Exploring the life experiences of school-aged children afflicted by tethered spinal cord syndrome: An interpretative qualitative study.

BACKGROUND: Children affected by tethered cord syndrome (TCS) encounter multifaceted challenges encompassing educational, familial and social spheres, underscoring the significance of a holistic comprehension of their subjective emotional well-being and life encounters. Nonetheless, healthcare professionals tend to prioritise the physical functionality of the afflicted individuals throughout the treatment and rehabilitation process, often neglecting the emotional experiences and requirements of these children as they transition into posthospitalization phases. AIM: To advance the subjective experiences and perceptions of children with TCS upon reintegration into their families, educational institutions and wider societal contexts subsequent to their discharge from medical facilities. METHODS: The study was conducted at the Children's Hospital in Zhejiang. Twelve children aged 8-15 with TCS were included in the study. The research design used an interpretative qualitative approach, utilising semi-structured interviews as the primary data collection method. Data analysis was performed using reflexive thematic analysis, facilitating a comprehensive exploration of emerging themes and patterns. RESULTS: Four major themes (and seven subthemes) were identified from the findings: (1) growing pains (a shameful secret, distance between ideal and reality); (2) inappropriate expressions of familial affection (knowing is not understanding, unspeakable guilt); (3) social estrangement (uncomfortable distinctions, familiar stranger) and (4) striving for independence and consistency. CONCLUSIONS: Children affected by TCS exhibit internal sensitivity and challenges in self-development, family dynamics and social interactions. They aspire to attain a future characterised by independence and freedom, akin to that of their typically developing peers. These findings can help health professionals, families and educators gain a deeper understanding of what it takes to be a child with TCS, and the findings can also serve as a platform for interventions that seek to promote self-expression in these children so that they can experience life as a meaningful and positive process. PATIENT OR PUBLIC CONTRIBUTION: This study received support from children with TCS and their guardians during data collection, as well as from the head nurse of the unit. Coresearchers also contributed to design, data collection, analysis and writing.

Stress among parents of infants with neural tube defect and its associated factors.

The purpose of this study was to assess the stress among parents (either the mother or the father) of infants with neural tube defects (NTDs) and its associated factors. Using Parenting Stress Index-Short Form (PSI-SF), 100 parents of infants with NTDs were compared with 100 parents as a normative group. The total mean score for parents of infants with NTDs was 104.0 (standard deviation (SD) = 22.9) compared with 84.3 (SD = 18.9) for parents of infants without NTDs. Fifty-three (53.5%) parents of infants with NTDs and 15% of the control group had clinically significant high total stress score. Parents of infants with NTDs had a significantly higher score of distress in all scales of PSI-SF compared with those of infants without NTDs. Multivariate analysis found that mothers of infants with NTDs had a significantly higher average score for parental distress, parent-child dysfunctional interaction and total stress than fathers. Parents' lower education, unemployed parents and lower family income per month were significantly associated with increased parent-child dysfunctional interaction and parental distress. Parents with lower education and lower family income are in need for psychological and emotional support from health-care professionals.

Improved symptoms and lifestyle more than 20 years after untethering surgery for primary tethered cord syndrome.

AIMS: Evaluation of the long-term effectiveness of untethering surgery on social adaptations to urinary symptoms at the time of the survey. METHODS: Postoperative analysis of the current activities of daily life implemented as a postal questionnaire survey. Completed questionnaires were returned by 33 of 52 patients with primary tethered cord syndrome (p-TCS) who had undergone untethering surgery more than 20 years ago, including 10 patients with tight filum terminale (TFT) and 23 with cauda equina adhesion syndrome (CEAS). RESULTS: Long-term follow-up surveys in patients with p-TCS after untethering surgery indicated excellent effects on lower urinary tract symptoms in 9 of 10 patients with TFT, and the progression of symptoms tended to improve or halted in over half of the 23 patients with CEAS. However, one patient with TFT in whom new symptoms of voiding difficulty appeared immediately after surgery and 7 with CEAS showed deterioration of urinary and/or bowel symptoms. Eighteen patients complained of some symptoms caused by lower urinary tract dysfunction after untethering surgery in terms of difficulty in activities of daily life. With the exception of one female p-TCS patient with short stature and low body weight, all data of physical status, as well as educational level, employment, occupational category and marriage statistics were within the normal respective ranges taken from the Japanese census. CONCLUSIONS: Postoperative long-term follow-up survey in patients with p-TCS indicated significant improvement of urological symptoms. However, 55% of patients had some lower urinary tract dysfunction that affected their activities in daily life.

Folic acid for the prevention of neural tube defects: the Danish experience.

Evidence from controlled trials suggests that ingestion of 0.4 mg of folic acid per day in the periconceptional period is effective in preventing neural tube defects (NTD). For this reason, most countries recommend that women planning pregnancy take folic acid supplements in the periconceptional period, and some countries even fortify stable foods with folic acid. Denmark exemplifies a country with a relatively conservative attitude with respect to taking action in these matters. In 1999, a national information campaign was launched that recommended women planning pregnancy take 0.4 mg of folic acid periconceptionally, but with the moderation that women who eat a healthy diet do not need to take folic acid supplement. The campaign was repeated during 2001. The results of the latter campaign were evaluated by using data from a national survey among pregnant women conducted simultaneously with the campaign by the Danish National Birth Cohort. An increase in the proportion of folic acid users took place concomitantly with the launching of the information events, but the increase was limited. Among women who did not plan their pregnancy, a small proportion had taken folic acid supplements periconceptionally, and this proportion did not change concomitantly with the campaign. Young age and low education were factors associated with low likelihood of taking folic acid. It seems that different and more efficient actions are needed if a more substantial proportion of Danish women and their fetuses are going to benefit from the knowledge that folic acid supplementation in the periconceptional period can prevent NTD.

Effects of vitamin B12 and folate deficiency on brain development in children.

Folate deficiency in the periconceptional period contributes to neural tube defects; deficits in vitamin B12 (cobalamin) have negative consequences on the developing brain during infancy; and deficits of both vitamins are associated with a greater risk of depression during adulthood. This review examines two mechanisms linking folate and vitamin B12 deficiency to abnormal behavior and development in infants: disruptions to myelination and inflammatory processes. Future investigations should focus on the relationship between the timing of deficient and marginal vitamin B12 status and outcomes such as infant growth, cognition, social development, and depressive symptoms, along with prevention of folate and vitamin B12 deficiency.

From disability to ability: comprehensive rehabilitation providing a holistic functional improvement in a child with neglected neural tube defect.

Neural Tube defects are one of the most common congenital disorders, presenting in a paediatric rehabilitation set-up. With its wide spectrum of clinical presentation and possible complications, the condition can significantly impact an individual's functional capacity and quality of life. The condition also affects the family of the child leaving them with a lifelong impairment to cope up with. Through this 16-year-old child, we shed light on the effects of providing rehabilitation, even at a later stage and its benefits. We also get a glimpse of difficulties in availing rehabilitation services in developing countries and the need to reach out many more neglected children like him with good functional abilities.

Toileting concerns, parenting stress, and behavior problems in children with special health care needs.

This study examined the relationship between toileting concerns, behavior problems, and parenting stress in parents of children with special health care needs (CSHCN). Participants included parents of 99 males and 71 females aged 4 to 12 years with neural tube defects (NTD), developmental-behavioral disabilities (DBD), or history of perinatal intraventricular hemorrhage (IVH). Parents completed the Achenbach Child Behavior Checklist (CBCL) and the Parenting Stress Index-Short Form (PSI-SF). Parents expressing toileting concerns on the CBCL reported significantly more personal distress and more externalizing problems versus those with continent children. Variation within subsamples suggested that expectations based on nature of disability may be a factor in parent adjustment. Direct assessment and intervention of toileting issues should be a high priority in secondary stress prevention with CSHCN and their families.

Psychological disturbance in children with physical disabilities: continuity and change in a 5-year follow-up.

This study measures continuity in behavioral disturbance over a 5-year period among 255 children with physical disabilities who were 6 to 18 years old at first assessment. The pattern of the results differed from that reported previously for a sample of the general population of children. Disabled children showed little stability in aggression, an area in which stability over time had been consistently reported for the general child population. Furthermore, important differences were observed between children with cystic fibrosis and children with cerebral palsy, myelodysplasia, or multiple physical handicaps, all conditions involving brain abnormality. Children with cystic fibrosis followed the trend toward better adjustment seen among the general population of children. In contrast, among children with conditions involving the brain, the majority of those who had been classified as psychologically severely impaired remained at this level of impairment 5 years later. In two behavioral domains, Mentation Problems and Isolation, children with conditions involving the brain maintained, on the average, the same markedly high score they had initially. Among children with physical conditions involving the brain, Mentation Problems and Isolation signify persistent problems that do not subside as the children mature.

Social consequences of long term impairments and disabilities: conceptual approach and assessment of handicap.

OBJECTIVES: To present a conceptual model of disablement adapted from the WHO model and to conduct a pilot study with a measurement tool (LIFE-H) of the concepts of life habits and handicap situations. DESIGN: Content validity and test-retest reliability study. SETTING: General community. PARTICIPANTS: A panel of 12 experts of rehabilitation for the process of content validity and 49 individuals with spinal cord disorders (adults and children) for the reliability study. OUTCOMES MEASURES: a person's life habits (activities of daily living and social roles). RESULTS: The LIFE-H questionnaire was designed to assess the handicap situations observed in daily life of individuals with disability. The experts concluded that the LIFE-H items covered most of a person's life habits (ADL and social roles) and that it could be used to determine the appearance of handicap situations. The LIFE-H total score showed a good level of reliability for the children and the adult samples (ICC = 0.73 and 0.74, respectively). Taken individually, a majority of life habit categories have shown a moderate to high reliability level (ICC > or = 0.50) while a few life habit categories such as the interpersonal relationship or nutrition showed a lower reliability level. CONCLUSION: The development of LIFE-H allows fulfillment of the need to determine the disruptions in life habits of persons with disabilities.

Australian survey on community knowledge and attitudes regarding the fortification of food with folic acid.

BACKGROUND: In response to the proposal that mandatory fortification be introduced in Australia to reduce the incidence of NTDs, the purpose of this study was to establish, for the adult community, baseline data on knowledge, attitudes, and behaviors towards the fortification of foods with folic acid. METHODS: A cross-sectional, Computer Assisted Telephone Interviewing survey with respondents recruited randomly from the electronic version of the Western Australian telephone directory was used. One thousand members of the community aged 18 years or older were interviewed for the study. RESULTS: Interviews were conducted in September 2006, with a response rate of 76%. Half the respondents were aware folate is currently added to some breakfast cereals (59%) and some breads (53%), but awareness of other food types that may be voluntarily fortified with folate was much lower. Only 13% of respondents were concerned about folate being added to bread, 10% believed folate should not be added to foods, and 9% said they would avoid foods with added folate. Generally, around half the respondents were uncertain about the benefits of fortifying foods with folate and around a quarter were uncertain about any risks or concerns with this practice. CONCLUSIONS: The community appears no more concerned about the fortification of bread with folate than they are about other existing fortification programs in Australia, and appear more likely to support rather than oppose the fortification of foods with folate, particularly if they are informed of the benefits of such a program.

Periconceptional use of folic acid in Shanxi Province of northern China.

OBJECTIVE: To understand the periconceptional use of folic acid and its association with selected characteristics in northern China. DESIGN: As part of a population-based case-control study on external structural birth defects, we analysed periconceptional use of folic acid among control mothers. SETTING: The study was conducted in four counties (Pingding, Xiyang, Taigu and Zezhou) in Shanxi Province of northern China. SUBJECTS: The subjects in this study were the control mothers, who were interviewed between January 2003 and May 2005. RESULTS: Of the 480 respondent mothers, 49 women (10.2%) reported ever using folic acid supplements, and only 16 women (3.3%) used it periconceptionally. The rates of any use and periconceptional use did not change significantly with study year. Periconceptional use of folic acid was significantly increased among women with more than high school education (odds ratio (OR) = 4.57, P < 0.10), women who were not farmers (OR = 4.72, P < 0.05), women with a history of birth-defect-affected pregnancy (OR = 32.73, P < 0.05) and women who planned their pregnancy (OR = 7.88, P < 0.10). CONCLUSION: The rate of periconceptional folic acid use was among the lowest of those reported from other countries. More work should be done to improve preconceptional use of folic acid with special attention on less educated women and women farmers.

Parental attitudes to the unclosed open neural tube defect.

Recent years have seen a trend away from the immediate closure to delayed or nonclosure of the back lesion in open neural tube defects (ONTD). Parental attitudes towards this more conservative surgical policy have not been investigated. We therefore surveyed 70 families in Northern Ireland of children who are still alive, over six months of age who had an ONTD which was not surgically closed immediately after birth. Fifty-eight parents (84%) responded. Most parents (84%) had no difficulty in handling or dressing the back, but three-quarters felt that the lesion was painful and 41% felt that its appearance was disturbing to other members of the family. In those who had delayed closure (n = 15) all parents confirmed that the child was more comfortable and easier to handle afterwards. Eleven of them (73%) felt that the child was more acceptable to the rest of the family and that surgery should have been carried out earlier. A third preferred immediate closure "to make the child more comfortable" and to "improve the general quality of life". However, 46% did not want surgery for fear of upsetting the status quo. This survey suggested that parental attitudes towards the open back lesion differ greatly and that these views should play a greater role in the decisions which influence the timing of closure of ONTD.

Screening for neural-tube defects and maternal anxiety.

Anxiety levels were studied in 176 women with raised serum alpha-fetoprotein levels at 16-18 weeks gestation, at the time they attended a central assessment clinic, and again 2-3 weeks later in those not found to have a fetus with a neural-tube defect. Methods of imparting information about the serum screening tests and the manner in which a normal amniotic fluid result was conveyed to the patient were also studied. Overall, women attending the clinic for further assessment were extremely anxious, irrespective of the source of their information. Anxiety scores 2-3 weeks after testing were greatly influenced by whether the patient had been given a definite normal result or whether she was told to assume that the result was normal if she did not hear from the clinic. Patients who, after reassessment, did not require amniocentesis had some residual anxiety in spite of verbal reassurance.

Psychological aspects of amniocentesis: anxiety feelings in three different risk groups.

A review of the literature about parents' experiences with amniocentesis is given in the first part of this paper. In the second part the results of a follow-up study in Belgium are presented. Three groups of women who had amniocentesis performed because of advanced maternal age, a previous child with Down syndrome or a previous child with neural tube defect, respectively, were interviewed at home about their experiences. Anxiety feelings were different between groups but also showed considerable variation within each group. The overall psychological evaluation of the procedure was positive, so that the majority of the women would opt for amniocentesis in a subsequent pregnancy and would recommend it to others. Later follow-up contact by mailed questionnaire revealed that almost all women elected for their subsequent pregnancies to be monitored by amniocentesis.

The psychological impact of false positive elevations of maternal serum alpha-fetoprotein.

The purpose of this study was to assess the psychological impact of maternal serum alpha-fetoprotein screening on pregnant women with false positive elevations of maternal serum alpha-fetoprotein and on their male partners. Subjects with maternal serum alpha-fetoprotein elevations and their partners exhibited significantly heightened anxiety as measured by the State-Trait Anxiety Inventory following notification of the elevation, and this anxiety persisted until normal results were obtained by further testing. Heightened anxiety was not alleviated by counseling prior to definitive testing. Once normal results were obtained, anxiety returned to control levels and heightened anxiety was not observed throughout the remainder of pregnancy. Maternal serum alpha-fetoprotein elevations had little effect on maternal attitudes toward pregnancy as measured by the Maternal Attitudes to Pregnancy Inventory. Similarly, no differences were observed between subjects with normal and elevated maternal serum alpha-fetoprotein levels with regard to depression, marital discord, work attendance, or work productivity.

Augmentation ileocystoplasty in children with myelodysplasia.

Over the past 8 years 20 children suffering from myelodysplasia were selected to undergo augmentation ileocystoplasty for urinary incontinence refractory to treatment by clean, intermittent catheterization and anticholinergic agents, or as part of urinary undiversion. The children ranged in age from 5 to 17 years, and the follow-up ranged from 6 months to 7 years. Three children underwent undiversion, two had vesicostomy closure and six had bladder outlet repair at the time of augmentation. In one patient, surgery was technically impossible. Postoperatively 16 children were fully continent on clean, intermittent catheterization and anticholinergic medication. Three boys failed to achieve satisfactory continence. Delayed perforation, a potentially serious complication, occurred in two patients several months postoperatively.