Familial Cancer

, Volume 8, Issue 1, pp 59–65 | Cite as

Psychosocial impact of Peutz-Jeghers Syndrome

  • Alice Woo
  • Amit Sadana
  • David T. Mauger
  • Maria J. Baker
  • Terri Berk
  • Thomas J. McGarrity
Article

Abstract

Peutz-Jeghers Syndrome (PJS) is an autosomal dominant inherited cancer predisposition syndrome and gastrointestinal hamartomatous polyposis syndrome characterized by the presence of distinct perioral freckling. To date, we have not found any tool that specifically assesses the psychosocial impact of PJS on patients. We developed a PJS quality of life questionnaire using expert opinions of 3 cancer genetic counselors and a survey of patients with PJS through recruitment of participants involved in a support group over the internet. We measured and compared our questionnaire results to the widely used Center for Epidemiologic Studies and Depression Scale (CES-D) and the Short Form 36 (SF-36). We recruited 38 patients for our study. Volunteers were mailed a consent form, the self-administered CES-D, SF-36 and our developed PJS questionnaire and were instructed to return the completed questionnaires by mail. Results showed that PJS patients suffer from mild depression even though physically they did not feel impacted by their condition compared to the general population. However, having PJS caused them to alter many important life decisions. The PJS Questionnaire correlated with data obtained from analysis of CES-D, as well as the SF-36. More uniquely, it provided specific information regarding the burden of disease and quality of life in patients affected with Peutz-Jeghers syndrome. Its ability to do so for other polyposis syndrome populations remains to be studied. These results are important in developing plan of care for these patients regarding genetic counseling and surveillance strategies for PJS patients.

Keywords

Peutz-Jeghers Syndrome Psychosocial impact Questionnaire development CES-D SF-36 

Notes

Acknowledgements

We would like to thank Ms. Beverly Bell and Mary Maiolo for their excellent secretarial assistance in the completion of this manuscript. We also acknowledge the contributions of Linda Nations and Christopher I. Amos, Ph.D., Department of Epidemiology and Biomathematics, UT M.D. Anderson, Houston, Texas.

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Copyright information

© Springer Science+Business Media B.V. 2008

Authors and Affiliations

  • Alice Woo
    • 1
  • Amit Sadana
    • 2
  • David T. Mauger
    • 3
  • Maria J. Baker
    • 4
  • Terri Berk
    • 5
  • Thomas J. McGarrity
    • 2
  1. 1.Penn State College of Medicine, Department of MedicineMilton S. Hershey Medical CenterHersheyUSA
  2. 2.Department of Medicine, Division of Gastroenterology/HepatologyMilton S. Hershey Medical Center, Penn State UniversityHersheyUSA
  3. 3.Department of Health Evaluations and SciencesMilton S. Hershey Medical CenterHersheyUSA
  4. 4.Department of Medicine and PediatricsPenn State Cancer Institute, Milton S. Hershey Medical CenterHersheyUSA
  5. 5.Digestive Diseases Clinical Research CentreFamilial GI Cancer Registry, Mount Sinai HospitalTorontoCanada

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