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Design of a framework for the deployment of collaborative independent rare disease-centric registries: Gaucher disease registry model
Journal article   Open access   Peer reviewed

Design of a framework for the deployment of collaborative independent rare disease-centric registries: Gaucher disease registry model

M.I. Bellgard, K.R. Napier, A.H. Bittles, J. Szer, S. Fletcher, N. Zeps, A.A. Hunter and J. Goldblatt
Blood Cells, Molecules, and Diseases, Vol.68, pp.232-238
2018
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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.

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Collaboration types
Domestic collaboration
Citation topics
1 Clinical & Life Sciences
1.155 Medical Ethics
1.155.1510 Orphan Drugs
Web Of Science research areas
Hematology
ESI research areas
Clinical Medicine
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