Physiotherapist inter-rater reliability of the Haemophilia Early Arthropathy Detection with Ultrasound protocol.

INTRODUCTION: Prevention of arthropathy is the major goal of haemophilia treatment, and early detection of the first signs of joint damage is important so that prevention strategies can be initiated to limit physical disability and improve quality of life. AIM: The aim of this study was to determine the inter-rater repeatability of the HEAD-US protocol when performed by haemophilia physiotherapists. METHODS: Sixty-three joints (21 elbows, 21 knees and 21 ankles) were examined in 21 patients (mean age; 29.14 ± 10.09 years) according to the HEAD-US protocol by four of six physiotherapists blinded to clinical information. Inter-rater repeatability was evaluated by comparing the concordance of scores between the different clinicians using weighted Kappa (κw ) statistics and 95% confidence intervals. RESULTS: Substantial agreement was found when evaluating synovial hypertrophy (κw  = 0.69), articular cartilage (κw  = 0.60) and bone (κw  = 0.74). Near perfect repeatability (κw  > 0.80) was found when scoring synovitis profiles of the elbow joint and bone profiles of the knee joint. Repeatability was moderate for scoring synovitis and articular cartilage profiles of the ankle joint. A moderate correlation between HEAD-US and HJHS was found for the elbow and ankle joint and a strong correlation for the knee joint. Discordance between clinical and ultrasound examination was 19.30%. CONCLUSION: Overall, we found repeatability of the HEAD-US protocol was good when performed by physiotherapists, and our results support the emerging view that US imaging complements the physical examination when screening and monitoring joint health of people with haemophilia at the point of care.

Management of muscle haematomas in patients with severe haemophilia in an evidence-poor world.

Treatment studies in haemophilia focus on joint bleeds; however, some 10-25% of bleeds occur in muscles. This review addresses management of muscle haematoma in severe haemophilia, defines gaps in the published evidence, and presents a combined clinician and physiotherapist perspective of treatment modalities. The following grade 2C recommendations were synthesized: (i) Sport and activity should be based on individual factor levels, bleeding history and physical characteristics, (ii) Musculoskeletal review aids the management of children and adults, (iii) 'Time to full recovery' should be realistic and based on known timelines from the healthy population, (iv) Diagnosis should be carried out by both a clinician and physiotherapist, (v) Severe muscle bleeds should be treated similarly to surgical patients: a 50% trough for 10-14 days followed by high-level prophylaxis, (vi) Protection, rest, ice, compression and elevation should be implemented in the acute stage, and (vii) Physiotherapy and rehabilitation should be divided into: control of haemorrhage (phase 1); restoration of Range of Movement (ROM) and strength (phase 2); functional rehabilitation and return to normal living (phase 3). Recommendations specifically for inhibitor patients include: (i) Minor to moderate bleeds should be managed by home-treatment within 1 h of bleed onset using either one injection of rFVIIa 270 µg kg(-1), or two to three injections of rFVIIa 90 µg kg(-1) (2-3 h intervals), or FEIBA 50-100 U kg(-1) (repeated at 12-hourly intervals, if necessary) and (ii) Severe muscle bleeds should be supervised by the treatment centre and include bypassing agents until clinical improvement is observed.