Current hemophilia guidelines are inadequate for the management of musculoskeletal health


A new review of current haemophilia guidelines found that all contained inadequate recommendations for the management of osteoporosis and the prevention of falls in patients with haemophilia (PWH) due to a lack of evidence in Literature.

Earlier diagnosis and treatment of osteoporosis in PWH is important, as fractures have been associated with significant morbidity and mortality.

“Determining the effectiveness of osteoporosis screening and treatment programs in adults and children with hemophilia will improve the management of musculoskeletal health in PWH,” study author Ayse Zengin wrote. , PhD, Department of Medicine, School of Clinical Sciences, Monash University.

Given the low bone mineral density (BMD) and increased prevalence of fractures in PWH, it is important for physicians to screen, diagnose, and treat these patients for osteoporosis. This study reviewed recent hemophilia guidelines regarding the musculoskeletal management of patient care.

Investigators searched Ovid MEDLINE to identify the most recent hemophilia guidelines, limiting them to the last 10 years. Inclusion criteria included recent general haemophilia guidelines from any country in the English language containing a section on osteoporosis and/or falls prevention.

The search ultimately resulted in 3 guidelines, including the 2020 UK guidelines, the 2020 World Federation of Hemophilia (WFH) and the 2016 Australian hemophilia guidelines.

WFH guidelines outline uncertainty about whether all PWH need routine osteoporosis screening, but suggest screening may be appropriate for those at high risk or with multiple clinical risk factors of osteoporosis.

The UK guidelines only recommend regular osteoporosis screening for PWH on antiretrovirals and state that the fracture risk assessment tool can be used as a guideline. Australian guidelines recommend screening according to local guidelines, in which a risk assessment should be performed in postmenopausal women and men > 50 years with ≥ 1 major risk factor for minimal traumatic fractures.

According to the data, the majority of fractures in PWH occurred much earlier than in the general population, with 81% occurring before the age of 50. Additionally, evidence has shown that the prevalence of fractures increases with the severity of haemophilia, as data show that fracture risk is 44% higher in people with severe disease, compared to severe disease. mild to moderate.

As such, current osteoporosis screening and diagnosis guidelines remain inadequate due to the limited data available to provide improved evidence-based recommendations.

Additionally, WFH guidelines state that anti-osteoporosis medications should be started in PWH with osteoporosis, minimal traumatic fractures, or at high risk of fracture and additionally started bisphosphonates if needed. However, UK guidelines do not recommend specific osteoporosis medications.

Although anti-osteoporosis drugs have been effective in improving BMD and subsequently reducing mortality risk, it remains to be assessed whether treatment benefits are seen in PWH.

Additionally, falls prevention is not specifically addressed in any of the guidelines. Australian and WFH guidelines recommend that PWH have a team of musculoskeletal specialists and undergo annual assessment for adults and every 6 months for children.

Investigators in the current study noted that falls are a major contributor to fractures in PWH, so the guidelines should include a section on fall prevention. Data show that 53-81% of fractures in PWH are due to falling from a standing height or below, with the annual prevalence of falls being 32-50% in PWH.

“Further studies are needed to assess the trajectory of bone health in PWH, the mechanism of bone loss in PWH, and the effectiveness of weight bearing exercises, fall prevention interventions, screening programs and anti-osteoporosis medication use in PWH across the life course,” Zengin noted.

The study, “Osteoporosis management and falls prevention in patients with hemophilia: Review of hemophilia guidelines,” was published in Haemophilia.


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