Impact of haemophilia on the everyday life of elderly patients

Rationale: Thanks to improved haemophilia care, life span of people with haemophilia (PWH) has increased enormously resulting in the first generation of elderly PWHs. Since studies in the assessment of health status and health-related quality of life (HR-QoL) in elderly PWHs are scarce, a retrospective case-control study was carried out in Italy. Methods: Patients ‡65 years with severe haemophilia were investigated for health status, every day functioning (ADL: Autonomy in Daily Life, IADL: Instrumental Activity of Daily Living, TGBA: Tinetti Gait and Balance Assessment Scale), cognitive functioning and emotional well-being (Geriatric Depression Scale, HR-QoL). HR-QoL was assessed with generic (EQ-5D, WHOQOL-BREF, WHOQoL-Old) and disease-specific questionnaires (Haem-A-QoLElderly). Male controls without bleeding disorders were matched for age and geographical environment. Results: Out of 49 elderly PWHs registered in Italy 39 were enrolled (79.5%) with a mean age of 69.5 years (SD = 3.7) and M = 8.8 bleeds in the last year (0–48); 85% had haemophilia A, 13% had inhibitors, 79% received on-demand treatment, 87% had chronic hepatitis C and 13% had HIV. PWHs reported significantly worse HR-QoL compared to matched controls in the dimensions (WHOQOL-BREF: 'physical health', 'psychological'; WHOQOL-OLD: 'past activities', 'social' and in all dimensions of the EQ-5D, but 'anxiety/depression') and had significantly worse scores in the Geriatric Depression Scale. High correlations were found for ADL and TGBA with WHOQOL-OLD 'social' and WHOQOL-BREF 'physical health', and for TGBA with EQ-VAS. Conclusions: The study showed that elderly PWHs had significantly more impairments in daily activities, physical functioning, HR-QoL and depression than matched controls