Fever is one of the most common symptoms that people with SCD report.
People living with sickle cell disease, in particular, sickle cell anaemia have a staggeringly high risk to severe bacterial infection. It is due to a reduced or often absent splenic function.
Impairment of the spleen begins as early as 2-3months old in a few infants to coincide with the period when their fetal haemoglobin starts declining. It leads to an increased risk of meningitis and septicemia, and the most offending pathogen being Streptococcus pneumoniae.
It is worth noting that before the advent of pneumococcal vaccination and penicillin prophylaxis, severe pneumonia had been nearly, always fatal.
Any febrile illness in a person with sickle cell disease should be considered an emergency for two reasons. First is the uncertainty of complete immunization, secondly, the possibility of penicillin-resistant organisms.
Because a fever is a rather unspecific symptom, even among patients living with sickle cell disease, the panel of experts derived the following recommendations in regards to the management of fever in sickle cell disease.
- Immediately evaluate with history and physical examination, complete blood count with differential, reticulocyte count, blood culture, and urine culture when appropriate, in any person with SCD who presents with a temperature ≥38.5°C (101.3°F).
- For children with SCD and a temperature ≥38.5°C (101.3°F), administer empiric parenteral antibiotics in accord with the national clinical guidelines. They should provide coverage against Streptococcus pneumoniae and gram-negative enteric organisms. In those that do not appear to be ill, outpatient management with an oral antibiotic is an acceptable approach.
- Hospitalize people with SCD and a temperature ≥39.5°C (103.1°F) and who appear ill for close observation and intravenous antibiotic therapy.
- In people with SCD whose febrile illness is accompanied by shortness of breath, tachypnea, cough, with or without rales, obtain an immediate x-ray to investigate for acute chest syndrome and manage accordingly.
- Lastly, include bacterial osteomyelitis as a differential, in febrile people with SCD who have localized or multifocal bone tenderness: manage accordingly.
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