The main focus of lower limb physical performance assessment in people with haemophilia (PWH) has usually been on function, muscle strength and joint flexibility. The impact of haemophilic arthropathy on balance and falls risk is relatively under-explored. The aim of this study was to evaluate balance and related performance in PWH compared with age and gender matched healthy controls. It involved a comprehensive suite of clinical and laboratory measures of static and dynamic balance, mobility, strength, physical activity and falls efficacy completed in 20 PWH (mean age 39.4, 100% male) and 20 controls. Fifty percent of PWH reported falls in the past 12 months. Moderate impairment of balance and related measures were identified in PWH compared with the controls, with an average 35% difference between groups. Significant differences were evident between groups on both clinical and laboratory measures, including measures of dynamic bilateral stance balance [limits of stability measures on the laboratory test, functional reach; (P < 0.001); dynamic single leg balance (Step Test, P < 0.001)], gait and mobility (gait speed, step width and turning measures on the laboratory test, timed up and go test; P < 0.001); muscle strength (timed sit to stand, P = 0.002; quadriceps strength, P < 0.001); and activity level and falls efficacy, (P < 0.004). The dynamic clinical and laboratory measures testing similar domains of balance, gait and mobility had moderate correlations (0.310 < r < 0.531, P < 0.01). Moderate impairments in balance, mobility and related measures were identified in PWH, compared with the control group. Clinicians should include assessments of balance and related measures when reviewing adults with haemophilia.

Adults with haemophilia and other bleeding disorders often develop lower limb musculoskeletal problems associated with bleeds into joints and muscles, which may affect balance performance and increase likelihood of falling. The aim of this study was to evaluate the effectiveness of an individualized balance and strength home exercise programme on improving balance and related outcomes for adults with haemophilia and other bleeding disorders. Twenty male adults with haemophilia and other bleeding disorders (mean age 39.4 years, 95% CI = 33.7-45.1) were recruited to participate. They underwent a comprehensive clinical and force platform assessment of balance and related measures. Based on assessment findings, the assessing physiotherapist provided an individualized home exercise programme of balance, strengthening and walking exercises. Re-assessment occurred after the 4-month exercise programme. Twelve participants (60%) completed the programme and were re-assessed. There were no safety problems or dropouts associated with the exercise programme aggravating joint status. Although there were no statistically significant changes in any of the measures (adjusted for multiple comparisons), there were improvements of between 5% and 22% on 10 of the 16 measures, with the Neurocom modified Clinical Test of Sensory Interaction on Balance (P = 0.036) and Timed Sit to Stand (P = 0.064) approaching significance. A tailored home exercise programme targeting balance, strengthening and walking is feasible for adults with haemophilia and other bleeding disorders. These results suggest that positive physical outcomes including improved balance and mobility may be achieved with this type of programme.

In oral surgery, patients with inherited bleeding disorders have historically had factor cover where possible. Factor support is expensive, time consuming to administer and places the patient at a potential risk of complications of therapy. A protocol employing rigorous local measures and minimal factor replacement was used to obtain haemostasis following simple and complex oral surgery on 50 consecutive patients with inherited bleeding disorders, referred to the Alfred Health Dental Unit from the Ronald Sawers Haemophilia Centre, Alfred Health, Melbourne. Excellent haemostasis was achieved using standardized local measures of 5% tranexamic acid solution, surgicel and monocryl sutures. Oral surgery may be considered safe to perform in patients with inherited bleeding disorders using minimal factor support and meticulous local haemostatic measures.

The risk of bleeding in patients with hereditary bleeding disorders (HBD) undergoing gastro-intestinal (GI) endoscopic procedures is unknown but guidelines generally recommend correction of factor deficiency. Investigate the safety of oral tranexamic acid (TA) without prophylactic factor replacement to prevent bleeding complications in patients with HBD undergoing elective GI endoscopic procedures. A prospective single-arm pilot study testing the feasibility of using TA, without prophylactic factor replacement or desmopressin preprocedure, for prevention of bleeding complications following elective standard risk (<1% risk of bleeding) endoscopic procedures in patients with HBD. Baseline factor levels, haemoglobin and iron studies (IS) were measured preprocedure. Primary outcome of bleeding (NCI CTCAE v3.0 Bleeding Scale) was undertaken by patient review and repeat Hb, IS on day 21. Twenty-eight patients underwent 32 GI endoscopic procedures from September 2010 until June 2012. The median age was 53 years (range 24-75 years) and disease types included mild haemophilia A/B (n = 12), severe haemophilia A/B (n = 9), von Willebrand disease (n = 5), FXI deficiency (n = 1) and FVII deficiency (n = 1). Procedures performed included 11 gastroscopies, 12 colonoscopies, 8 gastroscopies and colonoscopies and 1 flexible sigmoidoscopy. Fourteen standard risk procedures and two high risk procedures were performed. Two patients experienced Grade 1 bleeding and one patient experienced Grade 2 bleeding. This study suggests that TA without prophylactic factor replacement may be a safe approach for mild and moderate HBD patients undergoing standard risk endoscopic procedures, particularly where no biopsy is performed. These findings should be confirmed in a larger study.

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