[Improving the visibility of rare diseases in health care systems by specific routine coding]

Bundesgesundheitsblatt Gesundheitsforschung Gesundheitsschutz. 2017 May;60(5):532-536. doi: 10.1007/s00103-017-2534-9.
[Article in German]

Abstract

The evaluation of healthcare providers' routine data is an important basis for the analysis, planning and evaluation of measures in public health. The representation of rare diseases in the classifications that are used to record health data is not adequate. Coding rare diseases in a specific way is a challenge all around the world. There is still no general international solution for the routine coding of rare diseases.The double coding of rare diseases with ICD-10 Codes and Orphacodes is a short-term and low-cost alternative solution. Furthermore, this double coding enables international comparability. The specific encoding of rare diseases through this double coding can improve their capturing for statistical analysis and thus their visibility in healthcare systems. Nevertheless, the provision of a new classification is not enough to gather valid data. Some measures have already been adopted in Germany (and at the European level) in order to support the implementation of this double coding. Subsequently it would be possible to adopt more specific public health measures, based on better data, in order to provide better care to the more than four million people in Germany affected by rare diseases.

Keywords: Codification of rare diseases; Data sharing; Interoperability; Orphacode; RD-ACTION.

MeSH terms

  • Data Accuracy*
  • Electronic Health Records / organization & administration*
  • Germany
  • Humans
  • Information Storage and Retrieval / methods*
  • International Classification of Diseases / organization & administration*
  • Medical Record Linkage / methods
  • Quality Improvement / organization & administration*
  • Rare Diseases / classification*
  • Rare Diseases / diagnosis*