The Inherited Cancer Connect (ICCon) Partnership has established a national database of adult individuals carrying a pathogenic mutation in a cancer predisposition gene, identified through an FCC before the end of 2016 (n=11741, June 2018). Our aim is to enable a cohesive approach to drive translational research and improve the health of people with a hereditary cancer predisposition.
We present a summary of the number and nature of adult mutations carriers identified in Australia until December 2016. A minimal dataset collected during routine clinical care is entered for each carrier where available. Re-identifiable data can be extracted for the purposes of: identifying carriers who may benefit from new therapeutic interventions, responding to feasibility enquiries and identifying eligible patients for research and clinical trials, providing supportive data for health policy applications, and identifying shared families across FCCs. Collaborations have already been established with both national and international studies (ENIGMA; InSiGHT; AGHA; IMRC; Hide and Seek with Hereditary Cancer; Expanding Diagnostic Approaches for Lynch Syndrome), which utilise the ICCon database and/or partnership infrastructure to facilitate their own research activities.
While establishing the database, we encountered several barriers. (1) Ethics approval took four years to obtain in NSW and there was difficulty identifying the appropriate data custodian for a centralised state-based database. (2) Different jurisdictions required different processes in accessing data. (3) Variations in data management across FCCs meant that manual sourcing and entering of data from hard copy files was sometimes required where data was unavailable electronically. Data was not always complete and sometimes variable, requiring manual checking and/or entry. (4) Variations in clinical database software across FCCs resulted in an incompatibility in software used between several FCCs and the ICCon database; culminating in an inability to automatically transfer data to the ICCon database. (5) Few FCCs routinely receive prospective follow up data about mutation carriers unless the FCC has an established patient follow-up program.
Through the ICCon database we have identified the need for an agreed national minimal dataset to encourage the collection of clinically valuable and accessible data across all FCCs. In addition, it opens the discussion about ongoing clinical responsibility to identified mutation carriers and their families regarding cancer risk management to improve mortality and morbidity outcomes. Finally, the ICCon database can be used to develop a national registry of mutation carriers that will be essential for national health service planning and providing optimal clinical care, especially given the advent of mutation-targeted cancer therapies and rapidly changing advice around cancer risk-management practices. With the expansion of cancer genetic testing out of the FCC environment, a centralised register of mutation carriers is highly desirable.