The Role of Patient Organisations-Patients' and Parents' Views and Experience of Hirschsprung's Disease.

In many countries, patient organisations offer advice and the exchange of experiences to Hirschsprung's disease patients and their families. Professional treatment by experienced health care providers and the availability of life-long multidisciplinary follow-up care are essential. However, outside the clinic, patients and their families have to manage life on a day-to-day basis at home, which often brings up uncertainties and questions: Parents go through different stages during the diagnosis and treatment of their child, the affected children themselves go through many different stages of development, and even through the course of adulthood, new questions regarding the chronic disease may arise.

Ernica Clinical Consensus Statements on Total Colonic and Intestinal Aganglionosis.

Background: Hirschsprung disease is a congenital intestinal motility disorder characterized by an absence of enteric ganglion cells. Total colonic aganglionosis and near total or total intestinal aganglionosis, defined as absence of ganglion cells in the entire colon and with variable length of small bowel involved, are life-threatening conditions which affect less than 10 % of all patients with Hirschsprung disease. The aim of this project was to develop clinical consensus statements within ERNICA, the European Reference Network for rare congenital digestive diseases, on four major topics: Surgical treatment of total colonic aganglionosis, surgical treatment of total intestinal aganglionosis, management of poor bowel function in total colonic and/or intestinal aganglionosis and long-term management in total colonic and or intestinal aganglionosis.