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

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]

Actor brief (adolescent — Mia)

You are fourteen and fed up with heavy periods that have made you anaemic and miss school, and you are worried about what this diagnosis means. You want to know: (1) Is it serious, and will it get worse? (2) Why do I bruise and bleed so much when my friends don't? (3) What can I take for my periods, and can I still play sport? (4) When do I actually need to come to hospital? (5) Will I pass it on to my own children one day? You engage better when the candidate speaks to you directly, uses plain language, and respects that you are old enough to understand your own condition. You push back gently if the candidate talks only to your mother, uses jargon, or dismisses the impact on your school and sport. You settle when you feel the candidate has given you honest, practical answers and a clear plan. [1]

Actor brief (parent — Mrs Chen)

You are worried but supportive. You knew Mia bruised easily but did not connect it with her periods. You want to know: (1) Is it your fault she has it, and does it run in families? (2) Is there a cure? (3) What do you do if she has a bad nose-bleed or a heavy period at home? (4) Does she need to avoid anything, like certain pain medicines or contact sport? (5) Will it affect her having children? Push back gently if the candidate minimises the bleeding, does not check understanding, or does not give you a written plan. [1]

Exemplar candidate approach

Open and acknowledge. "Mia, Mrs Chen, thank you for coming in. I know the heavy periods and the bruising have been really disrupting things for you, Mia, especially at school and sport, and I want to explain clearly what we have found, what it means, and how we will manage it together so that you can get on with your life. I will talk mostly to you, Mia, because it is your condition, and Mrs Chen, please jump in at any point." [1]

Explain the diagnosis and cause in plain language. "Von Willebrand disease is the most common inherited bleeding condition. In your blood there is a protein called von Willebrand factor that has two jobs: it helps your platelets stick together to form a plug when a vessel is damaged, and it carries another clotting protein called factor eight so it does not get broken down. You have a mild form called type 1, which means you have less of this protein than most people, about three quarters of people with the condition have this type. That is why your nose-bleeds last longer, you bruise easily, and your periods are heavy. It is not your fault and it is not anything you did. It runs in families and is usually passed down from a parent, so it is quite likely one of your parents carries it too. It is not dangerous in the way some bleeding conditions are, and it does not get worse over time, but it does explain what you have been experiencing." [1] [5]

Explain the treatment options honestly. "The good news is that for your type we have several treatments that work well. The first is a medicine called desmopressin, which is given through a drip over half an hour and tells your body to release its stored von Willebrand factor, usually raising it two to four times. We do a test dose first to check you respond well. The second is a tablet called tranexamic acid that you take during your period or with dental work, which helps clots hold together, especially at surfaces like the nose, gums and womb lining. For your periods we can also use the contraceptive pill or a hormone coil to lighten the bleeding, and iron to treat your anaemia. Very few people with your type ever need a blood-clotting concentrate, and that is held in reserve only for surgery or a serious bleed." [4] [10]

Explain the period plan and emergency signs. "For your periods specifically, here is what I suggest: tranexamic acid during your period to reduce the flow, the contraceptive pill or a hormone coil to make the periods lighter and more regular, and iron to build your stores back up. Keep a note of how heavy they are so we can adjust. The signs that mean coming straight to hospital are: a nose-bleed that does not stop after twenty minutes of firm pressure, a period so heavy you are soaking through pads quickly or feeling dizzy, any bleeding after a fall or injury that does not stop, and a bad headache after a head injury. For the everyday nose-bleed, pinch the soft part of your nose, lean forward, and hold for ten to fifteen minutes. We will give you a written plan and a card that tells any doctor you have von Willebrand disease." [2] [4]

Address sport, lifestyle and future children. "You can absolutely keep playing sport. We will guide you on which ones are safest, but most activities are fine. Two things to avoid: pain medicines like ibuprofen and aspirin, because they interfere with platelets and make bleeding worse, so use paracetamol instead; and we plan any dental work or surgery ahead of time so we can cover you. As for your own children one day: because it is usually passed down from a parent, each of your future children would have a chance of inheriting it, but it is a mild condition that we manage very well, and many people with it have healthy families. When you are older we will make sure you understand the inheritance fully, and we will plan any pregnancy carefully because your von Willebrand factor naturally rises in pregnancy." [10] [1]

Check understanding and agree a plan. "Can I check, Mia, what is your biggest worry right now, and has anything I have said been unclear? Here is the plan: we will do your desmopressin test at the next visit, we start tranexamic acid and iron for your periods now, and we arrange the pill or coil with the gynaecology team if you would like. I will give you a written bleed plan and a medic alert card before you go, and we will see you regularly in the bleeding-disorder clinic with a team that includes nursing and social work. Does that sound right to both of you?" [2] [10]

Mark scheme (10 marks)

• Empathy and relationship, speaking to the adolescent (2): acknowledges the disruption to school and sport, addresses Mia directly, respects her autonomy, plain language. [1]
• Explains the diagnosis and cause clearly (2): von Willebrand factor and its two jobs, type 1 as the common mild form, inherited and not the patient's fault. [1] [5]
• Explains the treatment options with balanced pros and cons (2): desmopressin with a test dose and the expected rise, tranexamic acid, hormonal control of periods and iron; concentrate held in reserve. [4] [10]
• Explains the period plan and emergency signs honestly (2): tranexamic acid, pill or coil and iron for the periods, and the warning signs that mean coming straight to hospital including uncontrolled epistaxis and head injury. [2] [4]
• Addresses lifestyle and future children and agrees a plan (2): continued sport with guidance, avoidance of ibuprofen and aspirin with paracetamol preferred, inheritance and pregnancy planning, checks understanding, provides a written plan and medic alert card. [10] [1]