Disease-specific quality of life as part of the long-term follow-up for children born with esophageal atresia in an academic unit in South Africa-a pilot study.

As neonatal mortality rates have decreased in esophageal atresia (EA), there is a growing focus on quality of life (QoL) in these children. No study from Africa has reported on this topic. This pilot study aimed to describe disease-specific QoL in EA children and its applicability as part of long-term follow-up in an academic facility in South Africa. Disease-specific QoL in children born with EA was assessed utilizing the EA-QoL questionnaire for children aged 2-17 years during a patient-encounter. The parent-report for children aged 2-7 years compromised 17 items categorized into three domains: eating, physical health and treatment, and social isolation/stress. The 24-item EA-QL questionnaire for children aged 8-18 (child- and parent-report) explored four domains: eating, body perception, social relationships, and health and well-being. A total of 13 questionnaires for children aged 2-7 years were completed by five parents. A negative perceived impact on their child's eating was reported by 46-92% of parents, and less impact in the other two domains. A total of 27 questionnaires were completed by eight children aged 8-17 years and 10 parents. Similar percentages children and parents reported a negative impact in the eating, social relationships, and body perception domains. More than half reported a negative impact on the child's health and well-being. This study supports the concept that assessment of disease-specific QoL should play a vital role in the comprehensive follow-up approach for children born with EA. We identified that parents of younger children were more likely to report eating disorders, whereas parents of older children were more likely to report health difficulties with different perceptions when it came to the child's scar.

Schooling experiences in children with long-gap esophageal atresia compared with children with esophageal atresia and primary anastomosis: a Swedish study.

BACKGROUND: Children with long-gap esophageal atresia (LGEA) risk living with aerodigestive morbidity and mental health difficulties. No previous study has investigated their experiences of schooling, despite the importance of schools in children's development, learning and social relationships. We aimed to describe experiences of schooling in children with LGEA in Sweden in comparison with children with EA who had primary anastomosis. METHOD: Children with LGEA aged 3-17 were recruited nationwide in Sweden. One parent completed a survey on their child's school-based supports (according to definitions from the Swedish National Agency for Education), school absence, school satisfaction, school functioning (PedsQL 4.0), mental health (Strength and Difficulties Questionnaire) and current symptomatology. School data were compared between 26 children with LGEA to that from 95 children with EA who had PA, a hypothesized milder affected group. Mental health level was determined using validated norms; abnormal ≥ 90 percentile. Data were analyzed using descriptives, correlation and Mann-Whitney-U test. Significance level was p < 0.05. RESULTS: Formal school-based support was reported in 17 (65.4%) children with LGEA and concerned support with nutritional intake (60%), education (50%) and medical/special health needs (35%). The prevalence of school-based support was significantly higher compared to children with PA overall (36.8%, p = 0.013) and regarding nutritional intake support (20%, p < 0.001). In children with LGEA, school-based support was related to low birth weight (p = 0.036), young child age (p = 0.014), height ≤ -2SD for age/sex (p = 0.024) and an increased number of aerodigestive symptoms (p < 0.05). All children with LGEA who had abnormal mental health scores had school-based support, except for one child. Nine children with LGEA (36%) had school absence ≥ 1times/month the past year, more frequently because of colds/airway infections (p = 0.045) and GI-specific problems compared to PA (p = 0.003). School functioning scores were not significantly different from children with PA (p = 0.34) but correlated negatively with school-based support (< 0.001) and school absence (p = 0.002). One parent out of 26 reported their child's school satisfaction as "not good". CONCLUSIONS: Children with LGEA commonly receive school-based support, reflecting multifaceted daily needs and disease severity. School absence is frequent and related to poorer school functioning. Future research focusing on academic achievement in children with EA is needed.

Development and Validation of Symptom Score for Total Bladder-Bowel Dysfunction: Subscales for Overactive Bladder and Dysfunctional Voiding.

PURPOSE: Bladder-bowel questionnaires are an important tool in diagnosing nonneurogenic bladder-bowel dysfunction in children. We report the validity and reliability of a bladder-bowel questionnaire that has been in clinical use at our institution for decades. MATERIALS AND METHODS: The bladder-bowel questionnaire contains 13 questions, with answers ranging from never (score of 0) to daily (3). The questionnaire was answered by 139 healthy controls and 134 children 3 to 16 years old diagnosed with bladder-bowel dysfunction by a pediatric urologist/urotherapist. A subdiagnosis of overactive bladder or dysfunctional voiding was made in each patient. Bladder-bowel questionnaire scales were developed and evaluated against hypotheses of validity (known groups/convergent/discriminating) and reliability (internal consistency/retest reliability), sensitivity and specificity. Responsiveness was tested in 80 patients who answered the bladder-bowel questionnaire after treatment. RESULTS: A total bladder-bowel dysfunction score scale demonstrated the ability to discriminate between patients with bladder-bowel dysfunction and healthy subjects. It resulted in a ROC curve with AUC of 0.96. The maximized sensitivity was 94% and specificity was 89% for a cutoff score of 7. Two subscales were identified referring to 6 filling phase items and 3 voiding phase items. When tested in patients with overactive bladder and dysfunctional voiding, respectively, multivariable scales performed sufficiently to discriminate between those with and without overactive bladder, and those with and without dysfunctional voiding. All of these scales fulfilled the evaluated requirements for validity and reliability. At 1 year after treatment all scale scores corresponded to patient improvement (p <0.0001), suggesting the bladder-bowel questionnaire can detect clinical change over time. CONCLUSIONS: The bladder-bowel questionnaire is valid and reliable for diagnosing bladder-bowel dysfunction in pediatric patients, and overactive bladder and dysfunctional voiding in those with bladder-bowel dysfunction.

Children and adolescents with VACTERL association: health-related quality of life and psychological well-being in children and adolescents and their parents.

PURPOSE: VACTERL association is a rare and complex condition of congenital malformations, often requiring repeated surgery and entailing various physical sequelae. Due to scarcity of knowledge, the study aim was to investigate self-reported health-related quality of life (HRQoL), anxiety, depression and self-concept in children and adolescents with VACTERL association and self-reported anxiety and depression in their parents. METHODS: Patients aged 8-17 years with VACTERL association and their parents were recruited from three of four Swedish paediatric surgical centres during 2015-2019. The well-established validated questionnaires DISABKIDS, Beck Youth Inventories, Beck Anxiety Inventory and Beck Depression Inventory were sent to the families. Data were analysed using descriptives, t tests and multivariable analysis. Results were compared with norm groups and reference samples. RESULTS: The questionnaires were returned by 40 patients, 38 mothers and 33 fathers. The mean HRQoL was M = 80.4, comparable to children with asthma (M = 80.2) and diabetes (M = 79.5). Self-reported psychological well-being was comparable to the norm group of Swedish school children, and was significantly higher than a clinical sample. Factors negatively influencing children's HRQoL and psychological well-being were identified. The parents' self-reports of anxiety and depression were comparable to non-clinical samples. CONCLUSIONS: Although children and adolescents with VACTERL association reported similar HRQoL to those of European children with chronic conditions, their psychological well-being was comparable to Swedish school children in general. Nevertheless, some individuals among both children and parents were in need of extra support. This attained knowledge is valuable when counselling parents regarding the prognosis for children with VACTERL association.

The prevalence and role of coping strategies in the nutritional intake of children born with esophageal atresia: a condition-specific approach.

This study describes results of a condition-specific approach to the assessment of coping strategies in nutritional intake situations used by children with esophageal atresia. One hundred three families of children 2-17 years old with esophageal atresia participated (94% response rate). Following standardized focus groups with 30 families, nine coping items were developed, reflecting nine different coping strategies in nutritional intake situations. The coping items were pilot tested by 73 new families and evaluated for feasibility, validity, and reliability. The families also completed a validated condition-specific quality-of-life questionnaire for children with esophageal atresia, which included the scale Eating-Quality-of-life. Data were analyzed using descriptives, between-group analysis, and Spearman's rho (P < 0.05). Altogether, the coping items were feasible, valid, and reliable. Items reflecting problem-focused strategies revealed that 89% of 2-17 years old 'recognized their responsibility' and managed nutritional intake problems on their own, 79% 'tried to solve their feeding problems' testing different solutions, 79% took a 'confronting approach' to do what peers did in eating situations, and 54% 'sought other people's support'. Items reflecting emotion-focused strategies showed that 86% of the children 'accepted' their feeding difficulties, 68% 'reappraised feeding difficulties into positive outcomes' such as to eat only when food tasted good. Moreover, 63% of the children 'avoided' nutritional intake situations, 29% 'expressed worry or fear' when faced with these situations, while 25% 'distanced' themselves from eating problems by hiding or throwing away food. The children's use of coping strategies were mostly related to the existence of digestive symptoms (P < 0.05). Positive and negative coping strategies were identified. Of particular note was a correlation cluster of the so-called disengagement strategies 'avoidance', 'expression of emotional concerns' and 'distancing'. These strategies were negatively correlated with Eating-Quality-of-Life. Conversely, taking a 'confronting approach' correlated positively with Eating-Quality-of-life (P < 0.05). Hence, most children with esophageal atresia employ various coping strategies in nutritional intake situations. A good Eating-Quality-of-life may be positively affected by treating digestive morbidity and encouraging children to take an active approach to their eating problems rather than using disengagement coping.

Development and pilot-testing of a condition-specific instrument to assess the quality-of-life in children and adolescents born with esophageal atresia.

The survival rate of children with esophageal atresia has today reached 95%. However, children are at risk of chronic morbidity related to esophageal and respiratory dysfunction, and associated anomalies. This study describes the pilot testing of a condition-specific health-related quality-of-life instrument for children with esophageal atresia in Sweden and Germany, using a patient-derived development approach consistent with international guidelines. Following a literature review, standardized focus groups were conducted with 30 Swedish families of children with esophageal atresia aged 2-17 years. The results were used for item generation of two age-specific pilot questionnaire versions. These were then translated from Swedish into German with considerations of linguistic and semantical perspectives. The 30-item pilot questionnaire for children aged 2-7 years was completed by 34 families (parent report), and the 50-item pilot questionnaire for children aged 8-17 years was completed by 52 families (51 child report, 52 parent report), with an overall response rate of 96% in the total sample. Based on predefined psychometric criteria, poorly performing items were removed, resulting in an 18-item version with three domains (Eating, Physical health and treatment, Social isolation and stress,) for children aged 2-7 years and a 26-item version with four domains (Eating, Social relationships, Body perception, and Health and well-being) for children aged 8-17 years. Both versions demonstrated good internal consistency reliability and acceptable convergent and known-groups validity for the total scores. The study identified specific health-related quality-of-life domains for pediatric patients with esophageal atresia, highlighting issues that are important for follow-up care. After field testing in a larger patient sample, this instrument can be used to enhance the evaluation of pediatric surgical care.

Coping strategies used by children and adolescents born with esophageal atresia - a focus group study obtaining the child and parent perspective.

BACKGROUND: Esophageal atresia (EA) is a rare malformation, which requires surgical treatment. Survival rates today reach 95%, but EA remains a significant cause of chronic morbidity with increased risk of psychosocial problems and impaired health-related quality of life (HRQOL). No study of coping strategies of children with EA has been reported in the literature to date, but increased knowledge could lead to improved outcomes and better HRQOL. METHODS: Standardized focus groups with children with EA and their parents were conducted to identify issues related to health care needs and HRQOL, with group members relating their coping experiences. Identified coping statements were content analysed using a card sorting procedure and descriptive statistics. RESULTS: Thirty families (18 children 8-17 years; 32 parents of children with EA 2-17 years) participated in 10 focus groups. A total of 590 coping statements were recorded. Nine coping strategies were identified: problem solving (n = 116), avoidance (n = 95), recognizing responsibility (n = 71), confronting (n = 70), seeking social support (n = 63), positive reappraisal (n = 58), emotional expression (n = 46), acceptance (n = 40) and distancing (n = 31). Nine situational contexts were identified: nutritional intake (n = 227), communication of one's health condition (n = 78), self-perception when experiencing troublesome symptoms (n = 59), appearance of body or scar(s) (n = 57), physical activities like sport and play (n = 43), sleep (n = 34), hospital care (n = 33), stigmatization and social exclusion (n = 30) and medication intake (n = 29). CONCLUSIONS: Focus group methodology contributed to an increased understanding of disease-specific coping processes among children and adolescence with EA. Findings illustrate that they use several coping strategies, some of which they seem to adopt at early age and use in disease-related contexts of physical, social and emotional character. Such coping may influence health and HRQOL in children with EA. In view of the importance of establishing good coping strategies early in life, health care professionals should integrate coping aspects into care management. Future studies are warranted.