Fever is a common presenting symptom in many manifestations of sickle cell disease (SCD). In particular, fever is frequently the first indication of serious and life-threatening bacterial infections. It is also present in other serious SCD-associated conditions, such as acute chest syndrome or vasoocclusive crisis. 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 sickle-hemoglobin C disease (HbSC) and sickle cell-β+ thalassemia (HbS-β+ thalassemia) in a similar fashion when a predetermined level of fever develops.
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
In children with SCD, the major cause of death historically has been infection [1,2]. In the United States, prior to the availability of Haemophilus influenzae type b (H. influenzae) and pneumococcal vaccines, young children (below five years of age) with SCD 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 sickle cell disease remains significant at 0.8 to 4 percent [4,5]. (See "Overview of the management and prognosis of sickle cell disease", section on 'Introduction'.)