Cognitive disengagement syndrome in pediatric spina bifida.

The primary aim of this study was to characterize Cognitive Disengagement Syndrome (CDS) symptomatology in youth with spina bifida (SB). One hundred and sixty-nine patients aged 5-19 years old were drawn from clinical cases seen through a multidisciplinary outpatient SB clinic at a children's hospital between 2017 and 2019. Parent-reported CDS and inattention were measured using Penny's Sluggish Cognitive Tempo Scale and the Vanderbilt ADHD Rating Scale. Self-reported internalizing symptoms were measured with the 25-item Revised Children's Anxiety and Depression Scale (RCADS-25). We replicated Penny's proposed 3-factor structure of CDS with slow, sleepy, and daydreamer components. The slow component of CDS overlapped heavily with inattention, while the sleepy and daydreamer components were distinct from inattention and internalizing symptoms. Eighteen percent (22 of 122) of the full sample met criteria for elevated CDS, and 39% (9 of 22) of those patients did not meet criteria for elevated inattention. Diagnosis of myelomeningocele and presence of a shunt were associated with greater CDS symptoms. CDS can be measured reliably in youth with SB and can be discriminated from inattention and internalizing symptoms in this population. ADHD rating scale measures fail to identify a substantial portion of the SB population with attention-related challenges. Standard screening for CDS symptoms in SB clinics may be important to help identify clinically impairing symptoms and design targeted treatment plans.

Thalamic connectivity topography in newborns with spina bifida: association with neurological functional level but not developmental outcome at 2 years.

Spina bifida affects spinal cord and cerebral development, leading to motor and cognitive delay. We investigated whether there are associations between thalamocortical connectivity topography, neurological function, and developmental outcomes in open spina bifida. Diffusion tensor MRI was used to assess thalamocortical connectivity in 44 newborns with open spina bifida who underwent prenatal surgical repair. We quantified the volume of clusters formed based on the strongest probabilistic connectivity to the frontal, parietal, and temporal cortex. Developmental outcomes were assessed using the Bayley III Scales, while the functional level of the lesion was assessed by neurological examination at 2 years of age. Higher functional level was associated with smaller thalamo-parietal, while lower functional level was associated with smaller thalamo-temporal connectivity clusters (Bonferroni-corrected P < 0.05). Lower functional levels were associated with weaker thalamic temporal connectivity, particularly in the ventrolateral and ventral anterior nuclei. No associations were found between thalamocortical connectivity and developmental outcomes. Our findings suggest that altered thalamocortical circuitry development in open spina bifida may contribute to impaired lower extremity function, impacting motor function and independent ambulation. We hypothesize that the neurologic function might not merely be caused by the spinal cord lesion, but further impacted by the disruption of cerebral neuronal circuitry.

Comparing Cognitive Disengagement Syndrome Growth in Youth With and Without Spina Bifida.

OBJECTIVE: Cognitive disengagement syndrome (CDS; formally known as sluggish cognitive tempo), difficulties with social engagement, and lower levels of autonomy have been identified as maladaptive comorbidities in youth with spina bifida (SB). This study compared growth curves of CDS for youth with and without SB and examined whether these trajectories were associated with later functioning. METHODS: Longitudinal data spanning 8 years included youth with SB (n = 68, Mage = 8.34) and a demographically matched sample of typically developing (TD) peers (n = 68, Mage = 8.49). Adolescents, along with their caregivers and teachers, reported on youth social skills, behavioral functioning, and CDS. Growth curve models were examined by comparing CDS trajectories by SB status. RESULTS: Growth curves indicated that youth with SB had higher levels of teacher-reported CDS at ages 8 and 9, but growth curves were relatively stable for both groups. When predicting social skills, higher levels of teacher-reported (but not mother-reported) CDS at baseline predicted worse social functioning for both youth with and without SB in adolescence. For the slope findings, higher rates of mother-reported CDS over time predicted worse social skills (ß = -0.43) and lower levels of youth decision-making (ß = -0.43) for the SB group, while higher rates of teacher-reported CDS predicted worse social skills for the TD group. CONCLUSION: Next steps include understanding the impact that impaired social functioning and restricted autonomy have on youth with and without SB due to CDS to inform interventions. Additionally, advocacy for increased awareness of CDS-related impairment is needed, particularly for youth with chronic health conditions.

Foot deformity and quality of life among independently ambulating children with spina bifida in South Korea.

BACKGROUND: Children with spina bifida (SB) may have congenital or acquired foot deformities due to neurological defects in the spinal cord. As the musculoskeletal system keeps growing, foot deformities can develop or become aggravated. Thus, healthcare providers should provide constant monitoring and proper orthopedic management. Since foot deformities can affect not only the gait but also the daily life of children with SB, it is necessary to investigate the impact of foot deformities on everyday life. The purpose of this study was to examine the relationship between foot deformity and health-related quality of life (HRQoL) among independently ambulating children with SB. METHODS: This cross-sectional study examined the associations between foot deformity and HRQoL using two patient-reported outcome measures (Oxford Ankle Foot Questionnaire, Pediatric Outcomes Data Collection Instrument) in 93 children with SB aged 7-18 years between January 2020 and July 2021. RESULTS: Children with foot deformity (n = 54) reported lower scores in all subscales (physical, school and play, emotional, and footwear) of the Oxford Ankle Foot Questionnaire for children than those without foot deformity (n = 39; p < 0.001). Additionally, in terms of the Pediatric Outcomes Data Collection Instrument, children with foot deformity also reported poorer scores in four subscales (transfer and basic mobility, sports and physical functioning, comfort and pain, happiness with physical functioning; p < 0.001) than those without foot deformity, whereas upper extremity functioning was not significantly affected. Children with foot deformities, particularly those with bilateral foot deformities, equinus deformities, or mixed deformities, which are different types of right and left foot deformities, have a lower perceived HRQoL (p < 0.05). CONCLUSIONS: Among independently ambulating children with SB, those with foot deformities showed lower HRQoL. Moreover, children with foot deformities tend to have other clinical problems, including bladder and bowel dysfunction. Therefore, orthopedic management should consider the multifaceted factors that affect children's daily life and HRQoL.

Codevelopment of an illustration representative of people living with spina bifida for health educational materials.

BACKGROUND: The lack of health education resources specific to people with disabilities contributes to disparities in outcomes. Developing user-centered materials with representative images tailored to people with disabilities could help improve knowledge and outcomes. OBJECTIVE: As a first step in developing an online sexual health resource for adolescents with physical disabilities, we sought end-user feedback to create illustrated characters for use in educational materials. METHODS: Two styles of characters were developed by the research team, which included a professional disability artist. Verbal and online survey feedback was obtained at the Spina Bifida Association's Clinical Care Conference. A new image was created incorporating initial feedback. The new image and favored image from the first round were then tested through an online survey advertised on the Spina Bifida Association's Instagram story feed. Open-ended comments were organized by categories and overlapping themes. RESULTS: Feedback was obtained from 139 audience members and 25 survey respondents from the conference and 156 Instagram survey respondents. Themes included depiction of disability, nondisability diversity, other physical appearance, emotional response, and design style. Most frequently, participants suggested the inclusion of characters with a range of accurately depicted mobility aids and of characters without mobility aids. Participants also wanted a larger, more diverse group of happy, strong people of all ages. CONCLUSIONS: This work culminated in the codevelopment of an illustration that represents how people impacted by spina bifida view themselves and their community. We anticipate that using these images in educational materials will improve their acceptance and effectiveness.

A patient- and parent-centered approach to urinary and fecal incontinence in children and adolescents with spina bifida: understanding experiences in the context of other competing care issues.

INTRODUCTION: Despite significant and known challenges to urinary and fecal incontinence (UI and FI, respectively) among children with spina bifida (SB) and their families, few studies have identified patient-centered measures and approaches to assessing them. This study represents the first stage of a larger study to develop a patient-centered goal-setting tool to guide incontinence management (Figure). Our aim was to understand patient experiences of UI and FI, and what goals should be included in the tool. METHODS: We used a qualitative research approach integrated with human-centered design methods. We recruited, in clinic and online, children with SB (8-17 years old) and parents (>=18 years old) of children with SB (8-17 years old). Online activities were analyzed by four experienced design researchers using affinity diagramming, group analysis and modeling activities (mind maps, challenge maps, experience maps). Recruitment and thematic qualitative analysis continued until saturation was reached. RESULTS: Seventeen children with SB participated (9 female, 12 shunted, 13 using bladder catheterizations, 6 using Malone antegrade continence enema, median age: 15 years old). Fifteen parents participated (13 mothers/2 fathers, median age: 42 years old), including six mother-child dyads. Five major themes each were identified for UI and FI experiences: (1) negative emotional impact of incontinence, (2) unpredictability of incontinence, (3) challenging/unpleasant incontinence management, (4) inconvenient/unreliable incontinence management, (5) UI management having unpleasant complications and FI putting much responsibility on parents. We identified six UI goal domains and five FI goal domains. Four overlapping domains included: accidents, independence, interruptions at school and social/friends. Unique domains were: urinary tract infections (UI), catheterizations (UI) and enemas (FI). COMMENT: Findings of our study improve our understanding of children's and parents' experiences associated with incontinence in SB and potential continence goals. CONCLUSIONS: Children with SB experience incontinence as distressing and unpredictable. Incontinence management can be challenging and unreliable. Patient-centered continence goals cluster in domains, indicating how incontinence intermeshes with other life areas and offering potential approaches to structure continence goals in a goals-selection tool.

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.

Caregiver Expressed Emotion in Families of Youth With Spina Bifida: Demographic, Medical, and Family Correlates.

OBJECTIVE: Caregiver expressed emotion (EE), an interview-based measure of emotional valence within an interpersonal relationship, is associated with psychosocial outcomes across multiple conditions. Guided by a model implicating a bidirectional role of "Chronic Family Stress" in the unfolding of EE in family environments, the current study examined demographic, medical, and family-level variables in association with EE in caregivers of children with spina bifida (SB). METHODS: Data were combined from 2 distinct studies of families with a child with SB, resulting in a sample of 174 (ages 8-17). Linear regressions examined the family stressors and child variables in association with maternal and paternal warmth and criticism, as coded from EE interviews. RESULTS: Higher levels of family stress were associated with paternal criticism (p = .03), while having non-Hispanic White children was associated with both maternal and paternal criticism (ps < .005). Having children younger in age (ps < .01) and without a shunt (ps < .01) was associated with higher warmth. CONCLUSIONS: Family stressors, absence of the negative impacts of systemic racism, shunt status, and age appear to be associated with the expression of EE in caregivers of a child with SB. Findings highlight multiple assessment considerations, including assessing EE when children are younger to engage caregivers with children with SB when they are more likely to be expressing more warmth. Pinpointing factors associated with caregiver EE in SB will help to better identify families at risk for high levels of criticism and also aid in the development of targeted prevention and intervention programs.

Solo and Partnered Sexual Behavior Among an International Sample of Adults With Spina Bifida.

BACKGROUND: Spina bifida (SB) may differentially impact adults' participation in solo and partnered sexual behaviors, but little research investigates this topic. AIM: Describe solo and partnered sexual behaviors among an international sample of adult men and women with SB. MAIN OUTCOME MEASURES: Ever participated (no/yes) and recent participation (>1 year ago/within last year) in solo masturbation, cuddled with a partner, held hands with a partner, kissed a partner, touched a partner's genital, had genitals touched by a partner, gave a partner oral sex, received oral sex from a partner, vaginal sex, anal sex, and sex toy use. METHODS: Data were drawn from a larger cross-sectional, internet-based survey assessing the sexual behaviors of an international sample of men and women with SB. We used logistic regression to examine the impact of background (gender, age, independent living, and relationship status) and health (shunt status, ambulation, and genital sensation) factors on each outcome. RESULTS: The sample consisted of 345 respondents aged 18-73 years from 26 nations. Very few (<3%) had no lifetime experience with any solo or partnered behaviors; 25.0% reported participating in all behaviors at some point in their lives. The median number of past year sexual behaviors (of 16 total) was 7. Lifetime and recent participation were associated with demographic and health factors. CLINICAL IMPLICATIONS: Despite impairment, adults with spina bifida do participate in solo and partnered sexual behaviors. Medical personnel who work with this population should include discussions about sexuality as part of routine care. STRENGTHS & LIMITATIONS: Although this research measured solo and partnered sexual behavior in large international sample of adults with spina bifida, it is limited by its cross-sectional retrospective design and non-clinical convenience sample. CONCLUSION: Despite disability, many adults with SB participate in solo and partnered sexual behavior. Medical and psychosocial supports are needed to help adults in this population enjoy sexuality in a healthy and safe manner. Hensel DJ, Misseri R, Wiener JS, et al. Solo and Partnered Sexual Behavior Among an International Sample of Adults With Spina Bifida. J Sex Med 2022;19:1766-1777.

Nurse-led eHealth transition care program for adolescents with spina bifida: A feasibility and acceptability study.

PURPOSE: This study aimed to evaluate the feasibility and acceptability of a nurse-led eHealth transition care program for adolescents with spina bifida. DESIGN AND METHODS: This study used a single-arm, pretest-posttest intervention study. Adolescents with spina bifida, aged 12-15 years, and their parents participated in the program. A 6-week program was delivered through an online platform in real-time by nurses. We evaluated feasibility and acceptability using criteria such as the completion rate, program satisfaction, changes in transition readiness, social support, career preparation behavior, sexual knowledge, and sexual worries at three time points from July to September 2021. RESULTS: Thirteen adolescents completed all sessions and surveys (13/14, 92.9%). All adolescents expressed high satisfaction with both the content and delivery methods of the program. Significant benefits in transition readiness, career preparation behavior, and sexual knowledge were identified over the study period. However, the evaluation of social support and sexual worries did not demonstrate any significant improvements. Additionally, through family counseling, adolescents benefited from experiences such as reflecting on their current transition readiness, setting and achieving individualized goals and plans using a self-checklist with their parents and nursing professionals. CONCLUSION: This nurse-led eHealth intervention was feasible and acceptable for adolescents with spina bifida. Furthermore, our results highlight the practicability and the potential for strategic dissemination of using this eHealth program in transitional care during the COVID-19 pandemic. PRACTICE IMPLICATIONS: The eHealth transition care program contributes to broadening existing nursing interventions not only in medical areas but also in daily life areas.

Trajectories of Marital Satisfaction among Parents of Youth with Spina Bifida.

OBJECTIVE: This study examined marital satisfaction among parents of youth with spina bifida (SB) over an 8-year period and investigated the usefulness of the double ABCX model for understanding factors that predict change in marital satisfaction across child age. METHODS: Data from five time points of a longitudinal investigation of psychosocial outcomes in youth with SB were included. Mothers and fathers of children with SB (aged 8-17) reported on marital satisfaction and components of the double ABCX model. Change in marital satisfaction was examined across child age with components of the double ABCX model, as well as interactions between components, as predictors. RESULTS: Marital satisfaction was significantly higher in the present sample than in a normative sample of married couples. Although there were no significant changes in marital satisfaction for either parent as a function of child age, there was significant variability for the intercept and slope of maternal and paternal marital satisfaction within the sample. Family support predicted a higher intercept, and mental health symptoms predicted a lower intercept, for maternal and paternal marital satisfaction. More stressors and SB-related family stress predicted a lower intercept for paternal marital satisfaction. Family support and family stress attributed to SB moderated the relationship between child vulnerability and maternal marital satisfaction. Observed family cohesion and child psychosocial quality of life moderated the relationship between family stressors and paternal marital satisfaction. CONCLUSION: Findings demonstrate the usefulness of the double ABCX model for this population.

Working memory and cognitive flexibility predict growth trajectories of sluggish cognitive tempo in youth with spina bifida.

As sluggish cognitive tempo (SCT) shows similar inattention and neuropsychological dysfunction as youth with spina bifida (SB), it is important to examine whether neuropsychological functioning may affect the development of SCT in this population. Participants were 140 youth with SB and their parents who participated in five waves of a longitudinal study across eight years (ages 8-15 years at Time 1). At Time 1, teacher-, mother-, and father-report of SCT showed 9%, 8.3%, and 5.3% impairment in SCT respectively compared to other youth in the sample. Growth curves were used to examine changes over time in mother-, father-, and teacher-reported SCT. Four neurocognitive variables were included (attention, working memory, cognitive flexibility, plan/organize) as predictor variables of SCT growth and intercepts. Mother, father, and teacher-report of SCT were included in separate models. Age and shunt status were included as covariates in the growth models. Inattentive symptoms were also included as a covariate in working memory, shift, and plan/organize models. Worse working memory at Time 1 predicted an increase in mother-reported SCT symptoms over eight years (ß = -.28) and poorer cognitive shifting skills predicted higher rates of teacher-reported SCT over time (ß = -.61). All other neurocognitive variables predicted the intercept, but not the slope of SCT symptoms. Results indicate that worse neurocognitive functioning is associated with higher levels of SCT symptoms and that worse working memory and cognitive flexibility at baseline predicted an increase in SCT symptoms as youth with SB age. It may be important for clinicians to monitor and assess levels of SCT in youth with SB.

Parents' school-related concerns and perceived strengths in youth with spina bifida.

BACKGROUND: Although the academic difficulties of children with spina bifida (SB) are well-documented, there is limited literature on parents' views of their children's school experiences and school-related supportive services. Thus, the current study examined parents' school-related concerns, as well as perceived areas of strength, among children with SB. METHODS: Using a mixed-methods approach, 30 families (29 mothers and 19 fathers) of children with SB (ages 8-15 years) completed questionnaires and interviews. Content analysis was used to generate themes from interview data about parents' school-related concerns and perceptions of their child's strengths. RESULTS: Overall, six themes emerged when assessing both parents' concerns and perceived strengths. Some parents did not endorse school concerns or strengths for their child. However, other parents described concerns related to academic performance, cognitive abilities, lack of school support, missed school and/or class time and disengagement, as well as strengths such as academic skills, cognitive abilities, persistence, self-advocacy and agreeableness. Despite parents' concerns about their children's academic performance, quantitative data revealed that less than 50% of children had received a neuropsychological evaluation and/or academic accommodations; additional quantitative data supported the qualitative findings. CONCLUSIONS: The mixed-methods approach used in this study provides a richer understanding of parents' experiences in the school setting when they have a child with SB. Results can inform clinical practice, identifying a need to improve academic support for children with SB and help parents manage education-related stressors.

Assessment of health status in adolescents with spina bifida in the West Bank, Palestine: sense of coherence and self-perceived health.

PURPOSE: To assess self-perceived health and sense of coherence (SOC) in adolescents with spina bifida (SB) in the West Bank, Palestine, compared to a healthy reference group. Further, to assess the association between impairment levels in the adolescents with SB and their self-perceived health and SOC. METHODS: Fifty adolescents with SB and 150 healthy adolescents completed measures of self-perceived health - the Pediatric Quality of Life Inventory (PedsQLTM 4.0) - and SOC. The rehabilitation center nurses identified the physical impairments of the adolescents with SB from their medical records, and classified them by impairment severity. RESULTS: Adolescents with SB reported lower self-perceived health (PedsQL median 55, IQR 42-67), than the reference group (median 85, IQR 74-90), p < 0.001, and lower SOC (median 47, IQR 44-50) than the reference group (median 55, IQR 44-61), p < 0.001. Impairment level was inversely associated with both self-perceived health and SOC. CONCLUSIONS: The low self-perceived health and SOC among adolescents with SB in Palestine indicate the need for collaboration between policy makers and providers to improve these adolescents' physical and social environment and to promote the rehabilitation services provided for them.Implications for rehabilitationThe low self-perceived health and SOC among adolescents with SB in Palestine highlight the need to develop appropriate interventions to promote their health, independency, and self-care management.Collaboration of the caregivers and health care providers is required to develop, implement, and evaluate intervention programs that might promote coping abilities of the adolescents with SB.A multidisciplinary approach is needed to set goals that have a meaningful impact on social, emotional, behavioral, and educational conditions of the adolescents with SB and in different settings, for example, home, school, and community.The environment in Palestine presents barriers to the social engagement of adolescents with SB, and new ways are needed to enhance their participation in the society to promote their self-concept and wellbeing.

Psychosocial Adjustment in Emerging Adults With and Without Spina Bifida: A 14-Year Follow-up Study.

OBJECTIVE: A 14-year follow-up of youth with spina bifida (SB), as compared to a matched comparison sample without a chronic health condition, focused on psychosocial adjustment during emerging adulthood (ages 22-23). METHODS: The initial cohort at time 1 consisted of 68 families with an 8-year-old or 9-year-old child with SB and a matched comparison sample of 68 typically developing children and their families. At the final assessment (time 7), participants included 56 emerging adults with SB (82% of those who participated at time 1) and 62 comparison youth (91% of those who participated at time 1) who completed a set of self-report measures. RESULTS: Emerging adults with SB continue to lag behind across several important developmental milestones, including decision-making responsibility, involvement in social and romantic relationships, sexual intimacy, educational and vocational achievement, financial independence, and engagement in health-related behaviors. However, from a resilience perspective, these individuals also exhibited a number of strengths across the following areas: identity development, quality of social supports, and fewer problem behaviors (e.g., alcohol and other substance use). Most findings were still significant after controlling for a verbal intelligence quotient proxy. CONCLUSION: This study highlighted vulnerabilities and strengths of individuals with SB as they enter emerging adulthood, with considerable continuity across childhood, adolescence, and emerging adulthood.

Benefit-finding among young adults with spina bifida.

The purpose of the current study was to examine benefit-finding in a sample of young adults with SB and assess relationships between demographic and medical variables and benefit-finding. Young adults with SB (N = 329), ranging in age from 18 to 30 (M = 23.44 ± 2.93), completed the Benefit-Finding Scale (BFS), a 17-item self-report questionnaire, and reported on demographic and medical information related to their SB diagnosis. Participants endorsed a range of benefits associated with living with SB. The most frequently endorsed benefits were related to personal strengths, interpersonal relationships, and life philosophy. Younger age, majority race, higher education level, and employment were significantly associated with higher BFS scores. Young adults with SB report benefits associated with their chronic condition. Future research should examine these benefits in relation to psychosocial outcomes.

Anxiety, depression, and fatigue in middle-aged and older persons with spina bifida: a cross-sectional study.

PURPOSE: To study psychological distress and fatigue among persons with spina bifida (SB) 50 years or older and living in Norway. METHODS: In 2017, cross-sectional data were collected (n = 30). The Hospital Anxiety and Depression Scale (HADS) and the Fatigue Severity Scale (FSS) were used. Descriptive statistics, non-parametric tests, and Spearman correlations were performed. Relevant information from previous studies on adults with chronic spinal cord injury (SCI) and the general population, were collected for comparison. RESULTS: Participants were 18 women and 12 men, mean age 57.5 (SD 5.6), 26 with myelomeningocele, and six with hydrocephalus. Thirty percent scored above the HADS-A- and 20% above the HADS-D thresholds, thus in the same range as previous studies of SB, but higher compared to persons with SCI and norms. HADS-D correlated with pain and FSS scores. Forty percent reported fatigue symptoms (9/15 without hydrocephalus, 3/6 with hydrocephalus). CONCLUSIONS: The study revealed a high prevalence of fatigue symptomatology among middle-aged and older adults with SB. Symptoms of anxiety and depression were more common than among persons with chronic SCI and norms. SB follow-ups should include awareness of psychological distress and fatigue, and investigate pain and medication side effects among possible influencing factors.IMPLICATIONS FOR REHABILITATIONClinicians treating adult persons with SB should be aware of possible psychological distress and fatigue symptomatology among these patients.We suggest an initial screening for psychological distress and fatigue in persons with SB during follow-up visits and rehabilitation.Interventions to reduce pain may influence levels of psychological distress and/or fatigue in patients with SB.Clinicians should enquire about the effects of medication on fatigue when assessing and prescribing new medications; a thorough medication review helps to assess the benefits and risks.

Site, frequency, and duration of pain in young children with spina bifida.

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.

Assessment of Health Literacy and Self-reported Readiness for Transition to Adult Care Among Adolescents and Young Adults With Spina Bifida.

Importance: Health literacy has been shown to play an important role in transitions of care in adult populations, with low health literacy associated with adverse health outcomes. The role of health literacy in the transition from pediatric to adult care has been less well studied. Among adolescents and young adults with spina bifida, high rates of unsuccessful transition have been shown, but how patient health literacy affects transition readiness remains unknown. Objective: To determine whether health literacy is associated with transition readiness in adolescents and young adults with spina bifida. Design, Setting, and Participants: This cross-sectional study involved collection of patient-reported questionnaires between June 2019 and March 2020 at a multidisciplinary spina bifida center at a single, free-standing children's hospital. Patient demographic and clinical characteristics were obtained from medical record review. Patients were aged 12 years or older with a diagnosis of spina bifida (myelomeningocele and nonmyelomeningocele) whose primary language was English or Spanish. Data analysis was performed from October 2020 to March 2021. Exposures: Health literacy as assessed by the Brief Health Literacy Screening Tool. Main Outcomes and Measures: The primary outcome was total Transition Readiness Assessment Questionnaire (TRAQ) score, normalized into units of SD. Nested, multivariable linear regression models assessed the association between health literacy and TRAQ scores. Results: The TRAQ and Brief Health Literacy Screening Tool were completed by 200 individuals (median [range] age, 17.0 [12.0-31.0] years; 104 female participants [52.0%]). Most of the patients were younger than 18 years (110 participants [55.0%]) and White (136 participants [68.0%]) and had myelomeningocele (125 participants [62.5%]). The mean (SD) TRAQ score was 3.3 (1.1). Sixty-six participants (33.0%) reported inadequate health literacy, 60 participants (30.0%) reported marginal health literacy, and 74 participants (37.0%) reported adequate health literacy. In univariable analysis, health literacy, age, type of spina bifida, level of education, self-administration vs completion of the questionnaires with assistance, ambulatory status, and urinary incontinence were associated with total TRAQ score. In all nested, sequentially adjusted, multivariable models, higher health literacy remained a significant, stepwise, independent variable associated with higher TRAQ score. In the fully adjusted model, having adequate compared with inadequate health literacy was associated with an increase in normalized TRAQ score of 0.49 SD (95% CI, 0.19-0.79). Conclusions and Relevance: Patient-reported transition readiness is associated with health literacy, even after adjustment for education level and other demographic and clinical factors. Developing and implementing health literacy-sensitive care programs during the transition process may improve patient transition readiness.

'We did everything we could'- a qualitative study exploring the acceptability of maternal-fetal surgery for spina bifida to parents.

OBJECTIVE: To explore the concepts and strategies parents employ when considering maternal-fetal surgery (MFS) as an option for the management of spina bifida (SB) in their fetus, and how this determines the acceptability of the intervention. METHODS: A two-centre interview study enrolling parents whose fetuses with SB were eligible for MFS. To assess differences in acceptability, parents opting for MFS (n = 24) were interviewed at three different moments in time: prior to the intervention, directly after the intervention and 3-6 months after birth. Parents opting for termination of pregnancy (n = 5) were interviewed only once. Themes were identified and organised in line with the framework of acceptability. RESULTS: To parents opting for MFS, the intervention was perceived as an opportunity that needed to be taken. Feelings of parental responsibility drove them to do anything in their power to improve their future child's situation. Expectations seemed to be realistic yet were driven by hope for the best outcome. None expressed decisional regret at any stage, despite substantial impact and, at times, disappointing outcomes. For the small group of participants, who decided to opt for termination of pregnancy (TOP), MFS was not perceived as an intervention that substantially could improve the quality of their future child's life. CONCLUSION: Prospective parents opting for MFS were driven by their feelings of parental responsibility. They recognise the fetus as their future child and value information and care focusing on optimising the child's future health. In the small group of parents opting for TOP, MFS was felt to offer insufficient certainty of substantial improvement in quality of life and the perceived severe impact of SB drove their decision to end the pregnancy.