Evaluation of a web-based registry of inherited bleeding disorders: a descriptive study of the Brazilian experience with HEMOVIDAweb Coagulopatias

Abstract

BackgroundInherited bleeding disorders (IBD) consist of a group of rare heterogeneous diseases, which require treatment for life. Management of these disorders is complex and costly. Therefore, good quality data of the affected population is crucial to guide policy planning. The aim of this manuscript is to describe the impact of a national, web-based registry – the Hemovidaweb Coagulopatias (HWC) – in the management of the IBD in Brazil.MethodsThe system was developed in PHP 5.0 language and is available on the internet at http://coagulopatiasweb.datasus.gov.br. The system was validated in September 2008 and launched nationally with input from January 1, 2009. HWC collects variables related to socio-demographic, clinical, laboratory and treatment data of patients with IBD.ResultsWithin 7 years, there was an increment of 90.8% on the diagnosis of IBD altogether, which increased from 11,040 in December 2007 to 21,066 in December 2014. This is now the fourth and third largest world population of patients with haemophilia and von Willebrand’s disease (vWD), respectively, according to the most recent (2015) Annual Global Survey of the World Federation of Hemophilia. The data collected provided the basis for planning and implementing home therapy, prophylaxis and immune tolerance induction (ITI), recently initiated in Brazil.ConclusionHWC was an effective tool in the increment of registration of patients with IBD in Brazil. Furthermore, it was essential to support policy planning, monitoring, evaluation and treatment. Future development should focus on surveillance, health outcomes and research. Every country should implement a national registry on IBD.

Mortality of patients with haemophilia in Brazil: First report

“…In 2007, the Ministry of Health implemented a short‐home treatment programme, which comprised delivering three doses of factor concentrate for home treatment to be used for immediate replacement in case of bleeding. In 2009, a web‐based, national registry on inherited bleeding disorders was created . In 2012, prophylaxis, full home treatment and ITI programmes were implemented.…”

“…In 2009, a web-based, national registry on inherited bleeding disorders was created. 9 In 2012, prophylaxis, full home treatment and ITI programmes were implemented.…”

“…Haemophilia care in Brazil is guaranteed by the National Public Health System ( Sistema Único de Saúde) . In 2009, the Brazilian Ministry of Health initiated a web‐based registry of patients with inherited bleeding disorders, including haemophilia . From 2011 onwards, haemophilia care improved as a result of the implementation of health policies involving increased purchase of factor concentrates leading to the implementation of immune tolerance induction (ITI), prophylaxis and home treatment.…”

“…individuals registered in 2016, 8 Ministry of Health initiated a web-based registry of patients with inherited bleeding disorders, including haemophilia. 9 From 2011 onwards, haemophilia care improved as a result of the implementation of health policies involving increased purchase of factor concentrates leading to the implementation of immune tolerance induction (ITI), prophylaxis and home treatment. Since mortality studies can be useful as a tool to monitor progress of care in a population, it can be of value for policymakers and stakeholders to evaluate health policies.…”

Variation of rs3754689 at lactase gene and inhibitors in admixed Brazilian patients with hemophilia A

“…Treatment of hemophilia represents a burden of 350 million dollars per year with the purchase of coagulation factor concentrates, of which approximately 25% is spent annually for the treatment of immune tolerance induction and bleeding events for around 600 patients with clinically-relevant inhibitors. 4 Therefore, a predictive genetic marker of inhibitor development is urgently needed. Several studies have attempted to identify a predictive genetic marker of inhibitor development to be used in the clinical practice.…”