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International Health


the assumption that addressing the factors common to all rare diseases is more effective than looking at each particular condition separately. In this article we present the HemoRec database, established in Poland in 2006, as an example of a registry of rare bleeding disorders. It was implemented in selected treatment centres in the country and is being developed in order to collect data on inherited bleeding disorders and create the basis for data analysis and exchange between all relevant healthcare providers.


Registries of Inherited Bleeding Disorders European Principles of Haemophilia Care, formulated in 2008 by a panel of experts,7


stress the importance of collecting data on


haemophilia and related bleeding disorders, and recommends that each country should have a national haemophilia patient registry. Most countries across the world use different kinds of local databases, usually led independently by particular haemophilia treatment centres (HTCs), and a central database, held by the leading HTC of the country. The history and the current status of the Polish registry of patients with inherited bleeding disorders was described recently.8


A large database of patients with inherited bleeding disorders was established in the US. The national surveillance programme, initiated in 1998, enabled collection of data on more than 26,000 patients with inherited bleeding disorders treated in 140 HTCs across the country (www.cdc.gov/ncbddd/blooddisorders/udc/).6


This


database provides valuable, constantly updated information on prevalence, demographics, treatment modalities and complications of haemophilia and von Willebrand disease in the US, published regularly in the form of reports.9


Several attempts were made to create global databases of particular bleeding disorders or treatment modalities. There are three international registries of immune tolerance induction in patients with haemophilia and inhibitors: the International Immune Tolerance Registry,10 American Immune Tolerance Registry11 Registry.12


the North and the ISTH-SSC FIX Inhibitor


In 1996 in Milan a large international project commenced, called the Rare Bleeding Disorders Database (www.rbdd.org), which aimed to extend the knowledge of the rarest bleeding disorders (clotting factor deficiencies except for haemophilia A, haemophilia B and von Willebrand disease). A similar database was created in North America in 1999.13


which is available 24 hours a day, for each HTC using the application. Availability of both host servers and of the database itself is 99.9%.


Sending confidential data via the Internet requires strict safety measures; therefore, data gathered in the database are managed in accordance with global data privacy regulations valid in the European Union (e-Privacy), in the US (21 Code of Federal Regulations [CFR] part 21, the Health Insurance Portability and Accountability Act [HIPPA]) and Canada (the Personal Information Protection and Electronic Documents Act [PIPEDA]). Patient data are anonymised and sent between HTCs in a password-protected and strictly encrypted manner (using 128-bit Secure Sockets Layer [SSL] certificates). Personal information on each particular patient is stored exclusively in the application of the original HTC and can be accessed only by designated healthcare professionals from this centre. Back-up copies of data stored in the database are preserved in a professional data centre.


The HemoRec database has a broad functionality. Demographic and clinical data can be collected and stored, including physical examination and medical history, results of laboratory tests, details in terms of bleeding episodes and treatment. Each stored parameter can be analysed and presented in the form of a graph, which is useful in creating reports and economic analyses, as well as estimating demand for clotting factor concentrates. Handling the application is straightforward and does not require expertise in dealing with advanced computer databases. Each physician authorised to access the database uses an individual login and password. The MyHemorec module enables registration of bleeds and usage of factor concentrate by the patients themselves in their home setting. It can also serve as a communication platform between the patient and their HTC/attending physician.


HemoRec can be used to design and conduct clinical trials. Research projects, individually adjusted to the specific needs of each centre or country, can markedly influence the quality of care and optimise therapeutic procedures in the field of inherited bleeding disorders. Possible areas of research include the assessment and comparison of treatment schedules or dosing,15


arthropathy in patients on prophylaxis and the efficacy of physiotherapy. Data can be evaluated prospectively as well as retrospectively.


There are also several independent international registries of patients with different factor deficiencies such as the Seven Treatment Evaluation Registry (STER), founded by the International Factor VII Deficiency Study Group (www.targetseven.org), and the Greifswald Registry of FX deficiency.14


The HemoRec Project


The international HemoRec project was initiated in 2006. Its aim was to improve the registry of patients with haemophilia with and without inhibitors in Central and Eastern Europe. The project has evolved over the past few years and now includes patients with other inherited bleeding disorders. The Iinternet-based application HemoRec was developed by the Institute of Biostatistics and Analysis at the Masaryk University in Brno, Czech Republic and implemented in the largest HTCs in the following countries: Czech Republic, Slovakia, Poland, Serbia, Macedonia and Latvia.


The HemoRec database and the HemoRec website are hosted by two servers owned by the Masaryk University in Brno. The Institute of Biostatistics and Analysis provides technical support (Help Desk),


148


The status of the HemoRec database varies in each country. At the time when the project was started, a central/national registry was already held; therefore, in most countries, HemoRec serves as an independent registry of patients with haemophilia with and/or without inhibitors. In Serbia, HemoRec serves as a national patient registry and is used to monitor treatment, document the consumption of factor concentrates and estimate demand on these preparations by the Ministry of Health. In Macedonia it gained a similar status and became the national registry of patients with haemophilia.


Since 2006, 1,110 patients with inherited bleeding disorders have been registered in the Polish HemoRec, comprising 24.9% of all patients with inherited bleeding disorders registered in the Polish central registry, held at the Institute of Haematology and Blood Transfusion in Warsaw (status as of 2 November 2010). Fifteen HTCs located in the main cities of Poland have access to the HemoRec database: five paediatric and 10 adult (see Figure 1). A written informed consent for processing personal data was signed by each patient, in accordance with the Polish Personal Data Protection Act


EUROPEAN ONCOLOGY & HAEMATOLOGY


the efficacy of prevention of


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