Health-related quality of life and psychological well-being in elderly patients with haemophilia

S. Von Mackensen, A. Gringeri, S. M. Siboni, P. M. Mannucci

Research output: Contribution to journalArticlepeer-review


Many persons with severe haemophilia reach seniority thanks to effective treatment. There is no information on health-related quality of life (HRQoL) of these patients, who had lived for many years when regular replacement therapy was unavailable. Italian patients with severe haemophilia aged ≥65years born in the 1940s or earlier were compared with men without bleeding disorders matched for age and geography. HRQoL was assessed via generic and disease-specific questionnaires. Potential associations with concomitant illnesses, orthopaedic status, physical functioning, cognitive status and depression were evaluated. In addition, the newly adapted HRQoL questionnaire specific for elderly persons with haemophilia (Haem-A-QoL Eldlery) was psychometrically tested and validated. Thirty-nine patients, aged 65-78years, were investigated, 33 with haemophilia A and six with haemophilia B, and compared to 43 controls, aged 65-79years. Chronic blood borne viral infections, hypertension and arthropathy were more frequent in patients, whereas hypercholesterolemia and cardiovascular diseases were more frequent in controls. Psychometric characteristics of Haem-A-QoL Elderly showed good to excellent values for reliability and validity. HRQoL was worse in patients at EQ-VAS, WHOQOL-BREF and WHOQOL-Old. The highest impairments were found in patients by means of the haemophilia-specific Haem-A-QoL Elderly in such dimensions as 'physical activity & leisure', 'physical health' and 'view'. A poor orthopaedic status was negatively associated with HRQoL. Compared to age-matched controls elderly patients with haemophilia had an impaired HRQoL in association with their health status. The newly developed Haem-A-QoL Elderly proved to be a reliable and valid instrument for HRQoL assessment in elderly haemophilia patients.

Original languageEnglish
Pages (from-to)345-352
Number of pages8
Issue number3
Publication statusPublished - May 2012


  • Ageing
  • Cognitive status
  • Daily autonomy
  • Haemophilia
  • Health-related quality of life
  • Physical functioning

ASJC Scopus subject areas

  • Hematology
  • Genetics(clinical)


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