This National Institute for Health Research supported study was conducted by nurses and physiotherapists in 16 haemophilia centres in the UK, funded by a medical education grant from Pfizer UK.
In all, data were collected from 127 boys with mean age 12.26±2.6 (range 8-17) from 16 sites were enrolled. 110 had severe haemophilia A, 13 haemophilia B; 26/9 with past/current inhibitor. 120 boys were treated with prophylaxis (42.5% primary, 54% secondary) and 3 were treated on demand. The median HJHS score was 1 (range 0-37). In the preceding 6 months boys reported median 0 joint bleeds (range 0-8).
Boys reported good subjective physical functioning in:
- the Hep-Test-Q (M=79.88±16.2) with highest impairments in the domain ‘endurance’ (72.05±19.2)
- the PedHAL (M=84.98 ±19.0) with highest impairments in the domains ‘leisure activities & sports’ (M=81.85±23.6) and ‘sitting/kneeling/standing’ (M=82.75±20.5).
Boys reported generally good health-related quality of life in:
- the Haemo-QoL short version (M=23.09±15.1) with highest impairments in the domains ‘friends’ (M=27.22±29.9), ‘sport & school’ (M=26.44±25.4) and ‘treatment’ (M=26.32±21.43).
There was high correlation between the two subjective measures of physical functioning (HEP-Test-Q, PedHAL) of r=.583 (p<.0001). A high correlation was seen between subjective physical functioning (Hep-Test-Q) and HRQoL (Haemo-QoL) of r=-.531 (p<0.0001) and moderate correlation between PedHAL and Haemo-QoL r=-.454 (p<0.0001) implying that good physical function is related to low impairment in HRQoL.
The SO-FIT study has demonstrated that children with severe haemophilia in the UK generally report good health-related quality of life and subjective physical functioning. This is also reflected in objective assessment of joint health.