Health status in elderly persons with haemophilia

Rationale: Poor information on health and mental status and health-related quality of life (HR-QoL) of elderly people with haemophilia (PWH) is available. A multicenter case-control study has been designed assessing their health status, cognitive functioning and HR-QoL. Methods:Patients with severe haemophilia, aged ‡65 years, and controls without bleeding disorders, matched for age, sex and geographical environment, were evaluated for concomitant illnesses (Cumulative Illness Rating Scale–CIRS), orthopaedic status (WFH Orthopaedic Joint Score), physical activity levels (Autonomy in Daily Life–ADL, Instrumental Activity of Daily Living–IADL, Tinetti Gait and Balance Assessment Scale–TGBA) and cognitive status (Mini-Mental State Evaluation). Generic (WHOQoL-Bref, EQ-5D, WHO-QoL-Old) and disease-specific HR-QoL (Haem-A-QoLElderly) was assessed together with depression (Geriatric Depression Scale). Results: 39 patients, aged 65–78 years (mean 69), of whom 33 had haemophilia A and 79% received on demand treatment, and 44 controls, aged 65–79 years (mean 70) were enrolled. Significantly more PWH were suffering from chronic hepatitis B (10.8% versus 0) and C (86.8% versus 4.5%) with cirrhosis and hepatocarcinoma in 6 and 3 PWH respectively, HIV infection (13.2% versus 0) and blood hypertension (71.8% versus 45.5%). Controls were suffering significantly more from hypercholesterolemia (38.6% versus 2.7%) and cardiovascular diseases (36.4% versus 7.8%). All but 3 PWH had arthropathy (OJS: mean = 19.8, range: 0–67, versus controls mean = 1.36, range: 0–10). PWH showed worse ADL and TGBA, but similar for IADL and MMSE compared to controls. HR-QoL results are shown elsewhere. Conclusions: PWH have more co-morbidities and daily living problems, but similar mental health compared to controls.