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

Abstract

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.

Quality of Life in Parents of Children Born with Esophageal Atresia

“…Within a German-Swedish cooperation project aiming to develop a condition-specific instrument to assess QoL in children and adolescents born with EA from both self and proxy perspectives, the following study was conducted. 10 In the following analyses, only German families were included because parental QoL was only assessed in the German sample. All families of EA children aged 2 to 17 years at the time of the invitation who were ever treated either at the Hannover Medical School (MHH) or "Auf der Bult" Children's Hospital, Hannover were invited to participate in this cross-sectional study focusing on QoL in EA.…”

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

“…described HRQoL among children and adolescents born with EA, [24][25][26][27][28][29][30][31][32][33][34][35][36][37][38] three of which 32,34,38 also reported on HRQoL among adults (patients >18 years old). The remaining two articles described HRQoL among adults with EA only.…”

“…25,28,34 Moreover, six articles (41%) reported on HRQoL in international samples, in five of which HRQoL in Swedish-German pediatric EA cohorts was described. 26,[29][30][31]36 The remaining international study included the largest number of respondents, through collaboration with patient organizations. 38 Among 15 quantitative studies, sample sizes varied between 17 and 928 (median 46).…”

“…[41][42][43][44][45] Others were symptom-specific, focusing on swallowing or on the gastrointestinal tract. 39,[46][47][48][49] Three questionnaires were regarded as condition-specific, 24,26,28,30,32 two of which were lacking reports on validity and reliability. 28,32 ►Table 2 lists the seven HRQoL questionnaires with demonstrated validity and reliability.…”

“…In compliance with current standards of patient-reported outcome measurements, the Swedish-German authors developed and validated the first reported condition-specific HRQoL questionnaire for children and adolescents with EA. 24,26,30 Following pilot and field testing of the EA-QOL questionnaires, 26,30 the final version for children from 2 to 7 years old, and from 8 to 17, entails the domains presented in ►Table 2. Two further studies 28,32 have employed questionnaires adjusted to the EA population, although they seem to lack reports of validity and reliability.…”