The MISSION of the Canadian FH Registry is to bring together a multi-disciplinary group of physicians, basic and clinical researchers to improve the delivery of care to patients with severe lipoprotein disorders, especially FH, and to foster collaborative research.
Our VISION is to create a Canada-wide network of academic clinics, integrating lipid specialists, endocrinologists and cardiologists to treat patients with the highest standard of care and to create a collaborative research environment. Using a “hub and spoke” model, the registry will be extended in various communities to link primary care physicians with provincial academic centers.
The GOALS are to improve care to patients with FH and to reduce cardiovascular disease in this population at high risk.
The purpose of this initiative is to create a registry of subjects with FH across Canada. In Canada, the burden of disease is estimated to be approximately 83,500 patients, based on an allele frequency of 1/270 in Quebec and 1/500 in the rest of the country. Rare diseases of lipoprotein metabolism (orphan diseases) will also be included.
Given the wide geographic distribution of patients with FH in Canada, we provide a “hub and spoke” model specialized care. The hubs (red dots) are based in each province in centers recognized at the national level for their expertise in lipoprotein disorders and with advanced laboratory facilities (medical biochemistry, specialized assays, ultracentrifugation, genetic testing). Radiating from these centers are clinics that treat patients with FH.
We set up a three-tier registry (local, provincial and Canada-wide). The local database is nominative, used solely by the clinic or center and contains the information obtained by the local investigators and used for clinical purposes. From the data contained in local databases, data agreed upon by all members of the FH Registry initiative is captured and entered into the provincial database. This database has no identifiers other than the provincial health insurance number. This database can be used to generate province-specific health outcomes, resource utilization, health economics and comparative epidemiological/genetic/clinical features across provinces. The Canadian database is completely anonymous and will serve to generate country-wide statistics, public awareness and research collaborations. The data collected include medical and familial history, lifestyle and medication data, and laboratory findings.
Completely anonymous data, without any identifier –such as hospital ID number, date of birth, family relationships– will be made available to researchers, policy makers, governments and industry on a per need basis after a submission of a written request and approval by the Scientific Board in consultation with the Advisory Board.
The various steps associated with the inclusion of patients within the FH Canada Registry are presented below.
A patient who meets the requirements (FH criteria satisfied) is eligible to enter in the FH Canada registry and will have his data collected after approval.
The immediate relatives of an individual diagnosed with FH (parents, siblings, children) have a 50% risk or chance of having FH. We use the method of “cascade screening”, which involves testing of lipid levels in all primary (parents, siblings, and children), secondary (aunts, uncles, grandparents, grandchildren) and third-degree (cousins, nephews, nieces) relatives of the family member diagnosed with FH. This method has been used in other countries with a great deal of success and it has been established that this process of “seek and treat” saves both lives and money through this health promotion/disease prevention strategy.
The patient is followed up every year to collect data about his health, current treatment and laboratory findings.
Any patient who did not satisfy the FH criteria but has blood lipid abnormalities is entered in the SMASH (Systems and Molecular Approach of Severe Hyperlipidemia) part of the FH Canada registry. This will permit the capture of rare and orphan lipoprotein disorders.
The James Hogg Research Centre at St-Paul’s Hospital, UBC, Vancouver, provides the iCAPTURE platform used to capture the data from the Canadian FH Registry. The database software utilizes an Oracle backend and is firewalled and maintained in a separate non public network, and it is FDA (US Food and Drug administration), Health Canada and PIPEDA (Canada Personal Information Protection and Electronic Documents Act) compliant. It complies with privacy regulations in all provinces and uses passwords that allow limited access, with access tailored to each site. It allows researchers access under strictly controlled conditions after peer-review. The software is flexible enough to allow easy data entry for the provincial and Canadian databases. The James Hogg Research Centre maintains multi-level security for all database and computer systems.