Improving care for children with sickle cell disease/acute chest syndrome

Elizabeth A. Crabtree, M. Michele Mariscalco, Joy Hesselgrave, Suzanne F. Iniguez, Tanya J. Hilliard, Julie P. Katkin, Kathy McCarthy, Mireya Paulina Velasquez, Gladstone Airewele, Marilyn J. Hockenberry

Research output: Contribution to journalArticlepeer-review


BACKGROUND: Acute chest syndrome (ACS) is a leading cause of hospitalization and death of children with sickle cell disease (SCD). An evidence-based ACS/SCD guideline was established to standardize care throughout the institution in February 2008. However, by the summer of 2009 use of the guideline was inconsistent, and did not seem to have an impact on length of stay. As a result, an implementation program was developed. OBJECTIVE: This quality-improvement project evaluated the influence of the development and implementation of a clinical practice guideline for children with SCD with ACS or at risk for ACS on clinical outcomes. METHODS: Clinical outcomes of 139 patients with SCD were evaluated before and after the development of the implementation program. Outcomes included average length of stay, number of exchange transfusions, average cost per SCD admission, and documentation of the clinical respiratory score and pulmonary interventions. RESULTS: Average length of stay decreased from 5.8 days before implementation of the guideline to 4.1 days after implementation (P = .033). No patients required an exchange transfusion. Average cost per SCD admission decreased from $30 359 before guideline implementation to $22 368. Documentation of the clinical respiratory score increased from 31.0% before implementation to 75.5%, which is an improvement of 44.5% (P<.001). Documentation of incentive spirometry and positive expiratory pressure increased from 23.3% before implementation to 50.4%, which is an improvement of 27.1% (P < .001). CONCLUSIONS: Implementation of a guideline for children with SCD with ACS or at risk for ACS improved outcomes for patients with SCD.

Original languageEnglish (US)
Pages (from-to)e480-e488
Issue number2
StatePublished - Feb 2011
Externally publishedYes


  • Acute chest syndrome
  • Guideline development
  • Implementation
  • Quality improvement
  • Sickle cell disease

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health


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