Journal of Pediatric Rehabilitation Medicine - Volume 16, issue 4

Authors: Castillo, Jonathan | Thibadeau, Judy K. | Brei, Tim | Castillo, Heidi

Article Type: Editorial

Abstract: 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. Show more

Keywords: Myelomeningocele, Transition, guidelines, Global Health, International Collaboration

DOI: 10.3233/PRM-239020

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 16, no. 4, pp. 581-582, 2023

Authors: Fremion, Ellen | Kaufman, Melissa | Mukherjee, Shubhra | Murphy, Pamela | Smith, Kathryn

Article Type: Other

Abstract: 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. Show more

Keywords: Spina bifida, myelomeningocele, transition to adult care, adolescent care, care guidelines

DOI: 10.3233/PRM-230052

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 16, no. 4, pp. 583-593, 2023

Authors: Brun, Brittany | Wille, David A. | Schauer, Sonja M. | Moehrlen, Ueli | Meuli, Martin | Latal, Beatrice | Padden, Beth

Article Type: Research Article

Abstract: 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. Show more

Keywords: Spina bifida, ambulation, outcome, orthotics, assistive devices

DOI: 10.3233/PRM-220096

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 16, no. 4, pp. 595-604, 2023

Authors: Szymanski, Konrad M. | Adams, Cyrus M. | Alkawaldeh, Mohammad Y. | Austin, Paul F. | Bowman, Robin M. | Castillo, Heidi | Castillo, Jonathan | Chu, David I. | Estrada Jr , Carlos R. | Fascelli, Michele | Frimberger, Dominic C. | Gargollo, Patricio C. | Hamdan, Dawud G. | Hecht, Sarah L. | Hopson, Betsy | Husmann, Douglas A. | Jacobs, Micah A. | MacNeily, Andrew E. | McLeod, Daryl J. | Metcalfe, Peter D. | Meyer, Theresa | Misseri, Rosalia | O’Neil, Joseph | Rensing, Adam J. | Routh, Jonathan C. | Rove, Kyle O. | Sawin, Kathleen J. | Schlomer, Bruce J. | Shamblin, Isaac | Sherlock, Rebecca L. | Slobodov, Gennady | Stout, Jennifer | Tanaka, Stacy T. | Weiss, Dana A. | Wiener, John S. | Wood, Hadley M. | Yerkes, Elizabeth B. | Blount, Jeffrey

Article Type: Research Article

Abstract: 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. Show more

Keywords: Myelomeningocele, spinal dysraphism, hydrocephalus, cause of death, mortality

DOI: 10.3233/PRM-220086

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 16, no. 4, pp. 605-619, 2023

Authors: Partington, Michael D.

Article Type: Article Commentary

DOI: 10.3233/PRM-230068

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 16, no. 4, pp. 621-621, 2023

Authors: Yesehak, Bethelehem | Dorsey, Amanda | Zewdie, Kibruyisfaw | Kancherla, Vijaya | Ashagre, Yordanos

Article Type: Research Article

Abstract: 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. Show more

Keywords: Neural tube defects, spina bifida, congenital anomalies, folic acid, high risk prevention

DOI: 10.3233/PRM-230046

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 16, no. 4, pp. 623-628, 2023

Authors: Taha, Asma A. | Eisen, Aaron M. | Abdul Rahman, Hana Q. | Good, Kelsey E. | Freeman, Kurt A. | Kotzin, Jennifer D. | Wolf, Margaret H. | Azar, Najood G. | Davis, Kelley R. | Austin, J. Christopher

Article Type: Research Article

Abstract: 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. Show more

Keywords: Bowel continence, cecostomy, bowel incontinence, neurogenic bowel dysfunction, pediatrics, spina bifida

DOI: 10.3233/PRM-220123

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 16, no. 4, pp. 629-637, 2023

Authors: Whelan, JoAnne L. | Armstrong, Cheryl L.H. | Schroyer, Rebecca | O’Neil, Joseph

Article Type: Research Article

Abstract: 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. Show more

Keywords: Spina bifida, nutrition knowledge, physical activity, food access

DOI: 10.3233/PRM-230016

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 16, no. 4, pp. 639-647, 2023

Authors: Bent, Melissa A. | Padilla, Abigail N. | Wimmer, Sam P. | Lopez, Kelleen | Beltran, Veronica | Wren, Tishya A.L.

Article Type: Research Article

Abstract: 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. Show more

Keywords: Spina bifida, pressure injury, adolescents, temperature monitoring, technology

DOI: 10.3233/PRM-230030

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 16, no. 4, pp. 649-655, 2023

Authors: Dosa, Nienke P. | Ahmed, Yakob S. | Alriksson-Schmidt, Ann | Castillo, Heidi | Contepomi, Silvana | Locastro, Mary M. | Koning, Jan | Koutsouras, George | Kutwa, Elly | Mahorta, Anjali | MacFarland, Shade | Öhrvall, Ann-Marie | O’Neill, Patricia | Overvelde, Anneloes | Peny-Dahlstrand, Marie | Shaw, Andrea | Stockman, Jessica | Tovar-Spinoza, Zulma | Castillo, Jonathan

Article Type: Brief Report

Abstract: 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. Show more

Keywords: Spina bifida, global health, interprofessional education

DOI: 10.3233/PRM-230037

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 16, no. 4, pp. 657-663, 2023

Authors: Castillo, Heidi | Locastro, Mary M. | Fremion, Ellen | Malhotra, Anjali | Morales, Rosanna | Timmons, Kelly | Jarosz, Susan | Dosa, Nienke P. | Castillo, Jonathan

Article Type: Brief Report

Abstract: 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. Show more

Keywords: Health disparities, global health, myelomeningocele, minority health, spina bifida, three-hit model, socialprescribing

DOI: 10.3233/PRM-230036

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 16, no. 4, pp. 665-674, 2023

Authors: Kelly, Maryellen S. | Sherburne, Eileen | Kerr, Joy | Payne, Colleen | Dorries, Heather | Beierwaltes, Patricia | Guerro, Adam | Thibadeau, Judy

Article Type: Article Commentary

Keywords: Spina bifida, neurogenic bowel, fecal incontinence, bowel protocol

DOI: 10.3233/PRM-230060

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 16, no. 4, pp. 675-677, 2023

Article Type: Other

DOI: 10.3233/PRM-239018

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 16, no. 4, pp. 679-681, 2023

Authors: Pico, Elaine L.

Article Type: Other

DOI: 10.3233/PRM-239019

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 16, no. 4, pp. 683-683, 2023