Takeaway pearl: acute chest syndrome is the leading cause of death for patients with sickle cell disease and requires prompt recognition and management
- Sickle cell disease US data: 1 out of every 500 black children and 1 out of every 36,000 hispanic children is born with SCD
Acute Chest Syndrome
- typical presentation: some combination of fever, >2% decrease SpO2, tachypnea, increased WOB, CP, cough, wheezing, rales, and new pulmonary infiltrate on CXR
- hb often declines sharply
- causes: infection, bone marrow or fat embolism, atelectasis, pulmonary edema, intrapulmonary aggregates of sickled cells
- treatment: antibiotics, O2, pain control, fluids, simple vs exchange transfusion (based on severity)
- data is limited, but when performing exchange transfusions, expert opinion is oftentimes to target a hemoglobin S percentage <30 percent and end-hemoglobin of 10 g/dL
- Prevention: hydroxyurea: shown in trials to reduce ACS incidence by 50%
Ballas et al. Definitions of the phenotypic manifestations of sickle cell disease. Am J Hematol. 2010; 85 (1):6.
Melton CW, Haynes J. Sickle acute lung injury: role of prevention and early aggressive intervention strategies on outcome. Clin Chest Med. 2006; 27(3): 487.
National Heart, Lung, and Blood Institute. Disease and conditions index. Sickle cell anemia: who is at risk? Bethesda, MD: US Department of Health and Human Services, National Institutes of Health, National Heart, Lung, and Blood Institute; 2009. Available from:http://www.nhlbi.nih.gov/health/dci/Diseases/Sca/SCA_WhoIsAtRisk.html