Counsel the family of a newly diagnosed child with haemophilia A — OSCE

OSCE communication and shared-planning station: explaining a new diagnosis of severe haemophilia A to a parent, the meaning of factor VIII deficiency and the X-linked inheritance, the role of primary prophylaxis with factor concentrate or subcutaneous emicizumab, the management of bleeds at home including the emergency response to a head injury, the avoidance of intramuscular injections and certain medications, and the importance of comprehensive care, school planning and transition.

osce communication and shared planning5 min readVerification in progress

Prompt

A mother whose fourteen-month-old son has just been diagnosed with severe haemophilia A after two swollen knees wants to know what the condition is, how it will change his life, whether he will need injections forever, what to do when he bleeds, and whether it will affect his future children.

Candidate instructions

You are the paediatric registrar. You have eight minutes to speak with Mrs Patel, whose fourteen-month-old son Arjun has just been diagnosed with severe haemophilia A after presenting with two swollen knees. Arjun's maternal uncle needed injections for bleeding as a child. The team is starting him on prophylaxis. Explain what haemophilia A is in plain language, what the treatment options are including the choice between factor infusions and the subcutaneous medicine emicizumab, what to do when Arjun bleeds, the warning signs that mean coming straight to hospital, and how the condition may affect his future. Answer her questions, check her understanding, and agree a plan. [1]

Paediatrics Fellowship Pro

You have read the opening of this case. The complete unit — every section and its primary-source references — is part of the Paediatrics Fellowship fellowship atlas.

References6Show ledger

[1]Srivastava A, Santagostino E, Dougall A, et al. WFH Guidelines for the Management of Hemophilia, 3rd edition. Haemophilia, 2020.PMID 32744769
[2]Mannucci PM, Tuddenham EG The hemophilias--from royal genes to gene therapy. N Engl J Med, 2001.PMID 11396445
[3]Peyvandi F, Garagiola I, Young G The past and future of haemophilia: diagnosis, treatments, and its complications. Lancet, 2016.PMID 26897598
[5]Manco-Johnson MJ, Abshire TC, Shapiro AD, et al. Prophylaxis versus episodic treatment to prevent joint disease in boys with severe hemophilia. N Engl J Med, 2007.PMID 17687129
[9]Oldenburg J, Mahlangu JN, Kim B, et al. Emicizumab Prophylaxis in Hemophilia A with Inhibitors. N Engl J Med, 2017.PMID 28691557
[11]Young G, Sidonio RF, Liesner R, et al. A multicenter, open-label phase 3 study of emicizumab prophylaxis in children with hemophilia A with inhibitors. Blood, 2019.PMID 31697801