Acute Chest Syndrome- When Vosyn is wrong

A 18 year old African American male with history of sickle cell disease presents to the ED with severe chest pain. He is a student at the Juilliard school and was playing the clarinet -- but unfortunately the opera house they were performing in had the airconditioning break. He was sweating a lot and didn't drink much water. By the end of the show, he was exhausted and began having severe chest pain and shortness of breath. He presented to the ED where he was tachycardic to 110, tachypnea, hypertensive to 145/90 and a temp to 101.4. You ask him how he feels and says in broken sentences and in varying levels of consciousness, “palms sweaty, knees weak, arms are heavy” and his saturation is 75%. Which of the following is correct?

A. Aggressive fluid hydration is indicated in these scenarios due to risk of systemic infection

B. Acute chest syndrome is the second most common cause of death in sickle cell disease patients behind splenic rupture.

C. Atypical bacteria are the most common bacterial infection in acute chest syndrome.

D. In severe acute chest syndrome, exchange transfusions are always performed after simple blood transfusions

E. Acute chest syndrome is defined as chest pain in a patient with sickle cell disease


Field JJ, DeBaun MR. Acute chest syndrome in adults with sickle cell disease. In: UpToDate, Post, TW (Ed), UpToDate, Waltham, MA, 2018

Howard, Jo, Hart, Nicholas, Roberts-Harewood, Marilyn, Cummins, Michelle, Awogbade, Moji, Davis, Bernard; “Guideline on the management of acute chest syndrome in sickle cell disease”. BCSH Committee, 30 March 2015.

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.

Blake Briggs