Abstract

Background. Sport activities are considered beneficial for patients with haemophilia in terms of physical aspects, such as possible protection of joints and prevention of bleeds and deformity, and non-physical aspects, such as quality of life, socialisation and self-esteem. Patients’ report of their own experience of well-being and functioning becomes more and more important; therefore subjective evaluation should be combined with objective measurements. In the frame of the Haemophilia & Exercise Project (HEP) health-related quality of life (HRQoL) and subjective physical performance were evaluated together with objective measures such as orthopaedic joint score and EMG in haemophilia patients Germany. Aims. Assessment of HRQoL and subjective physical performance in adult patients with haemophilia attending the HEP sport camp. Methods. HEP is a sport camp, where adult haemophilia patients are trained twice a year. Participants were tested both objectively and subjectively concerning their physical performance, evaluated with the newly developed performance-specific HEP-Test-Q consisting of 25 items pertaining to 4 dimensions (mobility, strength & coordination, endurance, body perception), as well as regarding their HRQoL assessed with the generic SF-36 and the haemophilia-specific Haem-A-QoL questionnaire consisting of 46 items pertaining to 10 dimensions. Results. In total 33 haemophilia patients were enrolled in the HEP with a median age of 45 years (19-65). Almost all patients had haemophilia A (90.9%) and were severely affected by haemophilia (87.9%), 9.1% had inhibitors, 65.6% had chronic hepatitis C and 12.1% had HIV infection. Patients reported in average 6 bleeds in the previous 12 months (0-24) and 42.4% had target joints; 45.5% of all patients were on prophylaxis. HRQoL of these patients was significantly impaired in physical dimensions of the SF-36, as well as for emotional role functioning compared to the general population. In the haemophilia-specific Haem-A-QoL impairments were mainly found in the dimensions sport & leisure, future and physical health, 57.6% of patients reported that they couldn’t do as much sports as others, 51.5% suffered often/always from pain in joints. In the performance-specific HEP-Test-Q patients showed good values for mobility, but high impairments in coordination and endurance; 63.7% reported often/always problems in walking down stairs, 63.6% could never/seldom do exhausting activities. With the HEP-Test-Q 52% of the variance of the physical component of SF-36 could be explained, 34% of the mental component and 64% of the total score of Haem-A-QoL. Conclusions. Since most impairments in HRQoL were found in physical domains, especially in the dimension sport & leisure the additional assessment of subjective physical performance can be helpful in understanding specific problems of haemophilia patients, which can be measured by the newly developed HEP-Test-Q. It makes sense to combine objective assessments of physical performance with such subjective instruments in order to reveal aspects, which can not be measured yet objectively such as body perception. Since up to 64% of the variance of HRQoL could be explained by subjective physical performance it is important that haemophilia patients attend a supervised sport training which might improve physical status and body perception and consequently has a positive impact on quality of life.
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