title: Psychosocial impact of Von Hippel-Lindau disease: levels and sources of distress.
abstract: 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 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.
authors.affiliation: Department of Psychosocial Research & Epidemiology, The Netherlands Cancer Institute-Antoni van Leeuwenhoek hospital (NKI-AVL), Plesmanlaan 121, 1066 CX Amsterdam, the Netherlands.
authors.forename: C R M
authors.initials: CR
authors.lastname: Lammens
authors.forename: E M A
authors.initials: EM
authors.lastname: Bleiker
authors.forename: S
authors.initials: S
authors.lastname: Verhoef
authors.forename: F J
authors.initials: FJ
authors.lastname: Hes
authors.forename: M G E M
authors.initials: MG
authors.lastname: Ausems
authors.forename: D
authors.initials: D
authors.lastname: Majoor-Krakauer
authors.forename: R H
authors.initials: RH
authors.lastname: Sijmons
authors.forename: R B
authors.initials: RB
authors.lastname: van der Luijt
authors.forename: A M W
authors.initials: AM
authors.lastname: van den Ouweland
authors.forename: T A M
authors.initials: TA
authors.lastname: Van Os
authors.forename: N
authors.initials: N
authors.lastname: Hoogerbrugge
authors.forename: E B
authors.initials: EB
authors.lastname: Gómez García
authors.forename: C J
authors.initials: CJ
authors.lastname: Dommering
authors.forename: C M
authors.initials: CM
authors.lastname: Gundy
authors.forename: N K
authors.initials: NK
authors.lastname: Aaronson
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D016015:Logistic Models; D008297:Male; D011788:Quality of Life; D012944:Social Support; D011795:Surveys and Questionnaires; D006623:von Hippel-Lindau Disease / Q000523:psychology*