Illness perception and clinical treatment experiences in patients with M. Maroteaux-Lamy (mucopolysaccharidosis type VI) and a Turkish migration background in Germany

PLoS One. 2013 Jun 24;8(6):e66804. doi: 10.1371/journal.pone.0066804. Print 2013.

Abstract

Introduction: Mucopolysaccharidosis VI (MPS VI) is an inherited lysosomal storage disease caused by a mutation of the gene for arylsulfatase B (ASB). Of the thirty-one patients registered in Germany, almost fifty percent have a Turkish migration background. MPS VI is treated by enzyme replacement therapy (ERT), which is time-consuming and expensive.

Methods: This interdisciplinary study explored the illness perceptions and clinical treatment experiences among ten MPS VI patients with a Turkish migration background in two centers for metabolic diseases (Berlin and Mainz, Germany). The clinical treatment situation was observed and semi-structured interviews were conducted with patients and health care personnel, in addition to participatory observation in four patients' everyday environments in Berlin. The data from the interviews, patient records, and personal field notes were encoded, cross-related, and analyzed.

Results: Patients' acknowledgement of the disease and coping strategies are influenced predominantly by the perception of their individual health status and the handling of the disease within their family. Patients' willingness to cooperate with treatment strategies is further modified by their knowledge of the disease and the relationships with their health care providers. In this analysis, cultural factors turned out to be marginally relevant.

Conclusion: As with other chronic and debilitating diseases, effective treatment strategies have to reach beyond delivering medication. Health care providers need to strengthen the support for patients with a migration background. In this regard, they should respect the patients' cultural and social background and their personal perception of the disease and the therapy. Yet structural and social aspects (clinical setting, family and educational background) may be more crucial here than "cultural barriers."

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Demography
  • Emigration and Immigration*
  • Enzyme Replacement Therapy
  • Female
  • Germany
  • Health Knowledge, Attitudes, Practice*
  • Health Personnel
  • Humans
  • Male
  • Mucopolysaccharidosis VI / drug therapy*
  • N-Acetylgalactosamine-4-Sulfatase / therapeutic use
  • Physician-Patient Relations
  • Religion
  • Socioeconomic Factors
  • Treatment Outcome
  • Turkey / ethnology
  • Young Adult

Substances

  • N-Acetylgalactosamine-4-Sulfatase

Grant support

The research undertaken for this article was funded by an unrestricted grant from Bio-Marin Europe. The authors confirm independence from the sponsors; the content of the article has not been influenced by the sponsors. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.