Original article

Blood Transfusion - 2 2020 (March-April)

Physical activity improved by adherence to prophylaxis in an Italian population of children, adolescents and adults with severe haemophilia A: the SHAPE Study

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Key words: severe haemophilia, physical activity, adherence to treatment, FVIII replacement therapy, orthopaedic haemophilia scores
Publication Date: 2019-06-05

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.

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Authors

Ezio Zanon - Haemophilia Centre University Hospital of Padua, Padua, Italy

Annarita Tagliaferri - Regional Reference Centre for Inherited Bleeding Disorders, University Hospital of Parma, Parma, Italy

Samantha Pasca - Haemophilia Centre University Hospital of Padua, Padua, Italy

Cosimo P. Ettorre - Haemophilia and Thrombosis Center, "Giovanni XXIII" Hospital, Bari, Italy

Lucia D. Notarangelo - Oncology-Haematology and Bone Marrow Transplantation Unit, Children's Hospital, "Spedali Civili", Brescia, Italy

Chiara Biasioli - Haemophilia Center, "Bufalini" Hospital, Cesena, Italy

Anna B. Aru - Pediatric Onco-Haematology Center, "Microcitemico" Hospital Cagliari, Italy

Marta Milan - Haemophilia Centre University Hospital of Padua, Padua, Italy

Silvia Linari - Centre for Bleeding Disorders, "Careggi" University Hospital, Florence, Italy

Angiola Rocino - Haemophilia and Thrombosis Centre, "San Giovanni Bosco" Hospital, Naples, Italy

Fabio Gagliano - Haemophilia Center, "G. Di Cristina" Children's Hospital, Palermo, Italy

Giovanni Di Minno - Regional Reference Centre for Coagulation Disorders, "Federico II" University Hospital, Naples, Italy

Gabriella Gamba - Haemophilia and Congenital Coagulation disorders Center, "San Matteo" Hospital, Pavia, Italy

Rita C. Santoro - Haemostasis and Thrombosis Unit, Haemophilia Center, "Pugliese-Ciaccio" Hospital, Catanzaro, Italy

Piercarla Schinco - Haemostasis and Thrombosis Unit, "Molinette" Hospital Turin, Italy

Marco Marietta - Department of Oncology, Hematology and Respiratory System Diseases, University Hospital, Modena, Italy

Axel Seuser - Centre for Prevention, Rehabilitation and Orthopaedics, Bonn, Germany

Sylvia von Mackensen - Institute of Medical Psychology, University Medical Centre, Hamburg, Germany

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