Descriptive statistics were used for statistical comparisons. RESULTS: A total of 52 of 215 patients developed a clinical indication for urodynamics. Of these, 71 (33%) patients (8 of whom underwent testing twice) had urodynamics performed, resulting in a total of 79 urodynamic study encounters that were analyzed. Thirty-four (43%) urodynamic testing cases were performed due to a symptomatic change in lower urinary tract function; 14 (18%) were due to declining renal function or concern for upper tract deterioration based on imaging. The data obtained from urodynamic investigation led to new recommendations for urinary tract management in 59 (75%) of the urodynamic studies performed. A total of 32 of the 90 (35%) recommendations made were surgical interventions and 30 (33%) were for a change in medical management. Interestingly, 8 of the 18 (44%) routine or baseline urodynamic tests performed led to new recommendations in urinary tract management. CONCLUSION: A total of 24%of patients in the multidisciplinary spina bifida clinic developed an indication for urodynamic testing over a 7-year period which resulted in new recommendations for urinary tract management in most. As more patients with spina bifida enter adulthood, the indications for urodynamic evaluation may become more defined, since the results often lead to alterations in bladder management. [ABSTRACT FROM AUTHOR]

PURPOSE: Despite an increasing number of individuals with spina bifida reaching reproductive age, there has been a paucity of research into their reproductive health care needs. The objective of this study was to better understand the reproductive health experiences of self-identified women with spina bifida using qualitative methodology. METHODS: A phenomenological study design was used to address this objective. Women with spina bifida identified their interest in participating in a semi-structured interview after completing an online reproductive health survey. Interviews were recorded and transcribed verbatim. Qualitative analysis followed a phenomenological approach using Dedoose software. RESULTS: Twelve self-identified women with spina bifida participated. They described experiences in four domains: sexual education, pregnancy, labor and delivery, and postpartum. In addition, an intersecting domain of social justice and advocacy emerged. Numerous themes are described, including a lack of tailored sexual health information, impact of pregnancy on function, attitudes towards delivery method, and parenting challenges. CONCLUSION: This study explored the continuum of reproductive health experiences of women with spina bifida. They face unique reproductive health challenges that provide an opportunity for health care providers to offer more holistic care. [ABSTRACT FROM AUTHOR]

PURPOSE: To assess hand dexterity in children with myelomeningocele (MMC) and to explore factors related to hand dexterity in these children. METHODS: Ninety-four children with myelomeningocele, aged 4 to 18 years, were assessed. Demographic characteristics, disease factors, visual perception (Beery test of Visual Motor Integration), cognition (WeeFunctional Independence Measure), and self-care (Pediatric Evaluation of Disability Inventory) were assessed in relation to the Nine-Hole Peg Test (9HPT) for hand dexterity using Spearmen correlations and linear regressions. RESULTS: The children's performance on the 9HPT in both hands was significantly slower than the norms for their age groups. Children without a shunt showed significantly better function in both hands (p =.005) than those with a shunt. Factors most related to hand dexterity were neurological spinal level of MMC, presence of shunt, age, cognitive ability, and years of mother's education. CONCLUSION: Children with MMC appear to have poorer hand skills than typically developed children, which was related to pathology as well as functional and environmental factors. When addressing hand dexterity in children with MMC, it is important that rehabilitation professionals continue to work with these children as they get older, and put greater emphasis on parent education using materials that are adapted to varying educational levels. [ABSTRACT FROM AUTHOR]

PURPOSE: To investigate the: (1) percent of children with spina bifida (SB) complaining of pain, (2) frequency, duration, and cause of pain by sex, level of lesion type of SB, and ambulation status, (3) body sites reported to hurt, by variables in objective 2, and (4) associations between physical and mental/emotional health between caregiver and child. METHODS: Cross-sectional study of 101 caregivers of children (3 to 6 years old) with SB. Survey data and information from medical records were included. Pearson chi-square, one-way ANOVA, Fisher's exact test, logistic regressions, and bivariate correlations were used. RESULTS: Seventy percent reported that their child complained of pain, which did not significantly differ by sex, level of lesion, type of SB, or ambulation status. Most (86%) were reported to have experienced pain for less than 24 hours. The most frequently reported pain site was the head, followed by the abdomen and the lower body. Number of pain sites was moderately correlated with frequency of pain complaints. Correlations between how caregivers reported their own physical/mental/emotional health and how they rated that of their children ranged from weak (r = 0.22) to moderate (r = 0.55). CONCLUSION: Almost seven of ten children reportedly complained of pain ranging from at least once a month to everyday. Pain needs to be routinely assessed and treated in this population. [ABSTRACT FROM AUTHOR]

Whether it is for collaboration on folic acid fortification or the standardization of care efforts concerning neurogenic bowel dysfunction, a global forum on neural tube defects related issues is needed. Propitiously, the 2023 Spina Bifida World Congress sponsored by the Spina Bifida Association (SBA) was a catalyst for transnational dialog in the field of spina bifida (SB) research. Concurrently, the Journal of Pediatric Rehabilitation Medicine (JPRM) provides a platform for both international research as well as numerous clinical and educational projects, such as The Lifespan Bowel Management Protocol, and social interventions taught through the American Academy of Pediatrics' Spina Bifida Transition ECHO. Through this open access issue, work by colleagues in Ethiopia, the Nordic countries, and Switzerland, as well as among other transnational populations is highlighted. The development of the Spina Bifida Global Learning Collaborative is also showcased, representing a training initiative across four continents. Correspondingly in this issue, JPRM published an update to the Transition Guidelines for the Care of People with Spina Bifida. The clinical guidelines are a product of the SBA Collaborative Care Network cooperative agreement with the National Center on Birth Defects and Developmental Disabilities in the Centers for Disease Control and Prevention. While colleagues across the globe remain committed to native, immigrant, and displaced populations of individuals affected by SB, JPRM will continue to distribute premier research in multidisciplinary care, education, and advocacy. [ABSTRACT FROM AUTHOR]

BACKGROUND: People with childhood-onset disabilities face unique physical and social challenges in adulthood. Exercise interventions may improve physical performance in children, but there is a lack of research on adults. OBJECTIVE: To describe studies that investigate exercise interventions and to evaluate the quality of physical performance outcome measures for adults with childhood-onset disabilities. METHODS: Eligible studies reported on exercise interventions for adults (ages 16+) with cerebral palsy, spina bifida, or acquired brain injuries. Only randomized controlled trials published in English from 2008 to 2019 were included. MEDLINE, CINAHL, PEDro, EMBASE, and Cochrane Central Register of Controlled Trials were searched. Two reviewers independently screened studies and abstracted data. RESULTS: This scoping review included 4 trials reporting on cerebral palsy only. Three strength training programs found significant improvements in gait, and one mixed training program found significant improvements in strength and fitness. Only two outcome measures used are valid/reliable for adults (6 Minute Walk Test and Borg-20 Grades). CONCLUSION: Certain interventions may improve physical performance, but there is a lack of research on appropriate exercise interventions and physical performance outcome measures for adults with childhood-onset disabilities. Different exercise interventions should be investigated using larger sample sizes and outcome measures should be standardized. [ABSTRACT FROM AUTHOR]

Myelomeningocele (MMC) arises from an early neural developmental anomaly and results in a variety of structural abnormalities and associated functional neurologic deficits. As such, neurologic issues are central to virtually all clinical problems. Neurosurgical intervention strives to correct or improve these defects and prevent secondary complications. These interventions include closure of the open myelomeningocele and management (across the life span) of hydrocephalus, the Chiari II malformation (C2M) and tethered spinal cord (TSC). The development of pre-natal closure techniques and reports of improved outcome with in-utero closure (IUMC) have revolutionized the neurosurgical approach to myelomeningocele. Controversies remain surrounding patient selection, maternal risks, technique of IUMC (endoscopic vs. open) and long-term outcomes. However, real gains include reduced rates of hydrocephalus, modestly improved motor capabilities and reduction in C2M morbidity. For many decades, the cornerstone of treatment of hydrocephalus for many decades has been the placement and support of ventricular shunts. Endoscopic third ventriculostomy (ETV) with or without choroid plexus coagulation (ETV/CPC) is an appealing alternate strategy that avoids the morbidity and complications associated with shunts. The exact criteria for ETV-CPC candidacy and best metrics for outcome analysis remain active areas of debate and controversy. Similarly, neurosurgical management C2M, has centered upon the indications and clinical thresholds for performing posterior fossa surgical decompression. Tethered spinal cord management incorporates the diagnosis and surgical management of adhesions formed at the initial closure site, the consequent longitudinal traction related stress on the cord and the resulting neurologic signs and symptoms. [ABSTRACT FROM AUTHOR]