Explaining a primary immunodeficiency diagnosis and immunoglobulin replacement — OSCE

Candidate instructions (8-minute station)

You are the paediatric registrar in the general paediatric clinic. An eight-year-old girl has been referred for recurrent otitis media, sinusitis and pneumonia, with two hospital admissions in the past year and new crackles and wheeze suggesting early bronchiectasis. Her blood tests show low IgG and IgA and a poor response to a pneumococcal vaccine challenge, and the immunology team has confirmed a diagnosis of common variable immunodeficiency — a primary antibody deficiency. [7]

Your tasks are: [1]

1. Explain the diagnosis of common variable immunodeficiency in language the family can understand. [7]
2. Explain why immunoglobulin replacement therapy is needed, what it involves practically, and what it will and will not achieve. [7]
3. Cover the infection-prevention and vaccination strategy, including which vaccines are safe and which need coordination with immunology. [5]
4. Address the genetic counselling, the school and transition plan, and the father's belief that the recurrent infections were "just a phase". [1]

You should name the immunology team as the long-term lead for her care and explain what ongoing surveillance will involve. [5]