Joint health and functional ability in children with haemophilia who receive intensive replacement therapy |
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| Authors: | W. GROEN J. VAN DER NET K. BOS A. ABAD B. ‐M. BERGSTROM V. S. BLANCHETTE B. M. FELDMAN S. FUNK P. HELDERS P. HILLIARD M. MANCO‐JOHNSON P. PETRINI N. ZOURIKIAN K. FISCHER |
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| Affiliation: | 1. Child Development and Exercise Center, University Medical Center Utrecht, University Children’s Hospital, Utrecht, The Netherlands;2. Department of Child Health Evaluative Sciences, The Hospital for Sick Children, Toronto, ON, Canada;3. Division of Physical Therapy, Department of Paediatrics, Karolinska University Hospital, Stockholm, Sweden;4. Division of Hematology/Oncology, Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, ON, Canada;5. Division of Rheumatology, Departments of Pediatrics, Health Policy Management & Evaluation, and the Dalla Lana School of Public Health, University of Toronto, The Hospital for Sick Children, Toronto, Canada;6. Department of Pediatrics, University of Colorado Denver Hemophilia & Thrombosis Center, University of Colorado Denver Aurora, CO, USA;7. Department of Rehabilitation Services, The Hospital for Sick Children, Toronto, ON, Canada;8. Department of Pediatrics, University of Colorado Denver Hemophilia and Thrombosis Center, University of Colorado Denver and The Children’s Hospital, Aurora, CO, USA;9. Division of Blood Coagulation Disorders, Department of Pediatrics, Karolinska University Hospital, Stockholm, Sweden;10. Pediatric and Adult Comprehensive Hemostasis and Inhibitor Center, Sainte‐Justine University Centre Hospital, Montréal, QC, Canada;11. Van Creveldkliniek, Department of Haematology, and Julius Center for Health Sciences and Primary Care, University Medical Center Utrecht, Utrecht, The Netherlands |
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| Abstract: | 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 (CHAQ38). Two haemophilia‐specific domains were created by selecting items of the CHAQ38 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. |
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| Keywords: | activities children haemophilia limitations outcome |
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