title: Illness perception and clinical treatment experiences in patients with M. Maroteaux-Lamy (mucopolysaccharidosis type VI) and a Turkish migration background in Germany.
abstract: BACKGROUND
  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.
  
  
  CONCLUSIONS
  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."
authors.affiliation: Institute of Social and Cultural Antropology, Freie Universität Berlin, Berlin, Germany.
authors.forename: Hansjörg
authors.initials: H
authors.lastname: Dilger
authors.forename: Linn
authors.initials: L
authors.lastname: Leissner
authors.forename: Lenka
authors.initials: L
authors.lastname: Bosanska
authors.forename: Christina
authors.initials: C
authors.lastname: Lampe
authors.forename: Ursula
authors.initials: U
authors.lastname: Plöckinger
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D003710:Demography; D004641:Emigration and Immigration; D056947:Enzyme Replacement Therapy; D005260:Female; D005858:Germany; D007722:Health Knowledge, Attitudes, Practice; D006282:Health Personnel; D006801:Humans; D008297:Male; D009087:Mucopolysaccharidosis VI / Q000188:drug therapy*; D020051:N-Acetylgalactosamine-4-Sulfatase / Q000627:therapeutic use; D010817:Physician-Patient Relations; D012067:Religion; D012959:Socioeconomic Factors; D016896:Treatment Outcome; D014421:Turkey / Q000208:ethnology; D055815:Young Adult
chemical_list: D020051:N-Acetylgalactosamine-4-Sulfatase