Using a unique methodology to map an esophageal atresia patient’s journey, ERNICA is highlighting key issues and areas of good practice.

Esophageal atresia (EA) is a rare congenital condition with an estimated prevalence varying between 1 and 2 in 5,000 live births. Mortality from this condition has reduced to less than 10% since the first successful primary repair in 1941. However, some concerns remain about morbidities such as those of a gastroenterological or pulmonary nature. Esophageal atresia patients require life-long attention, ranging from surveillance to ongoing treatment. The psychological impact of the diseases both on patients and their families has also been increasingly recognised.

ERNICA has developed a ‘patient journey’ for esophageal atresia patients, under the leadership of ERNICA patient representatives from the international federation of EA support groups (EAT). The project, which started in 2018, adopts a unique methodology which deserves to be illustrated.

The patient journey describes the different key stages a patient has to go through during their life, from possible pre-natal diagnosis to adulthood. Each stage is depicted pictorially and is accompanied by text to describe in both medical and ‘lay-person’s’ language the key issues and relevant clinical good practice (e.g. follow-up and after-care regimes) at each of these stages.

The project is supported by a team from a wide range of medical disciplines including surgery, nursing, gastroenterology, speech and language therapy, and nutrition. The project involves an analysis of medical publications and good practice guides produced by EAT member organisations in Germany (KEKS), the Netherlands (VOKS), and by the French Centre for esophageal atresia in Lille. It also endeavours to synthesise with other projects undertaken within ERNICA (e.g. EA surgical consensus statements) and outside of the network, like the Transition project currently being undertaken by the International Network on EA. The patient journey points out existing gaps in good practice and areas where evidence is limited, highlighting possible areas for further research and/or future ERNICA projects. Two areas are highlighted as warranting further action: identifying good practice in ongoing treatment, and surveillance of adult patients.

Example of patient journey stage depicted pictorially 

Patient representatives representing the Federation of Esophageal Atresia and Tracheo-esophageal fistula support groups e.V. within the ERNICA work stream: Esophageal Diseases