The use of a co-design model in improving timely bleed reporting by adults with haemophilia living in the Auckland region of New Zealand.

Many adult patients diagnosed with phenotypically moderate and severe haemophilia living in the Auckland region of New Zealand do not report bleeding episodes within a timeframe that allows for optimal assessment and management. This can result in poor clinical outcomes for patients and poor oversight of the use of expensive clotting factor concentrates. Our goal was to improve both the number and speed at which bleeding episodes were reported to our centre, improving access to care and clinical oversight of the use of expensive factor concentrates and aiding the development of a care partnership with patients. We worked with 70 adult PWH living in the Auckland region of New Zealand with moderate and severe haemophilia A and B. Over a 5-month period between March and July 2013 we used a co-design model to develop and implement a range of strategies to improve the timing and frequency of bleed reporting. Mean bleed reporting time was reduced threefold, with a threefold increase in the number of bleeds reported per month. We reduced the number of bleeding episodes reported outside of a prespecified 48-h time limit by 68%. We significantly improved bleed reporting and time to report, indicating improved access to our services, improved clinical oversight and improved accountability to our national funder. We have achieved a care partnership and a reduction in factor consumption for the study population without compromising the quality of care they receive.

Conservative physiotherapeutic management of chronic haematomata and haemophilic pseudotumours: case study and comparison to historical management.

A conservative, non-operative physiotherapeutic regime for the management of chronic haematomata and pseudotumours in patients suffering from haemophilia is described in this article. Two cases are described where physiotherapy treatment is applied to large masses at the shoulder and femur respectively, where therapy commenced within the first 6 months following onset. These are presented relative to a case that was managed over a much longer period without early physiotherapy input, and the relative outcomes are examined. While both the early physiotherapy-managed cases showed a complete resolution at follow-up examination, the more established chronic pseudotumour required surgical excision, with significant residual muscle contractility, length and strength issues noted on clinical and magnetic resonance imaging reviews. No adverse symptoms or haemostatic issues were reported in response to this less invasive treatment regime by either patient in the two conservative physiotherapy cases.

Domiciliary application of CryoCuff in severe haemophilia: qualitative questionnaire and clinical audit.

The acute management of haemophilic bleeding episodes in the home setting is based on the concept of immediate factor replacement therapy and the PRICE regime--an acronym representing the concepts of Protection, Rest, Ice, Compression and Elevation [1,2]. Integral to this regime is the application of cold therapy, and yet little is known regarding the safe periods of application, or the relative safety of cryotherapy devices such as the CryoCuff when used in the home setting by patients suffering from severe haemophilia and related bleeding disorders. This study examines the subjective patient response to the application of the CryoCuff device in the home setting in terms of the effect on pain, joint swelling and the return to 'pre-bleed status' of the knee, ankle or elbow in patients with severe haemophilia A/B or type III von Willebrand's disease (VWD) immediately following haemarthrosis, and any potential adverse effects related to the device or recommended duration of application as stated in the PRICE guideline (Fig. 1). Twelve patients, either with severe haemophilia A/B or with VWD were recruited and asked to use the CryoCuff device as part of the PRICE regime immediately following the onset of knee-, ankle- or elbow bleeds for the next one year. Each subject was then sent a qualitative questionnaire to determine subjective responses to the device. All patients reported that the application protocol was easy to follow, they were able to apply the device as per the PRICE regime and they were able to tolerate it for the recommended period. Whereas, all the patients felt that the device had a significant impact on alleviation of pain and return to pre-bleed status, 78% of the patients felt that the device led to a significant reduction in swelling around the affected joint. There was no conclusive evidence that the device resulted in any reduction in the amount of factor used to treat the acute bleeding episode, however, no patients reported any perceived delay in achieving haemostasis or required extra factor replacement therapy consequent to the usage of the device. No other adverse effects were reported by participants in this study.