PEARL: Any patient that in your Emergency Department with a sickle cell disease (SCD)-related diagnosis requires incentive spirometry and frequent monitoring for acute chest syndrome (ACS).
BRIEF WHY: ACS is the most common cause of hospitalization and death in patients with SCD.1,2
Nearly half of all patients who develop ACS are admitted for diagnoses other than ACS. Of those not admitted with ACS, radiographic and clinical findings of ACS appeared a mean of 2.5 days after admission.2 It is because of this that all patients with SCD related diagnoses at presentation, must be treated as though they are in the prodrome stage of ACS, and all require incentive spirometry to reduce the risk of progression to ACS.2
More to come...
References
Platt OS, Brambilla DJ, Rosse WF, Milner PF, Castro O, Steinberg MH, Klug PP. Mortality In Sickle Cell Disease -- Life Expectancy and Risk Factors for Early Death. N Engl J Med 1994 330: 1639-1644
Vichinsky EP, Neumayr LD, Earles AN, Williams R, Lennette ET, Dean D, Nickerson B, Orringer E, McKie Vl, Bellevue R, Daeschner C, Manci EA, Abboud M, Moncino M, Ballas S, Ware R. The National Acute Chest Syndrome Study Group. Causes and Outcomes of the Acute Chest Syndrome in Sickle Cell Disease. N Engl J Med. 2000 342: 1855-1865
