OBJECTIVE: To determine whether aerobic capacity is normal in boys with different types of hemophilia compared with healthy peers and whether the level of aerobic capacity correlates with the amount of physical activity, joint health status, muscle strength, and anthropometrics.STUDY DESIGN: 47 patients (mean [SD] age, 12.9 [3.2] years; age range, 8.2-17.4 years) from the "Van Creveldkliniek" of the University Medical Center Utrecht, participated. Anthropometry, muscle strength, joint impairment, functional ability, and aerobic capacity were measured. The amount of energy expenditure during daily living was assessed.RESULTS: All boys were able to perform at maximal or near-maximal level on exercise tests, and none of them reported bleeds or other adverse events. Relative peak oxygen, peak heart rate, and peak working capacity were significantly lower compared with healthy control subjects. 30% had Z-scores >2 for weight. Total muscle strength was normal, and almost no joint impairment and no decrease in functional ability were found.CONCLUSION: The aerobic capacity of children with hemophilia is still lower than the normal population, whereas their overall muscle strength is comparable with healthy peers. The functional ability does not differ from healthy peers, and joint health status showed very minor impairments. A substantial proportion of Dutch children with hemophilia was overweight, without showing a reduction in the amount of self-reported physical activities.
DOCUMENT
OBJECTIVE: Repeated hemarthrosis in hemophilia causes arthropathy with pain and dysfunction. The Hemophilia Joint Health Score (HJHS) was developed to be more sensitive for detecting arthropathy than the World Federation of Hemophilia (WFH) physical examination scale, especially for children and those using factor prophylaxis. The HJHS has been shown to be highly reliable. We compared its validity and sensitivity to the WFH scale.METHODS: We studied 226 boys with mild, moderate, and severe hemophilia at 5 centers. The HJHS was scored by trained physiotherapists. Study physicians at each site blindly determined individual and total joint scores using a series of visual analog scales.RESULTS: The mean age was 10.8 years. Sixty-eight percent were severe (93% of whom were treated with prophylaxis), 15% were moderate (24% treated with prophylaxis), and 17% were mild (3% treated with prophylaxis). The HJHS correlated moderately with the physician total joint score (rs=0.42, P<0.0001) and with overall arthropathy impact (rs=0.42, P<0.0001). The HJHS was 97% more efficient than the WFH at differentiating severe from mild and moderate hemophilia. The HJHS was 74% more efficient than the WFH at differentiating subjects treated with prophylaxis from those treated on demand. We identified items on the HJHS that may be redundant or rarely endorsed and could be removed from future versions.CONCLUSION: Both the HJHS and WFH showed evidence of strong construct validity. The HJHS is somewhat more sensitive for mild arthropathy; its use should be considered for studies of children receiving prophylaxis.
DOCUMENT
Background: Joint bleeds are the hallmark of hemophilia, leading to a painful arthritic condition called as hemophilic arthropathy (HA). Exercise programs are frequently used to improve the physical functioning in persons with HA. As hemophilia is a rare disease, there are not many physiotherapists who are experienced in the field of hemophilia, and regular physiotherapy sessions with an experienced physiotherapist in the field of hemophilia are not feasible for persons with HA. Blended care is an innovative intervention that can support persons with HA at home to perform the advised physical activities and exercises and provide self-management information. Objective: The aim of this study was to develop a blended physiotherapy intervention for persons with HA. Methods: The blended physiotherapy intervention, namely, e-Exercise HA was developed by cocreation with physiotherapists, persons with HA, software developers, and researchers. The content of e-Exercise HA was compiled using the first 3 steps of the Center for eHealth Research roadmap model (ie, contextual inquiry, value specification, and design), including people with experience in the development of previous blended physiotherapy interventions, a literature search, and focus groups. Results: A 12-week blended intervention was developed, integrating face-to-face physiotherapy sessions with a web-based app. The intervention consists of information modules for persons with HA and information modules for physiotherapists, a graded activity program using a self-chosen activity, and personalized video-supported exercises. The information modules consist of text blocks, videos, and reflective questions. The patients can receive pop-ups as reminders and give feedback on the performance of the prescribed activities. Conclusions: In this study, we developed a blended physiotherapy intervention for persons with HA, which consists of information modules, a graded activity program, and personalized video-supported exercises.
LINK
Introduction: Throughout life, a patient with severe haemophilia is confronted with many treatment-related challenges. Insight into self-management and non-adherence could improve the quality of care for these patients. The aim of this study was to provide an overview of the current evidence on self-management and adherence to prophylaxis in haemophilia. Method: Based on series of studies and published literature, aspects of treatment were explored: learning and performing self-infusion, achieving self-management skills in adolescence, adherence issues and coping with haemophilia. Evidence-based and age-group-specific recommendations for haemophilia professionals were formulated. Results: Nearly, all severe haemophilia patients and parents were able to perform self-infusion and the quality level of infusion skills was acceptable. Learning self-infusion was generally initiated before the onset of puberty and full self-management was obtained 10 years later. Adherence was defined using a Delphi consensus procedure and was determined by skipping, dosing and timing of infusions. Adherence levels varied according to age, with highest levels in children (1–12 years) and the lowest among 25–40 years. Adherence to prophylaxis was acceptable (43%), yet 57% of the population struggled with prophylaxis. Qualitative research showed that the position of prophylaxis in life is the main driver of adherence. This position is influenced by acceptance and self-management skills. Regarding coping with haemophilia, the majority of patients used a problem-focused approach. Conclusion: Self-management and adherence to prophylaxis vary during the life span. Acceptance of the disease and self-management skills were important aspects that may require tailored professional support.
DOCUMENT
Background Regular physiotherapy with a physiotherapist experienced in the field is not feasible for many patients with haemophilia. We, therefore, developed a blended physiotherapy intervention for persons with haemophilic arthropathy (HA) (e-Exercise HA), integrating face-to-face physiotherapy with a smartphone application. Aim The aim of the study was to determine proof of concept of e- Exercise HA and to evaluate feasibility. Methods Proof of concept was evaluated by a single-case multiple baseline design. Physical activity (PA) was measured with an accelerometer during a baseline, intervention and post-intervention phase and analysed using visual inspection and a single case randomisation test. Changes in limitations in activities (Haemophilia Activities List [HAL]) and a General Perceived Effect (GPE) were evaluated between baseline (T0), post-intervention (T1) and 3 months post-intervention (T2) using Wilcoxson signed rank test. Feasibility was evaluated by the number of adverse events, attended sessions and open-ended questions. Results Nine patients with HA (90% severe, median age 57.5 (quartiles 50.5–63.3) and median HJHS 32 (quartiles 22–36)) were included. PA increased in two patients. HAL increased mean 15 (SD 9) points (p = .001) at T1, and decrease to mean +8 points (SD 7) (p = .012) at T2 compared to T0. At T1 and T2 8/9 participants scored a GPE > 3. Median 5 (range 4–7) face-to-face sessions were attended and a median 8 out of 12 information modules were viewed. No intervention-related bleeds were reported. Conclusion A blended physiotherapy intervention is feasible for persons with HA and the first indication of the effectiveness of the intervention in decreasing limitations in activities was observed.
MULTIFILE
The six-minute walk test (6MWT) is a self-paced, submaximal exercise test used to assess functional exercise capacity in patients with chronic diseases (Chang 2006, Solway et al 2001). It has been used widely in adults, and is being utilised increasingly in paediatric populations; it has been used as an estimate of physical fitness in, for example, children with severe cardiopulmonary disease, cystic fibrosis, and juvenile idiopathic arthritis (Hassan et al 2010).
DOCUMENT
Background: Accurate measurement of health literacy is essential to improve accessibility and effectiveness of health care and prevention. One measure frequently applied in international research is the Short Assessment of Health Literacy (SAHL). While the Dutch SAHL (SAHL-D) has proven to be valid and reliable, its administration is time consuming and burdensome for participants. Our aim was to further validate, strengthen and shorten the SAHL-D using Rasch analysis. Methods: Available cross-sectional SAHL-D data was used from adult samples (N = 1231) to assess unidimensionality, local independence, item fit, person fit, item hierarchy, scale targeting, precision (person reliability and person separation), and presence of differential item functioning (DIF) depending on age, gender, education and study sample. Results: Thirteen items for a short form were selected based on item fit and DIF, and scale properties were compared between the two forms. The long form had several items with DIF for age, gender, educational level and study sample. Both forms showed lower measurement precision at higher health literacy levels. Conclusions: The findings support the validity and reliability of the SAHL-D for the long form and the short form, which can be used for a rapid assessment of health literacy in research and clinical practice.
DOCUMENT
Background: Osteoarthritis is one of the most common chronic joint diseases, mostly affecting the knee or hip through pain, joint stiffness and decreased physical functioning in daily life. Regular physical activity (PA) can help preserve and improve physical functioning and reduce pain in patients with osteoarthritis. Interventions aiming to improve movement behaviour can be optimized by tailoring them to a patients' starting point; their current movement behaviour. Movement behaviour needs to be assessed in its full complexity, and therefore a multidimensional description is needed. Objectives: The aim of this study was to identify subgroups based on movement behaviour patterns in patients with hip and/or knee osteoarthritis who are eligible for a PA intervention. Second, differences between subgroups regarding Body Mass Index, sex, age, physical functioning, comorbidities, fatigue and pain were determined between subgroups. Methods: Baseline data of the clinical trial 'e-Exercise Osteoarthritis', collected in Dutch primary care physical therapy practices were analysed. Movement behaviour was assessed with ActiGraph GT3X and GT3X+ accelerometers. Groups with similar patterns were identified using a hierarchical cluster analysis, including six clustering variables indicating total time in and distribution of PA and sedentary behaviours. Differences in clinical characteristics between groups were assessed via Kruskall Wallis and Chi2 tests. Results: Accelerometer data, including all daily activities during 3 to 5 subsequent days, of 182 patients (average age 63 years) with hip and/or knee osteoarthritis were analysed. Four patterns were identified: inactive & sedentary, prolonged sedentary, light active and active. Physical functioning was less impaired in the group with the active pattern compared to the inactive & sedentary pattern. The group with the prolonged sedentary pattern experienced lower levels of pain and fatigue and higher levels of physical functioning compared to the light active and compared to the inactive & sedentary. Conclusions: Four subgroups with substantially different movement behaviour patterns and clinical characteristics can be identified in patients with osteoarthritis of the hip and/or knee. Knowledge about these subgroups can be used to personalize future movement behaviour interventions for this population.
LINK
Assessment of impairment and function is essential in order to monitor joint status and evaluate therapeutic interventions in patients with haemophilia. The improvements in the treatment of haemophilia have required the development of more sensitive tools to detect the more minor dysfunctions that may now be apparent. This paper outlines some of the recent developments in this field. The Haemophilia Joint Health Score (HJHS) provides a systematic and robust measure of joint impairment. The MRI Scoring System has been designed to provide a comprehensive scoring system combining both progressive and additive scales. The Functional Independence Score for Haemophilia (FISH) has been developed to assess performance of functional activities and can be used in conjunction with the Haemophilia Activities List (HAL) which provides a self report measure of function. It is recommended that both measures are evaluated as these tools measure different constructs. Further refinement and testing of the psychometric properties of all of these tools is in progress. More widespread use of these tools will enable the sharing of data across the world so promoting best practice and ultimately enhancing patient care.
DOCUMENT
In the Netherlands comparable levels of sports-participation between persons with haemophilia and healthy controls have been reported. This raises the question if children with haemophilia under the currently available prophylaxis do reach comparable levels of physical fitness and health-related quality of life (HRQoL) as their healthy peers. The aim of this study was to investigate the level of physical fitness, functional ability and quality of life and to determine the feasibility to safely test the exercise capacity of boys with severe haemophilia A. Thirteen subjects participated in this study. Physical fitness was determined using the measurement of maximal oxygen uptake (VO2peak) attained during a graded maximal exercise test to volitional exhaustion. Joint health, physical activity levels and health-related quality of life (Haemo-Qol) were also measured. Mean VO2peak was 1.86+/-0.77 L min-1 (Z-score: -0.39+/-1.61) which was not significantly different from reference values. Relative VO2peak was 47.42+/-8.29 mL min-1 kg-1 (Z-score: -0.52+/-1.43), which did not differ significantly from reference values either. One boy suffered a joint bleeding one day after the test. Haemo-Qol scores in parents and children ranged from 3.2% to 36.7% (100% reflects poor outcome). Relationship between the child or parent reports of Haemo-QoL and both absolute and relative VO2peak ranged from R=0.00 and R=0.4. Exercise testing in children with severe haemophilia A was a safe procedure. Patients with severe haemophilia A with good joint health and no limitations of activities have comparable physical fitness and physical active lifestyle with healthy peers and good HRQoL.
DOCUMENT
