Author: Erica Simon, DO, MHA (@E_M_Simon, EM Chief Resident, SAUSHEC, USAF) // Edited by: Alex Koyfman, MD (@EMHighAK, EM Attending Physician, UTSW / Parkland Memorial Hospital) and Brit Long, MD (@long_brit, EM Attending Physician, SAUSHEC, USAF)
Welcome to [email protected], an emdocs series designed to foster your working knowledge by providing an expedited review of clinical basics. We’ll keep it short, while you keep that EM brain sharp.
A 4-year old male with a history of sickle cell anemia presents to the ED for evaluation of fever of two days duration (Tmax 103.2) and progressively worsening shortness of breath. The patient’s parents report 48-hour hospitalization 6 months prior secondary to a vaso-occlusive crisis (pain localized to the extremities). ROS is negative for sick contacts. Medications include penicillin prophylaxis. Immunizations are up to date.
VS: HR 127, BP 97/64, RR 32, SpO2 91% on room air.
Neuro: GCS 15
HEENT: PERRLA, TMs clear bilaterally, nasal mucosa unremarkable, oropharynx clear and moist, no lymphadenopathy
CV: Tachycardia, cap refill 2 secs
Pulm: End-expiratory wheezing
Abdomen: ND, NT, no guarding or rebound
GU: Without findings
MSK: No TTP
Derm: No rashes
CXR demonstrates multi-lobar infiltrate.
What is the patient’s diagnosis? What’s the next step in your evaluation and treatment?
Answer: Acute Chest Syndrome (ACS)1-3
- Presentation: fever (>38.5°C or 101.3°F), tachypnea, cough, +/-chest pain, +/- wheezing and new infiltrate on CXR
- CXR, CBC, reticulocyte count, VBG, blood cultures, sputum culture2
- Consider underlying etiologies and evaluate as appropriate:
- Fat embolism
- Bronchodilators – improve peak expiratory flow2
- Incentive spirometry and analgesia – prevent development/worsening of atelectasis
- Empiric antibiotic therapy following procurement of culture samples (target Chlamydia, Mycoplasma, and Streptococcus)
- Oxygen therapy to maintain SpO2 >92%
- Fluid resuscitation targeting euvolemia (avoid iatrogenic pulmonary edema)
- Exchange transfusion (in consultation with heme/onc) for:
- Severe hypoxemia
- Multi-lobar involvement
- Worsening course
- Acute chest syndrome is the leading cause of death in sickle cell patients in the U.S. (12% mortality).2
- Infection is most common cause of ACS ( C. Pneumoniae and RSV).
- A normal pulmonary exam is most frequently associated with ACS.2
- ACS rapidly progresses to ARDS = admit to ICU level care
- Tintinalli J, Kelen G, Stapczynski J, Ma O, Cline D, et al. Tintinalli’s Emergency Medicine. 8th ed. New York: McGraw-Hill; 2016. Chapter 236, Sickle Cell Disease and Hereditary Hemolytic Anemias.
- Vichinsky E, Neumayr L, Earles A, Williams R, Lennette E, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. N Engl J Med. 2000; 342:1855-1865.
- Traill L and Barton M. Focus On: Acute Chest Syndrome – The Critical Cough. American College of Emergency Physicians Clinical and Practice Management. 2008. Available from: https://www.acep.org/Clinical—Practice-Management/Focus-On–Acute-Chest-Syndrome—The-Critical-Cough/