Counsel a family on recurrent meningococcal disease and a terminal complement deficiency — OSCE

OSCE communication and shared-planning station: explaining a likely terminal complement deficiency to a family after a child's second episode of invasive meningococcal disease, the need to confirm the defect with a CH50 and component assay before committing to a label, what meningococcal vaccination and antibiotic prophylaxis involve, the family implications of screening (including the X-linked properdin question), and the generally good prognosis with consistent prevention — while addressing fear and the instinct to dismiss recurrent meningococcaemia as 'unlucky'.

osce communication and shared decision-making2 min readVerification in progress

Prompt

The parents of a fourteen-year-old boy who has just recovered from his second episode of invasive meningococcal septicaemia in two years have been told his 'total complement' is undetectable. They are frightened he will die of the next infection, worried that something they did caused it, and confused about why he needs lifelong antibiotics when he is otherwise perfectly well. His maternal uncle died of meningococcal meningitis in childhood. Counsel them.

Candidate brief

You have eight minutes to counsel the parents of a fourteen-year-old boy in whom you suspect a terminal complement deficiency after his second episode of invasive meningococcal disease. The specific component is not yet confirmed. Use a structured, honest, empathic approach. ^[1] ^[2]

References4Show

[1]Bousfiha A, Moundir A, Tangye SG, et al. The 2022 Update of IUIS Phenotypical Classification for Human Inborn Errors of Immunity. J Clin Immunol, 2022.PMID 36198931
[2]Rauscher CK, Fajt ML, Bryk JA, et al. Clinical implications of C6 complement component deficiency. Allergy Asthma Proc, 2020.PMID 32867893
[3]Ladhani SN, Campbell H, Lucidarme J, et al. Invasive meningococcal disease in patients with complement deficiencies: a case series (2008-2017). BMC Infect Dis, 2019.PMID 31200658
[4]Walport MJ. Complement. First of two parts. N Engl J Med, 2001.PMID 11287977

Counsel a family on recurrent meningococcal disease and a terminal complement deficiency — OSCE

osce communication and shared decision-making2 min readVerification in progress

Candidate brief

References4Show

[1]Bousfiha A, Moundir A, Tangye SG, et al. The 2022 Update of IUIS Phenotypical Classification for Human Inborn Errors of Immunity. J Clin Immunol, 2022.PMID 36198931
[2]Rauscher CK, Fajt ML, Bryk JA, et al. Clinical implications of C6 complement component deficiency. Allergy Asthma Proc, 2020.PMID 32867893
[3]Ladhani SN, Campbell H, Lucidarme J, et al. Invasive meningococcal disease in patients with complement deficiencies: a case series (2008-2017). BMC Infect Dis, 2019.PMID 31200658
[4]Walport MJ. Complement. First of two parts. N Engl J Med, 2001.PMID 11287977