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Developmental Medicine and Child... Oct 2021To measure resilience and identify associated demographic and clinical factors in individuals with spina bifida.
AIM
To measure resilience and identify associated demographic and clinical factors in individuals with spina bifida.
METHOD
An anonymous survey was distributed via Facebook advertising to individuals with congenital urological conditions. Respondents 18 years or older with spina bifida were included in this study. Resilience was measured with the 10-item Connor-Davidson Resilience Scale. Mean resilience levels in the study population and a US general population sample were compared with Student's t-test. Multiple linear regression assessed demographic and clinical factors associated with resilience.
RESULTS
The mean resilience score for participants (n=195; 49 males, 146 females; mean age 40y 2mo [SD 12y 7mo] range 18-74y) was 27.2 (SD 7.5), which differed from a mean of 31.8 (SD 5.4) for a US general population sample (p<0.01). Multiple linear regression demonstrated significant positive associations between resilience and older age (p=0.04), prior urological surgeries (p=0.03), higher household education (p<0.01), and higher physical function (p<0.01).
INTERPRETATION
Resilience in individuals with spina bifida is moderately poor, relative to the general population, and is associated with certain demographic and clinical factors. As a modifiable construct with positive effects on quality of life, psychological well-being, and health-related behaviors, resilience is a promising target for intervention in individuals with spina bifida. What this paper adds Resilience in individuals with spina bifida is moderately poor. Resilience is lower in individuals with spina bifida than the general population. Resilience is associated with age, household education, physical function, and urological surgery.
Topics: Adolescent; Adult; Age Factors; Aged; Educational Status; Family Characteristics; Female; Humans; Male; Middle Aged; Physical Functional Performance; Resilience, Psychological; Spinal Dysraphism; Surveys and Questionnaires; Urologic Surgical Procedures; Young Adult
PubMed: 33987844
DOI: 10.1111/dmcn.14919 -
Journal of Pediatric Rehabilitation... 2020Self-management and independence behaviors are associated with improved health conditions common to spina bifida such as skin integrity and bowel and bladder management.... (Review)
Review
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 [1]. 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.
Topics: Adolescent; Adult; Child; Child, Preschool; Female; Functional Status; Humans; Infant; Male; Practice Guidelines as Topic; Self-Management; Spinal Dysraphism; Young Adult
PubMed: 33252094
DOI: 10.3233/PRM-200734 -
Sexual Medicine Reviews Apr 2021As most adults with spina bifida are either sexually active or interested in becoming sexually active, providers should understand how spina bifida impacts sexual... (Review)
Review
INTRODUCTION
As most adults with spina bifida are either sexually active or interested in becoming sexually active, providers should understand how spina bifida impacts sexual function and options for treatment.
OBJECTIVES
The objective of this study is to summarize the current literature describing how features of spina bifida impact sexual function in men and women, effective available treatment options for sexual dysfunction, and to identify research gaps.
METHODS
Searches were conducted in PubMed, CINAHL Complete, PsychInfo, Cochrane Central, Scopus, and Web of Science Core Collection databases using keywords related to spina bifida and sexual function. 34 primary research studies were included.
RESULTS
Most men (56-96%) can achieve an erection, although it may be insufficient for penetration. Although 50-88% ejaculate, it is often dripping, retrograde, or insensate. Twenty percent to 67% achieve orgasm. Generally, men with lower lesions and intact sacral reflexes have better outcomes, although some men with all levels of lesion report good function. Sildenafil is efficacious at treating erectile dysfunction for most men. The "TO-MAXimize sensation, sexuality, and quality of life" procedure may improve sexual function in selected men with low-level lesions. Female sexual function and treatment is less well understood. Women may experience decreased arousal, difficulties with orgasm, and pain. No treatment has been studied in women. Bowel and bladder incontinence during intercourse appears to be bothersome to men and women. Although both men and women have diminished sexual satisfaction, their sexual desire appears to be least impacted. Present studies are limited by studies' small, heterogeneous populations, the misuse of validated questionnaires in the sexually inactive population, and the lack of a validated questionnaire specific to people with spina bifida.
CONCLUSIONS
Spina bifida impacts the sexual function of both men and women. Future studies should seek a better understanding of female sexual function and treatment, use validated questionnaires appropriately, and ultimately create a validated sexual function questionnaire specific to this population. Streur CS, Corona L, Smith JE, et al. Sexual Function of Men and Women With Spina Bifida: A Scoping Literature Review. Sex Med Rev 2021;9:244-266.
Topics: Adult; Female; Humans; Male; Quality of Life; Sexual Behavior; Sexual Dysfunction, Physiological; Sexuality; Spinal Dysraphism
PubMed: 33608247
DOI: 10.1016/j.sxmr.2020.09.001 -
Child's Nervous System : ChNS :... Mar 2023Gut microbiota has recently been recognized to be influenced by a broad range of pathologies. Alterations of gut microbiota are known as dysbiosis and have found to be...
PURPOSE
Gut microbiota has recently been recognized to be influenced by a broad range of pathologies. Alterations of gut microbiota are known as dysbiosis and have found to be related to chronic constipation, a condition which affects also pediatric patients with spina bifida (SB).
METHODS
In this study, gut microbiota richness and composition were investigated by 16S rRNA sequencing and bioinformatic analysis in 48 SB patients (mean age, 11.9 ± 4.8 years) with secondary neurogenic constipation and 32 healthy controls (mean age, 18.0 ± 9.6 years). The study also aimed at exploring eventual effects of laxatives and transanal irrigation (TAI) adopted by SB subjects to get relief from the symptoms of neurogenic constipation.
RESULTS
Collected data demonstrated that the microbiota richness of SB patients was significantly increased compared to healthy controls, with a higher number of dominant bacteria rather than rare species. The absence of SB condition was associated with taxa Coprococcus 2, with the species C. eutactus and Roseburia, Dialister, and the [Eubacterium] coprostanoligenes group. On the other hand, the SB patients displayed a different group of positively associated taxa, namely, Blautia, Collinsella, Intestinibacter, and Romboutsia genera, the [Clostridium] innocuum group, and Clostridium sensu stricto 1. Bifidobacterium and the [Eubacterium] hallii group were also found to be positively associated with SB gut microbiome.
CONCLUSIONS
Among SB patients, the administration of laxatives and TAI did not negatively affect gut microbiota diversity and composition, even considering long-term use (up to 5 years) of TAI device.
Topics: Humans; Child; Adolescent; Young Adult; Adult; Neurogenic Bowel; Gastrointestinal Microbiome; RNA, Ribosomal, 16S; Laxatives; Spinal Dysraphism; Constipation
PubMed: 36180597
DOI: 10.1007/s00381-022-05688-0 -
Birth Defects Research May 2021Epidemiologists have consistently observed associations between prepregnancy obesity and spina bifida in offspring. Most studies, however, used self-reported body mass...
BACKGROUND
Epidemiologists have consistently observed associations between prepregnancy obesity and spina bifida in offspring. Most studies, however, used self-reported body mass index (potential for exposure misclassification) and incompletely ascertained cases of spina bifida among terminations of pregnancy (potential for selection bias). We conducted a quantitative bias analysis to explore the potential effects of these biases on study results.
METHODS
We included 808 mothers of fetuses or infants with spina bifida (case mothers) and 7,685 mothers of infants without birth defects (control mothers) from a population-based case-control study, the National Birth Defects Prevention Study (1997-2011). First, we performed a conventional epidemiologic analysis, adjusting for potential confounders using logistic regression. Then, we used 5,000 iterations of probabilistic bias analysis to adjust for the combination of confounding, exposure misclassification, and selection bias.
RESULTS
In the conventional confounding-adjusted analysis, prepregnancy obesity was associated with spina bifida (odds ratio 1.4, 95% confidence interval: 1.2, 1.7). In the probabilistic bias analysis, we tested nine different models for the combined effects of confounding, exposure misclassification, and selection bias. Results were consistent with a weak to moderate association between prepregnancy obesity and spina bifida, with the median odds ratios across the nine models ranging from 1.1 to 1.4.
CONCLUSIONS
Given our assumptions about the occurrence of bias in the study, our results suggest that exposure misclassification, selection bias, and confounding do not completely explain the association between prepregnancy obesity and spina bifida.
Topics: Bias; Body Mass Index; Case-Control Studies; Female; Humans; Odds Ratio; Pregnancy; Spinal Dysraphism
PubMed: 33605566
DOI: 10.1002/bdr2.1877 -
Journal of Pediatric Rehabilitation... 2020The 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....
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.
Topics: COVID-19; Disease Management; Global Health; Guidelines as Topic; Humans; Longevity; Pandemics; Quality of Life; SARS-CoV-2; Spinal Dysraphism
PubMed: 33325406
DOI: 10.3233/PRM-200029 -
PloS One 2021A systematic review was conducted in high-income country settings to analyse: (i) spina bifida neonatal and IMRs over time, and (ii) clinical and socio-demographic... (Meta-Analysis)
Meta-Analysis
OBJECTIVES
A systematic review was conducted in high-income country settings to analyse: (i) spina bifida neonatal and IMRs over time, and (ii) clinical and socio-demographic factors associated with mortality in the first year after birth in infants affected by spina bifida.
DATA SOURCES
PubMed, Embase, Ovid, Web of Science, CINAHL, Scopus and the Cochrane Library were searched from 1st January, 1990 to 31st August, 2020 to review evidence.
STUDY SELECTION
Population-based studies that provided data for spina bifida infant mortality and case fatality according to clinical and socio-demographical characteristics were included. Studies were excluded if they were conducted solely in tertiary centres. Spina bifida occulta or syndromal spina bifida were excluded where possible.
DATA EXTRACTION AND SYNTHESIS
Independent reviewers extracted data and assessed their quality using MOOSE guideline. Pooled mortality estimates were calculated using random-effects (+/- fixed effects) models meta-analyses. Heterogeneity between studies was assessed using the Cochrane Q test and I2 statistics. Meta-regression was performed to examine the impact of year of birth cohort on spina bifida infant mortality.
RESULTS
Twenty studies met the full inclusion criteria with a total study population of over 30 million liveborn infants and approximately 12,000 spina bifida-affected infants. Significant declines in spina bifida associated infant and neonatal mortality rates (e.g. 4.76% decrease in IMR per 100, 000 live births per year) and case fatality (e.g. 2.70% decrease in infant case fatality per year) were consistently observed over time. Preterm birth (RR 4.45; 2.30-8.60) and low birthweight (RR 4.77; 2.67-8.55) are the strongest risk factors associated with increased spina bifida infant case fatality.
SIGNIFICANCE
Significant declines in spina bifida associated infant/neonatal mortality and case fatality were consistently observed, advances in treatment and mandatory folic acid food fortification both likely play an important role. Particular attention is warranted from clinicians caring for preterm and low birthweight babies affected by spina bifida.
Topics: Female; Humans; Infant; Infant Mortality; Infant, Newborn; Pregnancy; Premature Birth; Spinal Dysraphism
PubMed: 33979363
DOI: 10.1371/journal.pone.0250098 -
Pediatrics International : Official... Jan 2022Patients with spina bifida suffer from motor paralysis and sensory disturbance, secondary deformation of the lower extremities, and development of decubitus ulcers. A...
BACKGROUND
Patients with spina bifida suffer from motor paralysis and sensory disturbance, secondary deformation of the lower extremities, and development of decubitus ulcers. A deep understanding of one's body, such as identifying the names, functions, relationships, homology (e.g. upper and lower limbs) and relative position of body parts, may prevent secondary disorders. The awareness of the body and its characteristics in children with spina bifida has not been fully elucidated. This study aimed to investigate how children with spina bifida perceive their bodies, especially their paralyzed parts and related or homologous ones.
METHODS
Participants included 36 children with spina bifida and 14 control children, aged 5-11 years. They were asked to draw self-portraits, and to answer questions about the names of body parts. The number of drawn body parts in the pictures and the correct response rates to the questions were quantified and compared between children with spina bifida and control children.
RESULTS
Children with spina bifida drew fewer hands, legs, and feet than control children. In the verbal tests, children with spina bifida had a lower correct response rate than control children on questions concerning the upper limbs, hands, trunk, and feet.
CONCLUSIONS
Children with spina bifida have diminished visuospatial and lexical-semantic body knowledge of the paralyzed parts and related organs.
Topics: Child; Humans; Semantics; Spinal Dysraphism
PubMed: 33780073
DOI: 10.1111/ped.14713 -
Urology Oct 2021To conduct a systematic review of self-reported experiences of sexual function and dysfunction in individuals with spina bifida (SB).
OBJECTIVE
To conduct a systematic review of self-reported experiences of sexual function and dysfunction in individuals with spina bifida (SB).
MATERIALS AND METHODS
Medline, Embase, and Web of Science were systematically searched. Studies included contained self-reported data from SB patients on one or more of the following sexual function domains: Genital sensitivity, orgasm, erectile function, ejaculation, lubrication, and/or dyspareunia. Two authors independently assessed eligibility, extracted data, and cross-checked results, with disagreements resolved by consensus. Studies included contained self-reported data from SB patients on one or more of the following sexual function domains: Genital sensitivity, orgasm, erectile function, ejaculation, lubrication, and/or dyspareunia.
RESULTS
Systematic search yielded 23 studies representing 1441 patients (816 males, 625 females). Eight utilized questionnaires validated in non-SB adults; the remainder used semi-structured interviews and non-validated instruments. Eleven assessed dysfunctions in both sexes, 10 in males, and 2 in females. Erectile function and orgasm were the most commonly assessed outcomes in males and females respectively. 12%-88% of males experienced erectile dysfunction; a majority (51%-90%) reported normal ejaculatory function. Many females were unable to experience orgasm (28%-63%).
CONCLUSION
Males with SB report significant erectile and ejaculatory dysfunction. Both sexes report impaired orgasms and genital sensitivity. SB-specific instruments assessing sexual dysfunction are needed in order to improve multidisciplinary care for this population.
Topics: Female; Humans; Male; Sexual Dysfunction, Physiological; Sexuality; Spinal Dysraphism
PubMed: 33930458
DOI: 10.1016/j.urology.2021.03.042 -
Journal of Pediatric Rehabilitation... 2022The purpose of this study was to assess if the incidence of lower extremity (LE) pressure injuries resulting in amputations was more frequent in ambulatory versus...
PURPOSE
The purpose of this study was to assess if the incidence of lower extremity (LE) pressure injuries resulting in amputations was more frequent in ambulatory versus non-ambulatory patients with spina bifida.
METHODS
The medical records of all patients at a large pediatric rehabilitation hospital who had spina bifida and a history of a pressure injury between 1997-2018 were retrospectively reviewed.
RESULTS
In this study, 112 patients with spina bifida developed LE pressure injuries, 53 patients walked with braces and/or an assistive device, and 59 patients used a wheelchair for mobility. Of the 11 patients who had amputations, 10 walked and 1 did not walk prior to their amputation. Ambulatory patients with LE pressure injuries had a 19% chance (10 out of 53 patients) of requiring an amputation. Non-ambulatory patients with a LE pressure injury had only a 2% chance (1 out of 59 patients) of requiring an amputation. Ambulatory patients with LE pressure injuries were at a much higher risk of requiring an amputation than patients who did not walk (p-value of < 0.005).
CONCLUSION
Patients with spina bifida who are ambulatory and develop a pressure injury have a high risk of ultimately needing an amputation and should be monitored and treated aggressively.
Topics: Humans; Child; Retrospective Studies; Spinal Dysraphism; Amputation, Surgical; Walking; Wheelchairs
PubMed: 36314223
DOI: 10.3233/PRM-210032