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Journal of Pediatric Psychology Jul 2016To examine (1) trajectories of sleep disturbances in adolescents with spina bifida (SB) compared with a typically developing (TD) group over a 10-year period and (2)...
OBJECTIVES
To examine (1) trajectories of sleep disturbances in adolescents with spina bifida (SB) compared with a typically developing (TD) group over a 10-year period and (2) individual, family, and socioeconomic determinants of changes in sleep disturbances.
METHODS
Participants were 68 families of youth with SB and 68 families of TD youth. Parent-report of adolescent sleep was collected every 2 years at 6 time points (T1: ages 8-9; T6: ages 18-19). Multiple informants and measures were used to examine internalizing, externalizing, and inattention symptoms, dyadic/family conflict, socioeconomic status (SES), and family income.
RESULTS
Sleep disturbances increased over the 10-year period. Youth with SB had greater sleep disturbances during early adolescence. Greater preadolescent externalizing symptoms, greater parent-child and marital conflict, and lower SES predicted increased sleep disturbances.
CONCLUSIONS
Sleep disturbances are common and persistent in adolescents with SB. Sleep assessment and management are important clinical and research priorities in this population.
Topics: Adolescent; Adolescent Development; Case-Control Studies; Child; Child Development; Family Conflict; Female; Humans; Longitudinal Studies; Male; Risk Factors; Sleep Wake Disorders; Socioeconomic Factors; Spinal Dysraphism
PubMed: 27118272
DOI: 10.1093/jpepsy/jsw021 -
BMC Pediatrics Jun 2023Children with spina bifida (SB) may have congenital or acquired foot deformities due to neurological defects in the spinal cord. As the musculoskeletal system keeps...
BACKGROUND
Children with spina bifida (SB) may have congenital or acquired foot deformities due to neurological defects in the spinal cord. As the musculoskeletal system keeps growing, foot deformities can develop or become aggravated. Thus, healthcare providers should provide constant monitoring and proper orthopedic management. Since foot deformities can affect not only the gait but also the daily life of children with SB, it is necessary to investigate the impact of foot deformities on everyday life. The purpose of this study was to examine the relationship between foot deformity and health-related quality of life (HRQoL) among independently ambulating children with SB.
METHODS
This cross-sectional study examined the associations between foot deformity and HRQoL using two patient-reported outcome measures (Oxford Ankle Foot Questionnaire, Pediatric Outcomes Data Collection Instrument) in 93 children with SB aged 7-18 years between January 2020 and July 2021.
RESULTS
Children with foot deformity (n = 54) reported lower scores in all subscales (physical, school and play, emotional, and footwear) of the Oxford Ankle Foot Questionnaire for children than those without foot deformity (n = 39; p < 0.001). Additionally, in terms of the Pediatric Outcomes Data Collection Instrument, children with foot deformity also reported poorer scores in four subscales (transfer and basic mobility, sports and physical functioning, comfort and pain, happiness with physical functioning; p < 0.001) than those without foot deformity, whereas upper extremity functioning was not significantly affected. Children with foot deformities, particularly those with bilateral foot deformities, equinus deformities, or mixed deformities, which are different types of right and left foot deformities, have a lower perceived HRQoL (p < 0.05).
CONCLUSIONS
Among independently ambulating children with SB, those with foot deformities showed lower HRQoL. Moreover, children with foot deformities tend to have other clinical problems, including bladder and bowel dysfunction. Therefore, orthopedic management should consider the multifaceted factors that affect children's daily life and HRQoL.
Topics: Child; Humans; Quality of Life; Cross-Sectional Studies; Foot Deformities; Surveys and Questionnaires; Spinal Dysraphism
PubMed: 37277711
DOI: 10.1186/s12887-023-04100-3 -
Postgraduate Medical Journal Jan 1995Recent advances in medical technology enable many children with complex disabilities to survive into adulthood and to have certain expectations of life. One of these...
Recent advances in medical technology enable many children with complex disabilities to survive into adulthood and to have certain expectations of life. One of these expectations is the continuity of specialist health care in an adult setting. This paper describes a new out-patient service which aims to provide optimum care, continuity and consistency of service for adults with spina bifida and/or hydrocephalus. The need for specialist health input into this service, in order to monitor the neurological, urological and psychosocial complications often associated with spina bifida and/or hydrocephalus is recognised. In one year (1992), 86 young adults with spina bifida and/or hydrocephalus attended for annual or more frequent assessment, either independently or with their families or carers. A variety of health and social problems were treated. In response to demand, a multi-disciplinary assessment unit, which includes the services of both medical and nursing specialists, occupational and physiotherapists, psychologists and access to specialist surgical opinions has recently opened at the Chelsea and Westminster Hospital. This new service attempts to meet some of the needs described in the outpatient audit. Adults with other disabilities are requesting to use this service. A longitudinal study to monitor quality, and outcome is indicated from this initial survey.
Topics: Adult; Ambulatory Care; Behavior; Continuity of Patient Care; Female; Hospitalization; Humans; Hydrocephalus; London; Male; Outpatient Clinics, Hospital; Patient Acceptance of Health Care; Patient Care Team; Patient Satisfaction; Spinal Dysraphism; Urinary Incontinence
PubMed: 7708585
DOI: 10.1136/pgmj.71.831.17 -
Annals of Physical and Rehabilitation... Mar 2023Scoliosis develops in a proportion of children with myelomeningocele; however, little is known about scoliosis in adulthood and in other forms of spina bifida (SB).
BACKGROUND
Scoliosis develops in a proportion of children with myelomeningocele; however, little is known about scoliosis in adulthood and in other forms of spina bifida (SB).
OBJECTIVES
The aims of this study were to describe the prevalence of scoliosis and identify risk factors for its development in a large cohort of adults with open and closed SB.
METHODS
This was a cross-sectional study of data from 580 adults with SB attending their first consultation at a French multidisciplinary referral centre for SB. Sex, anatomical location and type of SB (open or closed), neurological level, back pain and ambulatory status (new Functional Ambulation Classification [new FAC]) were compared in adults with and without scoliosis. These characteristics were used to determine scoliosis risk factors.
RESULTS
In total, 331 adults fulfilled the inclusion criteria: 221 had open and 110 had closed SB. Of these, 176 (53%) had scoliosis: 57% open and 45% closed SB. As compared with individuals without scoliosis, those with scoliosis more frequently had open SB (p=0.03), more cranially located SB (p<0.0001), more severe neurological deficits (p≤0.02) and poorer walking ability (mean new FAC score 3.5 [SD 3.3] vs 6.1 [2.6], [p<0.0001]). In total, 69% had chronic back pain, with no difference in frequency between those with and without scoliosis. The odds of scoliosis was associated with asymmetrical motor level and a new FAC score <4 (odds ratio 0.46, p<0.006, and 0.75, p<0.0001, respectively).
CONCLUSION
About half of adults with open and closed SB had scoliosis. Back pain was frequent in those both with and without scoliosis. Individuals with low walking ability and an asymmetrical motor level should be monitored early and continuously to limit the consequences of scoliosis during their lifetime. A major issue is to determine how scoliosis evolves and to determine appropriate monitoring and treatment strategies for individuals at risk.
Topics: Child; Humans; Adult; Spina Bifida Cystica; Scoliosis; Cross-Sectional Studies; Prevalence; Spinal Dysraphism; Risk Factors
PubMed: 35717001
DOI: 10.1016/j.rehab.2022.101685 -
Journal of Developmental and Behavioral... Apr 2022This study examined the transition to adult health care for individuals with spina bifida (SB) and explored demographic and relational associations with transition...
OBJECTIVE
This study examined the transition to adult health care for individuals with spina bifida (SB) and explored demographic and relational associations with transition status.
METHOD
Young adults with SB (18-30; n = 326) were recruited to complete an anonymous, online survey. Frequencies of reported experiences, behaviors, and satisfaction with the transition to adult health care were examined. Nonparametric tests and exploratory hierarchical regressions were used to examine demographic and relational factors with physicians between those (1) who had and had not yet transitioned and (2) who did and did not return to pediatric care.
RESULTS
Most of the sample reported having transitioned to adult health care, with three-quarters reporting that their primary physician is an adult primary care doctor. Individuals who had transitioned were more likely to be younger (p = 0.01) and to not have a shunt (p = 0.003). Beyond the effect of age and shunt status, relational factors with pediatric providers were not associated with transition (p > 0.1). After transition, over one-third reported returning to a pediatric provider. Those who did not return to pediatric care were more likely to have myelomeningocele, be a full-time student, and to not have a shunt (p < 0.001). Beyond the effect of age and shunt status, lower ratings of communication with adult providers were associated with a return to pediatric care (p = 0.04).
CONCLUSION
The results highlight the need for additional research about barriers and facilitators to the transition to adult health care to target interventions that support this critical milestone in young adults with SB.
Topics: Child; Delivery of Health Care; Demography; Humans; Physicians; Spinal Dysraphism; Transition to Adult Care; Young Adult
PubMed: 34570065
DOI: 10.1097/DBP.0000000000001001 -
Journal of Pediatric Rehabilitation... 2020Orthopedic or musculoskeletal problems are common in individuals with spina bifida. They can affect function and mobility and, in the case of spinal deformity, affect... (Review)
Review
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.
Topics: Adolescent; Adult; Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Male; Musculoskeletal Diseases; Orthopedics; Practice Guidelines as Topic; Spinal Dysraphism; Young Adult
PubMed: 33252095
DOI: 10.3233/PRM-200750 -
Developmental Disabilities Research... 2010Spina bifida myelomeningocele (SBM) is a specific type of neural tube defect whereby the open neural tube at the level of the spinal cord alters brain development during... (Review)
Review
Spina bifida myelomeningocele (SBM) is a specific type of neural tube defect whereby the open neural tube at the level of the spinal cord alters brain development during early stages of gestation. Some structural anomalies are virtually unique to individuals with SBM, including a complex pattern of cerebellar dysplasia known as the Chiari II malformation. Other structural anomalies are not necessarily unique to SBM, including altered development of the corpus callosum and posterior fossa. Within SBM, tremendous heterogeneity is reflected in the degree to which brain structures are atypical in qualitative appearance and quantitative measures of morphometry. Hallmark structural features of SBM include overall reductions in posterior fossa and cerebellum size and volume. Studies of the corpus callosum have shown complex patterns of agenesis or hypoplasia along its rostral-caudal axis, with rostrum and splenium regions particularly susceptible to agenesis. Studies of cortical regions have demonstrated complex patterns of thickening, thinning, and gyrification. Diffusion tensor imaging studies have reported compromised integrity of some specific white matter pathways. Given equally complex ocular motor, motor, and cognitive phenotypes consisting of relative strengths and weaknesses that seem to align with altered structural development, studies of SBM provide new insights to our current understanding of brain structure-function associations.
Topics: Arnold-Chiari Malformation; Brain; Cerebellum; Corpus Callosum; Humans; Magnetic Resonance Imaging; Meningomyelocele; Nerve Net; Neural Pathways; Spinal Dysraphism
PubMed: 20419768
DOI: 10.1002/ddrr.88 -
Disability and Health Journal Jul 2019More children with spina bifida (SB) are surviving into adulthood. Unfortunately, little data exist regarding the economic implications of modern SB care.
BACKGROUND
More children with spina bifida (SB) are surviving into adulthood. Unfortunately, little data exist regarding the economic implications of modern SB care.
OBJECTIVE
We examined economic data from two national databases to estimate the annual nationwide hospital and emergency charges of SB from 2006-14.
METHODS
We analyzed the 2006-2014 Nationwide Inpatient Sample (NIS) and Nationwide Emergency Department Sample (NEDS). SB patients were defined using ICD-9-CM codes. Demographic and charge data were obtained from each database. Multiple imputation was used to estimate missing data (1.6% for NIS and 22% in NEDS). The principal outcomes were mean, median, and total charges for encounters each year.
RESULTS
There were 725,646 encounters for individuals with SB between 2006 and 2014. The average age of captured SB patients who were admitted to a hospital or seen in an ER was 29 years. In 2014, the median charge for inpatient encounters was $31,071 (IQR: $15,947, $63,063) and for ER encounters was $2407.02 (IQR: $1321.91, $4211.35). In total, the sum of charges from all SB-related admissions in 2014 was $1,862,016,217 (95% CI: $1.69 billion, $2.03 billion), while the sum of charges of all SB-related ER encounters in 2014 was $176,843,522 (95% CI: $158 million, $196 million). There was a steady increase in charges over the study period.
CONCLUSION
Charges for SB-related inpatient and emergency care in the US in 2014 was in excess of $2 billion in contrast to $1.2 billion in 2006, after adjusting for inflation; this is an impressively high figure for a relatively small number of patients.
Topics: Adolescent; Adult; Child; Child, Preschool; Disabled Persons; Emergency Service, Hospital; Female; Forecasting; Hospitalization; Humans; Infant; Male; Pediatrics; Spinal Dysraphism; United States
PubMed: 30711573
DOI: 10.1016/j.dhjo.2019.01.007 -
Developmental Medicine and Child... Jul 2016To evaluate the prevalence of organ system disorders and describe healthcare utilization among adults with spina bifida at a regional clinic.
AIM
To evaluate the prevalence of organ system disorders and describe healthcare utilization among adults with spina bifida at a regional clinic.
METHOD
This study was a structured chart review using the Rochester Health Status Survey-IV. 65 males, 57 females aged 16 to 59 years were seen at the Spina Bifida Center of Central New York between January 2007 and December 2008 (annual hospitalization rate was 15 out of 100).
RESULTS
Hospitalizations and acute outpatient visits were associated with having shunted hydrocephalus, whereas visits to the emergency department were associated with having a decubitus ulcer. Logistic regression models revealed that older adults made proportionately fewer visits to primary care providers than younger adults (odds ratio 0.919; p=0.02). Yet for every 1-year increase in age, the odds of being hospitalized increased by 5% (odds ratio 1.051; p=0.03).
INTERPRETATION
Adults with spina bifida have multiple organ-system disorders. They have greater difficulty accessing services, and utilize emergency and inpatient healthcare at higher rates than the general population. In the future, adults with spina bifida will require access to more medical care and preventive services if they are to have optimal health, well-being, and functioning.
Topics: Adolescent; Adult; Cerebrospinal Fluid Shunts; Cognition Disorders; Delivery of Health Care; Female; Health Status; Health Surveys; Hospitalization; Humans; Hydrocephalus; Male; Middle Aged; Prevalence; Sex Factors; Spinal Dysraphism; Young Adult
PubMed: 26526690
DOI: 10.1111/dmcn.12952 -
Journal of Nursing Scholarship : An... Mar 2021This article focuses on the transition to adult health care in youth with spina bifida (SB) from the perspective of theory, measurement, and interventions.
PURPOSE
This article focuses on the transition to adult health care in youth with spina bifida (SB) from the perspective of theory, measurement, and interventions.
METHODS
The purpose of this article is to discuss (a) a theory of linkages between the transfer of medical responsibility from parent to child and the transition from pediatric to adult health care, as mediated by transition readiness; (b) measurement issues in the study of self-management and the transition to adult health care; and (c) U.S.-based and international interventions focused on the transition to adult health care in young adults with SB.
FINDINGS
Individuals with SB must adhere to a complex multicomponent treatment regimen while at the same time managing a unique array of cognitive and psychosocial challenges and comorbidities that hinder self-management, medical adherence, and the transition to adult health care. Moreover, such youth endure multiple transitions to adult health care (e.g., in the areas of urology, orthopedics, neurosurgery, and primary care) that may unfold across different time frames. Finally, three transition-related constructs need to be assessed, namely, transition readiness, transition completion, and transition success.
CONCLUSIONS
SB provides an important exemplar that highlights the complexities of conducting research on the transition to adult health care in youth with chronic health conditions. Many transition trajectories are possible, depending on the functioning level of the child and a host of other factors. Also, no single transition pathway is optimal for all patients with SB.
CLINICAL RELEVANCE
The success of the process by which a child with SB transitions from pediatric to adult health care can have life-sustaining implications for the patient.
Topics: Adolescent; Child; Humans; Models, Theoretical; Self-Management; Spinal Dysraphism; Transition to Adult Care; Young Adult
PubMed: 33482054
DOI: 10.1111/jnu.12626