The Jamaican Haemophilia Registry: Describing the burden of disease.

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.

Evolution of blood donation patterns in a hospital-based blood centre over a seven-year period: implications for donor recruitment and retention.

OBJECTIVE: The aim of this study is to investigate the effect of a public appeal to encourage voluntary blood donation by comparing the pattern of blood donations in 2000 and 2007. METHODS: A retrospective analysis of blood donation records was conducted at the University Hospital of the West Indies (UHWI) Blood Collection Centre from April to December of 2000 and 2007. Data were analysed to identify any significant changes in donation patterns and donor profiles. RESULTS: The total number of blood donor records reviewed was 3194 in 2000 and 2634 in 2007 representing 69.0% and 72.3% of the total blood donations, respectively. Autologous donations accounted for 1% in 2000 and 2.2% in 2007; however, there was no corresponding change in voluntary donations (3.4% in 2000 and 3.2% in 2007). Despite a reduction in the number of first-time donors (1539 in 2000 and 1115 in 2007), the percentage of units discarded for the presence of a marker of transfusion transmission infection (TTI) increased, being 6.5% in 2000 and 7.4% in 2007. Human T-lymphotropic virus (HTLV) was the most common infectious marker in 2000 (3.4% of donors) whereas reactive Venereal Disease Research Laboratory (VDRL) predominated in 2007 (3.6% of donors). CONCLUSION: The per capita donations (0.99% in 2000 and 0.88% in 2007) failed to meet the World Health Organization (WHO) recommendation for an adequate blood supply of 1-3%. Despite a national effort to improve voluntary donations, the positive changes in the pattern of blood donation over a period of seven years were limited to a decrease in the proportion of first-time donors and an increase in blood donors with one to four previous donations.

Chronic myeloid leukaemia at the University Hospital of the West Indies: a 17-year review.

OBJECTIVE: To determine the presenting features and evolution of patients diagnosed with chronic myeloid leukaemia between 1983 and 1999 at the University Hospital of the West Indies. METHODS: Forty-one records were retrospectively analyzed for the patients' demographics, reasons for referral, clinical features, laboratory investigations and the time to blast transformation and death. RESULTS: Seventy-one per cent were males and 29% were females. The male to female ratio was 2.4:1. The median age at presentation was 37 years (range 14-81 years). Seventy-eight per cent of the patients presented in the chronic phase. Weight loss and splenomegaly were the most frequent presenting features being seen in 54 and 83 per cent respectively. The median survival was 36 months. CONCLUSION: In this study, the clinical features and evolution were comparable to existing data. Improved accrual and routine Philadelphia chromosome testing would provide a more accurate reflection of the status of CML in our population.