Paeds Cases · haematology-oncology-and-transfusion
Explain hereditary spherocytosis and the splenectomy decision to a parent — OSCE
OSCE communication and shared-planning station: explaining the diagnosis of hereditary spherocytosis to a parent, the role of folic acid, the meaning of the spherocytes and the splenic destruction, the decision about splenectomy and its timing, the pre-splenectomy vaccination bundle and lifelong antibiotic prophylaxis, and the warning signs of the aplastic crisis and post-splenectomy infection.
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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]
References4ShowHide
- [1]Perrotta S, Gallagher PG, Mohandas N Hereditary spherocytosis. Lancet, 2008.PMID 18940465
- [2]Bolton-Maggs PH, Langer JC, Iolascon A, et al. Guidelines for the diagnosis and management of hereditary spherocytosis--2011 update. Br J Haematol, 2012.PMID 22055020
- [3]Liu Y, Jin S, Xu R, et al. Hereditary spherocytosis before and after splenectomy and risk of hospitalization for infection. Pediatr Res, 2023.PMID 35915237
- [4]Christensen RD, Yaish HM, Gallagher PG A pediatrician's practical guide to diagnosing and treating hereditary spherocytosis in neonates. Pediatrics, 2015.PMID 26009624