Acute chest syndrome (ACS) is a cause of frequent hospitalization and death in patients with sickle cell disease (SCD). Therapies commonly used include antibiotics, inhaled B-agonists, oxygen, high-dose dexamethasone and transfusions. Although corticosteroids appear to speed resolution of ACS, the frequency of rebound vasoocclusive crises has not been adequately assessed. We conducted a retrospective cohort study to characterize risk factors for readmission after ACS. We included patients <22 years of age hospitalized at Johns Hopkins Hospital from January 1998 to April 2004 for ACS. We identified cases using the hospital’s discharge database (ICD-9 codes for ACS, pneumonia, asthma, respiratory symptoms and respiratory failure) and by reviewing dictated summaries. We defined ACS as a new pulmonary infiltrate and ≥2 of the following: chest, rib or upper abdominal pain; fever; dyspnea; tachypnea; grunting; nasal flaring; or retractions. We defined severe ACS as lethargy, marked respiratory distress, an oxygen saturation of <85% with supplemental oxygen or extensive pulmonary infiltrates. We used a respiratory clinical severity score (RCSS) wherein normal respirations=0, tachypnea for age=1 and tachypnea and retractions=2. We recorded baseline characteristics, severity of illness, treatments given, duration of hospitalization and readmissions within 14 days of discharge. We compared variables by ANOVA and calculated odds ratios for readmission using univariate and multivariate logistic regression with adjustment for clustering and robust estimates of errors. We identified 62 patients with 127 episodes of ACS (mean age 12.5 years, range 1.2 to 21.9 years); 114 had definite new pulmonary infiltrates and 13 had probable. Nineteen episodes of ACS were severe; males had more episodes (64%) than females. Patients presented with tachypnea (90%), fever (83%) and pain (78%) and received antibiotics (98%), oxygen (80%) and bronchodilators (71%). Thirty-nine episodes were treated with corticosteroids (dexamethasone in 32 and prednisone in 5) and 42 with transfusions (exchange in 12). Corticosteroids were tapered in 18. Age, clinical severity, duration of ACS and frequency of wheezing and readmission were different among treatment groups. In the multivariate analysis, readmission was associated with use of inhalers at home before presentation (OR 6.2, 95% CI 1.7–22, p<0.01), higher SBP (OR 2.0 per 10 mm Hg increase, 95% CI 1.2–3.4, p<0.01) and RCSS (OR 7.2, 95% CI 2.1–25, p<0.005) at diagnosis and treatment with corticosteroids (OR 10, 95% CI 2.1–51, p<0.005). Transfusion (OR 0.02, 95% CI 0.001–0.3, p<0.01), but not corticosteroid taper, was associated with decreased readmission. Children and young adults with SCD are often readmitted after treatment for ACS. Risk factors for readmission likely include corticosteroids, severity of ACS and asthma. Our results suggest an unacceptable frequency of readmission after corticosteroids. Transfusion, but not a taper of corticosteroids, may reduce this risk. Limitations of this study include the retrospective design and small number of patients that received a taper.

Patient Characteristics by Treatment

Supportive N=64Corticosteroids N=20Transfusion N=23Corticosteroids and Transfusion N=19P-value
Age 13±6 11±4 13±5 9±3 <0.05 
RR 24±6 31±13 34±13 36±10 <0.0001 
Involved lobes 1.2±0.4 1.4±0.6 1.7±0.8 1.7±0.8 <0.005 
Wheezing (%) 11 15 42 <0.01 
ACS (days) 3.5±1.5 3.4±1.7 4.7±2.2 5±3.9 <0.0001 
Readmission (%) 13 60 26 <0.0001 
Supportive N=64Corticosteroids N=20Transfusion N=23Corticosteroids and Transfusion N=19P-value
Age 13±6 11±4 13±5 9±3 <0.05 
RR 24±6 31±13 34±13 36±10 <0.0001 
Involved lobes 1.2±0.4 1.4±0.6 1.7±0.8 1.7±0.8 <0.005 
Wheezing (%) 11 15 42 <0.01 
ACS (days) 3.5±1.5 3.4±1.7 4.7±2.2 5±3.9 <0.0001 
Readmission (%) 13 60 26 <0.0001 

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