Osteoporosis management and falls prevention in patients with haemophilia: Review of haemophilia guidelines

Abstract

Introduction: Patients with haemophilia (PWH) have a high prevalence of osteoporosis, falls and fractures at all ages. The role of haemophilia itself may contribute to low bone mineral density (BMD) due to coagulation factor deficiency. Guidelines for the management of osteoporosis, fracture and fall risk may help to reduce fracture and fall risk, and delay osteoporosis onset. Aim: We aim to review current haemophilia guidelines regarding osteoporosis prevention, screening, diagnosis and management, and fall prevention. Method: A database search (Ovid MEDLINE) revealed two haemophilia guidelines (World and British) published within the last ten years. Local Australian haemophilia guidelines were identified through a manual search. Results: All haemophilia guidelines were found to contain inadequate recommendations for osteoporosis management and fall prevention due to a lack of evidence in the literature. Conclusion: Further studies are required to assess the trajectory of bone health in PWH, the mechanism of bone loss in PWH, and the effectiveness of weight-bearing exercises, interventions for fall prevention, screening programmes, and use of antiosteoporosis medications in PWH across the lifecourse.

Association of bone mineral density and potential risk factors for osteoporosis in patients with severe haemophilia A

“…In our study, as many as 49.1% of the haemophilic patients had reduced bone mass. These results are worse than the literature data for adult PWH from recent years, 23–25 which report low BMD in approximately 27%−35% of haemophilic patients. Primary prophylaxis in children with severe haemophilia A was not introduced in Poland until 2008, while secondary prophylaxis in adults has been in place since 2012 2 .…”

“…22,23,26 In addition, HBV and HCV hepatotropic virus infections are independent risk factors for the development of secondary osteoporosis. 24,27 This study showed elevated inflammation parameters, including CRP and NLR, which were associated with newly diagnosed active HCV, HBV, and HIV infections and correlated with BMC. Some authors have suggested that NLR is an independent risk factor for the development of osteoporosis in elderly and menopausal women.…”

Pathogenesis and treatment of osteoporosis in patients with hemophilia

“…However, large-sample prospective drug therapy studies for osteoporosis in PWH are scarce. To date, only one study has evaluated the efficacy of ibandronate for osteoporosis in PWH [ 62 ]. In this study, 10 PWH with osteoporosis received ibandronate treatment for 12 months, and the lumbar spine BMD increased by 4.7%, but no significant change was found in the BMD of the total hip or femoral neck [ 71 ].…”

“…PWH have a predisposition to falls because of abnormal joint function, impaired mobility, and poor balance [61]. In PWH, the annual incidence of falls is 32-50%, and 53-81% of fractures are due to falls [62]. The WFH guidelines recommend that musculoskeletal assessment should be performed in adult PWH annually and in pediatric PWH every 6 months [59].…”

Diminished cortical bone density of long bones among children with haemophilic arthropathy

“…Despite these limitations, our investigation assessed a large sample of CwH with different disease severity. This investigation contributes to a better understanding of the direct and harmful consequences of decreased musculoskeletal health, 28 including sarcopenia 29 and osteoporosis, 2,3 which result in an increased propensity for falls, 30 fractures, 31 and hospitalizations, and, ultimately, premature death 32 . It is recommended that CwH engage in more physical activity, which may enhance muscle strength and eventually preserve bone strength.…”