Disadvantages include a high degree of operator dependency and inability to access the central surfaces of the joints of maximal interest in people with haemophilia. Standardized protocols for ultrasound assessment of ankles, knees and elbows have been published [19-21]. Recently, Martinoli and colleagues have reported details of a simplified ultrasound Sunitinib scanning protocol and scoring system for

use in people with haemophilia, the Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) [22]. Studies of ankles, knees and elbows for 49 subjects with haemophilia yielded good to excellent inter- and intra-observer reliability with this scoring system. Although promising, the HEAD-US method requires validation against physical examination, radiography and MRI in a larger series of individuals with haemophilia. The Haemophilia Activities List (HAL) is a patient-reported questionnaire developed by Dutch investigators that can be used as part of a test battery to evaluate the functional health status of adults with haemophilia. The investigators recommended that both a disease-specific activity measure (e.g. the HAL) and a performance test should be included BI 6727 mouse when assessing the functional health status of people with haemophilia [23]. The developmental studies of the HAL were conducted in adults with

haemophilia, most of whom had the severe form of the disorder [23, 24]. The investigators were careful to emphasize that additional studies in children <18 years of age and in adults with moderate/mild haemophilia A are required to determine if the current version of the HAL requires modification for use in these patient populations. A paediatric version of the HAL (pedHAL) has been developed and is being evaluated. The Functional Independence Score in Haemophilia (FISH) 上海皓元 is an objective, performance-based assessment tool developed by investigators at the Christian Medical College, Vellore, India to assess the functional ability of adults with haemophilia [25, 26]. When used by trained healthcare personnel

the current version of the tool has excellent measurement properties [26, 27]. A modification in the FISH, suitable for use in children with haemophilia treated with prophylaxis, is in development. There has been much debate over the definitions of activities and participation. The ICF (Fig. 1) defines activities as ‘the execution of a task or action by an individual’ while participation encompasses ‘involvement in a life situation’. As an example, running may be an activity a young boy with haemophilia can perform, whereas choosing to run with his friends in a soccer game would be an example of participation. Here, again the inclusion of disease-specific and generic measures is recommended. Several disease-specific measures are described below. Disease-specific QoL instruments with good measurement properties (i.e.