Abstract

Purpose Distal intestinal obstruction syndrome (DIOS) is a debilitating condition which is unique to Cystic Fibrosis (CF). Constipation is increasingly common in CF patients as a result of the altered fluid composition of the intestine caused by the CF transmembrane conductance regulator gene defect. The incidence of both DIOS and constipation is increased in CF-patients post lung transplant1-2. Methods A retrospective chart review was conducted on all patients with CF who received a lung transplantation in the same hospital between 2005 (lung transplant programme commencement) to January 2017. The ESPGHAN CF working group definition was used to define constipation and DIOS3. Results Fifty three patients (67.9% males and 32.1% females) with CF underwent lung transplant during the study period. The mean age was 32±7 years and mean BMI was 20.7±2.7kg/m2. No patients developed DIOS during their transplant admission however 30.2% (n=16) of patients developed constipation. Post-transplant the mean number of days before oral intake and enteral intake were commenced was 3 days and 2 days respectively. The mean days before pancreatic enzyme replacement therapy was commenced was 3 days. Of those who developed constipation, the median day of laxative commencement was day 4 post-transplant versus day 2 for those who did not develop constipation (P=0.005). 20.8% of the cohort required hospitalisation with constipation within 1 year post-transplant, and 8.5% (75% female) developed DIOS requiring hospitalisation within 2 years post-transplant. Of the patients admitted with constipation 10.5% (n = 2) required parenteral nutrition. Those who did not require a subsequent admission for the treatment of constipation or DIOS experienced weight gain during the first year post-transplant (+3.7 kg p=0.001, +3.8kg p Conclusion Early initiation of laxatives post lung transplant in the patients with CF may reduce the incidence of constipation and DIOS. Individuals that developed constipation and DIOS post-transplant gained significantly less weight in the first year post-transplant. This study highlights the negative impact DIOS and constipation can have on patients’ nutritional recovery post-transplant.

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