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CE: Understanding the Complications of Sickle Cell Disease
Paula Tanabe1, Regena Spratling, Dana Smith
1Paula Tanabe is a professor in the Schools of Nursing and Medicine and associate dean for research development and data science at Duke University, Durham, NC. Regena Spratling is the associate dean and chief academic officer for nursing in the Byrdine F. Lewis College of Nursing and Health Professions, Georgia State University, Atlanta. Dana Smith is a clinical nurse II in the ICU, and Peyton Grissom is the clinical team lead on a general medicine step-down unit, both at Duke University Hospital, Durham, NC. Mary Hulihan is a health scientist in the Division of Blood Disorders, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta. Contact author: Mary Hulihan, ibx5@cdc.gov. The authors and planners have disclosed no potential conflicts of interest, financial or otherwise.
Insights
Sickle cell disease (SCD) impacts red blood cells, leading to high hospitalization and mortality rates despite improved survival. Nurses can improve patient outcomes by understanding SCD complications and educating families.
Area of Science:
- Genetics
- Hematology
- Pediatrics
Background:
- Sickle cell disease (SCD) is a genetic disorder affecting hemoglobin, causing red blood cell abnormalities.
- While pediatric survival has improved, high rates of hospitalization, emergency department (ED) use, and mortality persist.
- Adult patients with SCD experience even higher healthcare utilization and a significantly lower median age at death.
Purpose of the Study:
- To provide a comprehensive overview of SCD for healthcare professionals, particularly nurses.
- To highlight the major complications associated with SCD.
- To outline nursing implications and provide educational resources for patients and caregivers.
Main Methods:
- Review of the genetic, hematologic, and clinical features of SCD.
- Description of major SCD-associated health complications.
- Discussion of nursing interventions and educational strategies.
Main Results:
- SCD is characterized by altered hemoglobin, leading to diverse clinical manifestations.
- Key complications include vaso-occlusion, acute chest syndrome, stroke, and organ damage.
- Nurses play a crucial role in managing complications and supporting patients/caregivers.
Conclusions:
- Nurses are pivotal in improving health outcomes and quality of life for individuals with SCD.
- Early recognition of complications and proactive patient education are essential.
- Accessible resources can empower clinicians, patients, and caregivers in managing SCD.
Abstract:
: Sickle cell disease (SCD) is an autosomal recessive genetic condition that alters the shape and function of the hemoglobin molecule in red blood cells. While the overall survival rate among children with SCD has improved in recent years, pediatric rates of hospitalization, ED use, and mortality from complications of SCD remain high. Among patients ages 18 and older, hospital admission and ED usage are even greater-and the median age at death of people with SCD is considerably lower than that of the general population. Nurses who care for patients with SCD have an opportunity to improve health outcomes and quality of life for these patients by recognizing the major SCD-associated complications and providing patients and their caregivers with appropriate educational information. The authors discuss the genetic, hematologic, and clinical features of SCD and describe the major associated health complications. In addition, they review the nursing implications of each complication and provide online links to resources for clinicians, patients, and caregivers.
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