Ezio Zanon1, Annarita Tagliaferri2, Samantha Pasca1, Cosimo P Ettorre3, Lucia D Notarangelo4, Chiara Biasioli5, Anna B Aru6, Marta Milan1, Silvia Linari7, Angiola Rocino8, Fabio Gagliano9, Giovanni Di Minno10, Gabriella Gamba11, Rita C Santoro12, Piercarla Schinco13, Marco Marietta14, Axel Seuser15, Sylvia von Mackensen16. 1. Haemophilia Centre University Hospital of Padua, Padua, Italy. 2. Regional Reference Centre for Inherited Bleeding Disorders, University Hospital of Parma, Parma, Italy. 3. Haemophilia and Thrombosis Center, "Giovanni XXIII" Hospital, Bari, Italy. 4. Oncology-Haematology and Bone Marrow Transplantation Unit, Children's Hospital, "Spedali Civili", Brescia, Italy. 5. Haemophilia Center, "Bufalini" Hospital, Cesena, Italy. 6. Pediatric Onco-Haematology Center, "Microcitemico" Hospital Cagliari, Italy. 7. Centre for Bleeding Disorders, "Careggi" University Hospital, Florence, Italy. 8. Haemophilia and Thrombosis Centre, "San Giovanni Bosco" Hospital, Naples, Italy. 9. Haemophilia Center, "G. Di Cristina" Children's Hospital, Palermo, Italy. 10. Regional Reference Centre for Coagulation Disorders, "Federico II" University Hospital, Naples, Italy. 11. Haemophilia and Congenital Coagulation disorders Center, "San Matteo" Hospital, Pavia, Italy. 12. Haemostasis and Thrombosis Unit, Haemophilia Center, "Pugliese-Ciaccio" Hospital, Catanzaro, Italy. 13. Haemostasis and Thrombosis Unit, "Molinette" Hospital Turin, Italy. 14. Department of Oncology, Hematology and Respiratory System Diseases, University Hospital, Modena, Italy. 15. Centre for Prevention, Rehabilitation and Orthopaedics, Bonn, Germany. 16. Institute of Medical Psychology, University Medical Centre, Hamburg, Germany.
Abstract
BACKGROUND: Physical activity in people with haemophilia (PWH) reduces the development of severe arthropathy, but it must be performed after regular, proper prophylaxis. Strict adherence to treatment is crucial to achieving effectiveness and established outcomes. The primary aim of this study was to collect prospective data on adherence to prophylaxis for over 36 months. A secondary aim was to verify whether adherence correlates with physical activity. MATERIALS AND METHODS: Italian patients with severe haemophilia A treated on prophylaxis with octocog alfa were included in the study. Physical findings were assessed by the Haemophilia and Exercise Project (HEP)-Test-Q and the Early Prophylaxis Immunologic Challenge (EPIC)-Norfolk Physical Activity Questionnaire; orthopaedic status was assessed by the Hemophilia Joint Health Score (HJHS). Adherence was measured as percentage of empty vials returned with respect to the prescribed amount. RESULTS: Forty-two PWH were enrolled: 31% children, 21.4% adolescents, and 47.6% adults. Type, frequency and impact of physical activities differed among the three groups. The HEP-Test-Q showed the highest impairments in the domains "endurance" and "strength/co-ordination". Eight percent of patients were classified as adherent to prophylaxis. Among them, 50% had at least one bleeding episode in the year before enrolment; this percentage dropped during the three years of the study. While remaining stable in the "non-adherent" group, the HJHS score decreased in the "adherent" patients. The mean number of school/work days lost was lower in adherent patients (from 3.4±6.8 to 0.2±0.9) than in non-adherent ones. DISCUSSION: PWH with better orthopaedic scores reported better physical performance. Adherence to long-term prophylaxis proved to be high and correlated with a reduction in bleeds, target joints, school/work days lost, and with a performance improvement in endurance sports activities over time.
BACKGROUND: Physical activity in people with haemophilia (PWH) reduces the development of severe arthropathy, but it must be performed after regular, proper prophylaxis. Strict adherence to treatment is crucial to achieving effectiveness and established outcomes. The primary aim of this study was to collect prospective data on adherence to prophylaxis for over 36 months. A secondary aim was to verify whether adherence correlates with physical activity. MATERIALS AND METHODS: Italian patients with severe haemophilia A treated on prophylaxis with octocog alfa were included in the study. Physical findings were assessed by the Haemophilia and Exercise Project (HEP)-Test-Q and the Early Prophylaxis Immunologic Challenge (EPIC)-Norfolk Physical Activity Questionnaire; orthopaedic status was assessed by the Hemophilia Joint Health Score (HJHS). Adherence was measured as percentage of empty vials returned with respect to the prescribed amount. RESULTS: Forty-two PWH were enrolled: 31% children, 21.4% adolescents, and 47.6% adults. Type, frequency and impact of physical activities differed among the three groups. The HEP-Test-Q showed the highest impairments in the domains "endurance" and "strength/co-ordination". Eight percent of patients were classified as adherent to prophylaxis. Among them, 50% had at least one bleeding episode in the year before enrolment; this percentage dropped during the three years of the study. While remaining stable in the "non-adherent" group, the HJHS score decreased in the "adherent" patients. The mean number of school/work days lost was lower in adherent patients (from 3.4±6.8 to 0.2±0.9) than in non-adherent ones. DISCUSSION: PWH with better orthopaedic scores reported better physical performance. Adherence to long-term prophylaxis proved to be high and correlated with a reduction in bleeds, target joints, school/work days lost, and with a performance improvement in endurance sports activities over time.
Authors: Karin Van Dijk; Johanna G Van Der Bom; Klaas N M A Bax; David C Van Der Zee; Marijke H Van Den Berg Journal: Haematologica Date: 2004-02 Impact factor: 9.941
Authors: A Tagliaferri; M Franchini; A Coppola; G F Rivolta; C Santoro; G Rossetti; G Feola; E Zanon; A Dragani; P Iannaccaro; P Radossi; P M Mannucci Journal: Haemophilia Date: 2008-06-05 Impact factor: 4.287