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.

Fear, isolation and the importance of support: A qualitative study of parents' experiences of feeding a child born with esophageal atresia.

OBJECTIVE: Feeding and swallowing difficulties in children are increasing due to improved survival rates of children with complex medical conditions. Despite being common complications of esophageal atresia (EA), EA related feeding difficulties have received little attention in research. Establishing positive feeding interactions and practices are important for child health and development, and for parental and child mental health. The current study aimed to investigate the parental experiences of feeding a child born with EA. METHODS: An international online survey was developed and disseminated to parents of children born with EA, aged 0-12 years, in collaboration with a patient charity for EA. Reflexive Thematic Analysis was used to analyze the qualitative survey responses. RESULTS: 176 participants were included in the qualitative sample from a larger international online survey study, chosen by a process of selective coding. Three themes were constructed during the analysis: 1) Anxiety, trauma and loss; 2) Isolated and unsupported; and 3) Supported. The results indicated that parents of children born with EA experienced significant anxiety related to their child's swallowing and feeding difficulties and traumatic experiences during feeding, and that these led to parents feeling a sense of loss and sadness. It was also found that support, or a lack of support, within parents' social environment might mediate parental experiences of child's feeding difficulties. CONCLUSIONS: This study highlighted the importance of support for parents of children born with EA, and suggested a need for improved guidance for feeding and swallowing difficulties.

Factors of family impact in a Swedish-German cohort of children born with esophageal atresia.

BACKGROUND: After repair of esophageal atresia (EA), childhood survivors commonly present with digestive and respiratory morbidity, and around 55% have associated anomalies. Although it is known that these problems can reduce health-related quality of life in children with EA, less is understood about the impact on the family. We aimed to identify factors related to family impact in children with EA. METHODS: One parent each of a child with EA (2-18 years) in 180 families from Sweden and Germany answered the PedsQL™ Family Impact Module as the dependent variable. The independent variables were the child's parent-reported health-related quality of life as measured by PedsQL™ 4.0, current symptoms, school situation, and parent/family characteristics together with child clinical data from the medical records. RESULTS: Stepwise multivariable regression analysis showed a multifactorial model of the total family impact scores (R2 = 0.60), with independent factors being the child's overall generic health-related quality of life, school-absence ≥ 1/month, severe tracheomalacia, a family receiving carer's allowance, and a parent with no university/college education, p < 0.05. Logistic regression analysis showed that an increased number of symptoms in the child the preceding 4 weeks lowered the family impact scores; however, the child's feeding (R2 = 0.35) and digestive symptoms (R2 = 0.25) explained more in the variation of scores than the child's respiratory symptoms (R2 = 0.09), p < 0.0001. CONCLUSIONS: Family functioning may be a contributing factor to the maintenance of child health. The study findings suggest multifactorial explanations to family impact in children with EA, which are essential when optimizing the support to these families in clinical and psychosocial practice. Future research should explore experiences of family impact from all family members' perspectives and multicenter studies are warranted to understand better the effectiveness of psychosocial-educational interventions to families of children with EA.

Traumatic stress, mental health, and quality of life in adolescents with esophageal atresia.

INTRODUCTION: We aimed to investigate QoL in EA patients in relation to comparison groups and to clinical factors including experienced traumatic stress. MATERIAL AND METHODS: Adolescents with EA in Norway born between 1996 and 2002 were included. Clinical assessment and patient's characteristics were collected. Quality of life (PedsQL), traumatic stress (IES-13) and mental health (SDQ-20) were compared to groups of healthy controls, children with acute lymphoblastic leukemia (ALL) and kidney transplanted children (TX). RESULTS: 68 EA adolescents participated. Total scores for PedsQL were not different from the healthy group and ALL patients, but significantly better than the TX patients. The subscale for physical performance was significantly lower than in healthy adolescents, and nine (17%) patients had scores ≤70 indicating reduced health status. Five EA adolescents (12%) had mental health scores suggesting a psychiatric disorder, and six (9%) reported high traumatic stress scores with a significant correlation to days on ventilator in the neonatal period. The strongest predictors for quality of life among EA adolescents were self-reported mental health, posttraumatic stress and GERD symptoms. CONCLUSION: Scores for Quality of life in the EA group are good except for subscale for physical performance. Symptoms of posttraumatic stress, mental strain and gastroesophageal reflux are predictors of reduced QoL.

Longitudinal Health Status and Quality of Life After Esophageal Atresia Repair.

OBJECTIVES: To longitudinally evaluate self-reported and proxy-reported health status (HS) and quality of life (QoL) of school-aged children born with esophageal atresia (EA). METHODS: We obtained Pediatric Quality of Life Inventory (HS) and DUX-25 (QoL) questionnaires from children born with EA between 1999 and 2011 at 8 and/or 12 years old. Children completed self-reports during neuropsychological assessments in a prospective longitudinal follow-up program. Parents filled out proxy-reports at home. Total and subscale scores were evaluated longitudinally and compared with sex-specific reference norms. RESULTS: In total, 110 participants (62% boys) were included. Self-reported HS improved significantly between 8 and 12 years for both boys (mean difference [md] 4.35, effect size [ES] 0.54, P = 0.009) and girls (md 3.26, ES 0.63, P = 0.004). Proxy-reported HS tended to improve over time, while self-reported and proxy-reported QoL tended to decline. Self-reported HS at 8 years was below normal for both boys (md -5.44, ES -0.35, P < 0.001) and girls (md -7.61, ES -0.32, P < 0.001). Girls' self-reported QoL was below normal at 8 (md -5.00, ES -0.18, P = 0.019) and 12 years (md -10.50, ES -0.26, P = 0.001). Parents reported normal HS at both ages, whereas they rated the QoL of their daughters below normal at 12 years (md -10.00, ES -0.16, P = 0.022). All above results are total scores. CONCLUSIONS: Self-reported and proxy-reported HS of children with EA improved between 8 and 12 years, while their QoL tended to decline. We recommend to consider HS and QoL as two separate concepts and to measure both simultaneously and longitudinally when evaluating the burden of disease.

Quality of Life Outcomes in Primary Caregivers of Children with Esophageal Atresia.

OBJECTIVE: To investigate the quality of life (QoL) impact on primary caregivers of children with esophageal atresia. STUDY DESIGN: We used a prospective cohort study design, inviting primary caregivers of children with esophageal atresia to complete the following questionnaires: Parent Experience of Child Illness (PECI), Patient-Reported Outcomes Measurement Information System (PROMIS) Anxiety, PROMIS Depression, 12-Item Short Form Survey (SF-12), and Pediatric Quality of Life Inventory (PedsQL). The PECI, PROMIS Anxiety and Depression, and SF-12 assessed caregiver QoL, and the PedsQL assessed patient QoL. Patients with Gross type E esophageal atresia served as controls. RESULTS: The primary caregivers of 100 patients (64 males, 36 females; median age, 4.6 years; range, 3.5 months to 19.0 years) completed questionnaires. The majority (76 of 100) of patients had Gross type C esophageal atresia. A VACTERL (vertebral anomalies, anorectal malformation, cardiac anomalies, tracheoesophageal fistula, renal anomalies, limb anomalies) association was found in 30, ≥1 esophageal dilatation was performed in 57, and fundoplication was performed in 11/100. When stratified by esophageal atresia types, significant differences were found in 2 PECI subscales (unresolved sorrow/anger, P = .02; uncertainty, P = .02), in PROMIS Anxiety (P = .02), and in SF-12 mental health (P = .02) and mental component summary scores (P = .02). No significant differences were found for VACTERL association, nor esophageal dilatation. Requirement for fundoplication resulted in lower SF-12 general health score, and lower PedsQL social and physical functioning scores. CONCLUSIONS: We have demonstrated that caring for a child with esophageal atresia and a previous requirement for fundoplication impacts caregiver QoL.

Health-Related Quality of Life in Patients after Repair of Esophageal Atresia: A Review of Current Literature.

Patients born with esophageal atresia (EA) require advanced reconstructive surgery and risk long-term digestive and respiratory morbidity. We describe the state of current literature on these patients' health-related quality of life (HRQoL) and give recommendations for future research. A literature search was conducted in PubMed, Cumulative Index to Nursing and Allied Health Literature (CINAHL), and PsychINFO and included articles from 2015 to December 28, 2019. Criteria for selecting articles were predefined. Seventeen articles describing HRQoL among children, adolescents, and adults were found, two of which focused on adults only. Six studies included international samples and among 15 quantitative studies sample sizes varied from 17 to 928 (median: 46). Seven different HRQoL questionnaires were used for children, three of which were specially adjusted for EA, of which one was reported validity and reliability. Four questionnaires were used for adults, one of which was generic and three symptom-specific, referring to swallowing or to the gastrointestinal tract. Hence, HRQoL outcomes were described differently. However, several studies of pediatric patients with EA revealed associations between lower HRQoL and particular congenital and surgical characteristics, which reflected disease severity. In two out of three articles, pediatric patients with EA had lower overall scores than healthy children. Digestive symptoms consistently lowered HRQoL scores in children and adults. Prior to 2015, only 12 studies were published, which illustrate a recent expansion in this field. Various HRQoL aspects and heterogeneous samples are examined, and outcomes differently reported. Current literature suggests that clinical subgroups of children with EA present with impaired HRQoL and that digestive symptomology influence HRQoL negatively. Conclusions of studies focusing on adults with EA are difficult to draw. Additional research is required.

The Impact of Congenital Esophageal Atresia on the Family Functioning.

PURPOSE: Most of the research in the field of esophageal atresia (EA) is focused on diagnostic problems and surgery. There is scarce literature addressing the impact of EA on the lives of families of patients. The aim of this paper is to investigate whether the presence of underlying associated malformations, disease-specific feeding problems and prematurity would have a significant influence on the family of a child after surgical repair of EA. DESIGN AND METHODS: The study sample consisted of 73 participants who were parents of children after surgery of EA. The impact of EA on families was assessed using an Authors-Designed Questionnaire (ADQ) to collect medical and sociodemographic background data as well as standardized questionnaire: the PedsQL™ Family Impact Module (PedsQL-FIM). RESULTS: The presence of cardiac impairment significantly (p = 0.037) affects the functioning of the family in the emotional domain. The coexistence of skeletal impairment seems to have the greatest impact on the functioning of the family, three statistically significant correlations have been demonstrated: (p = 0.021) - in the social domain, (p = 0.009) - in the cognitive domain and (p = 0.023) - in the domain of communication. The families of patients with tracheoesophageal fistula (TEF) had the statistically lower (p < 0.05) score of functioning in the emotional domain than those with children without TEF. CONCLUSION: Feeding problems and the presence of associated anomalies significantly affect the functioning of the family of the child with EA.

Assessment of the Concerns of Caregivers of Children with Repaired Esophageal Atresia-Tracheoesophageal Fistula Related to Feeding-Swallowing Difficulties.

The study aimed to assess concerns of caregivers of children with EA-TEF related to feeding-swallowing difficulties, compare the concerns according to type of atresia and repair time, and investigate its relationship with time to start oral feeding. Caregivers accompanying 64 children with EA-TEF were included. Age, sex, type of atresia, repair time, and time to start oral feeding were noted. Parents completed the Turkish version of the Feeding/Swallowing Impact Survey (T-FS-IS) to assess the concerns of caregivers related to feeding-swallowing difficulties. The T-FS-IS has three subscales including daily activities, worry, and feeding difficulties. The median age of patients was 3 (min = 1, max = 12) years, of which 57.8% were male. 43.8% of cases were isolated-EA, and 56.3% were EA-distal TEF. 57.8% of cases received early repair, and 42.2% had delayed repair. The median time to start oral feeding was 4 weeks (min = 1, max = 128). The mean scores of daily activities, worry, feeding difficulties, and total score from the T-FS-IS were 2.43 ± 1.18, 2.73 ± 1.28, 2.10 ± 0.97, and 2.44 ± 1.09, respectively. Caregivers of children with isolated-EA reported more problems in total score and all subscales of the T-FS-IS than EA-distal TEF (p < 0.01). Caregivers of children who received delayed repair reported more problems in total score and all subscales of the T-FS-IS than children with early repair (p < 0.05). Moderate to strong correlations were found between the T-FS-IS and time to start oral feeding (p < 0.01, r = 0.55-0.65). This study suggests that caregivers of children with isolated-EA and/or delayed repair and/or delay in oral intake may have higher concerns related to feeding-swallowing difficulties.

Clinical Factors Affecting Condition-Specific Quality-of-Life Domains in Pediatric Patients after Repair of Esophageal Atresia: The Swedish-German EA-QOL Study.

INTRODUCTION: We aimed to identify clinical factors affecting condition-specific health related quality of life (HRQOL) domains in children born with esophageal atresia (EA). This can facilitate preventive care to risk groups of HRQOL impairments. MATERIALS AND METHODS: A total of 124 Swedish and German families of EA children answered the validated EA-QOL questionnaires (response rate 68%), for evaluation of three HRQOL domains in children 2 to 7 years old (53 parents) and four HRQOL domains in children 8 to 17 years old (62 children/71 parents). Clinical data were collected through medical records and a questionnaire. Statistics included between-group analysis, univariable and stepwise multivariable regression analysis, p < 0.05. RESULTS: Between 2 to 7 years, no primary anastomosis (p = 0.022) and female gender (p = 0.026) predicted worse scores related to "physical health and treatment," and gastrostomy insertion related to "eating" (p = 0.0001), and "social isolation and stress" (p = 0.001). Between 8 to 17 years, no primary anastomosis (child report), prematurity, esophageal dilatation (parent report) predicted poor HRQOL related to "eating" (p < 0.05), associated anomalies to "body perception" (p = 0.031, parent report), female gender (p = 0.018, child report) and severe EA (p = 0.011 child report, p = 0.004 parent report) to "social relationships," and severe EA predicted worse "health and well-being" scores (p = 0.004, parent report). An increased number of digestive symptoms (difficulty swallowing food, heartburn, and vomiting), lowered all EA-QOL domain scores in both age groups (p < 0.001). An increased number of respiratory problems (cough, wheezing, airway infections. breathlessness, and chest tightness), lowered scores in two HRQOL domains among children 2 to 7 years (p < 0.05). CONCLUSION: Impairments within condition-specific HRQOL domains in EA children are found in congenital and surgical subgroups, and notably related to digestive symptoms throughout childhood.

Agreement between mothers', fathers', and children's' ratings on health-related quality of life in children born with esophageal atresia - a German cross-sectional study.

BACKGROUND: Esophageal atresia (EA) is a rare congenital malformation, which is characterized by the discontinuity of the esophagus. We investigated the agreement between mothers', fathers', and children's' ratings on health-related quality of life (HRQOL) in children born with EA. We aimed to broaden the understanding of subjective experiences of HRQOL from different perspectives. We hypothesized that the agreement between mother and father ratings would be high, whereas the agreement between child and mother ratings as well as child and father ratings would show more substantial differences. METHODS: We obtained data from 40 families (23 mother-father dyads of children aged 2-7 years and 17 mother-father-child triads of children and adolescents aged 8-18 years) with children born with EA, who were treated in two German hospitals. HRQOL was measured using the generic PedsQL™ questionnaires and the condition-specific EA-QOL© questionnaires. We calculated intraclass coefficients and performed one-way repeated measures ANOVAs to analyze differences for each domain as well as for the total scores. RESULTS: Intraclass correlation coefficients (ICCs) indicated a strong agreement (≥.80) between mother and father reports of children's HRQOL for both generic and condition-specific measurements. The ICCs for the generic HRQOL for mother/father-child-dyads revealed only fair to good agreement, whereas ICCs for condition-specific HRQOL showed high agreement for mother-child and father-child-agreement. Analyses of Covariance revealed differences in mother/father-child agreement in the generic domain School, both parents reporting lower HRQOL scores than the children themselves. Fathers reported significantly higher scores in the condition-specific domain Social than their children. CONCLUSIONS: Results showed that mothers' and fathers' reports corresponded to each other. Nonetheless, these reports might not be interchangeably used because mother-child and father-child agreement showed differences. Children might know the best on how they feel, and parent proxy-report is recommended when reasons such as young age, illness, or cognitive impairments do not allow to ask the child. But parent-report - no matter if reported by mother or father - should only be an additional source to broaden the view on the child's health status and well-being. The current study contributes to a better understanding of the complex family relationships involved when parenting a child born with EA.

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.

Developmental Status of Children Operated for Esophageal Atresia with or without Tracheoesophageal Fistula Along with Maternal Stress, Their Quality of life, and Coping Abilities at AIIMS, New Delhi.

INTRODUCTION: Esophageal atresia with or without tracheoesophageal fistula (EA with or without TEF) is one of the neonatal surgical emergencies requiring surgical intervention in the early neonatal period, influencing the developmental outcome in the operated children. This study was aimed to assess the developmental status of children operated for EA with or without TEF along with maternal stress, their quality of life (QOL), and coping abilities. MATERIALS AND METHODS: A descriptive cross-sectional survey was conducted on 51 children aged up to 5 years after EA with or without TEF repair and their mothers' in a tertiary care facility. The tools used were, namely, demographic datasheet of child and mother, anthropometry assessment, Developmental Assessment Scale for Indian Infants, and modified Vineland Social Maturity Scale for the developmental evaluation, Child Behavior Checklist (1.5-5 years) caregiver report form, Parental Stress Scale, WHOQOL BREF, and Coping Strategies Checklist. RESULTS: The majority of children had low weight (47.1%) and height (31.4%), for reference age. The overall and social developmental delay was observed in 40.7 and 37.5% of children, respectively, and few children (7.4%) had behavioral problems in the borderline range. Among the mothers, 47% had moderate stress and relatively poor QOL in environmental (60.1 ± 18.9) and psychological (60.8 ± 18.8) domains. The most commonly used coping strategy by the mothers was an emotional outlet (29.4%). CONCLUSION: Developmental delay was present in children operated for EA with or without TEF caused significant stress among mothers, affecting their QOL for which the emotional outlet was the most commonly used coping strategy.

Congenital Esophageal Atresia-Surgical Treatment Results in the Context of Quality of Life.

INTRODUCTION: Esophageal atresia (EA) is one of the most frequent congenital malformations of the gastrointestinal tract. The aim of the study was to assess surgical treatment results in the context of the quality of life (QoL) of children after correction of EA. MATERIALS AND METHODS: The data were collected among 73 patients after surgery of EA. The work diagnostic survey method was applied using authors' own questionnaire and standardized questionnaire, the PedsQL 4.0 generic core scales. Analysis of children growth was done based on World Health Organization percentile charts for body height and weight. Medical background for each patient, including demographic data, type of EA, type of delivery, the age of gestation, associated anomalies, mode of repair-primary and subsequent, if applicable, was collected from clinical records. Additional anomalies were divided into cardiac, skeletal, respiratory, renal, central nervous system, and other malformations (VACTERL association, CHARGE syndrome, trisomy chromosomes 18 and 21, and others). RESULTS: In this study, 23% of children in the study group weighted below third percentile, 36% were born between 33rd and 37th week of gestation; 56% had at least one associated congenital anomalies. The QoL of children born before 37th week of gestational age was lower (p < 0.034) in social functioning than children born in term. The presence of concomitant anomalies does affect the overall generic QoL. No statistical correlation was found between the type of anomaly (with/without TEF) and patients' QoL. CONCLUSION: It seems to be needed to extend psychological care of premature infants with EA.

Generic Health-Related Quality of Life after Repair of Esophageal Atresia and Its Determinants within a German-Swedish Cohort.

AIM: Despite advances of outcomes of esophageal atresia (EA), knowledge on patients' health-related quality of life (HRQoL) is sparse. Due to the heterogeneity of EA, larger cohorts need to be investigated to ensure reliability of data. Aim was to determine generic HRQoL after EA repair in a Swedish-German cohort. PATIENTS AND METHODS: Ethical approval was obtained. A total of 192 patients (2-18 years; 134 Swedish; 58 German) were included. Clinical data were reviewed. EA was classified in "severe" and "mild/moderate." Pediatric Quality of Life Inventory 4.0 Generic Core Scale (PedsQL 4.0) was used in appropriate versions (2-7 years; 8-18 years; self- [SR] and proxy report [PR]) to determine generic HRQoL. RESULTS: Swedish and German samples were clinically and demographically comparable. HRQoL was lower in "severe EA" versus "mild/moderate" (2-18 years; total score; PR 85.6 vs. 73.6; p < 0.001) and Gross A versus Gross C type EA (2-7 years; total score; PR 61.0 vs. 79.3; p = 0.035). Total HRQoL was higher in the Swedish versus German sample (2-18 years; total score; PR 82.3 vs. 72.7; p = 0.002). HRQoL was impaired in the German sample versus healthy population (2-18 years; total score; PR 72.7 vs. 82.7; p = 0.001). In German patients (8-18 years), HRQoL was higher in SR versus PR (80.7 vs. 74.7; p = 0.044). Patients' age and presence of VACTERL association or isolated anorectal malformations did not affect HRQoL. Various differences were detected regarding different dimensions of PedsQL 4.0. CONCLUSION: In this first international study, we found several differences in perception of generic HRQoL. HRQoL appears to be determined by the type of EA and severity rather than patients' age or the presence of typical associated malformations. Country-specific differences may be culturally dependent, but further investigations are suggested. A condition-specific instrument validated for EA may provide additional insights.

Health-related quality of life experiences in children and adolescents born with esophageal atresia: A Swedish-German focus group study.

BACKGROUND: Esophageal atresia (EA) is a rare malformation of the esophagus, which needs surgical treatment. Survival rates have reached 95%, but esophageal and respiratory morbidity during childhood is frequent. Child and parent perspectives and cultural and age-specific approaches are fundamental in understanding children's health-related quality of life (HRQoL) and when developing a pediatric HRQoL questionnaire. We aimed to increase the conceptual and cross-cultural understanding of condition-specific HRQoL experiences among EA children from Sweden and Germany and investigate content validity for an EA-specific HRQoL questionnaire. METHODS: Eighteen standardized focus groups (FGs) with 51 families of EA children aged 2-17 years in Sweden (n = 30 families) and Germany (n = 21 families) were used to explore HRQoL experiences, which were content analyzed into HRQoL domains. The Swedish HRQoL domains were analyzed first and used as framework to evaluate HRQoL content reported in the German FGs. HRQoL experiences were then categorized as physical, social, and emotional HRQoL burden or resource. RESULTS: One thousand nine hundred eight HRQoL statements were recorded. All nine EA-specific HRQoL domains identified in the Swedish FGs (eating, social relationships, general life issues, communication, body issues, bothersome symptoms, confidence, impact of medical treatment, and additional difficulties due to concomitant anomalies) were recognized in the FGs held in Germany, and no additional EA-specific HRQoL domain was found. The HRQoL dimensions referenced physical burden (n = 655, 34.5%), social burden (n = 497, 26.0%), social resources (n = 303, 15.9%), emotional burden (n = 210, 11.0%), physical resources (n = 158, 8.3%), and emotional resources (n = 85, 4.5%). CONCLUSION: This first international FG study to obtain the EA child and his or her parents' perspective on HRQoL suggests Swedish-German qualitative comparability of the HRQoL domains and content validity for a cross-cultural EA-specific HRQoL questionnaire. EA children make positive and negative HRQoL experiences, but prominently related to physical and social burden, which underlines appropriate follow-up care and future research.

The Esophageal-Atresia-Quality-of-life Questionnaires: Feasibility, Validity and Reliability in Sweden and Germany.

OBJECTIVES: Esophageal atresia (EA) is a rare malformation characterized of discontinuity of the esophagus, concurrent with or without a tracheoesophageal fistula (TEF). We report the feasibility validity and reliability of a condition-specific quality-of-life (QOL) tool for EA/TEF children, the age-adapted EA-QOL-questionnaires, when used in Sweden and Germany. METHODS: A total of 124 families of children with EA/TEF participated in the study; 53 parents completed the EA-QOL-questionnaire for children aged 2 to 7 years; 62 children/71 parents the EA-QOL-questionnaire for children 8 to 17 years. Feasibility was determined from the percentage of missing item responses. Based on clinical data and previously validated generic QOL-instruments (PedsQL 4.0, DISABKIDS-12), the final EA-QOL scores were evaluated against hypotheses of validity (known-groups/concurrent/convergent) and reliability (internal consistency/retest reliability of scores for 3 weeks). Significant level was P < 0.05. RESULTS: In the questionnaire for EA/TEF children aged 2 to 7 years, 16/18 items were completed with missing values <6% (range 0%-7.5%), and in the questionnaire for 8 to 17-year-olds, 24/24 child-reported items (range 0%-4.8%) and 21/24 parent-reported items (range 0%-7.0%). In both age-specific EA-QOL-questionnaires, desirable standards for known-groups and concurrent validity were fulfilled; digestive symptoms and feeding difficulties negatively impacted EA-QOL-Total-scores (P < 0.001), and as hypothesized, in 2 to 7-year-olds, respiratory symptoms decreased EA-QOL-Total-scores (P = 0.002). Correlations between the EA-QOL and generic QOL questionnaires supported convergent validity. Internal consistency reliability was satisfactory. The level of agreements of EA-QOL-scores between the field- and retest study were good to excellent. CONCLUSIONS: The overall psychometric performance of the EA-QOL-questionnaires for EA/TEF children is satisfactory and can enhance outcome evaluations in future research and clinical practice.

Mother's Lived Experience During Repair of Long-Gap Esophageal Atresia: A Phenomenological Inquiry.

BACKGROUND: Infants born with long-gap esophageal atresia (LGEA) pose unique physiologic risks in the newborn period. Anatomic and physiologic anomalies require an extended hospitalization with procedural analgesia and sedation that impact the mother's experience of birth, maternal response, and nurturing of her infant. PURPOSE: The aim of this study was to understand the meaning of experiences that mothers of infants born with LGEA encounter in the neonatal intensive care unit while their infant undergoes esophageal repair. METHODS: A hermeneutical phenomenological design was used to guide this inquiry. Three mothers were interviewed on 3 separate occasions. The conversations were audio-recorded and transcribed verbatim. The findings were analyzed using fundamental existential lifeworld themes. RESULTS: The essence that conceptualized the study was "making connections: day-by-day." Themes that emerged are (a) the many phases; (b) the long and winding road; (c) a new me, my purpose; and (d) our new community. IMPLICATIONS FOR PRACTICE: Nurses' knowledge and understanding of maternal experiences of having an infant with LGEA will enable for increased physical closeness, optimizing time spent together to learn their infant's unique personality. Creating partnerships with mothers can enhance our understanding of their perspectives, concerns, needs, and guide interventions. IMPLICATIONS FOR RESEARCH: Further exploration of family dynamics including fathers, siblings, and contextual factors may illuminate interventions to enhance relationships and communication that may influence developmental outcomes for families of infants with LGEA.

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.