The Jamaican Haemophilia Registry: Describing the burden of disease.

IntroductionJamaica has an estimated 200 persons with haemophilia (PWH), who face significant constraints in access to specialized haemophilia care, including access to clotting factor concentrates. AimThe aim of this paper is to establish the current burden of disease in PWH in Jamaica. MethodsPWH 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. ResultsIn all, 45 PWH were registered (mean age: 29, range: 0.17-69years), including 13 children (<18years 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. ConclusionsThere 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.