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Infections In Sickle Cell Disease: Overview

by Lynn Cates, M.D., F.A.A.P.
reviewed by Lewis Hsu, M.D., Ph.D.
While you may know that infections in children with sickle cell disease can be more serious than in other children, you may not be aware of some of the complex but important facts about these infections. When discussing the increased risk for infection, it is important to distinguish between sickle cell disease and sickle cell trait. In general, people with sickle cell disease have a markedly increased risk for problems with infections, while those with sickle cell trait appear to actually be at least partially protected from one serious kind of infection, malaria.

Types of infections in sickle cell disease
It is very important for parents of children with sickle cell disease to be aware that their children are at much higher risk of serious-and potentially fatal-bacterial infections than most other children. Overwhelming infections are the leading early, preventable cause of death in sickle cell disease. For the most part, children with sickle cell disease develop infections caused by the same germs that afflict other children, but they get these infections more often, become sicker faster, and are more likely to develop severe complications or even die. In children with sickle cell disease, what seems like a simple cold can turn into a much more dangerous condition very quickly. Better prevention and treatment for infections have made a huge improvement in sickle cell care in the past 15 years.

The most worrisome infections are caused by a few types of bacteria including Streptococcus pneumoniae (the pneumococcus or pneumococcal infections), Haemophilus influenzae type b (Hib), Neisseria meningitidis (the meningococcus or meningococcal disease), and Salmonella. These bacteria are capable of causing a broad spectrum of diseases, ranging from middle ear infections to invasive infections of the bloodstream (sepsis), lung (pneumonia), brain (meningitis), and bone (osteomyelitis). Because their immune systems do not work normally, children with sickle cell disease may develop sepsis or meningitis from the same bacteria that just cause ear infections in most other children.

Protecting your child with sickle cell disease from infections
There are several ways parents can help decrease the risk of infections:
  • Find comprehensive health care. Parents need to develop a close working relationship with a medical team that has experience dealing with sickle cell disease. They must be able to quickly reach healthcare providers who are knowledgeable about both sickle cell disease and their child's medical history.


  • Follow all prevention recommendations. Parents of children with sickle cell disease should see that their child receives proper preventive care such as long-term antibiotic prophylaxis (that is, antibiotics that are given routinely to head off possible infections) and immunizations.


  • Get prompt treatment. Infections can get out of control very quickly in children with sickle cell disease. Antibiotic prophylaxis and immunizations markedly decrease the chance that children with sickle cell disease will get serious infections, but they can never provide complete protection. For example, children are still susceptible to serious infections with penicillin-resistant bacteria and bacteria that are not included in their routine immunizations such as Salmonella. Therefore, even if a child receives every single dose of penicillin prophylaxis and gets all his immunizations on time, he still must be evaluated right away if it looks like he is developing an infection.
Parents should work closely with their child's healthcare provider to learn how to detect the first signs of infection, and they need to learn exactly what they should do when their child becomes ill. It is particularly important that parents know to contact their child's healthcare provider right away whenever he starts developing a fever. In general, they also should seek advice early on for worrisome nonspecific symptoms that can be caused by either infections or other complications of sickle cell disease. Such symptoms include:

  • Fever

  • Trouble breathing

  • Tiredness

  • Headache

  • Pain

  • Weakness

  • Just plain looking sick.
Again, parents seeking advice about these kinds of non-specific symptoms should make sure to tell the healthcare provider that their child has sickle cell disease, because that information can be a very important factor in choosing a course of action.

Infections in sickle cell trait
In contrast to people with sickle cell disease, people with sickle cell trait have no increased susceptibility to infections other than a slightly increased risk for urinary tract infections. They actually are at a decreased risk for severe malaria, a potentially life-threatening parasitic infection that is found in some of the same regions of the world where sickle cell disease is most prevalent. Although people with sickle cell trait can develop malaria, they are less likely to die from it.

 RELATED INFORMATION
*  Types of Infections in Sickle Cell Disease
*  Sickle Cell Disease


Created February 15, 2001
Reviewed February 19, 2001
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