Interventions for the Management of Distal Intestinal Obstruction Syndrome in Cystic Fibrosis

Abstract

Distal intestinal obstruction syndrome (DIOS) is a major gastrointestinal complication in cystic fibrosis (CF). It arises when thick mucus and viscid faecal material combine in the bowel, commonly at the terminal ileum or caecum, leading to partial obstruction (incomplete DIOS) or complete obstruction (complete DIOS). There is limited evidence for the efficacy and safety of laxatives used to manage DIOS and insufficient knowledge of current practices used to treat it; consequently, this thesis aimed to evaluate these matters in children and adults.
A Cochrane review was conducted to evaluate the effectiveness and safety of laxatives used for the prevention of DIOS. Of 2631 studies identified, only 1 study was included so meta-analysis could not be performed. The study was a double-blind, placebo-controlled, crossover trial investigating the efficacy of cisapride (a prokinetic drug) in 17 patients with a history of DIOS. Radiograph scores revealed no difference between cisapride and placebo. There were no adverse effects. However, total gastrointestinal symptom scores favoured cisapride with a mean difference of -7.60(95% CI -14.73 to -0.47). However, cisapride is no longer licenced due to cardiac side effects, limiting its clinical applicability.
A quantitative survey was conducted to establish the current treatments for constipation and DIOS in UK CF centres. Results varied greatly, especially for the treatment of DIOS: incomplete DIOS had 23 different 1st line combinations in adults and 22 in children; complete DIOS had 25 1st line combinations in adults and 17 in children. Over 99% respondents recognised limited evidence for their treatment decisions.
This thesis demonstrates that there is a lack of evidence for the prevention of DIOS and little consensus for the treatment of DIOS, highlighting a need for research, which at present is pitifully lacking.