Frailty and frailty syndromes in persons with haemophilia: a review.

Abstract

Advances in treatment for persons with haemophilia (PWH) have improved life expectancy, but PWH face new challenges, including frailty. Frailty is a health state of increased functional vulnerability. It is associated with ageing and is linked to adverse outcomes such as falls, hospitalisation and institutionalisation. Although well-characterised in the general population, its prevalence and impact in PWH remain underexplored. Emerging evidence suggests PWH currently have increased frailty, particularly those with severe disease and joint damage from inadequate early prophylaxis. This review examines frailty in PWH including the impact of frailty syndromes (falls, immobility, incontinence, cognitive impairment, polypharmacy) and the potential role of the haemophilia multidisciplinary team in screening and management. The Clinical Frailty Score can be used by non-specialists and can aid in early identification and subsequent intervention including referral to Geriatric Medicine when appropriate. Importantly, interventions such as the comprehensive geriatric assessment have been shown to reverse or slow progression of frailty with improved health outcomes. An individualised multidisciplinary approach, including falls prevention, tailored exercise regimes, and medication review, is key. Cognitive impairment and dementia may impact self-management, necessitating dementia-friendly healthcare strategies. Given the growing population of older PWH, it is crucial to increase awareness of frailty for both the haemophilia multidisciplinary team and PWH, to facilitate screening, evidence-based interventions, optimisation of care pathways, and ensure comprehensive multidisciplinary support to improve quality of life. Future studies should address the specific needs of ageing PWH, including women, and further investigate the impact of frailty on clinical outcomes in PWH.