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| |    Types of Infections in Sickle Cell Disease by Lynn Cates, M.D., F.A.A.P. reviewed by Lewis Hsu, M.D., Ph.D. Although it's true that any signs of infection need to be taken seriously in a child with sickle cell disease, certain kinds of bacterial infections are known to be particularly serious, with the potential to cause long-term problems or even be fatal. Understanding why these infections are so serious and what parents can do to recognize--or help prevent--them is an important step toward insuring a better life for children living with this disease. Encapsulated bacteria The types of infections that are most common--and most worrisome--in children with sickle cell disease are caused by bacteria with an outer coating that protects them from the body's immune system. These are known as encapsulated bacteria and include the pneumococcus, Haemophilus influenzae type b (Hib), and the meningococcus. These infections are especially serious because they are capable of spreading throughout the bloodstream (sepsis) and causing shock, which can be fatal within as little as four hours from the start of fever for a child with sickle cell disease. They also can invade vital organs such as the lungs or the brain, causing pneumonia or meningitis, respectively. Children who don't have sickle cell disease can develop infections with these encapsulated bacteria, but they get them less often than children with sickle cell disease do. In addition, although these infections are always considered very serious in any child, they are not as likely to cause permanent damage or death in most children who do not have sickle cell disease. Encapsulated organisms like the pneumococcus normally inhabit certain areas of the body--the nose in particular. Ordinarily they cause no problem, but when a respiratory viral infection such as a cold or the flu comes along, it can weaken the tissues of the nose and throat (or middle ear or sinus), or the immune system, permitting these bacteria to invade the bloodstream and spread to vital organs. For that reason, a child does not have to be exposed to pneumococcal disease in someone else to get sick--he can become infected from bacteria that reside within his own body. Excellent vaccines are available, however, to help reduce the risk of all three of these kinds of infections, and penicillin prophylaxis markedly reduces the risk of pneumococcal disease. Salmonella Children with sickle cell disease also are more susceptible to Salmonella infections. Salmonella disease usually is limited to the gastrointestinal tract, causing fever, vomiting, and diarrhea. However, children with sickle cell disease are much more prone than other children to develop serious invasive Salmonella infections of the bone (osteomyelitis), as well as sepsis and meningitis. Poultry, eggs, dairy products, fruits, chitlins, and vegetables can all transmit Salmonella infections, so careful food handling is extremely important. Salmonella also can be transmitted by a variety of pets, especially reptiles and turtles, so it's a good idea to check with your child's healthcare provider before bringing one home. Early antibiotic therapy Early in the course of an illness, it can be difficult, if not impossible, for even skilled healthcare providers to distinguish between a simple viral infection and a potentially deadly bacterial infection, since both can have the same nonspecific symptoms like fever, tiredness, stomachache, runny nose, cough, or headache. Since the risk of serious bacterial infections is greater in children with sickle cell disease, healthcare providers generally play it very safe and treat them with antibiotics at the earliest sign of infection, before the exact cause of the illness has been determined. It is extremely important for the family to let healthcare providers know that this is a child with sickle cell disease when discussing how to handle a fever. Advice given for fever in most children is not the same as advice for fever in a child with sickle cell.
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