This case is written by Dr. Carla Angelski. She has completed both a PEM fellowship at Dalhousie and a MEd in Health Sciences Education. She now works in the Pediatric Emergency Department at the Royal University Hospital in Saskatchewan and is intimately involved in the delivery of high-fidelity simulation at the their sim centre. She is currently working on a curriculum to deliver in-situ simulation for ongoing faculty CME within the division and department.
Why it Matters
Patients with sickle cell disease are subject to a host of crises that can be difficult to manage. This case highlights the unique management of acute chest syndrome. In particular:
- Recognition of acute chest syndrome as a possibility in the sickle cell patient with respiratory distress
- Judicious use of fluids in patients with possible acute chest syndrome
- The possible need for exchange transfusion in patients with severe acute chest syndrome
Clinical Vignette
You are working the day shift at a tertiary children’s hospital. A mother brings in her son, James, a four-year old boy with known sickle cell disease (HbSS). She is concerned since he’s had low energy and a cough for two days. Now he’s had a fever since this afternoon.
Case Summary
A 4-year-old boy with known sick cell disease presents with two days of cough and a one afternoon of fever. The patient is initially saturating at 88%, looks unwell and is in moderate-severe distress. During the case, the patient’s oxygenation with drop and the emergency team is expected to provide airway support. They will also need to pick appropriate induction agents for intubation. The case will end with ICU admission. During the case, the mother will also be challenging/questioning the team until a team member is delegated to help keep the mother calm.
Download the case here: Acute Chest Syndrome
CXR for the case found here:
(CXR source: http://reference.medscape.com/features/slideshow/sickle-cell#8)
Post-intubation CXR for the case found here:
(CXR source: http://www.swjpcc.com/critical-care/?currentPage=4)
EM Sim Cases 