From the Guidelines
Sickle cell anemia significantly weakens the immune system, making patients more susceptible to infections that require specific preventive measures. The disease compromises immunity through several mechanisms: the spleen often becomes damaged or non-functional (functional asplenia), reducing the body's ability to filter bacteria from the bloodstream; white blood cell function may be impaired; and patients frequently experience poor circulation that allows infections to develop more easily. To prevent infections, patients should receive all recommended vaccinations, including pneumococcal, meningococcal, Haemophilus influenzae type b, and annual flu vaccines, as supported by the guidelines for immunization 1. Children with sickle cell disease typically require daily prophylactic antibiotics, usually penicillin (125mg twice daily for children under 3, 250mg twice daily for older children) until at least age 5, as recommended by studies on preventing pneumococcal disease 1. Good hygiene practices are essential, including:
- Regular handwashing
- Avoiding sick contacts
- Prompt cleaning of cuts or wounds Patients should seek immediate medical attention for any fever (temperature above 101.3°F/38.5°C), as this could indicate a serious infection requiring urgent treatment, and staying well-hydrated and avoiding extreme temperatures also helps prevent sickle cell crises that could further compromise immunity 1. These precautions are critical because infections remain a leading cause of illness and death in people with sickle cell anemia.
From the Research
Sickle Cell Anemia and the Immune System
- Sickle cell anemia affects the immune system by increasing the risk of infections, particularly those caused by Streptococcus pneumoniae 2, 3, 4.
- The increased risk of infection is due to splenic dysfunction, which is a major contributor to the immune system's inability to fight off infections 2.
- Abnormalities in immunologic defense mechanisms, including synthesis of polyclonal IgG and IgM, the alternative complement pathway, opsonic activity, and T and B cell interaction, may also enhance the risk of infection 2.
Precautions to Prevent Infections
- Prophylactic antibiotics, such as penicillin, may be effective in reducing the incidence of pneumococcal disease in children with sickle cell anemia 3, 5, 4.
- Vaccination against pneumococcal disease is also recommended, although the effectiveness of current vaccines in young patients with sickle cell anemia is modest 2, 6.
- Early institution of antibiotic therapy for febrile episodes and antimicrobial prophylaxis may also be effective in reducing the incidence of pneumococcal disease 4.
- Specific guidelines for infection prevention, including the use of prophylactic antibiotics and vaccination, should be followed to reduce the risk of infection in individuals with sickle cell anemia 4.
Infection Risk in Adults with Sickle Cell Anemia
- Adults with sickle cell disease are also at risk of pneumococcal sepsis and meningitis, although this is less common than in children 6.
- The use of the 23-valent polysaccharide vaccine is recommended in adults with sickle cell disease to reduce the risk of pneumococcal disease 6.
- High fever, generalized weakness, and neck stiffness are common symptoms of pneumococcal sepsis and meningitis in adults with sickle cell disease, and prompt medical attention is necessary to prevent complications and mortality 6.