Study of physical function in adolescents with haemophilia: The SO‐FIT study

• Published in: Haemophilia : the official journal of the World Federation of Hemophilia 2017-11, Vol.23 (6), p.918-925
• Main Authors: Khair, K., Holland, M., Bladen, M., Griffioen, A., McLaughlin, P., Mackensen, S., Wilkinson, Melanie, Basingstoke, Anna Wells, Charles, Lisa, Bingham, Gordon, Harvey, Andrew, Harbridge, Hannah, Emmitt, Shaun, Stephensen, David, Ward, June, Hook, Susan, Reid, Jenna, Preston, Suzanne, Flannery, Thuvia, South, Anne, Hubert, Nicola, Phillott, Anica, Bhandari, Trupti, Sexton, Julia, Pollard, Debra, Forsyth, Kate, Patel, Vish, Jones, April, Hopper, David, Oyesiku, Lara, Taylor, Stephanie, Vidler, Vicky, Sutcliffe, Candice, Bell, Tricia, Webster, Caroline, Garland, Dairin
• Format: Article
• Language: English
• Subjects: Adolescent; Adolescents; Child; Children; haemophilia; haemophilia joint health score; haemo‐QoL; Health Status; Health Surveys - methods; Health Surveys - statistics & numerical data; health‐related quality of life; Hemophilia; Hemophilia A - drug therapy; Hemophilia A - physiopathology; Hemorrhage - physiopathology; Hemorrhage - prevention & control; HEP‐Test‐Q; Humans; Joints - physiopathology; Male; PedHAL; physical function; Quality of Life; Self Report; subjective assessment; United Kingdom
• ISSN: 1351-8216; 1365-2516
• DOI: 10.1111/hae.13323

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