Patient-related outcomes of familial adenomatous polyposis (FAP): compliance, distress and life course

K.F.L. Douma1, N. K. Aaronson1, H.F.A. Vasen2,3 , C.M. Gundy1, S. Verhoef4, M.A. Gerritsma1, E.M A. Bleiker1

Division of Psychosocial Research & Epidemiology, The Netherlands Cancer Institute - Antoni van Leeuwenhoek Hospital, Amsterdam1; The Netherlands Foundation for the Detection of Hereditary Tumours, Leiden2; Department of Gastroenterology, Leiden University Medical Center3; Family Cancer Clinic, The Netherlands Cancer Institute - Antoni van Leeuwenhoek Hospital, Amsterdam4

Purpose: The primary aim of this nationwide, cross-sectional study was to determine the extent to which individuals with (a high risk of having) FAP comply with recommended colorectal cancer (CRC)-screening schedules, experience heightened levels of psychosocial distress, and experience an altered life course.

Methods: All individuals from FAP families registered at the Netherlands Foundation for the Detection of Hereditary Tumours were invited to complete a self-report questionnaire that included questions about compliance, generalized (MHI-5), cancer-specific (CWS) and FAP-specific (IES) distress, and about the effect of FAP on interpersonal relationships, work and finances. Additionally, data on objective compliance with screening advice was extracted from the STOET medical records.

Results: In total, 525 individuals (response 64%) completed the questionnaire. Preliminary results indicated that 23% of those individuals at-risk of developing FAP, and 6% of those who have undergone surgery were under-compliant with screening advice. This involved delays in undergoing screening and/or undergoing fewer screenings than had been recommended. Higher perceived risk of developing FAP and use of sedatives during screening were positively associated with compliance (p<0.05). Levels of generalized distress were comparable to those of the general Dutch population. However, approximately 20% of respondents reported having moderate to severe levels of FAP-specific distress. Distress levels were associated most strongly with psychosocial variables (e.g., family functioning and risk perception), rather than sociodemographic or clinical variables. In total, 43% of the variance in distress could be explained by these sets of variables combined. Approximately 25% of those who reported being moderately to severely distressed had received any professional psychosocial support. One-quarter of those with a FAP-diagnosis (n=340), indicated that the disease had a negative impact on relationships, work or finances (e.g., insurance).

Conclusions: Approximately one-quarter of individuals with (a high risk of having) FAP undergo screening less often than is recommended. Steps that can be taken to increase compliance rates include ensuring that individuals have a realistic understanding of their actual risk, and routinely offering sedatives as part of the screening procedure. A substantial minority of individuals have moderate to high levels of distress associated with FAP, and experience negative social, work and financial consequences meriting professional psychosocial support. We would recommend the use of a brief screening questionnaire to identify those individuals at risk for and in need of such support.