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Study comparing two types of screening provision for people with von Hippel-Lindau disease

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Abstract

Patients diagnosed with von Hippel-Lindau disease (vHL) require life-long surveillance for this multi-system disease. It is therefore important to assess the comprehensiveness of screening provision, as well as identify what type of screening service is most likely to encourage regular patient attendance. This descriptive study reports on two types of screening service: single appointment One Stop (OS) clinics and multiple appointment Ad Hoc (AH) clinics. One hundred and seventeen vHL patients from eight regional genetics centres were approached to take part. Seventy-two (61.5%) returning a completed study questionnaire: fifty-four (75%) were screened at OS clinics and eighteen (25%) at AH clinics. Comprehensiveness of screening, attendance rates, patient ratings of quality of care and levels of psychological morbidity were compared between the two types of service. While levels of disease severity were similar in patients screened at OS and AH clinics, those seen at OS clinics were screened for twice as many site-specific vHL manifestations compared to those seen at AH clinics (P < 0.0001). More patients at OS clinics regularly attended their screening appointments compared to those at AH clinics (P = 0.0045). There was no difference in the quality of care reported by patients attending the two types of screening service and few problems were reported. Twenty-nine percent of respondents were categorised as clinically anxious and 13% as clinically depressed. These findings suggest that an optimum vHL screening service is one based on OS clinics offering regular comprehensive surveillance and psychological support.

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Abbreviations

vHL:

von Hippel-Lindau disease

OS:

One Stop

AH:

Ad Hoc

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Acknowledgements

We would like to acknowledge the support received from members of the Steering Committee for the British Family Cancer Record (John Burn, Tim Bishop, Eamonn Sheridan, Lauren Padmore, Peter Sasieni, Huw Thomas, Gwen Turner, Richard Turner, Robin Waxman); the consultants (Anneke Lucassen, Fiona Douglas, Fiona Lalloo, Joan Paterson, Sozia Miedzybrodzka) and genetics counsellors (Gillian Crawford, Peter Risby, Carol Giblin, Helen Burton, Sheila Goff, Deborah Leatham) at participating centres; and Teresa Mielniczek for help in collating the data. Without their help this study would not have been possible. This work was supported by grants from Cancer Research UK and the National Lottery Charities Board.

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Authors and Affiliations

  1. Gynaecological Oncology, UCL Elizabeth Garrett Anderson Institute for Women’s Health, 49 Tottenham Court Road, London, W1T 7DN, UK

    Lindsay Fraser

  2. Department of Clinical Genetics, Guy’s Hospital, London, UK

    Sally Watts

  3. Cancer Research UK, Centre for Statistics in Medicine, Institute of Health Sciences, University of Oxford, Oxford, UK

    Anna Cargill

  4. Department of Public Health & Primary Care, University of Cambridge, Cambridge, UK

    Stephen Sutton

  5. St George’s Hospital, University of London, London, UK

    Shirley Hodgson

Authors
  1. Lindsay Fraser
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  2. Sally Watts
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  3. Anna Cargill
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  4. Stephen Sutton
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  5. Shirley Hodgson
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Corresponding author

Correspondence to Lindsay Fraser.

Appendix

Appendix

Table 6 VHL Disease Severity Index
Full size table

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Fraser, L., Watts, S., Cargill, A. et al. Study comparing two types of screening provision for people with von Hippel-Lindau disease. Familial Cancer 6, 103–111 (2007). https://doi.org/10.1007/s10689-006-9111-y

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  • DOI: https://doi.org/10.1007/s10689-006-9111-y

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