Gastroenterology

Gastroenterology

Volume 154, Issue 3, February 2018, Pages 736-745.e14
Gastroenterology

Special Report
Cystic Fibrosis Colorectal Cancer Screening Consensus Recommendations

https://doi.org/10.1053/j.gastro.2017.12.012Get rights and content

Background & Aims

Improved therapy has substantially increased survival of persons with cystic fibrosis (CF). But the risk of colorectal cancer (CRC) in adults with CF is 5−10 times greater compared to the general population, and 25−30 times greater in CF patients after an organ transplantation. To address this risk, the CF Foundation convened a multi-stakeholder task force to develop CRC screening recommendations.

Methods

The 18-member task force consisted of experts including pulmonologists, gastroenterologists, a social worker, nurse coordinator, surgeon, epidemiologist, statistician, CF adult, and a parent. The committee comprised 3 workgroups: Cancer Risk, Transplant, and Procedure and Preparation. A guidelines specialist at the CF Foundation conducted an evidence synthesis February−March 2016 based on PubMed literature searches. Task force members conducted additional independent searches. A total of 1159 articles were retrieved. After initial screening, the committee read 198 articles in full and analyzed 123 articles to develop recommendation statements. An independent decision analysis evaluating the benefits of screening relative to harms and resources required was conducted by the Department of Public Health at Erasmus Medical Center, Netherlands using the Microsimulation Screening Analysis model from the Cancer Innervation and Surveillance Modeling Network. The task force included recommendation statements in the final guideline only if they reached an 80% acceptance threshold.

Results

The task force makes 10 CRC screening recommendations that emphasize shared, individualized decision-making and familiarity with CF-specific gastrointestinal challenges. We recommend colonoscopy as the preferred screening method, initiation of screening at age 40 years, 5-year re-screening and 3-year surveillance intervals (unless shorter interval is indicated by individual findings), and a CF-specific intensive bowel preparation. Organ transplant recipients with CF should initiate CRC screening at age 30 years within 2 years of the transplantation because of the additional risk for colon cancer associated with immunosuppression.

Conclusions

These recommendations aim to help CF adults, families, primary care physicians, gastroenterologists, and CF and transplantation centers address the issue of CRC screening. They differ from guidelines developed for the general population with respect to the recommended age of screening initiation, screening method, preparation, and the interval for repeat screening and surveillance.

Section snippets

Methodology

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,

Results

The task force found limited information on CRC screening in adults with CF, and available reports consisted mostly of retrospective reviews of patient records and case−control studies. The task force identified no randomized clinical trials comparing results in screened vs nonscreened patients or reports comparing results of colonoscopy with results of less-invasive screening procedures.

The task force developed ten recommendations (Table 1). Based on the quality and limited number of studies,

Conclusions

The CF CRC screening task force recommends screening with colonoscopy beginning at age 40 years for non-transplanted patients with CF and age 30 years for persons who have undergone and successfully recovered from a transplantation procedure. All decisions around CRC screening should be made in concert with the adult with CF. These discussions should include the consideration of comorbidities, safety, treatments, and quality of life. These recommendations are similar to the guidelines for

Raising Awareness of Colorectal Cancer Risk and Acceptability of Screening

Because of the absence of any information on other screening procedures, the task force recommends colonoscopy screening as the current best screening procedure. Thus, a key issue is acceptability of this procedure by the CF community. One member of the task force contacted several CF care centers and found a high degree of compliance when colonoscopy was recommended, with level of patient education on bowel preparations being a key factor for acceptance. Because many patients and center

Acknowledgments

Cystic Fibrosis Colorectal Cancer Screening Task Force: Amy Leigh Braid, Community Advisor to the Cystic Fibrosis Foundation; Joanne Cullina, MSN, APN, ACNS-B, Northwestern University; Anne Daggett, MSW, LCSW, St. Luke’s Cystic Fibrosis Center of Idaho; Aliza Fink, DSc, Medical Department, Cystic Fibrosis Foundation; Andrea Gini, MSc, Department of Public Health Erasmus MC, Rotterdam, The Netherlands; Denis Hadjiliadis, MD, MHS, Paul F Harron Jr Associate Professor of Medicine Perelman School

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    Conflicts of interest The authors disclose no conflicts.

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