Standards of Care

The activities grouped under this area of work are linked to ERNICA’s objective to maximise the quality of care provided to patients with rare inherited and congenital digestive disorders.

Guideline / Consensus statement development:

For some rare or complex conditions, there are no clinical guidelines or consensus documents available at present. For some diseases, these are available, but they are in need of review and revision. ERNICA seeks to develop and/or revise existing clinical guidelines and consensus documents, to ensure clinical recommendations are made available to doctors and patients based on research of the highest quality. Patient representatives and healthcare professionals are involved in guideline development and revision.

Esophageal Atresia: 

Consensus statement on the management of patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF):  In October 2018, members of the ERNICA Esophageal Workstream performed a conference to establish consensus on the management of patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) based on the latest evidence from the literature and expert opinion. The manuscript has now been published in the European Journal of Pediatric Surgery and the full text is available to read here

Consensus statement on the Perioperative, Surgical and Long-term Management of Patients with Long-gap Esophageal Atresia (EA): In development. The 2nd ERNICA consensus conference on this topic took place in November 2019.

Hirschsprung Disease:  A guideline is in development and due for publication.

Total colonic aganglionosis:  Discussions have started in regards to the development of a guideline. 

Gastroschisis:  Discussions have started in regards to the development of a guideline. 

Patient Journeys: 

ERNICA's patient representatives within the Esophageal Diseases working group are currently working on developing a ‘patient journey’ document, alongisde clinicians within the network. Creation of these documents asks patient representatives to reflect on their own personal journey with the rare disease they represent and map what their needs were against what would have been the 'ideal clinical care' at each stage. 

Representatives from ERN GENTURIS have recently published a paper outlining the 'Patient Journey' approach and its benefits. Click here to read.