Paeds Cases · genetics-dysmorphology-and-metabolism
Communicating a new Wilson disease diagnosis — OSCE
OSCE communication and shared decision-making station: explaining to the parents of a 13-year-old boy what a new Wilson disease diagnosis means, why copper accumulated and caused the tremor and liver changes, what the lifelong treatment involves and why it must never be stopped, what the Kayser-Fleischer rings tell the clinician, the family screening obligations for his siblings, and the role of liver transplantation - while addressing guilt, the fear of a restricted life, and the question of cure.
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Counsel the parents. You have five minutes. Demonstrate an organised, empathic and accurate explanation that addresses the four questions a fellowship communication station rewards: what happened to their son and why, what the immediate and lifelong treatment is and why it must never be stopped, what the family screening means for their other children, and what the honest prognosis is - including the role of liver transplantation. The management framework follows the 2022 AASLD Practice Guidance. [1]
References4ShowHide
- [1]Schilsky ML, Roberts EA, Bronstein JM, et al. A multidisciplinary approach to the diagnosis and management of Wilson disease: Executive summary of the 2022 Practice Guidance on Wilson disease from the American Association for the Study of Liver Diseases. Hepatology, 2023.PMID 36152019
- [3]Schilsky ML. Wilson Disease: Diagnosis, Treatment, and Follow-up. Clin Liver Dis, 2017.PMID 28987261
- [7]Litwin T, Dzieżyc K, Członkowska A. Wilson disease-treatment perspectives. Ann Transl Med, 2019.PMID 31179305
- [9]Schilsky ML. Liver transplantation for Wilson's disease. Ann N Y Acad Sci, 2014.PMID 24820352