Study of physical function in adolescents with haemophilia: The SO‐FIT study. Issue 6 (14th August 2017)

• Record Type: Journal Article
• Authors: Khair, K.; Holland, M.; Bladen, M.; Griffioen, A.; McLaughlin, P.; von Mackensen, S.
• Other Names: Wilkinson Melanie investigator.; Basingstoke Anna Wells investigator.; Charles Lisa investigator.; Bingham Gordon investigator.; Harvey Andrew investigator.; Harbridge Hannah investigator.; Emmitt Shaun investigator.; Stephensen David investigator.; Ward June investigator.; Hook Susan investigator.; Reid Jenna investigator.; Preston Suzanne investigator.; Flannery Thuvia investigator.; South Anne investigator.; Hubert Nicola investigator.; Phillott Anica investigator.; Bhandari Trupti investigator.; Sexton Julia investigator.; Pollard Debra investigator.; Forsyth Kate investigator.; Patel Vish investigator.; Jones April investigator.; Hopper David investigator.; Oyesiku Lara investigator.; Taylor Stephanie investigator.; Vidler Vicky investigator.; Sutcliffe Candice investigator.; Bell Tricia investigator.; Webster Caroline investigator.; Garland Dairin investigator.
• Is Part Of: Haemophilia. Volume 23:Issue 6(2017)
• Page Start: 918
• Page End: 925
• Publication Date: 2017-08-14
• Subjects: adolescents -- haemophilia -- haemophilia joint health score -- haemo‐QoL -- health‐related quality of life -- HEP‐Test‐Q -- PedHAL -- physical function -- subjective assessment; Hemophilia -- Periodicals; 616.1572005
• DOI: 10.1111/hae.13323
• Languages: English
• ISSNs: 1351-8216

Abstract

Abstract : Introduction: Contemporary haemophilia care demands Patient‐Reported Outcomes. SO‐FIT is a UK multi‐centre study, assessing self‐reported function, health‐related quality of life (HRQoL) 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 PedHAL (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 HRQoL 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 PedHAL and Haemo‐QoL SF r = −.561 ( P < .0001) implying that good perceived physical function is related to good HRQoL. Conclusions: The SO‐FIT study has demonstrated that children with severe haemophilia in the UK report good HRQoL and have good joint health as reflected in low HJHS scores.