THE UNIQUE ROLE IMMUNOLOGISTS PLAY IN NAVIGATING THE CARE OF CRITICALLY ILL PEDIATRIC PATIENTS

Abstract

<h3>Introduction</h3> Immunologists can play a critical role in navigating prognostic determinations and thus, treatment choices in patient populations with cultural/religious beliefs that diverge from recommended aggressive medical care. <h3>Case Description</h3> An 18-month-old Amish female presented with fever, respiratory distress, hypoxemia and diffuse lymphadenopathy. Chest CT revealed diffuse lymphadenopathy with resultant occlusion of her left mainstem bronchus. Bronchoscopy was performed after which she had respiratory failure requiring extracorporeal membrane oxygenation (ECMO) support. Bronchoalveolar lavage was positive for Blastomyces antigen, respiratory culture positive for <i>Streptococcus anginosus</i> and serum positive for EBV. Flow cytometry showed normal levels of T cells, naïve T cells and B cells but absent natural killer (NK) cells. She was treated for pulmonary blastomycosis and superimposed bacterial pneumonia with excellent clinical improvement. Flow cytometry subsequently repeated with improving but low NK cells (1%). <h3>Discussion</h3> Immunology was consulted to help determine if an underlying immunodeficiency placed her at increased risk for such a severe infection and prognosis given need for ECMO. NK cells are part of the innate immune defense against infection. Reassuringly, she initially had absent NK cells while she was critically ill and being treated with systemic corticosteroids and her levels improved alongside her clinical status. The rest of her immune work-up was also reassuring including newborn screen (including wild-type RMRP/RAG1), immunoglobulin levels, complement studies and dihydrorhodamine test.This case highlights the importance of specialized immunology expertise to assist in discussions with families and providers regarding complicated disease courses in the context of cultural/religious preferences and ethical dilemmas.