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Study of physical function in adolescents with haemophilia: The SO‐FIT study
Author(s) -
Khair K.,
Holland M.,
Bladen M.,
Griffioen A.,
McLaughlin P.,
Mackensen S.
Publication year - 2017
Publication title -
haemophilia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.213
H-Index - 92
eISSN - 1365-2516
pISSN - 1351-8216
DOI - 10.1111/hae.13323
Subject(s) - medicine , haemophilia , haemophilia a , pediatrics
Contemporary haemophilia care demands Patient‐Reported Outcomes. SO‐ FIT is a UK multi‐centre study, assessing self‐reported function, health‐related quality of life ( HRQ oL) and joint health in boys with severe haemophilia. Methods Subjective physical function (PedHAL, HEP‐Test‐Q) and HRQoL (Haemo‐QoL Short Form [SF]) were assessed alongside joint health using the objective Haemophilia Joint Health Score (HJHS v2.1). Demographic and clinical data were collected. Results Data from 127 boys mean age 12.38 ± 2.5 (range 8‐17) treated at 16 sites were analysed. One‐hundred‐and‐thirteen had haemophilia A, 25/9 past/current inhibitor, 124 were treated prophylactically (46.8% primary) and three on‐demand. In the preceding 6 months, boys reported median 0 joint bleeds (range 0‐8) with a median HJHS score of 1 (range 0‐30). Boys reported good physical functioning; HEP ‐Test‐Q (M = 80.32 ± 16.1) showed the highest impairments in the domain “endurance” (72.53 ± 19.1), in Ped HAL (M = 85.44 ± 18.9) highest impairments were in the domains “leisure activities & sports” (M = 82.43 ± 23.4) and “lying/sitting/kneeling/standing” (M = 83.22 ± 20.3). Boys reported generally good HRQ oL in Haemo‐QoL SF SF (M = 22.81 ± 15.0) with highest impairments in the domains “friends” (M = 28.81 ± 30.5) and “sports & school” (M = 26.14 ± 25.1). HJHS revealed low correlations with the Haemo‐QoL SF ( r = .251, P < .006), the PedHAL ( r = −.397, P < .0001) and the HEP‐Test‐Q ( r = −.323, P < .0001). A moderate correlation was seen between HEP ‐Test‐Q and Haemo‐QoL SF of r = −.575 ( P < .0001) and between Ped HAL and Haemo‐QoL SF r = −.561 ( P < .0001) implying that good perceived physical function is related to good HRQ oL. Conclusions The SO ‐ FIT study has demonstrated that children with severe haemophilia in the UK report good HRQ oL and have good joint health as reflected in low HJHS scores.