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Joint health and functional ability in children with haemophilia who receive intensive replacement therapy
Author(s) -
GROEN W.,
VAN DER NET J.,
BOS K.,
ABAD A.,
BERGSTROM B. M.,
BLANCHETTE V. S.,
FELDMAN B. M.,
FUNK S.,
HELDERS P.,
HILLIARD P.,
MANCOJOHNSON M.,
PETRINI P.,
ZOURIKIAN N.,
FISCHER K.
Publication year - 2011
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/j.1365-2516.2011.02606.x
Subject(s) - medicine , haemophilia , interquartile range , ankle , physical therapy , arthropathy , haemophilia a , range of motion , enzyme replacement therapy , pediatrics , surgery , disease , osteoarthritis , pathology , alternative medicine
Summary. Joint physical examination is an important outcome in haemophilia; however its relationship with functional ability is not well established in children with intensive replacement therapy. Boys aged 4–16 years were recruited from two European and three North American treatment centres. Joint physical structure and function was measured with the Haemophilia Joint Health Score (HJHS) while functional ability was measured with the revised Childhood Health Assessment Questionnaire (CHAQ 38 ). Two haemophilia‐specific domains were created by selecting items of the CHAQ 38 that cover haemophilia‐specific problems. Associations between CHAQ, HJHS, cumulative number of haemarthroses and age were assessed. A total of 226 subjects – mean 10.8 years old (SD 3.8) – participated; the majority (68%) had severe haemophilia. Most severe patients (91%) were on prophylactic treatment. Lifetime number of haemarthroses [median = 5; interquartile range (IQR) = 1–12] and total HJHS (median = 5; IQR = 1–12) correlated strongly (ρ = 0.51). Total HJHS did not correlate with age and only weakly (ρ = −0.19) with functional ability scores (median = 0; IQR = −0.06–0). Overall, haemarthroses were reported most frequently in the ankles. Detailed analysis of ankle joint health scores revealed moderate associations (ρ = 0.3–0.5) of strength, gait and atrophy with lower extremity tasks (e.g. stair climbing). In this population, HJHS summating six joints did not perform as well as individual joint scores, however, certain elements of ankle impairment, specifically muscle strength, atrophy and gait associated significantly with functional loss in lower extremity activities. Mild abnormalities in ankle assessment by HJHS may lead to functional loss. Therefore, ankle joints may warrant special attention in the follow up of these children.