Counsel the family of a newly diagnosed adolescent with von Willebrand disease — OSCE

OSCE communication and shared-planning station: explaining a new diagnosis of type 1 von Willebrand disease to an adolescent girl and her parent, the meaning of von Willebrand factor and the autosomal inheritance, the role of desmopressin and tranexamic acid, the management of heavy menstrual bleeding, the warning signs that mean coming to hospital, and the importance of comprehensive care, school planning and the avoidance of certain medications.

osce communication and shared planning6 min readVerification in progress

Prompt

A fourteen-year-old girl whose heavy menstrual bleeding and iron deficiency have led to a diagnosis of type 1 von Willebrand disease wants to know what the condition is, why she bruises and bleeds, whether it will affect her sport and future children, what to do about her periods, and when she should come to hospital.

Candidate instructions

You are the paediatric registrar. You have eight minutes to speak with fourteen-year-old Mia and her mother, Mrs Chen. Mia has just been diagnosed with type 1 von Willebrand disease after presenting with heavy menstrual bleeding and iron deficiency, along with lifelong easy bruising and prolonged nose-bleeds. Explain what von Willebrand disease is in plain language, what causes her bruising and heavy periods, how it is treated including desmopressin and tranexamic acid, what the plan is for her periods, the warning signs that mean coming straight to hospital, and how the condition may affect her sport and her future. Answer their questions, check their understanding, and agree a plan. ^[1]

References5Show

[1]Leebeek FW, Eikenboom JC Von Willebrand's Disease. N Engl J Med, 2016.PMID 27959741
[2]Nichols WL, Hultin MB, James AH, Manco-Johnson MJ, et al. von Willebrand disease (VWD): evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) expert panel report. Haemophilia, 2008.PMID 18315614
[4]Mannucci PM Treatment of von Willebrand's Disease. N Engl J Med, 2004.PMID 15306670
[5]Sadler JE A revised classification of von Willebrand disease. For the Subcommittee on von Willebrand Factor of the Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis. Thromb Haemost, 1994.PMID 8052974
[10]Mannucci PM, Federici AB, James AH, Kessler CM von Willebrand disease in the 21st century: current approaches and new challenges. Haemophilia, 2009.PMID 19624761

Counsel the family of a newly diagnosed adolescent with von Willebrand disease — OSCE

osce communication and shared planning6 min readVerification in progress

Candidate instructions

References5Show

[1]Leebeek FW, Eikenboom JC Von Willebrand's Disease. N Engl J Med, 2016.PMID 27959741
[2]Nichols WL, Hultin MB, James AH, Manco-Johnson MJ, et al. von Willebrand disease (VWD): evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) expert panel report. Haemophilia, 2008.PMID 18315614
[4]Mannucci PM Treatment of von Willebrand's Disease. N Engl J Med, 2004.PMID 15306670
[5]Sadler JE A revised classification of von Willebrand disease. For the Subcommittee on von Willebrand Factor of the Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis. Thromb Haemost, 1994.PMID 8052974
[10]Mannucci PM, Federici AB, James AH, Kessler CM von Willebrand disease in the 21st century: current approaches and new challenges. Haemophilia, 2009.PMID 19624761