Explain hereditary spherocytosis and the splenectomy decision to a parent — OSCE

Candidate instructions

You are the paediatric registrar. You have five minutes to speak with Mrs Chen, whose seven-year-old daughter Lily has just been diagnosed with hereditary spherocytosis after presenting with fatigue, pallor and an enlarged spleen. Mrs Chen had a splenectomy herself as a child and knows the condition runs in the family. The team is considering a splenectomy for Lily. Explain what hereditary spherocytosis is in plain language, what the treatment options are, what removing the spleen would mean for Lily including its benefits and risks, and what they need to watch for in the future. Answer her questions, check her understanding, and agree a plan. [1]

Actor brief (parent — Mrs Chen)

You are anxious but informed, because you had the same condition and had your spleen removed as a child. You want to know: (1) Is Lily going to have the same problems you had, and is it your fault she inherited it? (2) Does she really need the operation — you managed without major problems, so can she wait? (3) What are the risks of removing the spleen — you remember being told you have to be careful about infections? (4) What do you do if she gets sick? (5) Will this affect her having children one day? Push back if the candidate uses jargon, dismisses your experience, or rushes the decision. You settle when you feel the candidate respects your knowledge and gives you honest, balanced information. [1]

Exemplar candidate approach

Open and acknowledge. "Mrs Chen, thank you for coming in. I know this is a condition close to your own experience, and that gives us a real head start in looking after Lily. I want to explain clearly what hereditary spherocytosis is, what the options are for Lily, and what removing the spleen would mean — the benefits and the risks — so that together we can make the right plan for her." [1]

Explain the diagnosis in plain language. "Hereditary spherocytosis is an inherited condition where the red blood cells, which carry oxygen around the body, are shaped slightly differently — they are small and round instead of the usual doughnut shape. Because of that shape, they get caught and broken down in the spleen, an organ in the tummy that normally filters old blood cells. That is why Lily has been tired and pale and yellow, and why her spleen is enlarged. It is not your fault — it is inherited, and because you carry it too, there was a fifty-fifty chance Lily would have it. Having it does not mean it is more severe or less severe than yours was." [1] [2]

Explain the treatment options and the splenectomy decision. "There are two parts to the treatment. The first is a vitamin called folic acid, which Lily takes every day to help her body keep making new red blood cells. The second is the bigger decision: whether to remove the spleen. Removing the spleen stops the red cells from being broken down, so the anaemia goes away and Lily will feel much better. The operation is done with keyhole surgery and most children recover quickly. The reason we consider it is that Lily has moderate disease — her blood count is quite low and she is symptomatic. We would not rush it; we like to wait until a child is at least six, and Lily is seven, so the timing is right if we decide to go ahead. Whether we do total or partial removal is something we can discuss, but most surgeons now favour total removal for the best long-term outcome." [2]

Explain the risks of splenectomy honestly. "The spleen has an important job beyond filtering blood cells — it helps fight certain infections, particularly from bacteria that have a capsule around them. Without a spleen, Lily would be at higher risk of a serious infection from those bacteria, and that risk is lifelong. We reduce that risk in three ways. First, before the operation we give Lily vaccinations against those bacteria — pneumococcus, meningococcus and Haemophilus influenzae. Second, after the operation she takes a low dose of an antibiotic every day, for life, as a safety net. Third, and most importantly, if she ever gets a fever we treat it as an emergency — she comes straight to hospital for stronger antibiotics through a drip. With those precautions in place, most children who have had their spleen removed live completely full and active lives." [3]

Address the inheritance and future children question. "You asked whether this will affect Lily having children one day. Hereditary spherocytosis does not affect fertility or the ability to have children. Because it is inherited, each of Lily's future children would have a fifty-fifty chance of having the condition, and we would screen them at birth. It is entirely manageable, and many people with hereditary spherocytosis have healthy families. When Lily is older we will make sure she understands the condition and the inheritance pattern." [1] [4]

Check understanding and agree a plan. "Can I check — what is your biggest concern right now, and has anything I have said been unclear? Here is what I suggest as a plan: Lily starts folic acid today, we arrange the vaccination course over the next few weeks, and we book the splenectomy with the surgeon once the vaccinations are complete. I will give you a written summary and a card that explains what to do if Lily ever has a fever. You know this condition better than most, and I want to make sure you feel confident about every step. Does that sound right to you?" [2] [3]

Mark scheme (10 marks)

• Empathy and relationship (2): acknowledges the parent's own experience, respects her knowledge, plain language, does not rush. [1]
• Explains the diagnosis clearly (2): hereditary spherocytosis as inherited, spherocytes trapped in the spleen, not the parent's fault. [1] [2]
• Explains the splenectomy decision with balanced benefits and risks (2): folic acid as baseline, splenectomy curative for the haemolysis, timing at six years, and the infection risk. [2] [3]
• Explains the precautions bundle honestly (2): pre-splenectomy vaccination, lifelong antibiotic prophylaxis, and the febrile-illness action plan. [3]
• Addresses the inheritance question and agrees a plan (2): fifty-fifty inheritance, no effect on fertility, checks understanding, sets out next steps. [1] [4]