-
The Pan African Medical Journal 2022
Topics: Humans; Spinal Dysraphism; Meningomyelocele
PubMed: 36338552
DOI: 10.11604/pamj.2022.42.258.35894 -
Pediatrics Sep 2018Patients with spina bifida (SB) typically develop serious secondary conditions and undergo surgical procedures related to neurologic disorders, orthopedic abnormalities,...
BACKGROUND AND OBJECTIVES
Patients with spina bifida (SB) typically develop serious secondary conditions and undergo surgical procedures related to neurologic disorders, orthopedic abnormalities, bladder and bowel dysfunction, and skin breakdown. In this study, we describe the age distribution of common surgical procedures and health outcomes in patients with SB.
METHODS
Using serial cross-sectional data from the National Spina Bifida Patient Registry (2009-2013; = 4664), we examined surgical procedures (gastrointestinal, neurologic, orthopedic, skin, urologic, and other) and health outcomes (fecal continence, urinary continence, skin breakdown, and ambulation status) of patients with SB by age and SB type (myelomeningocele and nonmyelomeningocele).
RESULTS
All patients who were enrolled had available health outcome data, and 81.5% ( = 3801) of patients had complete surgical procedure data, which totaled 18 891 procedures across their lifetimes. Almost all procedures (91.4%) occurred among participants with myelomeningocele SB. For both types of SB, the distribution of procedures varied by age. The most frequent procedures were neurologic, with approximately half (53%) occurring in patients <1 year of age; orthopedic and urologic procedures followed in frequency but tended to occur at older ages. The health outcomes for patients with myelomeningocele SB revealed lower frequencies of positive health outcomes than those for patients with nonmyelomeningocele SB across all age groups. Overall, the rates of fecal and urinary continence and skin breakdown increased with age whereas the ability to ambulate declined with age.
CONCLUSIONS
Understanding the surgical procedures and health outcome variations by age and SB type can help clinicians and populations that are affected set expectations regarding the occurrence of these procedures and the outcomes throughout the patients' life spans.
Topics: Adolescent; Adult; Child; Child, Preschool; Cross-Sectional Studies; Female; Humans; Infant; Male; Registries; Spinal Dysraphism; Treatment Outcome; Young Adult
PubMed: 30158199
DOI: 10.1542/peds.2017-3730 -
Birth Defects Research Aug 2019The National Spina Bifida Patient Registry (NSBPR) was established in 2008, as a partnership between the CDC and spina bifida (SB) clinics throughout the United States.... (Review)
Review
BACKGROUND
The National Spina Bifida Patient Registry (NSBPR) was established in 2008, as a partnership between the CDC and spina bifida (SB) clinics throughout the United States. The purpose of this study is to explore the initial work of this project through a literature review of published studies from the NSBPR and provide a description of how this body of literature has developed overtime.
METHODS
We included studies indexed in MEDLINE by means of PubMed from January 2009 through April 2018. Included articles were appraised to identify key themes relevant to SB demographics, interventions, and outcomes. Additionally, information regarding objectives, hypotheses, and results of each study was summarized.
RESULTS
We identified 13 articles meeting inclusion criteria. These publications described findings or explored associations using NSBPR variables. They were grouped into four categories: general characteristics (4 studies), mobility and skin injury (2 studies), bowel continence (3 studies), and bladder continence (5 studies).
CONCLUSIONS
The NSBPR represents one of the largest described clinical samples of individuals living with SB. The first decade of studies have focused primarily on descriptive analyses and on identifying predictors of clinical outcomes. These initial results may help develop interventions (including culturally appropriate initiatives), be a resource for developing international evidence-based standards of care and best-practices, and lead to improved outcomes for individuals living with SB globally. Additionally, the results underscore the strengths of the NSBPR's design and highlight the potential breadth of research topics that could be addressed in the future.
Topics: Humans; Registries; Spinal Dysraphism; United States
PubMed: 30403011
DOI: 10.1002/bdr2.1407 -
Journal of Pediatric Rehabilitation... 2023This article provides an update to the 2018 Spina Bifida Association's Transition to Adult Care Guidelines. (Review)
Review
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.
Topics: Young Adult; Humans; Quality of Life; Transition to Adult Care; Spinal Dysraphism; Self-Management; Chronic Disease
PubMed: 38160373
DOI: 10.3233/PRM-230052 -
Fetal Diagnosis and Therapy 2015
Topics: Animals; Fetal Diseases; Fetal Therapies; Humans; Prenatal Diagnosis; Spinal Dysraphism
PubMed: 25721029
DOI: 10.1159/000375329 -
Journal of Pediatric Rehabilitation... 2020While the neuropsychological profile for individuals with Spina Bifida (SB) can vary, often certain patterns of strengths and weaknesses are evident across the lifespan.... (Review)
Review
While the neuropsychological profile for individuals with Spina Bifida (SB) can vary, often certain patterns of strengths and weaknesses are evident across the lifespan. Understanding variability related to neural structure, genetics, ethnicity, and the environment is key to understanding individual differences in outcomes and can be vital in planning interventions and tracking progress. This article outlines the SB Guideline for the Neuropsychological Care of People with Spina Bifida from the 2018 Spina Bifida Association's Fourth Edition of the Guidelines for the Care of People with Spina Bifida and acknowledges that further research in SB neurocognitive profiles is warranted.
Topics: Adolescent; Adult; Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Male; Nervous System Diseases; Practice Guidelines as Topic; Spinal Dysraphism; Young Adult
PubMed: 33285647
DOI: 10.3233/PRM-200761 -
The Orthopedic Clinics of North America Jul 2018Obesity is a common problem in children and adolescents with neuromuscular disease. The available literature on obesity in cerebral palsy, spina bifida, and Duchenne... (Review)
Review
Obesity is a common problem in children and adolescents with neuromuscular disease. The available literature on obesity in cerebral palsy, spina bifida, and Duchenne muscular dystrophy as it relates to orthopedic treatment is reviewed, including the demographics and measurement of obesity as well as the mechanisms of obesity in these individuals. In addition, the effect of obesity on function, patient evaluation, and orthopedic treatment are reviewed.
Topics: Cerebral Palsy; Humans; Muscular Dystrophy, Duchenne; Neuromuscular Diseases; Obesity; Spinal Dysraphism
PubMed: 29929714
DOI: 10.1016/j.ocl.2018.02.006 -
Journal of Pediatric Rehabilitation... 2020Nutritional challenges and a lack of activity can lead to health problems across the lifespan for people with spina bifida. Children and adults with spina bifida are... (Review)
Review
Nutritional challenges and a lack of activity can lead to health problems across the lifespan for people with spina bifida. Children and adults with spina bifida are also at greater risk of being classified as overweight or obese compared to their peers without the condition. Therefore, early recognition of nutrition problems, weight management counseling, and timely referrals for evaluation and management of diet and activity can help those with spina bifida and their families achieve a healthy lifestyle. This article details the development of the Nutrition, Metabolic Syndrome and Obesity Guidelines, which are part of the 2018 Spina Bifida Association's Fourth Edition of the Guidelines for the Care of People with Spina Bifida. It discusses the identification and management of poor nutrition and prevention of obesity for children, adolescents, and adults with spina bifida and highlights areas requiring further research.
Topics: Adolescent; Adult; Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Male; Metabolic Syndrome; Nutritional Physiological Phenomena; Obesity; Practice Guidelines as Topic; Spinal Dysraphism; Young Adult
PubMed: 33325412
DOI: 10.3233/PRM-200753 -
The Urologic Clinics of North America May 2024The health care needs children with spina bifida evolve over their lifetime; continued, regular contact with appropraitely trained, multidisciplinary providers is... (Review)
Review
The health care needs children with spina bifida evolve over their lifetime; continued, regular contact with appropraitely trained, multidisciplinary providers is crucial to a patient's health and quality of life. Substantial research has been conducted to improve the transition process starting at an early age; however, there continue to be strong barriers to successful transition. This article reviews key aspects of the care of patients with spina bifida, the impact of inadequate transition to adult care, barriers to transition, and offers a potential vision for the future.
Topics: Adult; Child; Humans; Transitional Care; Quality of Life; Spinal Dysraphism
PubMed: 38609191
DOI: 10.1016/j.ucl.2024.01.006 -
Journal of Rehabilitation Medicine Sep 2021Individuals with spina bifida often have cognitive impairments leading to dif-ficulties in education and daily activities. The aims of this study were to explore...
BACKGROUND AND OBJECTIVE
Individuals with spina bifida often have cognitive impairments leading to dif-ficulties in education and daily activities. The aims of this study were to explore cognitive impairments in adults with spina bifida and to consider associations between impairments, educational outcome and per-formance of daily activities, comparing individuals with and without intellectual disability.
METHODS
Data were collected on 35 adults with spina bifida via cognitive tests and Assessment of Motor and Process Skills (AMPS). Participants were divided into 3 groups: individuals without intellectual disability who completed compulsory education (NID-C); those without intellectual disability, who failed to successfully pass compulsory education (NID-F); and those with intellectual disability failed to successfully pass compulsory education (ID-F).
RESULTS
All individuals with intellectual disability failed to successfully pass compulsory education (group ID-F) and had poorer scores across almost all measures than group NID-F and significantly poorer scores than group NID-C. All except 6 individuals scored below cut-off levels for effort and safety on both AMPS motor and process scales; more significant associations were seen between the cognitive tests and the motor rather than process scale.
CONCLUSION
Cognitive impairments, irrespective of intellectual disability, impact on the performance of eve-ryday activities and on educational achievement, and thus need to be considered in assessments and inter-ventions to improve outcomes and promote independence in people with spina bifida.
Topics: Activities of Daily Living; Adult; Cognition; Humans; Neuropsychological Tests; Spinal Dysraphism
PubMed: 34448489
DOI: 10.2340/16501977-2868