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Establishment of a condition-specific quality-of-life questionnaire for children born with esophageal atresia aged 2-7 across 14 countries

International EA-QOL Group. Front Pediatr. .

Abstract

Background: Esophageal atresia (EA) is a rare congenital anomaly characterized by a discontinuity of the esophagus. Following surgical repair, survival rates have improved dramatically the past decenniums and today exceed 90%, but the children commonly present with esophageal and respiratory morbidity. In 2018, a condition-specific quality-of-life questionnaire for children with esophageal atresia (EA) aged 2-7 in Sweden-Germany was finalized (The EA-QOL questionnaire). The study aim was to describe the evaluation of the new translations across 12 new countries in Europe, Asia, Africa, Central-and North America.

Methods: Following forward-backward translation into the new languages, the 17-item EA-QOL questionnaire was tested in cognitive debriefing interviews with parents of children with EA aged 2-7. Parents rated if each item was easy to understand (clarity) and sensitive to answer (interference with personal integrity). They could skip responding to a non-applicable/problematic item and give open comments. Predefined psychometric criteria were used; item clarity ≥80%/item sensitive to answer ≤20%/item feasibility ≤5% missing item responses. The decision to modify the translation was based on native expert, patient stakeholder, and instrument developer review, and the need for harmonization between translations.

Results: Similar to findings in the Swedish-German cognitive debriefing, the cross-cultural analysis of input from 116 parents from 12 new countries (4-14 parents, median 9 parents/country) showed that all items in the EA-QOL questionnaire fulfilled the criteria for item clarity ≥80% and sensitive to answer (ranging from 1%-4.5%), although results varied between countries. Four items had missing responses between 5.2% and 13.4%, three within the same domain and were in line with parents' explanations. Poor translations and feasibility were improved.

Conclusions: Based on parent input, the collaboration between native experts, patient stakeholders, and instrument developers, a linguistic version of the EA-QOL questionnaire for children aged 2-7 for use in and across 14 countries has been established. These efforts have set the conditions for a cross-cultural field test of the EA-QOL questionnaire and will open the doors for a new chapter in outcome research, registries, and clinical practice concerning children with EA. In the long-term, this will help increase knowledge of the disease's burden, promote patient-centeredness, exchange of information between nations, and strengthen evidence-based treatments for children born with EA.

Keywords: children; cognitive debriefing; esophageal atresia; quality of life; rare disease; translation; validity.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Presentation of subtypes of esophageal atresia according to the Gross classification system, and their prevalence. The red color illustrates the esophagus, and the gray color represents the windpipe. Gross A; interrupted esophagus without any connection to the windpipe. Gross B; interrupted esophagus with a connection to the windpipe from the upper (proximal) esophageal segment. Gross C; interrupted esophagus with a connection to the windpipe from the lower (distal) esophageal segment. Gross D; interrupted esophagus with a connection to the windpipe from both the proximal and the distal esophageal segments, and Gross E/H-type refers to a connection to the windpipe without any interruption of the esophagus. The illustration is reprinted with permission from Vladimir Gatzinsky.
Figure 2
Figure 2
The conceptual aim and structure of the EA-QOL questionnaire for children aged 2−7.
Figure 3
Figure 3
The translational procedure of the EA-QOL questionnaire.
Figure 4
Figure 4
The cognitive procedure used in the evaluation of the EA-QOL questionnaire for children aged 2−7.
See this image and copyright information in PMC

References

    1. Stoll C, Alembik Y, Dott B, Roth MP. Associated malformations in patients with esophageal atresia. Eur J Med Genet. (2009) 52(5):287–90. 10.1016/j.ejmg.2009.04.004 - DOI - PubMed
    1. Chittmittrapap S, Spitz L, Kiely EM, Brereton RJ. Oesophageal atresia and associated anomalies. Arch Dis Child. (1989) 64(3):364–8. 10.1136/adc.64.3.364 - DOI - PMC - PubMed
    1. van Lennep M, Singendonk MMJ, Dall'Oglio L, Gottrand F, Krishnan U, Terheggen-Lagro SWJ, et al. Oesophageal atresia. Nat Rev Dis Primers. (2019) 5(1):26. 10.1038/s41572-019-0077-0 - DOI - PubMed
    1. Global PaedSurg Research Collaboration. Mortality from gastrointestinal congenital anomalies at 264 hospitals in 74 low-income, middle-income, and high-income countries: a multicentre, international, prospective cohort study. Lancet. (2021) 398(10297):325–39. 10.1016/S0140-6736(21)00767-4 - DOI - PMC - PubMed
    1. Coppens CH, van den Engel-Hoek L, Scharbatke H, de Groot SAF, Draaisma JMT. Dysphagia in children with repaired oesophageal atresia. Eur J Pediatr. (2016) 175(9):1209–17. 10.1007/s00431-016-2760-4 - DOI - PMC - PubMed