A comprehensive study of current haemophilia care and outcomes in Singapore

Abstract

As an island nation, Singapore has a small haemophilia population. Through a hybrid health care model which taps on state-funding, medical insurance, the national retirement savings fund and co-payment by patients, our haemophiliacs receive on-demand therapy as standard of care with opportunities for prophylactic treatment if indicated. Recently, we embarked on a comprehensive crosssectional review of our haemophilia A and B populations to determine their current demographic profile and clinical status in order to gauge the performance of our management model and permit forward planning.This study was conducted in 2013 at the three haemophilia treatment centres in Singapore: Singapore General Hospital (SGH), National University Hospital (NUH) and Kandang Kerbau Women's and Children's Hospital (KKH). SGH houses a Comprehensive Haemophilia Treatment Centre (CHTC) and maintains the National Haemophilia Registry. The Registry provided the baseline demographic information on our haemophiliacs while population and health care statistics on Singapore were obtained from the Department of Statistics, Singapore [1]. All haemophiliacs listed in the Registry were invited to participate in this study. Consenting participants answered a set of questionnaires that explores further details of their treatment, complications and socio-economic data. Participants above the age of 4 were also assessed by a site investigator for the status of their joints using the Haemophilia Joint Health Scores (HJHS) version 2.1 [2]. Joint assessments were only conducted in the absence of recent joint bleed or other acute joint symptoms. Data on factor concentrates usage in 2013 were obtained from units dispensing clotting factors at the respective hospitals.Singapore has 195 identified people living with haemophilia A and another 40 people with haemophilia B among a resident population of 3.85 million [2].The prevalence of haemophilia A and B is 10.31 per 100 000 males and 2.11 per 100 000 males respectively. Demographic and clinical characteristics of these individuals are shown in Table 1. Inhibitors developed in 17.9% of our haemophilia A population [8.2% persistent, 5.6% high responders]. Only one haemophilia B individual had inhibitors. Immune tolerance induction (ITI) therapy was successfully given to two haemophilia A adults with inhibitors using a dose regimen of 100 IU kg À1 day À1 without additional immunosuppressive therapy. One third (30.2%) of our haemophilia A and 20% of our haemophilia B population had no family history of haemophilia.Ninety-two individuals with haemophilia A and 16 with haemophilia B from the ages of 1-79 consented to contributing further socio-economic and clinical data. They represented 19.4%, 56.2% and 67.2% of the mild, moderate and severe haemophilia A population respectively and 40% of the haemophilia B population. The bleeding profile and bleeding-related complications of participating individuals as well as their current mode of treatment, type of product used and access to home treatment is shown in Table...

Burden of mild haemophilia A: Systematic literature review

“…Because data were scarce and estimates varied, we consulted studies not meeting inclusion criteria to provide context for ABR. Two studies were found with an ABR estimate for mild HA patients of all ages (including children); mean ABR centred on 0.5‐0.6 episodes per year . Mild HA patients in our review also experienced other morbidity, QoL impacts and economic impacts.…”

Principles of haemophilia care: The Asia‐Pacific perspective

“…Both environmental and genetic factors are implicated in the development of inhibitors. Exposure to the coagulation factor antigen, at high doses in the first year of life and during inflammatory danger signals, has been associated with higher risk of inhibitors . The comparative rate of inhibitor development with von Willebrand factor containing intermediate purity pdCFC or rCFC has been a matter of long‐standing debate.…”

“…Exposure to the coagulation factor antigen, at high doses in the first year of life and during inflammatory danger signals, has been associated with higher risk of inhibitors. 37 The comparative rate of inhibitor development with von Willebrand factor containing intermediate purity pdCFC or rCFC has been a matter of long-standing debate. A recent randomized controlled study has found higher risk of inhibitors with rCFC only among previously untreated and minimally treated children.…”

<b>Características sociodemográficas de homens com hemofilia no sul do brasil / Sociodemographic characteristics of men with hemophilia in the south of brazil<b>

“…A hemofilia A ocorre com mais frequência que a hemofilia B e representa em torno de 80% dos casos (1) . Outros estudos (2,(8)(9)(12)(13)(14) também apontam o predomínio da hemofilia A. Ambas são classificadas de acordo com o nível de atividade coagulante dos fatores VIII e IX. É considerada grave quando o nível plasmático desses fatores for inferior a 1%, moderada entre 1 a 5% e leve de 5 a 40% (1) .…”

“…Na forma grave os sangramentos mais comuns são as hemartroses e os hematomas musculares ligados a traumas ou, comumente, espontâneos (1,8) . Quanto à gravidade, outros estudos corroboram com os resultados encontrados nesse trabalho, com maior ocorrência da forma grave (8,12,14) . Em relação à idade, o tratamento profilático melhora significativamente a qualidade de vida dos usuários e minimiza as hemorragias que oferecem risco de vida (15)(16) , com impacto positivo na expectativa de vida.…”