G Wharfe1, L Buchner-Daley1, T Gibson1, P Hilliard2, K Usuba3,4, A Abad3, L Boma-Fischer5, V Bouskill6, G Floros5, D Lillicrap7, Y Lowe1, D Lowe1, N Palmer-Mitchell1, M L Rand8,9,10, J Teitel5, A Tuttle7, A Watson1, R White1, N L Young3,4, V S Blanchette3,10,11. 1. University Hospital, University of the West Indies (UWI), Mona, Kingston, Jamaica. 2. Department of Rehabilitation, The Hospital for Sick Children, Toronto, ON, Canada. 3. Child Health Evaluative Sciences Program, Research Institute, The Hospital for Sick Children, Toronto, ON, Canada. 4. School of Rural and Northern Health and ECHO Research Centre, Laurentian University, Sudbury, ON, Canada. 5. Department of Hematology, Comprehensive Care Hemophilia Program, St. Michael's Hospital, Toronto, ON, Canada. 6. Department of Nursing, The Hospital for Sick Children, Toronto, ON, Canada. 7. Department of Pathology and Molecular Medicine, Queen's University, Kingston, ON, Canada. 8. Departments of Laboratory Medicine & Pathobiology, Biochemistry and Paediatrics, University of Toronto, Toronto, ON, Canada. 9. Translational Medicine Program, Research Institute, The Hospital for Sick Children, ON, Canada. 10. Division of Haematology/Oncology, The Hospital for Sick Children, Toronto, ON, Canada. 11. Department of Paediatrics, University of Toronto, Toronto, ON, Canada.
Abstract
INTRODUCTION: Jamaica has an estimated 200 persons with haemophilia (PWH), who face significant constraints in access to specialized haemophilia care, including access to clotting factor concentrates. AIM: The aim of this paper is to establish the current burden of disease in PWH in Jamaica. METHODS: PWH were enrolled through the University Hospital of the West Indies, Jamaica. The impact of haemophilia was assessed using a comprehensive battery of heath outcome measures that included the following: laboratory, clinical information and validated outcome measures of joint structure and function, activity, and health-related quality of life (HRQoL) to provide a health profile of the Jamaican haemophilia population. RESULTS: In all, 45 PWH were registered (mean age: 29, range: 0.17-69 years), including 13 children (<18 years of age) and 32 adults. In this sample, 41 had haemophilia A (30 severe) and 4 had haemophilia B (3 severe); 10 patients with haemophilia A were inhibitor positive. The results indicate that adults with haemophilia in Jamaica have significant joint damage: mean Haemophilia Joint Health Score (HJHS) = 42.1 (SD = 17.3); moderate activity levels - mean Haemophilia Activities List (HAL) score = 64.8 (SD = 17.8); and low HRQoL scores - mean Haemo-QoL-A score = 62.3 (SD = 19.4). Results for children are also reported but should be interpreted with caution due to the small sample size. CONCLUSIONS: There is a very high burden of disease in PWH in Jamaica. The health profiles reported in this paper are an essential first step in advocating for a multidisciplinary Comprehensive Care Program for assessment and care of PWH in Jamaica.
INTRODUCTION: Jamaica has an estimated 200 persons with haemophilia (PWH), who face significant constraints in access to specialized haemophilia care, including access to clotting factor concentrates. AIM: The aim of this paper is to establish the current burden of disease in PWH in Jamaica. METHODS: PWH were enrolled through the University Hospital of the West Indies, Jamaica. The impact of haemophilia was assessed using a comprehensive battery of heath outcome measures that included the following: laboratory, clinical information and validated outcome measures of joint structure and function, activity, and health-related quality of life (HRQoL) to provide a health profile of the Jamaican haemophilia population. RESULTS: In all, 45 PWH were registered (mean age: 29, range: 0.17-69 years), including 13 children (<18 years of age) and 32 adults. In this sample, 41 had haemophilia A (30 severe) and 4 had haemophilia B (3 severe); 10 patients with haemophilia A were inhibitor positive. The results indicate that adults with haemophilia in Jamaica have significant joint damage: mean Haemophilia Joint Health Score (HJHS) = 42.1 (SD = 17.3); moderate activity levels - mean Haemophilia Activities List (HAL) score = 64.8 (SD = 17.8); and low HRQoL scores - mean Haemo-QoL-A score = 62.3 (SD = 19.4). Results for children are also reported but should be interpreted with caution due to the small sample size. CONCLUSIONS: There is a very high burden of disease in PWH in Jamaica. The health profiles reported in this paper are an essential first step in advocating for a multidisciplinary Comprehensive Care Program for assessment and care of PWH in Jamaica.
Authors: K John Pasi; Michael Laffan; Savita Rangarajan; Tara M Robinson; Nina Mitchell; Will Lester; Emily Symington; Bella Madan; Xinqun Yang; Benjamin Kim; Glenn F Pierce; Wing Yen Wong Journal: Haemophilia Date: 2021-08-11 Impact factor: 4.263
Authors: Erna C van Balen; Brian O'Mahony; Marjon H Cnossen; Gerard Dolan; Victor S Blanchette; Kathelijn Fischer; Deborah Gue; Jamie O'Hara; Alfonso Iorio; Shannon Jackson; Barbara A Konkle; Diane J Nugent; Donna Coffin; Mark W Skinner; Cees Smit; Alok Srivastava; Fred van Eenennaam; Johanna G van der Bom; Samantha C Gouw Journal: Res Pract Thromb Haemost Date: 2021-03-06
Authors: Koyo Usuba; Victoria E Price; Victor Blanchette; Audrey Abad; Carmen Altisent; Loretta Buchner-Daley; Jorge D A Carneiro; Brian M Feldman; Kathelijn Fischer; John Grainger; Susanne Holzhauer; Koon-Hung Luke; Sandrine Meunier; Margareth Ozelo; Ling Tang; Sandra V Antunes; Paula Villaça; Cindy Wakefield; Gilian Wharfe; Runhui Wu; Nancy L Young Journal: Res Pract Thromb Haemost Date: 2019-04-23
Authors: Isolde A R Kuijlaars; Janjaap van der Net; Tyler W Buckner; Christine L Kempton; Roger E G Schutgens; Kathelijn Fischer Journal: Haemophilia Date: 2021-09-08 Impact factor: 4.263