Management of fever in sickle cell disease
- Zora R Rogers, MD
Zora R Rogers, MD
- Professor of Pediatrics
- The University of Texas Southwestern Medical Center
- Section Editors
- Donald H Mahoney, Jr, MD
Donald H Mahoney, Jr, MD
- Section Editor — Pediatric Hematology
- Professor of Pediatrics
- Baylor College of Medicine
- Stanley L Schrier, MD
Stanley L Schrier, MD
- Editor-in-Chief — Hematology
- Section Editor — Myeloproliferative Disorders; Red Cell Disorders
- Professor of Medicine
- Stanford University School of Medicine
Fever is a common presenting symptom for many manifestations of sickle cell disease (SCD). In particular, fever is frequently the first indication of serious and life-threatening bacterial infections. While commonly discussed in pediatric care, this is a problem throughout the lifespan. Fever is also present in other serious SCD-associated conditions such as acute chest syndrome or vasoocclusive pain episodes. As a result, patients with SCD and fever should be evaluated and treated promptly to avoid significant morbidity and mortality.
Although patients with sickle cell anemia (hemoglobin SS; HbSS) and the closely related sickle cell-β0 thalassemia (HbS-β0 thalassemia) are at highest risk of bacteremia because of their predictable early loss of splenic function, many centers evaluate patients with the variant hemoglobinopathies such as sickle-hemoglobin C disease (HbSC) and sickle cell-β+ thalassemia (HbS-β+ thalassemia) in a similar fashion when a predetermined level of fever develops. Splenic dysfunction worsens with age in patients with all genotypes, and all adults with SCD and fever should be evaluated promptly according to similar guidelines.
The management of the patient with SCD and fever will be reviewed here. Overviews of the other clinical manifestations and their management in patients with SCD are presented separately. (See "Overview of the clinical manifestations of sickle cell disease" and "Overview of the management and prognosis of sickle cell disease".)
The prevention of infections is a major goal of the management of individuals with SCD, especially young children. This is discussed separately. (See "Overview of the management and prognosis of sickle cell disease", section on 'Infection prevention'.)
RATIONALE FOR MANAGEMENT APPROACH
Despite the widespread availability of pneumococcal and Haemophilus influenzae vaccines and the general use of penicillin prophylaxis, individuals with SCD and fever remain at risk for invasive infections from pneumococcus and other organisms. In children with SCD, the major cause of death historically has been infection [1,2]. Prior to the availability of H. influenzae type b and pneumococcal vaccines, young children (below five years of age) with SCD in the United States had a 13 percent risk of developing bacterial sepsis or meningitis with mortality rates of 30 and 10 percent in patients with sepsis and meningitis, respectively . Although mortality has significantly decreased since the introduction of vaccines, particularly since licensure of the conjugate pneumococcal vaccine (Prevnar) in 2000, approximately one-quarter of deaths between 1999 and 2002 in children with SCD in the first nine years of life continue to be due to infectious causes . Case series published since the introduction of the conjugate pneumococcal vaccine indicate that the prevalence of bacteremia in febrile patients with SCD remains significant at 0.8 to 4 percent [4,5]. (See "Overview of the management and prognosis of sickle cell disease", section on 'Introduction'.)
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- RATIONALE FOR MANAGEMENT APPROACH
- ACUTE MANAGEMENT
- Empiric antibiotic therapy
- Evaluation for associated conditions
- Inpatient versus outpatient management
- - Decision about admission
- - Inpatient management
- - Outpatient management
- Specific infections
- MANAGEMENT IN VARIANT SICKLE CELL SYNDROMES
- Hemoglobin SS
- Hemoglobin SC
- Sickle cell-beta thalassemia
- DISCHARGE INSTRUCTIONS
- SUMMARY AND RECOMMENDATIONS