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

Chantal R. M. Lammens, Eveline M. A. Bleiker, S. Verhoef, Frederik J. Hes, M. G. E. M. Ausems, Danielle Majoor-Krakauer, Rolf H. Sijmons, Rob B. van der Luijt, Ans M. W. van den Ouweland, Theo A. Mvan Os, N. Hoogerbrugge, E. B. Gomez Garcia, C. J. Dommering, C. M. Gundy, Neil K. Aaronson*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

30 Citations (Web of Science)

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.
Original languageEnglish
Pages (from-to)483-491
JournalClinical Genetics
Volume77
Issue number5
DOIs
Publication statusPublished - May 2010

Keywords

  • distress
  • psychosocial impact
  • quality of life
  • Von Hippel
  • Lindau disease
  • worries

Cite this