Cystic fibrosis associated liver disease in children

Table 7 Pre and post-transplant protocol for prevention and treatment of distal intestinal obstructive syndrome

Pre and post-transplant protocol
Low risk	(1) 600 mg N-acetyl-cysteine in 120 mL water orally/nasogastric tube twice/day. Senna twice daily; (2) 2 liters of Klean prep per day post-transplant; (3) Consider early nasogastric tube in patients with delayed gastric emptying studies pre-operatively; (4) All patients in intensive care unit should only receive only elemental feed via nasogastric tube as this does not require pancreatic enzyme replacement. Once transferred to ward, can be restarted on regular feeding and pancreatic enzyme supplements; (5) Try and reduce opiates early during hospital stay; and (6) Treat all patients with proton pump inhibitors.
High risk	(1) As per low risk management; and (2) High risk of developing DIOS and subsequent surgical gut decompression is associated with a high mortality. So these patients should receive a prophylactic loop ileostomy.
Treatment of DIOS	(1) Stop feeding, nasogastric tube on free drainage and intravenous fluids; (2) 100 mL gastrografin in 400 mL water enterally and repeat after 6 h; (3) Subsequent management is with Klean prep in 1 L water over 1 h via oral/nasogastric tube and can be repeated up to 4 times every 24 h until bowel movement is achieved; and (4) If no improvement after 48 h, then it is unlikely to resolve without surgery to decompress the gut and also consider total parenteral nutrition.

DIOS: Distal intestinal obstructive syndrome.