Risk for bacteremia low among children with sickle cell disease, fever

Key takeaways:

• Bacteremia was uncommon among children and young adults with sickle cell disease who presented at the ED with a fever.
• The large study included patients aged younger than 22 years.

The risk for bacteremia was low — just over 1% — among children and young adults with sickle cell disease who presented at the emergency department with a fever, according to the results of a large study published in JAMA Network Open.

The study included more than 35,000 ED encounters with more than 11,000 patients at 36 pediatric hospitals in the United States.

“To our knowledge, this is one of the largest studies to date describing the risk of bacteremia in this population,” the authors wrote.

According to the authors, sickle cell disease is the most common genetic disease in the world, with an estimated 400,000 children born with it each year, including 2,000 in the U.S.

“Bacteremia and invasive bacterial infections are important causes of death in children and young adults with [sickle cell disease], especially those younger than 5 years, due to the functional asplenia, immune system defects, and anatomic predisposition toward osteomyelitis inherent to the underlying disease,” the authors wrote. “Before development of the conjugate pneumococcal vaccine and hydroxyurea treatment, bacteremia was common, and the likelihood of death from an episode of bacteremia was 15% to 20%.”

“Given the historical high risk of mortality due to bacteremia, guidelines in the U.S. recommend prompt emergency department evaluation for fever higher than 38.5 °C and standardized care for this high-risk population, including the collection of laboratory studies, blood cultures, and the administration of antibiotics,” they continued.

Using the Pediatric Health Information Systems database, the researchers assessed data from 35,548 ED encounters with 11,181 children and young adults with sickle cell disease and fever from 2016 to 2021.

Just 405 of the total encounters — 1.1% — involved a diagnosis of bacteremia within 3 days of the index visit.

The researchers found that a prior history of bacteremia (OR = 1.36; 95% CI, 1.01-1.83), central line-associated bloodstream infection (OR, = 6.39; 95% CI, 3.02-13.52) or apheresis (OR = 1.77; 95% CI, 1.22-2.55) was associated with a diagnosis of bacteremia.

“Prospective studies on children and young adults with [sickle cell disease] presenting with fever are needed to develop decision models and risk stratification tools to refine our approach and avoid unnecessary antibiotic exposure and hospitalization in this population,” they wrote.

In an editorial commentary published alongside the study, Suzie A. Noronha, MD, director of the pediatric sickle cell program at the University of Rochester Medical Center, and John J. Strouse, MD, PhD, a pediatric hematology-oncology specialist at Duke Health, noted that newborn screening for sickle cell disease in the U.S. and early prophylaxis with penicillin were associated with greatly reducing the rate of invasive pneumococcal infections in children.

“These rates decreased further with the widespread use and expanded serotype coverage of the conjugate pneumococcal vaccines, such that invasive pneumococcal infections, although still much more frequent than the general population, have become a rare cause of serious illness and death in children with [sickle cell disease] in the United States,” they wrote.

Noronha and Strouse also noted the effect that usual treatment can cause on pediatric patients.

“The impact of emotional trauma to young children who undergo repeated blood cultures or work time lost for parents who must bring their child for evaluation has not been measured,” they wrote. “Perhaps it is time to critically reexamine our approach to fever in children with [sickle cell disease] with a more comprehensive analysis of contemporary risks and benefits.”

References:

Noronha S, et al. JAMA Network Open. 2023;doi:10.1001/jamanetworkopen.2023.18837.

Rineer S, et al. JAMA Netw Open. 2023;doi:10.1001/jamanetworkopen.2023.18904.