Total Energy Expenditure and Body Composition of Children with Developmental Disabilities

Background Obesity prevalence is increased in children with developmental disabilities, specifically in children with spina bifida and Down syndrome. Energy expenditure, a critical aspect of weight management, has been extensively studied in the typically developing population, but not adequately studied in children with developmental disabilities. Objective Determine energy expenditure, fat-free mass and body fat percentile and the impact of these findings on recommended caloric intake in children with spina bifida and Down syndrome. Methods/Measures This pilot study included 36 children, 18 with spina bifida, 9 with Down syndrome and 9 typically developing children. Half of the children with spina bifida were non-ambulatory. Doubly labeled water was used to measure energy expenditure and body composition. Descriptive statistics described the sample and MANOVA and ANOVA methods were used to evaluate differences between groups. Results Energy expenditure was significantly less for children with spina bifida who primarily used a wheelchair (p = .001) and children with Down syndrome (p = .041) when compared to children without a disability when adjusted for fat-free mass. However, no significant difference was detected in children with spina bifida who ambulated without assistance (p = .072). Conclusions Children with spina bifida and Down syndrome have a significantly decreased energy expenditure which directly impacts recommended caloric intake. No significant difference was detected for children with spina bifida who ambulated, although the small sample size of this pilot study may have limited these findings. Validating these results in a larger study is integral to supporting successful weight management of these children

Family functioning guidelines for the care of people with spina bifida

Research supports a resilience-disruption model of family functioning in families with a child with spina bifida. Guidelines are warranted to both minimize disruption to the family system and maximize family resilience and adaptation to multiple spina bifida-related and normative stressors. This article discusses the spina bifida family functioning guidelines from the 2018 Spina Bifida Association’s Fourth Edition of the Guidelines for the Care of People with Spina Bifida, and reviews evidence-based directions with the intention of helping individuals with spina bifida achieve optimal mental health throughout their lifespan. Guidelines address clinical questions pertaining to the impact of having a child with spina bifida on family functioning, resilience and vulnerability factors, parenting behaviors that may facilitate adaptive child outcomes, and appropriate interventions or approaches to promote family functioning. Gaps in the research and future directions are discussed

The development of number concepts in children with differing degrees of spina bifida and hydrocephalus

The main contributions of the work in this thesis are summarised in testing the following hypotheses: (i) children with differing degrees of spina bifida and hydrocephalus pass through normal stages in the development of number concepts as postulated by Piaget; (ii) spina bifida children without a shunt are significantly more successful overall in Piagetian number tests than those with; (iii) there is a significant negative correlation between operativity in the Piagetian number tests and degree of overall handicap as reflected by the Pultibec Scale; (iv) spina bifida boys are significantly more successful educationally, particularly with respect to the development of number concepts, than spina bifida girls; (v) spina bifida children have specific perceptual problems; (vi) the well-attested progress in pre-school spina bifida children's acquisition of vocabulary skills is not maintained thereafter to the same extent; (vii) the level of reading attainment of spina bifida children overall is below normal at each age level

A Closer Look at the Global Management of Spina Bifida: The Implementation of Endoscopic Third Ventriculostomy in the Treatment of Spina Bifida-Related Hydrocephalus in Africa

Spina bifida, specifically myelomeningocele, is a debilitating neural tube defect that affects patients and families throughout the world. Traditional management and treatment methods are described, followed by an explanation of why this is often inadequate in providing care for those in impoverished areas of the world, including many regions of Africa. Endoscopic third ventriculostomy is proposed as a promising alternative to traditional methods for treating hydrocephalus, an often associated condition, and the implementation of this treatment method in Africa is explored. In order to relieve Africa of the burden of spina bifida-related hydrocephalus and improve global management of spina bifida, it is imperative that the paucity of neurosurgeons in Africa be addressed and successful models for training be expanded

Educating School Nurses to Improve Bowel Continence in Children with Spina Bifida

Children born with spina bifida, a neural tube defect, can have associated loss of bowel control resulting in bowel incontinence. The National Patient Spina Bifida Registry found that 87% of those living with spina bifida had bowel incontinence, and less than 30% were bowel continent (Sawin et al., 2015; Wiener et al., 2017). Unfortunately, providers may never start a child living with spina bifida on a bowel management program. Evidence suggests that children with spina bifida should begin a bowel management program early, using a stepwise approach. School nurses, who interact with children living with spinal bifida while attending school, have an opportunity to provide support to children living with bowel incontinence but may lack knowledge and skills on bowel management. This evidence-based practice project aimed to educate school nurses in a county school district about the best bowel management guidelines for children attending school with spina bifida. The project’s goal was to improve the lives of children with spina bifida, especially in school, by motivating school nurses to play a more active role in the child’s bowel management routines towards increasing bowel continence. The Iowa Model of Evidence-Based Practice guided the steps of the project. Eighty-six school nurses received education about spina bifida and bowel management asynchronously via an online voiceover PowerPoint presentation. Nurses completed a pre-and post-knowledge test, and a significant improvement (p \u3c 0.001) in test scores by approximately three points was seen from the pre-test to the post-test. Educating school nurses about spina bifida and bowel management made them better prepared to support and manage bowel incontinence in children living with spina bifida

Sleep-disordered breathing on respiratory polygraphy in neonates with spina bifida

INTRODUCTION Studies have shown a high prevalence of sleep-disordered breathing (SDB) in children with spina bifida. International standards for regular testing for SDB in this population are lacking. While there are studies investigating the prevalence of SDB in children with spina bifida, there are close to no studies in neonates. AIM AND OBJECTIVE To evaluate if routine respiratory polygraphy (RPG) testing is indicated for neonates with spina bifida and if yes, with what therapeutic consequence. METHODS We conducted a retrospective cohort study of all neonates with spina bifida at the University (Children's) Hospital Zurich after fetal spina bifida repair born between 2017 and 2022, who had undergone at least 1 RPG evaluation during hospitalization on the neonatal ward. RPG were evaluated by a blinded group of experienced pediatric pulmonologists. Based on the neonatal RPG results and pediatric pulmonologist's recommendation for caffeine therapy the spina bifida cohort was divided into two groups. Neonatal baseline RPG and follow-up RPG at the age of the 3 months were evaluated. RESULTS 48 neonates with RPG were included. Compared to the standard values in healthy neonates, the RPG results of this spina bifida cohort showed findings of SDB with central apnea and hypopnea. 22 (45.8%) neonatal RPG evaluations detected central SDB, prompting caffeine therapy. Follow-up RPG conducted after 3 months showed significant improvement of SDB with (almost) no need for continuation of caffeine. CONCLUSION We recommend the implementation of routine RPG testing in neonates with spina bifida to detect SDB and facilitate early targeted treatment

ADHD and Attention Problems in Children With and Without Spina Bifida

Objectives: To identify differences in the diagnosis and treatment of attention deficit/hyperactivity disorder (ADHD) between typically developing children and children with spina bifida. Method: Sixty-eight children with spina bifida and 68 demographically matched, typically developing children participated in a larger, longitudinal study. Rates of maternal, paternal, and teacher reports of attention problems, as well as rates of maternal reports of ADHD diagnosis, diagnosing provider, pharmaceutical treatment, mental health treatment, and academic accommodations were obtained at 5 time points over a period of 8 years and were compared across groups. Results: Children with spina bifida were more likely to have an ADHD diagnosis and attention problems. Attention problems and ADHD diagnoses were first reported at earlier time points for children with spina bifida than typically developing children. Among children with ADHD or attention problems, children with spina bifida were more likely to be treated with medication, but they were just as likely to use mental health services and receive resource services at school. Conclusions: Children with spina bifida were diagnosed with ADHD and identified as having attention problems more frequently and at an earlier age. This finding could be due to earlier symptom development, greater parental awareness, or more contact with providers. Among those with ADHD or attention problems, stimulant medication was more likely to be prescribed to children with spina bifida, despite research that suggests it may not be as beneficial for them. Further research on the effectiveness of ADHD pharmacological treatment for children with spina bifida is recommended

Preserving Shoulder Integrity for Pediatric Wheelchair Users

According to the Centers for Disease Control and Prevention (CDC) (2019), 1,645 babies are born with spina bifida each year. There are three different types of spina bifida, with myelomeningocele being the most common. As for prevalence, “Hispanic women have the highest rate of having a child affected by spina bifida, when compared with non-Hispanic white and non-Hispanic black women” (CDC, 2019, para. 2). Children diagnosed with spina bifida may face motor, sensory, and cognitive impairments that can affect their quality of life and participation in occupations. According to Murray et al. (2015), children between the ages of 8 and 15 with spina bifida reported lower health-related quality of life compared to other children with chronic health conditions, making children with spina bifida a population of great interest to occupational therapists. Furthermore, the lifetime cost of care for a person with spina bifida is $791,900 (CDC, 2019). According to the American Spinal Injury Association (ASIA) (2020), about 20% of SCIs occur in children and adolescents. Around 1,455 children are admitted to hospitals each year for treatment for SCIs in the United States (ASIA, 2020). The leading cause of pediatric SCIs are car accidents followed by falls. Males are also two times more likely to obtain a SCI than females (Chin, 2018)

J Urol

PurposeWe describe the characteristics, treatments and survival of patients with spina bifida in whom end stage renal disease developed from 2004 through 2008 in the United States Renal Data System.Materials and MethodsWe used ICD-9-CM code 741.* to identify individuals with spina bifida using hospital inpatient data from 1977 to 2010, and physician and facility claims from 2004 to 2008. We constructed a 5:1 comparison group of patients with end stage renal disease without spina bifida matched by age at first end stage renal disease service, gender and race/ethnicity. We assessed the risk of mortality and of renal transplantation while on dialysis using multivariate cause specific proportional hazards survival analysis. We also compared survival after the first renal transplant from the first end stage renal disease service to August 2011.ResultsWe identified 439 patients with end stage renal disease and spina bifida in whom end stage renal disease developed at an average younger age than in patients without spina bifida (41 vs 62 years, p <0.001) and in whom urological issues were the most common primary cause of end stage renal disease. Compared to patients with end stage renal disease without spina bifida those who had spina bifida showed a similar mortality hazard on dialysis and after transplantation. However, patients with end stage renal disease without spina bifida were more likely to undergo renal transplantation than patients with spina bifida (HR 1.51, 95% CI 1.13\u20132.03). Hospitalizations related to urinary tract infections were positively associated with the risk of death on dialysis in patients with end stage renal disease and spina bifida (HR 1.42, 95% CI 1.33\u20131.53).ConclusionsSpina bifida was not associated with increased mortality in patients with end stage renal disease on dialysis or after renal transplantation. Proper urological and bladder management is imperative in patients with spina bifida, particularly in adults.CC999999/Intramural CDC HHS/United States2016-02-01T00:00:00Z25167993PMC448186

J Urol

Purpose:Most children with spina bifida now survive into adulthood, although most have neuropathic bladder with potential complications of incontinence, infection, renal damage and diminished quality of life. In this study we sought to 1) describe contemporary bladder management and continence outcomes of adults with spina bifida, 2) describe differences from younger individuals and 3) assess for association with socioeconomic factors.Materials and Methods:We analyzed data on bladder management and outcomes in adults with spina bifida from the National Spina Bifida Patient Registry. A strict definition of continence was used. Results were compared to young children (age 5 to 11 years) and adolescents (12 to 19). Statistical analysis compared cohorts by gender, ethnicity, spina bifida type, lesion level, insurance status, educational attainment, employment status and continence.Results:A total of 5,250 patients with spina bifida were included, of whom 1,372 (26.1%) were adults. Of the adult patients 45.8% did not take medication, but 76.8% performed clean intermittent catheterization. Continence was decreased in adults with myelomeningocele (45.8%) vs those with non-myelomeningocele spina bifida (63.1%, p <0.0001). Continence rates were higher in the older cohorts with myelomeningocele (p <0.0001) but not in those with non-myelomeningocele spina bifida (p 1/4 0.1192). Bladder management and history of urological surgery varied among age groups. On univariate analysis with spina bifida related or socioeconomic variables continence was significantly associated with educational level but on multivariable logistic regression analysis bladder continence was significantly associated with employment status only.Conclusions:Bladder management techniques differ between adults and children with spina bifida. Bladder continence outcomes were better in adults, with nearly half reporting continence. Continence was significantly associated with employment status in patients age 25 years or older.20182019-12-18T00:00:00ZU01 DD000744/DD/NCBDD CDC HHS/United StatesU01 DD001070/DD/NCBDD CDC HHS/United StatesU01 DD001073/DD/NCBDD CDC HHS/United StatesU01 DD001072/DD/NCBDD CDC HHS/United States29588216PMC69193136997vault:3426