A few core measures prevent most early deaths in children with sickle cell disease, and they are easy to overlook when a child presents acutely.
Treat fever as an emergency: a child with sickle cell disease and a temperature needs urgent assessment and prompt broad-spectrum antibiotics because of the risk of overwhelming pneumococcal sepsis from functional asplenia. Ensure penicillin prophylaxis in early childhood and full pneumococcal and other vaccinations. Start and maintain hydroxyurea to reduce pain crises and acute chest syndrome, and teach families to recognise acute chest syndrome, splenic sequestration and stroke. Keep children well hydrated and manage pain adequately during crises.
The habit worth keeping is to have a standing plan for the febrile child with sickle cell disease, so that antibiotics are not delayed while the diagnosis is reconsidered.
- Treat fever in a child with sickle cell disease as an emergency with prompt antibiotics.
- Maintain penicillin prophylaxis in early childhood and complete pneumococcal vaccination.
- Use hydroxyurea to reduce pain crises and acute chest syndrome.
- Teach families to recognise acute chest syndrome, splenic sequestration and stroke.
- Ensure good hydration and adequate analgesia during crises.
Why it matters
Functional asplenia makes a simple fever potentially fatal, and the protective measures are cheap and well established.
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