Haemophilia. 2018;1–8. wileyonlinelibrary.com/journal/hae
|
1 © 2018 John Wiley & Sons Ltd
Accepted: 18 April 2018
DOI: 10.1111/hae.13517
ORIGINAL ARTICLE
The Jamaican Haemophilia Registry: Describing the burden of
disease
G. Wharfe
1
| L. Buchner-Daley
1
| T. Gibson
1
| P. Hilliard
2
| K. Usuba
3,4
|
A. Abad
3
| L. Boma-Fischer
5
| V. Bouskill
6
| G. Floros
5
| D. Lillicrap
7
|
Y. Lowe
1
| D. Lowe
1
| N. Palmer-Mitchell
1
| M. L. Rand
8,9,10
| J. Teitel
5
| A. Tuttle
7
|
A. Watson
1
| R. White
1
| N. L. Young
3,4
| V. S. Blanchette
3,10,11
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
Correspondence
Victor Blanchette, Division of Hematology/
Oncology, The Hospital for Sick Children,
Toronto, ON, Canada.
Email: victor.blanchette@sickkids.ca
Funding information
Shire Global; Hospital for Sick Children;
Novo Nordisk Haemophilia Foundation;
World Federation of Hemophilia
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), includ-
ing 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 cau-
tion 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 multidis-
ciplinary Comprehensive Care Program for assessment and care of PWH in Jamaica.
KEYWORDS
burden of disease, haemophilia, health assessment, Jamaica, outcome measures, registry