The Jamaican Haemophilia Registry: Describing the burden of disease

G Wharfe; L Buchner-Daley; T Gibson; P Hilliard; K Usuba; A Abad; L Boma-Fischer; V Bouskill; G Floros; D Lillicrap; Y Lowe; D Lowe; N Palmer-Mitchell; M L Rand; J Teitel; A Tuttle; A Watson; R White; N L Young; V S Blanchette

doi:10.1111/hae.13517

The Jamaican Haemophilia Registry: Describing the burden of disease

Haemophilia. 2018 Jul;24(4):e179-e186. doi: 10.1111/hae.13517. Epub 2018 May 31.

Authors

G Wharfe¹, L Buchner-Daley¹, T Gibson¹, P Hilliard², K Usuba^{3

4}, A Abad³, L Boma-Fischer⁵, V Bouskill⁶, G Floros⁵, D Lillicrap⁷, Y Lowe¹, D Lowe¹, N Palmer-Mitchell¹, M L Rand^{8

9

10}, J Teitel⁵, A Tuttle⁷, A Watson¹, R White¹, N L Young^{3

4}, V S Blanchette^{3

10

11}

Affiliations

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.

PMID: 29855125
DOI: 10.1111/hae.13517

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.

Keywords: Jamaica; burden of disease; haemophilia; health assessment; outcome measures; registry.

MeSH terms

Adolescent
Adult
Aged
Child
Child, Preschool
Cost of Illness*
Female
Hemophilia A / economics*
Hemophilia A / epidemiology*
Hemophilia B / economics*
Hemophilia B / epidemiology*
Humans
Infant
Jamaica / epidemiology
Male
Middle Aged
Quality of Life
Registries*
Surveys and Questionnaires
Young Adult