Leiden University Scholarly Publications

Persistent URL of this record https://hdl.handle.net/1887/110389

In Collections

This item can be found in the following collections:

Lammens, C.R.M.; Bleiker, E.M.A.; Verhoef, S.; Hes, F.J.; Ausems, M.G.E.M.; Majoor-Krakauer, D.; ... ; Aaronson, N.K. (2010)

Psychosocial impact of Von Hippel-Lindau disease: levels and sources of distress

Article / Letter to editor
Von Hippel-Lindau disease (VHL) is a hereditary tumor susceptibility syndrome, characterized by an increased risk of developing multiple benign and malignant tumors at various sites and ages with limited preventive options. This study evaluates the prevalence of distress among VHL family members and factors associated significantly with such distress. Forty-eight families with a VHL mutation were identified via the nine family cancer clinics in the Netherlands. In total, 171 family members (carriers, 50% at-risk, non-carriers) were approached, of whom 123 (72%) completed a self-report questionnaire. Approximately 40% of the VHL family members reported clinically relevant levels of distress, approaching 50% among the carriers and, possibly even more striking, 36% among the non-carriers. Having lost a first degree relative due to VHL during adolescence (OR 11.2; 95% CI 1.4-86.9) was related significantly to heightened levels of distress. Approximately, only one-third of those who...Show moreVon Hippel-Lindau disease (VHL) is a hereditary tumor susceptibility syndrome, characterized by an increased risk of developing multiple benign and malignant tumors at various sites and ages with limited preventive options. This study evaluates the prevalence of distress among VHL family members and factors associated significantly with such distress. Forty-eight families with a VHL mutation were identified via the nine family cancer clinics in the Netherlands. In total, 171 family members (carriers, 50% at-risk, non-carriers) were approached, of whom 123 (72%) completed a self-report questionnaire. Approximately 40% of the VHL family members reported clinically relevant levels of distress, approaching 50% among the carriers and, possibly even more striking, 36% among the non-carriers. Having lost a first degree relative due to VHL during adolescence (OR 11.2; 95% CI 1.4-86.9) was related significantly to heightened levels of distress. Approximately, only one-third of those who reported heightened levels of distress had received professional psychosocial support. A substantial percentage of family members experience clinically relevant levels of distress. We would recommend the introduction of a procedure for screening for distress in this vulnerable population. Special attention should be paid to those individuals who have lost a close relative due to VHL during adolescence.Show less
distress psychosocial impact quality of life Von Hippel Lindau disease worries hereditary breast-cancer colorectal-cancer psychological distress health survey risk family perceptions patient history sf-36
All authors
Lammens, C.R.M.; Bleiker, E.M.A.; Verhoef, S.; Hes, F.J.; Ausems, M.G.E.M.; Majoor-Krakauer, D.; Sijmons, R.H.; Luijt, R.B. van der; Ouweland, A.M.W. van den; Os, T. van; Hoogerbrugge, N.; Garcia, E.B.G.; Dommering, C.J.; Gundy, C.M.; Aaronson, N.K.
Date
2010-05-31
Journal
Clinical Genetics
Volume
77
Issue
5
Pages
483 - 491
DOI
doi:10.1111/j.1399-0004.2010.01333.x