Journal of Pediatric Rehabilitation Medicine - Volume 11, issue 4

Article Type: Editorial

DOI: 10.3233/PRM-180483

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 11, no. 4, pp. 211-, 2018

Authors: Castillo, Jonathan

Article Type: Editorial

Abstract: Globally there is substantial variation in birth prevalence rates of neural tube defects. In the United States, for instance, the birth prevalence is seven cases per 10,000 live births, whereas in certain regions of Latin America, it has been reported to be as high as 96 cases per 10,000. While immigration from Latin America is often a result of social determinants, immigration itself can be understood as a social determinant of health (SDH). The Centers for Disease Control and Prevention has stated that when attempting to mitigate racial/ethnic health disparities, it should be remembered that SDH effectively determine longevity and …quality of life (QOL). Subsequently, investigators have begun to recognize existing disparities through the use of the National Spina Bifida Patient Registry (NSBPR). In the face of these documented health disparities among minority populations with spina bifida, the need for timely and culturally-competent study of QOL among Hispanics/Latinos, who have the highest spina bifida prevalence, is self-evident. Furthermore, social variables have been linked with worse outcomes in national patient registries, illustrating the importance of accounting for SDH in multicenter comparative analysis. Therefore, accounting for these individual-level differences becomes even more critical, when making comparisons within SB care – a condition with a known ethnic/racial-gradient in incidence. As we face an increasingly global community, with growing travel and immigration, fresh approaches will be required, such as community-based participatory research and culturally-competent learning collaboratives , in order to address the challenges ahead. Show more

Keywords: Social determinants of health, quality of life, community-based participatory research, immigrant health, minority health, three-hit model, learning collaborative

DOI: 10.3233/PRM-180484

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 11, no. 4, pp. 213-216, 2018

Authors: Antiel, Ryan M. | Janvier, Annie | Feudtner, Chris | Blaine, Kallie | Fry, Jessica | Howell, Lori J. | Houtrow, Amy J.

Article Type: Research Article

Abstract: BACKGROUND: Prenatal surgery for myelomeningocele (MMC) has been demonstrated to have benefits over postnatal surgery. Nevertheless, prenatal surgery requires a significant emotional, physical, and financial commitment from the entire family. METHODS: Mixed methods study of parents’ perceptions regarding provider communication, treatment choices, and the family impact of having a child with MMC. RESULTS: Parents of children with MMC (n = 109) completed questionnaires. Parents were well informed and reported gathering information about prenatal surgery from a wide range of sources. After a fetal diagnosis of MMC, most learned …about their options from their obstetrician, although one-third were not told about the option of prenatal surgery. About one-fourth of these parents felt pressure to undergo one particular option. Half of parents said that having a child with MMC has had a positive impact on them and their family, while the other half indicated that having a child with MMC has had both positive and negative impacts. The most commonly noted positive impacts were changes in parental attitudes, as well as having new opportunities and relationships. The most frequently reported negative impacts concerned relational and financial strain. The vast majority of parents indicated that they would still undergo prenatal surgery if they could travel back in time with their present knowledge. CONCLUSIONS: A better understanding of the parental experiences and perspectives following prenatal surgery will play an important role in providing overall support for parents and family members. Show more

Keywords: Myelomeningocele, prenatal diagnosis, maternal-fetal surgery, parental decision-making, parental perspectives, clinical ethics, family impact

DOI: 10.3233/PRM-170483

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 11, no. 4, pp. 217-225, 2018

Authors: Houtrow, Amy J. | Burrows, Pamela K. | Thom, Elizabeth A.

Article Type: Research Article

Abstract: PURPOSE: To evaluate for differences in neurodevelopmental outcomes at 30 months of age for children enrolled in the Management of Myelomeningocele Study (MOMS) based on the presence of hydrocephalus and cerebral shunts. METHODS: Children with no hydrocephalus (N = 27), children with shunted hydrocephalus (N = 108), and children with unshunted hydrocephalus (N = 36) were compared at 30 months of age on the Bayley II Mental and Psychomotor Indices, the Peabody Developmental Motor Scales-2 and the Preschool Language Scale, 4 …th edition. Generalized linear models were used to adjust for factors significantly different between the groups at baseline. Additional analyses were conducted to evaluate the impact of the severity of hydrocephalus. RESULTS: In unadjusted comparisons, statistically significant differences were noted between the three groups on the Peabody Gross Motor Quotient and thus the Total Motor Quotient. After adjustment, no statistically significant differences were identified. In subanalyses, children with more severe hydrocephalus fared worse on the Peabody Fine Motor Quotient (median 88 versus 94, p = 0.005), the Total Motor Quotient (median 70 versus 73, p = 0.02) and both Preschool Language Scale subtests (auditory comprehension: median 93 versus 104, p = 0.02 and expressive communication: median 95 versus 104.5, p = 0.01) and thus the total score (median 92 versus 105, p = 0.004). These results remained significant in the multivariable adjusted model. CONCLUSION: No neurodevelopmental differences were noted with children enrolled in MOMS across the three hydrocephalus/shunt groups, although severity of hydrocephalus was associated with poorer outcomes. Show more

Keywords: Hydrocephalus, ventriculoperitoneal shunt, myelomeningocele, neurodevelopmental

DOI: 10.3233/PRM-170481

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 11, no. 4, pp. 227-235, 2018

Authors: Conklin, Michael J. | Hopson, Betsy | Arynchyna, Anastasia | Atchley, Travis | Trapp, Courtney | Rocque, Brandon G.

Article Type: Research Article

Abstract: PURPOSE: Recent studies on patients with spina bifida have noted an increased incidence of skin breakdown with more proximal functional neurologic level. We hypothesized that there would be an inverse relationship between skin breakdown of the foot and severity of functional level of lesion, because patients with more caudal levels of lesion spend more time ambulating. METHODS: The National Spina Bifida Patient Registry (NSBPR) at Children’s of Alabama was queried for the presence of skin breakdown of the foot, ambulatory status, functional neurologic level, and diagnosis of myelomeningocoele (MMC) vs. non-myelomeningocoele (non-MMC). Univariate and multivariate analysis …were performed. RESULTS: Of 491 total patients, 378 were MMC and 113 were non-MMC. Eighty-five of 378 (22.5%) patients with MMC and 5 of 113 (4.4%) non-MMC patients reported skin breakdown (p = 0.009). Thoracic and lumbar levels were compared to the sacral level for statistical analysis. Skin breakdown occurred in 26.2% of thoracic (p = 0.001), 33.3% of high-lumbar (p = 0.001), 21.5% of mid-lumbar (p = 0.008), 26.2% of low-lumbar (p = 0.001), and 6.1% of sacral level patients. Ambulatory status was not significant on multivariate analysis. CONCLUSION: A diagnosis of MMC is a significant, independent risk factor for skin breakdown of the foot. Compared to sacral level, thoracic and lumbar levels of function were also independently significant. Ambulatory status was not significant. Show more

Keywords: Spina bifida, skin breakdown, ambulation

DOI: 10.3233/PRM-170520

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 11, no. 4, pp. 237-241, 2018

Authors: Crytzer, Theresa M. | Cheng, Yu-Ting | Bryner, Mary Jo | Wilson III, Robert | Sciurba, Frank C. | Dicianno, Brad E.

Article Type: Research Article

Abstract: PURPOSE: To describe pulmonary function and determine the impact of neurological level, scoliosis, and obesity on pulmonary function in people with spina bifida (SB). METHODS: Participants with SB (N = 29) (15 females; age, 30 ± 12 years) completed spirometry and body plethysmographic lung volume testing. Univariate and multivariate regression analyses were used to describe the factors associated with pulmonary function in people with SB. RESULTS: Distribution of category of impairment in pulmonary function was: 55% (n = 16) restricted, …6.9% (n = 2) spirometric restricted, 1 combined obstructed and restricted, and 35.5% (n = 10) normal. In univariate analyses, neurological level was negatively associated with pulmonary function parameters, i.e., forced vital capacity (FVC) (p = 0.005), forced expiratory volume in 1 second (FEV 1 ) (p = 0.008), total lung capacity (TLC) (p = 0.001), and degree of scoliosis were inversely associated with FVC (p = 0.005), FEV 1 (p = 0.003), and TLC (p = 0.004). In multivariate models, level of lesion and degree of scoliosis independently contributed to the degree of lung function impairment. Restrictive pulmonary function was observed in 9/10 (90%) of those with thoracic neurological levels and was associated with decreased inspiratory capacity (IC) and expiratory reserve volume (ERV). Lumbar level lesions were associated with either normal lung function or an isolated reduction in FVC due to reduction in only ERV and preserved TLC representing spirometric restriction. CONCLUSIONS: High prevalence of restrictive pulmonary physiology is present in people with SB, with more rostral neurological levels and greater degree of scoliosis associated with a higher degree of pulmonary function impairment. Show more

Keywords: Spina bifida, myelomeningocele, pulmonary function, scoliosis, neurological level, restrictive lung disease, total lung capacity, wheelchair, spirometry, lung volume, spirometric restriction, mobility impairment

DOI: 10.3233/PRM-179451

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 11, no. 4, pp. 243-254, 2018

Authors: Alriksson-Schmidt, Ann | Josenby, Annika Lundkvist | Lindquist, Barbro | Westbom, Lena

Article Type: Research Article

Abstract: PURPOSE: To increase knowledge about pain and general health in adults with myelomeningocele, a health condition with several risk factors for pain such as musculoskeletal deformities, shunt dysfunctions, bowel problems, and urinary tract infections/stones. METHODS: Descriptive correlational pilot study (N = 51, 53% males). Chi-square tests were used to test associations among presence, impact, severity, and pain site in relation to sex and age. ANOVA was used to analyze associations between sex, age, and general health (today). RESULTS: Seventy-three percent reported pain in the past four weeks. No …significant sex or age differences were associated with the presence of pain. Women were significantly more likely to report that pain interfered with work, χ 2 (1, N = 41) = 5.53, p = 0.02. There were significant main effects for sex and age on general health (today), F (2, 44) = 5.63, p = 0.007, adjusted R 2 = 0.17. Women scored lower on general health (today) (mean = 63.58) than men (mean = 76.33). Older individuals reported worse general health (today) than did younger (B = - 0.89, t = - 2.79, p = 0.008). CONCLUSIONS: Pain was frequent, and pain sites differed widely. Women were more likely to report that pain interfered with work, and scored lower on health, as did older persons. Show more

Keywords: Pain, myelomeningocele, adults, self-reported health, spina bifida, quality of life

DOI: 10.3233/PRM-170517

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 11, no. 4, pp. 255-264, 2018

Authors: Marreiros, Humberto

Article Type: Review Article

Abstract: BACKGROUND CONTEXT: Patients with spina bifida (SB) are at risk for pathological fractures and low bone mineral density (BMD). PURPOSE AND METHODS: This article reviews the literature and provides a comprehensive overview of how the characteristics of SB and its associated comorbidities intersect with bone fragility to identify possible pathophysiological mechanisms of fractures and low BMD. RESULTS: Bone fragility occurs early in the life of patients with SB as a result of a disturbance that determines changes in bone shape, quantity, and quality, as poor mineralization reduces bone stiffness. Bone fragility in SB …occurs due to local and systemic factors and may be considered a state of impaired bone quality of multifactorial aetiology, with complex interacting influences of neurological, metabolic, and endocrinological origins and the presence of smaller bones. Bone fragility should be evaluated globally according to skeletal age and Tanner staging. The phases of the evolution of Charcot joints seem to intercept the evolution of epiphyseal fractures. Charcot arthropathy in SB may be initiated by the occurrence of repetitive trauma and fractures in epiphyseal and subepiphyseal regions, where there is a deficit of bone mineralization and greater bone mass deficits. CONCLUSION: Bone fragility in MMC potentially has a multifactorial neuro-endocrinological-metabolic-renal dimension, with smaller bones, lower bone mass, and mineralization deficits affecting bone strength. Show more

Keywords: Spina bifida, bone fragility, fractures, bone mineral density, Charcot joints, prevention

DOI: 10.3233/PRM-180555

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 11, no. 4, pp. 265-281, 2018

Authors: Atchley, T.J. | Dangle, P.P. | Hopson, B.D. | Graham, A. | Arynchyna, A.A. | Rocque, B.G. | Joseph, D.B. | Wilson, T.S.

Article Type: Research Article

Abstract: PURPOSE : The purpose of this study is twofold: 1) to determine the age when a child with spina bifida (SB) will most likely transition from caregiver clean intermittent catheterization (CIC) to self-CIC, and 2) to identify factors associated with self-CIC in children older than that age. METHODS : This is a retrospective, single-institution cohort study of individuals with SB. Data were collected prospectively as part of the National Spina Bifida Patient Registry. For Aim 1, we identified all individuals who perform self-CIC and who had a documented transition from caregiver-CIC. We then determined the age of transition …to self-CIC. For Aim 2, we compared individuals over age 10 years (age cutoff determined by Aim 1) who use self-CIC to those who use caregiver-CIC to determine what variables were associated with self-CIC. RESULTS : From our SB population, 206 individuals used self-CIC. Of these, 64 patients had documented ages of transition from caregiver- to self-CIC. 46 (71.9%) and 56 (87.5%) patients had transitioned to self-CIC by 10 and 14 years, respectively. For Aim 2, we used age 10 as a cutoff, based on the findings from Aim 1, and found that 287/696 patients were ⩾ 10 years and using CIC. Factors independently associated with lower likelihood of self-CIC were thoracic spinal lesions (odds ratio (OR) 0.45) and Medicaid insurance (OR 0.24). CONCLUSIONS : The ages at self-CIC transition vary, although most patients transition by age 10. Thoracic-level spinal lesions and Medicaid insurance are associated with lower odds of self-CIC. Show more

Keywords: Spina bifida, adult, pediatric, clean intermittent catheterization, urologic management

DOI: 10.3233/PRM-170518

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 11, no. 4, pp. 283-291, 2018

Authors: Ambartsumyan, Lusine | Rodriguez, Leonel

Article Type: Research Article

Abstract: Spina bifida is a birth defect that commonly causes bowel and bladder dysfunction in children with a significant negative impact on quality of life and emotional wellbeing. Fecal continence improves satisfaction and the quality of life of both children and their caretakers. Bowel management in children with spina bifida is hampered by limited controlled studies and variable practice within different institutions and subspecialists. The goals of a successful bowel management program in children with spina bifida consist of predictable bowel movements, social continence, and eventual independence. Treatment options range from conservative interventions such as diets and oral laxatives that modify …stool consistency and transit, to trans-anal irrigations and antegrade continence enemas that facilitate predictable recto-sigmoid emptying and provide a greater degree of independence. In children, the treatment approach should be implemented in the context of the child’s developmental age in order to allow for optimum social integration with their age-appropriate peers. We present a review of a stepwise approach to bowel management in children with spina bifida and the challenges related to the proposed treatment options. Show more

Keywords: Children, spina bifida, neurogenic bowel, bowel management, motility, constipation, fecal incontinence

DOI: 10.3233/PRM-170533

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 11, no. 4, pp. 293-301, 2018

Authors: Van Batavia, Jason P. | Weiss, Dana A. | Long, Christopher J. | Madison, Julian | McCarthy, Gus | Plachter, Natalie | Zderic, Stephen A.

Article Type: Research Article

Abstract: PURPOSE: The era of the electronic health record (EHR) generates the ability to systematically collect and record innumerable data for complex procedures such as videourodynamic studies (VUDS). We developed a Structured Data Entry System (SDES) that would serve as a way to better standardize VUDS for both quality improvement and research capabilities. METHODS: A working group convened to design a SDES form for VUDS in a flow sheet format in our hospital’s EHR, allowing for easy integration of the information into the clinical encounter note and for weekly export of data to clinicians in spreadsheet form. …RESULTS: Analysis of weekly VUDS data revealed that entries were missing in 3% of cells in all SDES forms completed. The availability of the data in an Excel spreadsheet allows for easy manipulation, calculation of clinical variables, and streamlined analysis in figures or graphs to identify patients at the highest risk. CONCLUSION: Designing and implementing a SDES based on a flowsheet that can allow data to be placed seamlessly in the clinical record and to be integrated into a searchable database for quality improvement and research purposes allows one to harness the true potential of the EHR. Show more

Keywords: Electronic medical records, standardized data entry system, urodynamics, pediatric urology, health information technology

DOI: 10.3233/PRM-170525

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 11, no. 4, pp. 303-309, 2018

Authors: Sawin, Kathleen J. | Heffelfinger, Amy | Cashin, Susan E. | Brei, Timothy J.

Article Type: Research Article

Abstract: PURPOSE: Measuring self-management behaviors in adolescents and young adults with chronic health conditions has become a priority in health care, yet there is a paucity of instruments that capture these behaviors. The purpose of this psychometric study was to evaluate the reliability and validity of the 17-item generic Adolescent/Young Adult Self-Management and Independence Scale II (AMIS II). METHOD: Data were collected from 201 adolescents/young adults (AYA) with spina bifida and 129 of their parents. Exploratory factor analysis, confirmatory factor analysis, Cronbach alpha, frequencies, Pearson correlations, and intraclass correlations were used to evaluate the data. …RESULTS: The exploratory factor analysis of parent data supported two related self-management factors (Condition Self-Management and Independent Living Self-Management). Confirmatory factor analysis of AYA data confirmed these two factors and an overall scale with good fit statistics (GFI and CFI = 0.86–0.95; RMSEA = 0.057). Internal reliabilities ranged from α = 0.72–0.89. Intraclass correlation analysis supported the stability of the instrument (ICC parent report = 0.82, AYA report = 0.84). Concurrent validity was supported with low to moderate correlations to six related but distinct variables. CONCLUSION: Psychometric analysis supports this expanded measure of self-management for AYA with spina bifida. Evaluation of this instrument in AYA with other chronic health conditions is underway. Show more

Keywords: Adolescents, self-management, young adults, independent living behaviors, chronic health conditions

DOI: 10.3233/PRM-170479

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 11, no. 4, pp. 311-322, 2018

Authors: Hopson, Betsy | Rocque, Brandon G. | Joseph, David B. | Powell, Danielle | McLain, Amie B. (Jackson) | Davis, Richard D. | Wilson, Tracey S. | Conklin, Michael J. | Blount, Jeffrey P.

Article Type: Research Article

Abstract: PURPOSE: To describe the development and implementation of the Children’s of Alabama (COA) Spina Bifida (SB) Lifetime-Care-Model, including standardized care protocols and transition plan. METHODS: In 2010, members of the pediatric team at COA began to evaluate limitations in access to care for patients with SB at various stages of life. Through clinic surveys, observations, and caregiver report, a Lifetime-Care-Model was developed and implemented. Partnerships were made with adult medicine colleagues to create an interdisciplinary model at each stage. Since developing this program, it has evolved to include standardized care protocols. RESULTS: Since …2011, there have been 42 prenatal clinics; 114 families received counseling and prenatal care. Of these, 106 have delivered at our center and established care in our pediatric clinic. There are currently 474 patients in the pediatric and 218 in the adult clinics. CONCLUSIONS: Our institutional experience suggests that patients with SB benefit from continuity of care throughout their lifetime. This article describes early failures which led to an evolution in approach and implementation of a Lifetime-Care-Model which results in a smooth transition between all phases of life. We hope that other institutions may adapt and build upon it to create programs unique to their specific patient needs. Show more

Keywords: Spina bifida, transition, care model, disability, care coordination

DOI: 10.3233/PRM-180548

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 11, no. 4, pp. 323-334, 2018

Article Type: Other

DOI: 10.3233/PRM-180485

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 11, no. 4, pp. 335-336, 2018

Article Type: Other

DOI: 10.3233/PRM-180486

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 11, no. 4, pp. 337-340, 2018

Article Type: Other

DOI: 10.3233/PRM-180487

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 11, no. 4, pp. 341-, 2018