Journal of Pediatric Rehabilitation Medicine - Volume 13, issue 4
Purchase individual online access for 1 year to this journal.
Price: EUR 105.00
The Journal of Pediatric Rehabilitation Medicine (JPRM): An Interdisciplinary Approach Throughout the Lifespan is designed to parallel the multidisciplinary teams caring for children, adolescents and adults with childhood-onset physical disabilities and complex care needs worldwide. Published quarterly, topics include, and are not limited to, cerebral palsy, traumatic brain injury, spinal cord injury, spina bifida, limb deficiency, muscular dystrophy, stroke, cancer, developmental delays, and rare disorders. Furthermore, the journal welcomes papers dedicated to pediatric rehabilitation from a global health perspective.
The aim of JPRM is to engage a diverse group of international experts with the goal of providing readers with comprehensive information regarding children and adolescents requiring rehabilitation. JPRM brings together specialists from medicine, nursing, psychology, social work, nutrition, child life, family centered care, and occupational, physical, and speech therapy. For manuscript submissions, authorship involving at least two different specialties is encouraged, although not required, to facilitate a transdisciplinary and collaborative approach. Manuscripts are blinded and peer reviewed including biostatistical analysis. Authors are invited to submit original research, systematic and scoping reviews, guidelines, protocols, care pathways, case reports, book reviews, commentaries, editorials, and dates for future conferences.
Abstract: The COVID-19 pandemic has reminded us that, if of nothing else, we live in a globalized community. Enthusiasm for evidenced-based medical knowledge is also contagious. Just as the incidence of SARS-CoV-2, the associated coronavirus, has had a borderless impact on global public health, so too neural tube defects have widespread significance. Previously, the concept of “blue marble health” was introduced as a policy framework to illustrate trends in the geographic distribution of health disparities affecting at-risk populations that live, not only in low-income countries, but also in pockets of the populace in wealthier nations. Subsequently, the Spina Bifida Association’s Collaborative…Care Network, through a cooperative agreement with the Centers for Disease Control and Prevention, recently produced the “Guidelines for the Care of People with Spina Bifida.” While language differences, immigration, cultural beliefs, acculturation, local resources and social determinants of health, must be taken into account when these guidelines are implemented across the globe, they could not come at a more suitable time. The current digital age, as well as open access to this special issue, will ensure their ongoing wide distribution.
Keywords: Guidelines, myelomeningocele, social determinants of health, blue marble health, immigration, global health, spina bifida
Abstract: “Guidelines for the Care of People with Spina Bifida” provide the best, most up-to-date recommendations for care across the lifespan, from newborn to adult. This special issue of the Journal of Pediatric Rehabilitation Medicine is a collection of key sections of the 2018 Guidelines . The sections of the Guidelines published herein have been expanded from their original format to include more background information about key topics and why they are important in the care of people with SB. It is the hope of SBA that these and future Guidelines will promote and standardize best practice…regardless of the characteristics of individuals with SB or where their care was received. It is through providing better care that we will ultimately achieve a better future for all those living with SB.
Keywords: Spina bifida, spinal dysraphism, case management, standards
Abstract: As the diagnosis of Spina Bifida (SB) is often made prenatally, SB-specific prenatal counseling is needed. It is essential to provide information about medical care and lifelong impact of this diagnosis, treatment options available to women carrying fetuses affected, and resources that will assist in the care of individuals with SB. This article outlines the SB Prenatal Counseling Guidelines 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 prenatal counseling is warranted.
Abstract: Myelomeningocele (MMC) arises from an early neural developmental anomaly and results in a variety of structural abnormalities and associated functional neurologic deficits. As such, neurologic issues are central to virtually all clinical problems. Neurosurgical intervention strives to correct or improve these defects and prevent secondary complications. These interventions include closure of the open myelomeningocele and management (across the life span) of hydrocephalus, the Chiari II malformation (C2M) and tethered spinal cord (TSC). The development of pre-natal closure techniques and reports of improved outcome with in-utero closure (IUMC) have revolutionized the neurosurgical approach to myelomeningocele. Controversies remain surrounding patient selection, maternal…risks, technique of IUMC (endoscopic vs. open) and long-term outcomes. However, real gains include reduced rates of hydrocephalus, modestly improved motor capabilities and reduction in C2M morbidity. For many decades, the cornerstone of treatment of hydrocephalus for many decades has been the placement and support of ventricular shunts. Endoscopic third ventriculostomy (ETV) with or without choroid plexus coagulation (ETV/CPC) is an appealing alternate strategy that avoids the morbidity and complications associated with shunts. The exact criteria for ETV-CPC candidacy and best metrics for outcome analysis remain active areas of debate and controversy. Similarly, neurosurgical management C2M, has centered upon the indications and clinical thresholds for performing posterior fossa surgical decompression. Tethered spinal cord management incorporates the diagnosis and surgical management of adhesions formed at the initial closure site, the consequent longitudinal traction related stress on the cord and the resulting neurologic signs and symptoms.
Keywords: Spina bifida, myelomeningocele, intra-uterine myelomeningocele closure (IUMC), hydrocephalus, ventricular shunt, endoscopic third ventriculostomy (etv), endoscopic third ventriculostomy with choroid plexus coagulation (ETV-CPC), chiari II malformation (C2M), tethered spinal cord (TSC), health care guidelines, neural tube defects
Abstract: PURPOSE: The life expectancy for people with spina bifida has increased, thus resulting in greater need for guidelines in urologic care in order to protect normal renal function, to develop strategies for urinary continence, and to advance independence through adult years. METHODS: The English literature was assessed from 2002–2015; greater than 300 publications identified. Case reports and opinion pieces were eliminated leaving 100 for in depth review. Clinical questions were then established for each age group that allowed for focused assessment. RESULTS: There was no Level 1 evidence for any of the defined…clinical questions. This resulted in group consensus for all questions throughout all age groups. Guidelines were provided for identifying a symptomatic urinary infection, the role of urodynamic bladder testing and identification of bladder hostility, determining methods of renal function assessment and surveillance, the initiation of continence control, and transitioning to self-care through the teen and adult years. CONCLUSION: Urologic guidelines continue to be based on clinical consensus due to the lack of high level evidence-based research. Further research is required in all aspects of urologic management. While not the “Standard of Care,” these guidelines should be considered “Best Practice”.
Abstract: PURPOSE: Bowel dysfunction, such as constipation and fecal incontinence, has a significant impact on health, activities of daily living, and quality of life among people with spina bifida. Secondary complications may result from bowel dysfunction and include urologic dysfunction, loss of skin integrity, shunt (hydrocephalus) function, as well as loss of social opportunities and employability. METHODS: Using a consensus building methodology, the guidelines for management of bowel dysfunction in spina bifida were written by experts in the field of spina bifida and bowel function and care. RESULTS: The evidence-based guidelines are presented in…table format and provide age-specific recommendations to achieve fecal continence without constipation. Recommended treatments are presented from least to most invasive options. Literature supporting the recommendations and the interval research published to date is also presented. CONCLUSION: These guidelines present a standardized approach to management of bowel dysfunction in spina bifida. Bowel management in children and young adults with spina bifida is limited by variability in clinical practice and paucity of robust research in neurogenic bowel. Collaborative multi-institutional efforts are needed to overcome research barriers and provide innovative solutions.
Abstract: Care coordination is the deliberate organization of patient care activities between two or more participants (including the patient) involved in a person’s care to facilitate the appropriate delivery of health care services. Organizing care involves the marshalling of personnel and other resources needed to carry out all required patient care activities. It is often managed by the exchange of information among participants responsible for different aspects of care [1 ]. With an estimated 85% of individuals with Spina Bifida (SB) surviving to adulthood, SB specific care coordination guidelines are warranted. Care coordination (also described as case management services) is a process…that links them to services and resources in a coordinated effort to maximize their potential by providing optimal health care. However, care can be complicated due to the medical complexities of the condition and the need for multidisciplinary care, as well as economic and sociocultural barriers. It is often a shared responsibility by the multidisciplinary Spina Bifida team [2 ]. For this reason, the Spina Bifida Care Coordinator has the primary responsibility for overseeing the overall treatment plan for the individual with Spina Bifida[3 ]. Care coordination includes communication with the primary care provider in a patient’s medical home. This article discusses the Spina Bifida Care Coordination Guideline from the 2018 Spina Bifida Association’s Fourth Edition of the Guidelines for the Care of People with Spina Bifida and explores care coordination goals for different age groups as well as further research topics in SB care coordination.
Keywords: Spina Bifida, myelomeningocele, care coordination, multidisciplinary care, case management neural tube defects
Abstract: Individuals with Spina Bifida (SB) have unique lifelong medical and social needs. Thus, when considering how to promote health and offer preventive care, providers must adapt general healthcare screening and counseling recommendations to their patients’ physical and cognitive impairments along with discerning how to monitor secondary or chronic conditions common to the population. This article provides an update on the health promotion and preventive health care guidelines developed as part of the Spina Bifida Association’s fourth edition of the Guidelines for the Care of People with Spina Bifida. The guidelines highlight accommodations needed to promote general preventive health, common secondary/chronic…conditions such as obesity, metabolic syndrome, hypertension, musculoskeletal pain, and considerations for preventing acute care utilization for the SB population throughout the lifespan. Further research is needed to understand the effectiveness of preventive care interventions in promoting positive health outcomes and mitigating potentially preventable acute care utilization.
Keywords: Spina bifida, myelomeningocele, disabilities, health promotion, preventative health
Abstract: Spina bifida’s (SB) impact on cognitive, physical, and psychosocial functioning places individuals at risk for mental health concerns. This article discusses the SB Mental Health 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 SB achieve optimal mental health throughout the lifespan. Guidelines address clinical questions pertaining to the psychosocial impact of SB on mental health and adaptation, domains of mental health that are affected in individuals with SB, areas of resilience, common maladaptive behaviors that may impact…people with SB, and resources or practices that are helpful in mitigating mental health issues in this population. Gaps in the research and future directions are discussed.
Keywords: Spina Bifida, myelomeningocele, neural tube defects, mental health
Abstract: 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.
Keywords: Spina bifida, myelomeningocele, family functioning, neural tube defects
Abstract: PURPOSE: Skin-related issues have a significant impact on health, activities of daily living, and quality of life among people with spina bifida. Data presented by select clinics that participate in the National Spina Bifida Patient Registry reported that 26% of individuals had a history of pressure injuries with 19% having had one in the past year. The spina bifida community lack direct guidelines on prevention of these and other skin related issues. The Integument (skin) Guidelines focus on prevention, not treatment, of existing problems. METHODS: Using a consensus building methodology, the guidelines were written by experts…in spina bifida and wound care. RESULTS: The guidelines include age-grouped, evidence-based guidelines written in the context of an understanding of the whole person. They are presented in table format according to the age of the person with spina bifida. CONCLUSION: These guidelines present a standardized approach to prevention of skin-related issues in spina bifida. Discovering what results in successful minimization of skin-related issues with testing of technology or prevention strategies is the next step in protecting this vulnerable population.
Abstract: It is estimated that a significant percentage of individuals with spina bifida (SB) are shorter than their age-matched typical peers. Parents of children with spina bifida may ask if human growth hormone is appropriate for their child. This article discusses short stature and the use of human growth hormone among children with SB. This guideline was developed for SB Healthcare Guidelines from the 2018 Spina Bifida Association’s Fourth Edition of the Guidelines for the Care of People with Spina Bifida.
Keywords: Spina bifida, short stature, human growth hormone, obesity, myelomeningocele, neural tube defects
Abstract: Children with spina bifida are at greater risk of developing central precocious puberty (CPP) compared to others. Therefore, early recognition and timely referral for further evaluation by a pediatric endocrinologist allows appropriate management that reduces the impact of CPP. This article discusses the diagnosis and management of CPP in children with spina bifida. This guideline was developed for SB Transition Healthcare Guidelines from the 2018 Spina Bifida Association’s Fourth Edition of the Guidelines for the Care of People with Spina Bifida.
Abstract: Quality of Life (QOL) and Health-Related Quality of Life (HRQOL) are important concepts across the life span for those with spina bifida (SB). This article discusses the SB Quality of Life Healthcare Guidelines from the 2018 Spina Bifida Association’s Fourth Edition of the Guidelines for the Care of People with Spina Bifida. The focus of these QOL Guidelines was to summarize the evidence and expert opinions on how to mitigate factors that negatively impact QOL/HRQOL or enhance the factors positively related to QOL/HRQOL, the measurement of QOL/HRQOL and the gaps that need to be addressed in future research.
Keywords: Myelomeningocele, spina bifida, pain, continence, quality of life, health-related quality of life, neural tube defects
Abstract: Self-management and independence behaviors are associated with improved health conditions common to spina bifida such as skin integrity and bowel and bladder management. While most children with spina bifida ultimately achieve basic self-care behaviors, (e.g., dressing appropriately, planning activities with peers, or cooking pre-planned meals), they often lag 2–5 years behind their typically-developing peers in these activities . Valid and reliable condition-specific assessments of self-management and independence are critical to optimizing outcomes for this population. Partnerships among parents, clinicians, and youths with spina bifida are essential to implementing tailored interventions based on these assessments. The guidelines delineated in this article…are informed by current self-management research for people with spina bifida and offer recommendations to promote self-management and independence across the lifespan.
Abstract: An estimated 85% of individuals with spina bifida (SB) survive into adulthood, warranting SB-specific transition to adult healthcare guidelines to address the diverse and complex medical, adaptive, and social needs particular to this condition. Latex allergy constitutes one important health concern for this population that requires ongoing and life-long evidence-based management. This article discusses management of latex allergy according to the SB Latex Allergy Healthcare Guidelines from the 2018 Spina Bifida Association’s Fourth Edition of the Guidelines for the Care of People with Spina Bifida, reviews current care models in which such latex allergy guidelines can be implemented, and explores…further relevant research topics in SB care relative to latex allergy.
Abstract: Sexual development is not only comprised of the changes to a person’s body during puberty; it is a part of social development and should be considered in the context of basic and routine human desires for connectedness and intimacy, beliefs, values and aspirations. As is true for everyone, it is important that individuals with spina bifida have opportunities to acquire developmentally appropriate, relevant and accurate sexual health knowledge. Those with spina bifida need to be able to negotiate sexual desire, intimacy and sexual expression. They also need education about their sexual health and how to limit the negative outcomes of…sexual activity related to sexually transmitted infections, unplanned pregnancy or sexual coercion, violence, abuse or exploitation. This article discusses the Spina Bifida Sexual Health and Education Healthcare Guidelines from the 2018 Spina Bifida Association’s Fourth Edition of the Guidelines for the Care of People with Spina Bifida using the World Health Organization’s framing of sexual health and reviews the literature on sexual health and education for individuals with spina bifida.
Keywords: Spina bifida, myelomeningocele, neural tube defects, sexuality, sex education, sexual health
Abstract: The Spina Bifida Association (SBA) is the organization that represents the needs of the population with spina bifida (SB). They are tasked with advocacy, education, optimizing care, and providing a social voice for those with spina bifida. In response to the tenet of optimizing care they were tasked with developing up to date clinical care guidelines which address health care needs for those impacted by spina bifida throughout their lifespan. This article will discuss the SB Mobility Healthcare Guidelines from the 2018 Spina Bifida Association’s Fourth Edition of the Guidelines for the Care of People with Spina Bifida.
Abstract: Orthopedic or musculoskeletal problems are common in individuals with spina bifida. They can affect function and mobility and, in the case of spinal deformity, affect pulmonary function. We discuss the current treatment guidelines developed through collaboration with the Spina Bifida Association and the Orthopedics and Mobility working group using a specific methodology previously reported [1,2]. General considerations are discussed followed by evaluation and treatment guidelines for specific age ranges. References are provided where applicable, but where data is lacking treatment guidelines fall under the umbrella of clinical consensus. This leaves “research gaps” where areas of possible future study could be…considered.
Abstract: 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.
Abstract: Women and girls with spina bifida have specific health care concerns. It is essential that they, and their health care providers have access to information to help them make healthy choices throughout their lifespan. This article aims to address key aspects of health pertinent to girls and women with spina bifida and outlines the SB Women’s Health Guidelines for the Care of People with Spina Bifida. Further research into this area is needed.
Abstract: 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.
Abstract: PURPOSE: The majority of behavioral intervention technologies (BITs) have been designed and targeted towards the general population (i.e., typically-developing individuals); thus, little is known about the use of BITs to aid those with special needs, such as youth with disabilities. The current study assessed adolescents and young adults with spina bifida (AYA-SB) for: 1) their technology usage, and 2) anticipated barriers to using technology to help manage their health. METHODS: AYA-SB completed a survey of their media and technology usage. A card sorting task that ranked and grouped anticipated barriers to using a mobile app to…manage health was also completed. Ranked means, standard deviations, and the number of times a barrier was discarded were used to interpret sample rankings. RESULTS: AYA-SB reported less frequent technology and media use than the general population. However, differences emerged by age, with young adults endorsing higher usage than their younger counterparts. Top concerns focused on usability, accessibility, safety, personal barriers due to lack of engagement, technological functioning, privacy, and efficacy. CONCLUSIONS: AYA-SB appear to be selective users of technology. It is therefore critical that the design of BITs address their concerns, specifically aiming to have high usability, accessibility, and engagement.
Keywords: Spina bifida, behavioral intervention technologies, access barriers, adolescents and young adults, technology use
Abstract: PURPOSE: Neurogenic bowel dysfunction (NBD) is a common comorbidity of myelomeningocele (MMC), the most common and severe form of spina bifida. The National Spina Bifida Patient Registry (NSBPR) is a research collaboration between the CDC and Spina Bifida Clinics. Fecal continence (continence) outcomes for common treatment modalities for NBD have not been described in a large sample of individuals with MMC. NSBPR patients with MMC and NBD were studied to determine variation in continence status and their ability to perform their treatment independently according to treatment modality and individual characteristics. METHODS: Continence was defined as <…1 episode of incontinence per month. Eleven common treatments were evaluated. Inclusion criteria were established diagnoses of both MMC and NBD, as well as age ⩾ 5 years (n = 3670). Chi-square or exact statistical tests were used for bivariate analyses. Logistic regression models were used to estimate the odds of continence outcomes by age, sex, race/ethnicity, level of motor function, and insurance status. RESULTS: At total of 3670 members of the NSBPR met inclusion criteria between November 2013 and December 2017. Overall prevalence of continence was 45%. Prevalence ranged from 40–69% across different treatments. Among continent individuals, 60% achieved continence without surgery. Antegrade enemas were the most commonly used treatment and had the highest associated continence rate. Ability to carry out a treatment independently increased with age. Multivariable logistic regression showed significantly higher odds of continence among individuals aged ⩾ 12 years, female, non-Hispanic white, and with private insurance.