Since 1966 when the Cystic Fibrosis Foundation Patient Registry (CFFPR) was founded, clinicians have witnessed significant advances in both the quality and quantity of life for patients living with Cystic Fibrosis (CF). As patients with CF live longer and fuller lives, increasing encumbrances from gastrointestinal manifestations of CF will be observed. This article serves to discuss “below the diaphragm” concerns involving the large intestine (Distal Intestinal Obstruction Syndrome, Constipation, and Colorectal Cancer). Avenues for development and implementation of clinical care protocols, particularly regarding proactive management of known associated conditions and cancer screening, will continue to be refined in the coming years. It falls to the multidisciplinary CF care team to be actively engaged in addressing these concerns effectively as priority shifts from relative acuity (typically related to early nutrition and lung function) to the travails of longevity as the CF population continues to age.
Bibliographical noteFunding Information:
The authors would like to thank the Cystic Fibrosis Foundation (Bethesda, MD, USA) and Faculty Members of the Developing Innovative GastroEnterologic Specialty Training (DIGEST) Program (Dr. Sarah Jane Schwarzenberg, Dr. Drucy Borowitz, Dr. Maria Mascarenhas, Dr. Steven Freedman, and Dr. Michael Wilschanski) for their unwavering support, both through their consistent mentorship and financial support via the DIGEST Career Development Award (Dr. Abraham, ABRAHA14GE0 ).
- Colon cancer
- Colorectal cancer
- Cystic fibrosis
- Distal intestinal obstruction syndrome