Sickle Cell Disease - Complications

Pneumonia and Other Infections

Infections are common and an important cause of severe complications in sickle cell patients. Before early screening for sickle cell disease and the use of preventive antibiotics in children, 35% of sickle cell infants were lost to infections. Fortunately, with screening tests for sickle cell now required for newborns in most states and with the use of preventive antibiotics in babies who are born with the disease, this terrible mortality rate has dropped significantly.

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Infections in Infants and Toddlers with Sickle Cell Disease. The most common organisms causing infection in children with sickle cell disease are the following:

Streptococcus pneumoniae (can cause blood infections or meningitis)
Haemophilus influenza (a cause of meningitis)

Such infections pose a grave threat to infants and very young children with sickle cell disease. They can progress to fatal pneumonia with devastating speed in infants, and death can occur only a few hours after onset of fever. The risk for pneumococcal meningitis, a dangerous infection of the central nervous system, is also significant.

Infections in Children and Adults. Infections are also common in older children and adults with sickle cell disease, particularly respiratory infections such as pneumonia, kidney infections, and osteomyelitis, a serious infection in the bone. The organisms causing them, however, tend to differ from those in young children. The incidence of pneumococcal infections decrease and those caused by other bacteria increase, including the following:

Chlamydia and Mycoplasma pneumoniae. These are the important infectionsin acute chest syndrome (see above).
Gram-negative bacteria. This group ofbacteria mostly infects hospitalized patients and can cause serious pneumonias and other infections.

General Approach to Treating Infections. Fever in any sickle cell patient should be considered an indication of infection. Temperatures over 101 F in children warrant a call to the doctor. Adults with sickle cell should call the doctor if they have a have fever over 100 F and any signs of infection including chest pain, productive cough, urinary problems, or any other symptoms. Some approaches for treating infections are as follows:

Hospitalization for infections. When sickle cell patients develop infections, they are nearly always hospitalized immediately and treated with intravenous or high dose injections of antibiotics in order to prevent septicemia, the dangerous spread of the infection throughout the body. Antibiotics called cephalosporins (e.g., cefotaxime [Claforan], ceftriaxone [Rocephin] or cefuroxime [Ceftin]) are typically used. Repeated hospitalizations are very disruptive for both children and adults. Studies have found that older children whose fever is below 38.5 C (101 F) and who have no serious infection or other complications may not need hospitalization. Children who have indications of serious complications of infection (higher fevers, pain, a history of pneumonia, and signs of dehydration) should remain in the hospital.
Treatment of osteomyelitis. If osteomyelitis, an infection in the bone, occurs, a 6-week antibiotic course is needed, most of it intravenous. An accurate diagnosis of osteomyelitis is sometimes difficult to make, because bone damage from sickling can cause similar symptoms. It should be strongly considered in children with signs of pain and swelling in the legs, a high white blood cell count, high fever, and high levels of a test that measures so-called sedimentation rates. It is important, however, to confirm the presence of an actual infection before administering antibiotics, because the antibiotic treatment required for osteomyelitis is so intensive and prolonged. The most common cause of osteomyelitis in children is Salmonella.
Treatment of urinary tract infections. Urinary tract infections may be difficult to manage and can be a serious problem for pregnant women with sickle cell disease. Doctors should take a urine culture before beginning antibiotic treatment and another culture1 to2 weeks after treatment to be sure the infection has cleared up.