Question 57: Has acute chest syndrome (ACS) occurred in the last two years?

Acute chest syndrome (ACS) is a term used to identify symptoms of chest pain, cough, fever, decreased oxygen (hypoxia), and lung infiltrates. Due to the sickling nature of red blood cells as a result of sickle cell disease, ACS may result in pulmonary infarction / emboli or viral / bacterial pneumonia. Diagnosis of ACS should be made based on clinical judgement.

Indicate if the recipient was clinically diagnosed with ACS in the last two years. If diagnosed, report Yes. If not clinically diagnosed or was diagnosed more than two years prior to the start of the preparative regimen / infusion, report No and go to question 62. If no information is available to determine if ACS was clinically diagnosed, report Unknown and go to question 62.

Questions 58 – 59: Total number of episodes within the last two years

Indicate if the total number of ACS episodes in the last two years is Known or Unknown. If Known, specify the number of episodes. If the number of ACS episodes are not known, select Unknown.

In this context, an “episode” is defined as having clinical symptoms requiring intervention (i.e., antibiotics, steroids, etc.) followed by resolution of symptoms and discontinuation of intervention. This should not be interpreted as the number of days the recipient was receiving treatment or had active symptoms. An episode may also be referred to as an “event.”

Question 60: Were red blood cell transfusions required for treatment of ACS in the last two years? (report any simple or exchanged transfusions prior to infusion)

Report Yes if RBC transfusion support was required to offset the clinical symptoms of ACS for any episode(s) within the last two years. This includes both simple and exchange transfusions. If RBC transfusions were not required in the last two years, report No. If no information is available to determine if RBC transfusion were required, report Unknown.

Question 61: Was intubation / mechanical ventilation required for treatment of ACS in the last two years?

A history of intubation and/or mechanical ventilation may impact the recipient’s pulmonary function post-HCT.

If the recipient was intubated and/or placed on mechanical ventilation due to ACS within the last two years, report Yes. If the recipient was not intubated and/or placed on mechanical ventilation within the last two years, report “No.” If no information is available to determine if the recipient was intubated and/or placed on mechanical ventilation, report Unknown.

Do not report Yes if the recipient uses a BIPAP or CPAP machine for sleep apnea.

Last modified: Jul 26, 2020

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