Introduction: Intestinal failure (IF) is a complex condition of severe intestinal malabsorption caused by short bowel syndrome (SBS), congenital diseases of enterocyte development, and severe motility disorders (total or subtotal aganglionosis or chronic intestinal pseudo-obstruction syndrome). Children with IF require long-term total or partial parenteral nutrition (PN) to preserve nutritional status and prevent complications. When a child depends on long-term PN, home parenteral nutrition (HPN) is the best alternative to prolonged hospital stay and is recognized as the best option for improving the quality of life of these children and their families. In our country, during many years all paediatric patients with IF were managed individually within the hospital setting of paediatric gastroenterology referral centres.

Methods: We present the process of developing the first national HPN program for paediatric patients with IF, and the current practice of establishing and managing children on HPN.

Results: Five patients were managed on HPN for prolonged period, four of those are currently on HPN. There is a multidisciplinary team involved in the process. With regards to the aetiology, three patients have SBS, one patient has tufting enteropathy and one total aganglionosis with severe dysmotility and postoperative SBS. For administration of HPN we place a tunnelled single lumen central venous catheter (CVC) surgically. We use commercial premixed PN solutions with individual adjustments. The regime, monitoring and prevention of complications follow the European HPN guidelines.

Conclusion: Although PN is a life-saving therapeutic option for children with IF, it is a high-risk therapy with potential acute and long-term complications. The rate of complications can be high, particularly in inexperienced hands. Management of HPN by centralised units with expertise in the investigation of IF rehabilitation and with a multidisciplinary team to support care at home would minimise complications and improve outcome. This program will continue to expand and to improve the management at all levels.