FACIAL SWELLING, RESPIRATORY FAILURE, FLUCTUATING COMPLEMENT 4 LEVELS, AND RELIGIOUS CONSIDERATION IN A PEDIATRIC PATIENT

Abstract

Listen

Translate article

<h3>Introduction</h3> Hereditary Angioedema is a rare condition defined by acute, recurring episodes of edema, with the upper airway being particularly dangerous. HAE Type 2 is caused by the dysfunction of the C1-esterase inhibitor with low C4 levels. <h3>Case Description</h3> A 10-year-old female presented with facial and airway swelling unresponsive to steroids, antihistamines, and epinephrine that required intubation. C4 complement was low but then was normal prior to treatment. The patient's religion did not allow for plasma products, but a plasma derived C1 esterase inhibitor product was given emergently with improvement in her swelling. C1 Esterase Inhibitor Function was found to be low. A non-formulary recombinant C1 esterase inhibitor product was arranged for hospital and later home use. At 11 years she was admitted in respiratory failure. She decompensated with failed attempts at intubation and required cricothyrotomy. A recombinant C1 esterase inhibitor product was given. She remained with tracheostomy for 7 days then was de-cannulated. She was discharged with an oral plasma Kallikrein inhibitor, emergency recombinant C1 esterase inhibitor product, and a HAE emergency card. In addition to formulary HAE treatment, a recombinant product was placed in restricted use. A best practice advisory was developed in the EMR to advise physicians of the patient's HAE status. <h3>Discussion</h3> Although HAE Type 2 is well characterized, this is a rare report of a Pediatric patient with HAE requiring intubation on initial presentation and later cricothyrotomy. The challenge was her respiratory failure as a presenting sign of HAE, fluctuating C4 levels, religious consideration, and implementation of an EMR advisory.