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Notes: The aim of this study was to investigate the validity of the Dutch Arthritis Impact Measurement Scales 2 (D-AIMS2). Hence, D-AIMS2 data of individuals with severe haemophilia were correlated with clinical and perceived health-related quality of life data. Patients with severe haemophilia, who visit the Van Creveldkliniek on a regular basis, were administered the D-AIMS2. In addition, health-related quality of life was measured by the Sickness Impact Profile (SIP). As clinical indices, range of movement (which was converted into Joint Alignment and Motion scores) and muscle strength were recorded during the routine visit. Extensive descriptive and correlational (linear) analyses between corresponding datasets were performed. Thirty-one individuals with severe haemophilia were included. Their scores on the D-AIMS2 demonstrated moderate to very high internal consistency for scales and components (Cronbach’s α=0.62–0.92). The physical health components of the D-AIMS2 and the SIP were significantly correlated (Pearson’s r=0.53; P 〈 0.05). The psychological health and social interaction components of the D-AIMS2 did not correlate significantly with the psychosocial component of the SIP. The physical health component of the D-AIMS2 correlated significantly with the clinical data for the lower extremities (r=0.52 and r =−0.45; P 〈 0.05). These data support the reliability and validity of the physical aspects of the D-AIMS2 in patients with severe haemophilia. The next step should be to extend the investigation of psychometric qualities of this health-related questionnaire in a larger population.

Notes: Summary.  The World Federation of Hemophilia scoring system (WFH-1) evaluates primarily body functions and structures, whereas assessment of the whole area of functional health status is considered nowadays to be a better measure. In addition, the WFH-1 lacks psychometric properties (reliability, validity and sensitivity to change). This study aimed to gain insight into the clinimetric assessment of functional health status in patients with haemophilia by way of a systematic literature search. A standardized literature search and selection was performed on the databases of CINAHL (1982–2001), Medline (1966–2001), and PubMed (January–July 2001). Clinimetric instruments applied in the selected articles were classified based on the international classification of functioning, disability, and health (ICF) and analysed for their psychometric properties. In 19 articles published between 1979 and 2001, 34 clinimetric instruments were used to assess functional health status. Instruments were classified as measuring the ICF components body structure and function (n = 17), activities (12) and participation (4). Reliability was measured in four articles on three different instruments, the validity (construct) of the instruments for patients with haemophilia was reported in six articles on five instruments, and sensitivity to change in three articles on three instruments. The populations under study varied in number (7–9 35), mean age (21.6–50.8 years), and in the distribution of haemophilia severity. Reports on the evaluation of functional health status in patients with haemophilia are increasing. Further research on the psychometric properties of the instruments is warranted in more groups of patients with haemophilia. Development of the new core set of clinimetric instruments, the WFH-2, might benefit from this strategy.

Notes: Articular contractures in haemophilia are impairments that can not be cured by means of physiotherapy because of the pathophysiology of the joint. Rehabilitation, however, tries to diminish the disabilities and prevent handicaps caused by the impairments. Physiotherapy aims at pain reduction by means of manual traction. Next to manual traction the intensive physiotherapy programme includes mobilization techniques, muscle strengthening exercises and stretching, joint stability training, postural and gait training, and functional training. In all 50 haemophilia patients have undergone this intensive 4-week clinical rehabilitation programme. Data of 20 of these severe haemophilia patients show that the mean range of motion at the start of the rehabilitation period, after 4 weeks and after 5 years do not differ. In spite of progressing arthropathy after 5 years the activities of daily living (ADL), walking range and pain are equal or better according to 13 of 15 patients.

Notes: Summary.  A multicentre study was performed to compare clotting factor use and outcome between on-demand and prophylactic treatment strategies for patients with severe haemophilia. Data on treatment and outcome of 49 Dutch patients with severe haemophilia, born 1970–80, primarily treated with prophylaxis, were compared with those of 106 French patients, who were primarily treated on demand. Dutch patients received intermediate dose prophylaxis, for a median duration of 12.7 years. Patients primarily treated with prophylaxis had fewer joint bleeds per year (median 2.8 vs. 11.5), a higher proportion of patients without joint bleeds (29% vs. 9%), lower clinical scores (median 2.0 vs. 8.0), and less arthropathy as measured by the Pettersson score (median 7 points vs. 16 points). Mean annual clotting factor use was equal at 1488 ± 783 IU kg−1 year−1 (mean ± standard deviation) for patients primarily treated with prophylaxis and 1612 ± 1442 IU kg−1 year−1 for patients primarily treated on demand. These findings suggest that, compared with a primarily on-demand treatment strategy, a primarily prophylactic treatment strategy leads to better outcome at equal treatment costs in young adults with severe haemophilia.

Notes: The aim of this pilot study was to examine the usefulness of the Dutch version of the Arthritis Impact Measurement Scales 2 (D-AIMS2)in assessing the health status of Dutch individuals with haemophilia. Sixty-eight individuals with mild, moderate, and severe haemophilia attending our clinic for their annual check-up participated. They first completed the Canadian Occupational Performance Measure (COPM). The D-AIMS2 was filled in afterwards at home. With the COPM, individuals rated their specific problematic activities of daily life (ADL), as well as the severity and importance of each problem. The D-AIMS2 is a comprehensive, self-administered questionnaire that evaluates functional health status. Fifty-seven individuals completed and returned the D-AIMS2. Reliability analysis demonstrated good internal consistency for the scales (Cronbach’s α=0.76–1.00), as well as for the components (α=0.80–0.88), except for the component ‘social interaction’ (α=0.44). Criterion validity of the D-AIMS2 was assessed by comparison with COPM outcomes; 80% of the problematic ADLs were included in the questionnaire, 20% were missing. Correlations between the D-AIMS2 components ‘physical health’ and ‘symptoms’ with predicted scores of those individuals by a highly experienced physiotherapist (r=0.63 and 0.53, respectively) substantiated its concurrent validity. Based on these results we concluded that the D-AIMS2, with minor adjustments, can be an appropriate tool for assessing the health status of Dutch haemophilia patients.

Notes: Summary.  People with haemophilia experience a progressive deterioration of their functional health status. Regular clinical assessment of functional health status provides insight into their process of disablement. As such, the development of a core-set of measurement tools is warranted. The aim of this study was to gather data to prepare a (preliminary) core set of clinically relevant and feasible instruments to assess the functional health status of adults with haemophilia, and to indicate their psychometric qualities. Therefore, clinimetric instruments frequently used in two haemophilia-resembling diseases (Rheumatoid Arthritis and Osteoarthritis) were reviewed from the literature. An extensive search in Medline yielded 13 relevant review articles, incorporating a total of 182 instruments, of which 40 were appropriate for haemophilia. Of these 40 instruments 3 measure body structures, 13 body functions, 19 activities (of which 5 are performance based and 14 self-report based), and 3 measure participation. This classification is based on the International Classification of Functioning, Disability and Health. Detailed information regarding the psychometrics (reliability, validity and responsiveness) of four instruments is described fully in the literature, whereas the psychometrics of the majority of the other instruments are only partly described. The results of this literature study may contribute to the formation of a (preliminary) core set of clinimetric instruments to assess the functional health status of adults with haemophilia. Decisions on the final core set should be held within the Musculoskeletal Committee of the World Federation of Haemophilia.

Notes: The demand for patient information in modern medical care is increasing and sound information for patients is becoming a necessity. For haemophilia patients, information about their disease and its complications is already widely available. In order to increase the organization of this information, a ‘Patient Information Dossier’ (PID) and communication check lists were developed at the Dutch National Hemophilia Center, the Van Creveldkliniek, in cooperation with the Department of Patient Education of the University Medical Center Utrecht. The PID has an unique double function: (1) it contains patient tailored information about the practical facts of hospital care; and (2) it provides a communication checklist used by various members of the comprehensive care team, in order to supply patients with more uniform information. In order to gain a better insight of the gaps in information supply, according to patients and healthcare workers, the Department of Patient Education formulated a questionnaire. The PID itself was written by a study group consisting of members of the comprehensive care team. The entire process was developed, edited and coordinated by an advisor of the Department of Patient Education. The above-mentioned study group developed a specific PID on haemophilia care. Its 10 chapters provide information and guidelines, and advise patients where to find more information about this subject. Each chapter includes a checklist for patients, enabling them to prepare subjects for discussion during clinical visits. The team also developed a communication checklist to be used by various team members during a patient’s visit to the clinic, as well as specific checklists covering the possible problem subjects of the PID. The PID is the lifelong property of the patient, and can be used during each visit to the clinic. The PID was implemented in February 2000, and within 4 months, was distributed among 200 patients visiting the Van Creveldkliniek. Evaluation by use of a questionnaire showed that most patients found the information in the PID sufficient and in accordance with that which they had received previously.

Notes: Summary.  Haemophilia is characterized by intra-articular and intramuscular bleeding episodes. Although much work has been carried out into post-bleeding mechanisms in both synovial joints and muscles, the picture is not yet clear. A firm post-bleeding rehabilitation protocol is of utmost importance for people with haemophilia. The use of the International Classification of Functioning Disability and Health reveals that information worldwide is on body level, whereas the goals of patients with haemophilia implicitly aim at optimal functional recovery. This interferes much more with activities and participation in society. The Functional Independence Score for Haemophilia and the Post-bleeding Protocol Based on Functional Milestones are examples of post-bleeding approaches that deal more with function. Two cases will emphasize the value of this approach.