Abstract
Background: Sickle cell disease (SCD) is the most common genetic non-cancer blood disorder (American Society of Hematology, 2021). Many complications can occur with SCD, including acute chest syndrome (ACS), the most common cause of mortality, the second most common cause of morbidity, and the second most common cause of hospitalization in patients with SCD (Brewin & Howard, 2017). In addition, the development of ACS can lead to increased length of hospital stay, increased episodes of ACS, worse long-term health outcomes, and decreased patient satisfaction.|Purpose: This quality improvement project aimed to develop and implement an evidence-based Sickle Cell Disease Stay Healthy Bundle for pediatric patients with SCD admitted to the pediatric inpatient setting to facilitate the prevention and early detection of ACS.|Methods: This project took place on a 14-bed general pediatric and pediatric hematology/oncology unit at a Midwest children’s hospital during an eight-week study period. The bundle included orders and guidelines related to ambulation, up-to-chair for meals, incentive spirometry use, pain management, assessment and scoring utilizing the Respiratory Assessment Score, and decision making based on SCD Stay Healthy Guidelines (Ahmad et al., 2011). In addition, education was provided to the nurses about ACS and the SCD Stay Healthy Bundle before implementation.|Evaluation: Data analysis was completed, including demographic information, use of the SCD order set, compliance with the bundle, and whether patients developed ACS during their hospitalization. Six patients with SCD were admitted during the study period, with one patient developing ACS. While nursing compliance with the bundle was poor, significant improvements in nursing knowledge and understanding of ACS were noted.|Implications for clinical practice: Continued education about hematologic and oncologic emergencies will significantly improve the quality of care provided to pediatric hematology and oncology patients.