title: Cultural aspects in the management of inborn errors of metabolism.
abstract: European Health Care Systems have not yet accommodated both previous and current migration waves. Children from immigrant families, especially children with chronic conditions, are particularly affected from the shortcomings in medical care. One condition, phenylketonuria (PKU), is an inborn error of metabolism (IEM) which results in intellectual disability unless treated with a lifelong phenylalanine (Phe) restricted diet. In our PKU clinic, patients from families who previously had emmigrated from the geographic area of Turkey to Austria, exhibited worse blood Phe control and cognitive development than comparable patients from native Austrian families. Using structured and semi-structured interviews, questionnaires, and illness narratives, we identified language, psychosocial, economic, educational and cultural barriers as factors influencing adherence to treatment. Our findings led us to conclude that access to interpreter services, exploration of the socio-cultural background and of family ecology, as well as bi-directional communication and medical decision making according to the "best interest of the child" principle, may improve outcomes in patients requiring complex treatment and care.
authors.affiliation: Division of Biochemical Diseases, Department of Pediatrics, British Columbia Children's Hospital, University of British Columbia, Vancouver, Canada.
authors.forename: Sylvia
authors.initials: S
authors.lastname: Stockler
authors.forename: Dorothea
authors.initials: D
authors.lastname: Moeslinger
authors.forename: Marion
authors.initials: M
authors.lastname: Herle
authors.forename: Banu
authors.initials: B
authors.lastname: Wimmer
authors.forename: Osman S
authors.initials: OS
authors.lastname: Ipsiroglu
mesh_terms: D001317:Austria; D002648:Child; D003142:Communication; D003466:Cultural Characteristics; D004641:Emigration and Immigration; D005006:Ethnicity; D005191:Family Characteristics; D005260:Female; D006801:Humans; D007802:Language; D008297:Male; D008661:Metabolism, Inborn Errors / Q000191:economics / Q000523:psychology / Q000628:therapy*; D010649:Phenylalanine / Q000097:blood; D010661:Phenylketonurias / Q000097:blood / Q000178:diet therapy / Q000191:economics / Q000523:psychology; D012959:Socioeconomic Factors; D014421:Turkey / Q000208:ethnology
chemical_list: D010649:Phenylalanine