Cystic fibrosis is a progressive monogenetic disorder that causes persistent pulmonary disease, but also affects other organ systems including the digestive tract. Recent advances in treatment and care of CF patients including the use of new and highly effective CFTR modulators, have led to a dramatic increase in survival; young patients with CF can now expect to live to or beyond middle age, where cancer is more frequent. CF patients are now known to face an increased risk of digestive tract cancer-particularly cancer of the colon. The risk, which could be triggered by associated CF-related conditions or other genetic mechanisms, is even greater in patients who received a transplant. There is also some evidence that CF patients develop adenomatous polyps more frequently and at an earlier age than non-CF subjects. To reduce the excess risk of intestinal cancer in CF patients, the Cystic Fibrosis Foundation has developed colonoscopy-based guidelines. For non-transplanted patients, colonoscopy should commence at age 40, with rescreening at five-year intervals; the screening interval should be shortened to three years if adenomatous polyps are discovered. For transplanted patients, screening should start at age 30, or within two years of the transplant operation. Prior to colonoscopy, it is essential for CF patients to undergo a special, more intensive bowel preparation than normally used for non-CF persons. Whether the new drugs that have dramatically improved morbidity and mortality for CF patients will alter the risk of cancer is unknown and needs to be assessed in future studies.
Copyright © 2021. Published by Elsevier Inc.