Document Type

Journal Article

Publisher

Elsevier

School

School of Medical and Health Sciences

RAS ID

24346

Funders

Wellcome Trust Pathfinder Award [REF 104746] with financial contribution to this award by Shire Australia Pty. Ltd.

Australian National Health and Medical Research Council

EU FP7 Project [HEALTH.2012.2.1.1-1-C]: RD Connect

Grant Number

NHMRC Numbers : APP634485, APP1055319

Comments

Bellgard, M. I., Napier, K. R., Bittles, A. H., Szer, J., Fletcher, S., Zeps, N., … Goldblatt, J. (2018). Design of a framework for the deployment of collaborative independent rare disease-centric registries: Gaucher disease registry model. Blood Cells, Molecules, and Diseases, 68, 232-238.

https://doi.org/10.1016/j.bcmd.2017.01.013

Abstract

Orphan drug clinical trials often are adversely affected by a lack of high quality treatment efficacy data that can be reliably compared across large patient cohorts derived from multiple governmental and country jurisdictions. It is critical that these patient data be captured with limited corporate involvement. For some time, there have been calls to develop collaborative, non-proprietary, patient-centric registries for post-market surveillance of aspects related to orphan drug efficacy. There is an urgent need for the development and sustainable deployment of these ‘independent’ registries that can capture comprehensive clinical, genetic and therapeutic information on patients with rare diseases. We therefore extended an open-source registry platform, the Rare Disease Registry Framework (RDRF) to establish an Independent Rare Disease Registry (IRDR). We engaged with an established rare disease community for Gaucher disease to determine system requirements, methods of data capture, consent, and reporting. A non-proprietary IRDR model is presented that can serve as autonomous data repository, but more importantly ensures that the relevant data can be made available to appropriate stakeholders in a secure, timely and efficient manner to improve clinical decision-making and the lives of those with a rare disease.

DOI

10.1016/j.bcmd.2017.01.013

Creative Commons License

Creative Commons Attribution 4.0 License
This work is licensed under a Creative Commons Attribution 4.0 License.

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