HAPII Impact and prevalence study
Research type
Research Study
Full title
The prevalence and impact of blood induced ankle arthritis in patients with moderate and severe haemophilia A and B: The HAPII study
IRAS ID
206141
Contact name
Richard Wilkins
Contact email
Sponsor organisation
University of Leeds
Duration of Study in the UK
2 years, 6 months, 31 days
Research summary
Summary of Research
Haemophilia impairs bleeding control because of an absence of clotting factor VIII (haemophilia A) or factor IX (haemophilia B) resulting in bleeding into muscles and joints. Although treatment with artificial clotting factor has some effect on frequency of bleeds, most patients still experience ongoing spontaneous and traumatic bleeds in joints. Prolonged exposure of joint structures to blood leads to cartilage, structural and functional damage, ultimately resulting in pain and disability. Damage also leads to changes in joint function furthering the risk of subsequent mechanical joint and soft tissue haemorrhage. Ultimately, this vicious cycle results in cumulative and irreversible damage to joints with severe reduction in mobility. The introduction of clotting factor substitution, both prophylactically and on-demand, has dramatically improved life expectancy and quality of life in patients with haemophilia. The paradigm shift from treating a bleeding episode, to prevention with regular replacement therapy (prophylaxis) has seen a reduction in bleeding frequency. Since the introduction of clotting factor, the most common site for musculoskeletal bleeding has also changed from the knee to the tibiotalar (ankle) joint. Bleeding into the ankle joint is now estimated to account for 20% of all haemophilia-related bleeds in the UK. Despite this, little has been reported on the prevalence and impact of chronic ankle haemarthrosis.Summary of Results
Haemophilia impairs bleeding control because of an absence of clotting factor VIII (haemophilia A) or factor IX (haemophilia B) resulting in bleeding into muscles and joints. Although treatment with artificial clotting factor patients may have experienced bleeding into joint caused by spontaneous or traumatic bleeding. Prolonged exposure of joint structures to blood leads to cartilage, structural and functional damage, ultimately resulting in pain and disability. Damage also leads to changes in joint function furthering the risk of subsequent mechanical joint and soft tissue haemorrhage. Ultimately, this vicious cycle results in cumulative and irreversible damage to joints with severe reduction in mobility. Since the introduction of clotting factor, the most common site for musculoskeletal bleeding has also changed from the knee to the tibiotalar (ankle) joint. Bleeding into the ankle joint is now estimated to account for 20% of all haemophilia-related bleeds in the UK. Despite this, little has been reported on the prevalence and impact of chronic ankle haemarthrosis.
This study has identify that even the most compliant of patients with factor treatment still experience bleeding into their joints. Whilst the incidence of bleeding is low the ankle joint is disproportionately affected by blood induced ankle arthritis. A subsequent multi-centre questionnaire was conducted at 18 haemophilia centres across the Uk. A total of 243 patients with blood induced ankle arthritis reported poor health related quality of life and foot and ankle outcomes equivalent to patients with ankle osteoarthritis awaiting joint surgery to relieve pain. Patients in this study had moderate to severe levels of joint arthritis and pain. The study has provided insight to the main joint affected by blood induced arthritis and provided direction for future research into the management of the condition.REC name
London - Queen Square Research Ethics Committee
REC reference
16/LO/2251
Date of REC Opinion
13 Jan 2017
REC opinion
Further Information Favourable Opinion