PURPOSE
The purpose of this study was to examine the health-related quality of life in a sample of Canadian adults diagnosed with familial adenomatous polyposis and desmoid tumor.
METHODS
The study was conducted in two parts. Seven individuals participated in a focus group prior to a mail-out survey. A cross-sectional mail-out survey was administered to eligible individuals who were actively followed at the Familial Gastrointestinal Cancer Registry at Mount Sinai Hospital.
RESULTS
Forty-one individuals (25 female and 16 male) were available to be contacted by the registry coordinator and 23 questionnaire packages were completed, resulting in a 56.1 percent participation rate. The results of this study demonstrated a reduced health-related quality of life for individuals living with familial adenomatous polyposis and desmoid tumor for over 10 years. The main predictors of health-related quality of life in this analysis included marital status (married vs. not married), prior knowledge of desmoid tumor in the family, and current level of hopelessness (R 2 = 0.856, df = 13, F = 26.8, P < 0.001). The qualitative content analysis of themes from the focus group indicated that ongoing medical uncertainty and lack of information from health care professionals, isolation, and family communication were the main challenges in living with desmoid tumors.
CONCLUSIONS
The findings from this study suggest that ongoing education of health care professionals is warranted and that information and support interventions may be beneficial to this clinical population.
Similar content being viewed by others
References
T Berk B Bapat HS Stern (1993) ArticleTitleScreening, management, and surveillance for families with familial adenomatous polyposis Gastrointest Endosc Clin N Am 3 725–36
LA Belchetz T Berk BV Bapat Z Cohen S Gallinger (1996) ArticleTitleChanging causes of mortality in patients with familial adenomatous polyposis Dis Colon Rectum 39 384–7
T Iwama Y Mishima J Utsunomiya (1993) ArticleTitleThe impact of familial adenomatous polyposis on the tumorigenesis and mortality at the several organs. Its rational treatment Ann Surg 217 101–8
L Bertario S Presciuttini P Sala C Rossetti M Pietroiusti (1994) ArticleTitleCauses of death and postsurgical survival in familial adenomatous polyposis: results from the Italian Registry Sem Surg Oncol 10 225–34
T Berk Z Cohen RS McLeod HS Stern (1992) ArticleTitleManagement of mesenteric desmoid tumours in familial adenomatous polyposis Can J Surg 35 393–5
AL Knudsen S Bülow (2001) ArticleTitleDesmoid tumour in familial adenomatous polyposis. A review of the literature Fam Cancer 1 111–9
SK Clark RK Phillips (1996) ArticleTitleDesmoids in familial adenomatous polyposis Br J Surg 83 1494–504
M Schnitzler Z Cohen M Blackstein et al. (1997) ArticleTitleChemotherapy for desmoid tumors in association with familial adenomatous polyposis Dis Colon Rectum 40 798–801
MA Rodriguez-Bigas MC Mahoney CP Karakousis N Petrelli (1994) ArticleTitleDesmoid tumors in patients with familial adenomatous polyposis Cancer 74 1270–4
C Penna E Tiret R Parc et al. (1993) ArticleTitleOperation and abdominal desmoid tumors in familial adenomatous polyposis Surg Gynecol Obstet 177 263–8
G Moslein RR Dozois (1997) ArticleTitleDesmoid tumors associated with familial adenomatous polyposis Perspect Colon Rectal Surg 10 109–26
AK Gurbuz FM Giardiello GM Petersen et al. (1994) ArticleTitleDesmoid tumors in familial adenomatous polyposis Gut 35 377–81
AC Dudokdewit A Tibben HJ Duivenvoorden MF Niermeijer J Passchier RW Trijsburg (1998) ArticleTitleDistress in individuals facing predictive DNA testing for autosomal dominant late-onset disorders: comparing questionnaire results with in-depth interviews. Rotterdam/Leiden Genetics Workgroup Am J Med Genet 75 62–74
AC Dudokdewit HJ Duivenvoorden J Passchier MF Niermeijer A Tibben (1998) ArticleTitleCourse of distress experiences by persons at risk for an autosomal dominant inheritable disorder participating in a predictive testing program: an explorative study. Rottendam/Leiden Genetics Workgroup Psychosom Med 60 543–9
AC Dudokdewit A Tibben HJ Duivenvoorden MF Niermeijer J Passchier (1998) ArticleTitlePredicting adaptation to presymptomatic DNA testing for late onset disorders: who will experience distress? Rotterdam/Leiden Genetics Workgroup J Med Genet 35 745–54
S Michie M Bobrow TM Marteau (2001) ArticleTitlePredictive genetic testing in children and adults: a study of emotional impact J Med Genet 38 519–26
AM Codori KL Zawacki GM Peterson et al. (2003) ArticleTitleGenetic testing for hereditary colorectal cancer in children: long-term psychological effects Am J Med Genet 116A 117–28
CY Ko LC Rusin DJ Schoetz et al. (2001) ArticleTitleLong-term outcomes of the ileal pouch anal anastomosis: the association of bowel function and quality of life 5 years after surgery J Surg Res 98 102–7
T Berk (1999) The impact of living with hereditary colorectal disease J Utsunomiya JJ Mulvihill W Weber (Eds) Familial cancer & prevention John Wiley & Sons New York 315–9
SL Franzoi SA Shields (1984) ArticleTitleThe body esteem scale: multidimensional structure and sex differences in a college population J Pers Assess 48 173–9
IG Sarason HM Levine RB Basham BR Sarason (1983) ArticleTitleAssessing social support: The Social Support Questionnaire J Pers Soc Psychol 44 127–39
NB Epstein LM Baldwin DS Bishop (1983) ArticleTitleThe McMaster Family Assessment Device J Marital Fam Ther 9 171–80
AT Beck A Weissman D Lester L Trexler (1974) ArticleTitleThe measurement of pessimism: the hopelessness scale J Consult Clin Psychol 42 861–5
CE Ferrans MJ Powers (1992) ArticleTitlePsychometric assessment of the quality of life index Res Nursing Health 15 29–38
CE Ferrans (1990) ArticleTitleDevelopment of a quality of life index for patients with cancer Ontario Nurses Federation 17 (Suppl) 15–21
B Robb T Pritts G Gang et al. (2002) ArticleTitleQuality of life in patients undergoing ileal pouch-anal anastomosis at the University of Cincinnati Am J Surg 183 353–60
E Leger R Ladouceur MH Freeston (2002) ArticleTitleAnxiety and physical limitation: a complex relation Encephale 28 205–9
D Neumar-Sztainer M Story MD Resnick A Garwick RW Blum (1995) ArticleTitleBody dissatisfaction and unhealthy weight-control practices among adolescents with and without chronic illness: a population-based study Arch Pediatr Adolesc Med 149 1330–5
CD Thomas RJ Freeman (1990) ArticleTitleThe body esteem scale: construct validity of the female subscales J Pers Assess 54 204–12
H Cecil MA Stanley (1997) ArticleTitleReliability and validity of adolescents’ scores on the body esteem scale Educational Psychol Measurement 57 340–56
M Horowitz E Sundin A Zanko R Lauer (2001) ArticleTitleCoping with the grim news from genetic tests Psychosomatics 42 100–5
D Wellisch E Gritz W Schain H Wang J Siau (1992) ArticleTitlePsychological functioning of daughters of breast cancer patients, Part II: characterizing the distressed daughter of the breast cancer patient Psychosomatics 33 171–9
ACKNOWLEDGMENTS
The authors thank all of the patients who are associated with the Polyposis Registry and those who participated in this study. The authors also thank journal reviewers for their helpful comments.
Author information
Authors and Affiliations
Corresponding author
Additional information
A Research Scientist Career Award from the National Cancer Institute of Canada with funds from the Canadian Cancer Society supports Mary Jane Esplen.
About this article
Cite this article
Esplen, M., Berk, T., Butler, K. et al. Quality of Life in Adults Diagnosed With Familial Adenomatous Polyposis and Desmoid Tumor. Dis Colon Rectum 47, 687–696 (2004). https://doi.org/10.1007/s10350-003-0121-7
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10350-003-0121-7