Acute Chest Syndrome (Sickle Cell Crisis)

Key Points:

--Acute chest syndrome (chest pain, hypoxia, decreasing Hb levels, multi-lobar pneumonia on CXR) is the leading cause of death amongst patients with sickle cell disease.

--Vichinsky et al.: 30 center study, 671 episodes of the acute chest syndrome in 538 patients with sickle cell disease

• 72% of patients were initially admitted with another diagnosis (e.g. pain), then diagnosed with acute chest syndrome 2.5 days later (on average). 
• Most common symptoms:
• fever (80%)
• cough (62%)
• chest pain (44%)
• tachypnea (45%)
• SOB (41%)
• extremity pain (37%)
• abdominal pain (35%)
• rib/sternal pain (21%)
• Mortality mostly due to respiratory failure from pulmonary emboli (marrow, fat, or thrombosis) and pneumonia.

Treatment:
--broad antibiotics (including a macrolide)
--bronchodilators (assume airway hyperreactivitiy even if no wheezing)
--early transfusions for patients at high risk of complications.

10-SECOND TAKEAWAY:
--Sickle Cell + cardio/pulm complaints: think about Acute Chest Syndrome
--Symptoms: non-specific
--PE's and pneumonias suck
--Treatment Toolbox: antibiotics, bronchodilators, transfusions

Reference(s): Vichinsky EP, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med. 2000 Jun 22;342(25):1855-65.

Submitted by T. Boyd.