Recent advances in the treatment of cardiorespiratory complications in patients with cystic fibrosis (CF) have improved median survival and resulted in gastrointestinal complications becoming more evident.^1,2 The distal intestinal obstruction syndrome (DIOS) develops due to the accumulation of viscid muco-faeculent material in the terminal ileum and right hemicolon.³ Reduced intestinal water content, lower luminal acidity of the foregut, and accumulation of intraluminal macromolecules contribute to the development of DIOS.⁴ Typically, patients develop progressive symptoms and signs of small bowel obstruction. A plain abdominal x ray will reveal dilated loops of centrally placed small bowel, with faecal loading at the terminal ileum and right hemicolon (see fig 1⇓). Most cases will respond to the aggressive use of laxative agents administered orally and rectally, intravenous hydration, pancreatic supplements, and N-acetylcysteine (Parvolex) and gastrografin (meglumine diatrizoate) orally or per rectum.^5–,⁸ Surgical decompression has been reserved for refractory cases but carries …