Cystic Fibrosis Colorectal Cancer Screening Consensus Recommendations

Denis Hadjiliadis; Alexander Khoruts; Ann G. Zauber; Sarah E. Hempstead; Patrick Maisonneuve; Albert B. Lowenfels


Gastroenterology. 2018;154(3):736-745. 

In This Article


The CRC screening task force convened in April 2015 at the CF Foundation Headquarters. The 18-member task force consisted of pulmonologists, gastroenterologists, a social worker, nurse coordinator, surgeon, epidemiologist, statistician, adult with CF, and a parent of a child with CF. The committee was divided into 3 workgroups: Cancer Risk, Transplant, and Procedure and Preparation.

At the initial meeting, the task force determined the scope of the document; developed (PICO) Population, Intervention, Comparison, and Outcome questions; and determined relevant search terms. The American Gastroenterological Association Governing Board reviewed and approved the PICO questions.

PubMed was searched for relevant, published, articles in February−March 2016. The search terms used can be found in the Supplementary Material. Task force members also conducted their own independent searches.

A total of 1159 articles were retrieved. Of these articles, 198 articles were read at the full-text level. In total, 50 articles were included in the final manuscript (Figure 1).

Figure 1.

Search strategy diagram.

The initial review of the literature at the title level was conducted by a guidelines specialist at the CF Foundation and task force members. Task force members then reviewed the relevant abstracts and full articles. After a thorough review of the records, each workgroup drafted recommendation statements based on their PICO questions. Ten recommendation statements were considered and voted on, and an 80% agreement threshold was agreed on before the meeting.


The CF Foundation collaborated with the Department of Public Health of Erasmus Medical Center, University Medical Center Rotterdam, Netherlands, to conduct modeling on CRC screening strategies for adults with CF. The goal of the modeling was to provide estimates of the benefits and costs of screening in the CF population given that life expectancy and CRC risk differ from the general population. Data including age distribution and life expectancy used for this modeling came were calculated using 2010−2014 CF Foundation Patient Registry data.[28] Most of the excess mortality risk in CF is not due to CRC. The modeling was conducted using the MISCAN-Colon model, which is part of the Cancer Intervention and Surveillance Modeling Network.[29]

In addition to the 10 listed recommendations drawn up by the task force, the modeling group developed a unique program to determine the most effective strategy for screening this high-risk population. A brief description of the methodology and main findings of the modeling procedures are presented here, more detailed information is available in a separate report in this journal.[30]

Microsimulation Screening Analysis-colon Model

The existing Microsimulation Screening Analysis-Colon model for the general US population was adjusted to reflect the increased CRC risk and the elevated all-cause mortality in CF patients. Two versions of this model were developed: one for CF patients with an organ transplant and the other for CF patients without an organ transplant. Subsequently, we used these models to predict the benefits, harms, and resources associated with implementation of 101 different screening strategies that varied by test (colonoscopy or fecal immunochemical test [FIT]), age to begin and end screening, and screening interval. We used incremental cost-effectiveness analysis to determine the impact of various screening strategies for the CF population with and without organ transplant.

External Review

The manuscript and recommendation statements were distributed to the CF Community, Lung Transplant Community, and Gastrointestinal Community through CF Foundation, International Society of Heart and Lung Transplantation and American Gastroenterological Association listservs reviewers were given 2 weeks to submit comments and feedback using an online survey tool, Survey Monkey.[31] Committee chairs reviewed and responded to comments and updated the manuscript.