How fit are children and adolescents with haemophilia in Germany? Results of a prospective study assessing the sport‐specific motor performance by means of modern test procedures of sports science

Abstract

There are a lot of publications on the physical fitness of patients with haemophilia (PWH), however, most studies only reflect individual sport-specific motor capacities or focus on a single fitness ability. They involve small patient populations. In this respect principal objective of this study was to compare the physical fitness in all respects and the body composition of young PWH to healthy peers based on the most valid data we could get. Twenty-one German haemophilia treatment centres were visited from 2002 to 2009. PWH between 8 and 25 years were included. They performed a five-stage fitness test covering the sport-specific motor capacities for coordination, measured by one leg stand, strength, aerobic fitness and mobility as well as body composition. The patients' results were compared with age- and gender-specific reference values of healthy subjects. Two hundred and eighty-five PWH (mean age 13.2 ± 4.5 years, 164 PWH with severe disease) were included prospectively in the study. PWH are significantly below the reference values of healthy subjects in the one-leg stand test, the mobility of the lower extremity, the strength ratio of chest and back muscles and the endurance test. In body composition, the back strength and the mobility of the upper extremity PWH are significantly above the reference values. There are no significant differences in abdominal strength. In conclusion we found specific differences in different fitness abilities between PWH and healthy subjects. Knowing this, we are able to work out exercise programmes to compensate the diminished fitness abilities for our PWH.

Evaluation of early musculoskeletal disease in patients with haemophilia

“…Decline in joint function negatively impacts HRQoL through decreased activity and participation [45,46], and deterioration in joint scores confirm the importance of lifelong prophylactic clotting factor replacement therapy [47]. Improvements in joint strength and ROM in patients with haemophilia, such as those observed through exercise therapy [45,48], have been linked with improvements in daily functioning and HRQoL [49,50].…”

“…Improvements in joint strength and ROM in patients with haemophilia, such as those observed through exercise therapy [45,48], have been linked with improvements in daily functioning and HRQoL [49,50]. Both generic (SF-36) and disease-specific HRQoL tools can be used to assess patients with haemophilia, dependent on the QoL domains being evaluated [49].…”

The effect of resistance exercise on strength and safety outcome for people with haemophilia: A systematic review

“…Several studies verified a deficit in the basic motor functions in people with haemophilia (PwH), this is why a multimodal training is necessary. Regarding strength performance, studies have shown reduced strength in certain muscle groups and patient groups: adults with haemophilia seem to have strength deficits in the upper and lower extremities as well as in the back muscles.…”

Validation of the Haemophilia & Exercise Project‐Test‐Questionnaire (HEP‐Test‐Q)—An instrument for the assessment of subjective physical functioning in children with haemophilia

“…In clinical practice, physical functioning is mainly assessed through ClinROs such as the HJHS and/or PROs such as the PedHAL or the HEP‐Test‐Q . In research or gait and motion laboratories, physical functioning is evaluated by clinimetric measures, Performance Outcomes (PerfOs) such as 1 leg stand, 6‐minute walk test or the Functional Independence Score for Hemophilia (FISH) or apparatus testing such as pedabarography, EMG or motion analysis . The self‐reported PedHAL focuses on activities of daily living such as use of transport, self‐care and household tasks, whereas the HEP‐Test‐Q assesses aspects of physical functioning based on motor ability (mobility, strength, coordination and endurance).…”

“…12 In research or gait and motion laboratories, physical functioning is evaluated by clinimetric measures, 29 Performance Outcomes (PerfOs) such as 1 leg stand, 6-minute walk test or the Functional Independence Score for Hemophilia (FISH) 30 or apparatus testing such as pedabarography, EMG or motion analysis. 31 The self-reported PedHAL focuses on activities of daily living such as use of transport, self-care and household tasks, whereas the HEP-Test-Q assesses aspects of physical functioning based on motor ability (mobility, strength, coordination and endurance). PedHAL is an internationally recognized instrument with use in clinical trials, yet there is limited published evidence of its use.…”