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Updated: Jun 5, 2026

Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
Published on: March 14, 2017
Improving care for children with sickle cell disease/acute chest syndrome
Elizabeth A Crabtree1, M Michele Mariscalco, Joy Hesselgrave
1Evidence Based Outcomes Center, Texas Children’s Hospital, Houston, Texas 77030, USA. eacrabtr@texaschildrens.org
Insights
Implementing a clinical practice guideline for children with sickle cell disease (SCD) and acute chest syndrome (ACS) significantly improved patient outcomes. The guideline led to shorter hospital stays and reduced costs for pediatric SCD patients.
Area of Science:
- Pediatric Hematology
- Clinical Quality Improvement
- Evidence-Based Medicine
Background:
- Acute Chest Syndrome (ACS) is a major cause of mortality and hospitalization in children with Sickle Cell Disease (SCD).
- An evidence-based guideline was established in 2008 to standardize ACS/SCD care, but its use became inconsistent by 2009.
- An implementation program was developed to address inconsistent guideline adherence and improve clinical outcomes.
Purpose of the Study:
- To evaluate the impact of a clinical practice guideline for pediatric patients with SCD and ACS on clinical outcomes.
- To assess the effectiveness of a developed implementation program for the ACS/SCD guideline.
Main Methods:
- A quality-improvement project evaluated 139 pediatric patients with SCD before and after the implementation program.
- Key outcomes measured included average length of stay, exchange transfusion rates, cost per admission, and documentation of respiratory scores and pulmonary interventions.
Main Results:
- Average length of stay decreased from 5.8 to 4.1 days (P = .033).
- No patients required exchange transfusions post-implementation.
- Average cost per admission decreased from $30,359 to $22,368.
- Documentation of clinical respiratory score increased by 44.5% (P < .001), and pulmonary interventions increased by 27.1% (P < .001).
Conclusions:
- The implementation of the ACS/SCD clinical practice guideline significantly improved clinical outcomes for pediatric patients with SCD.
- The quality improvement initiative demonstrated success in standardizing care and enhancing patient management for ACS in children with SCD.
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.
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