Do not miss the diagnosis of sepsisor pneumonia in a patient with sickle cell disease and fever

Do not miss the diagnosis of sepsisor pneumonia in a patient with sickle cell disease and fever: Excerpt from Avoiding Common Pediatric Errors

Author: Emily Riehm Meier, MD

What to Do - Interpret the Data

Sickle cell anemia (SCD) is a chronic hemolytic anemia affecting 1 in 400 African Americans in the United States. End-organ damage from the persistent anemia and vaso-occlusion from irreversibly sickled cells has been well documented. One of the earliest organs affected is the spleen, placing children with SCD at increased risk for overwhelming bacterial sepsis. Patients with SCD also have poor opsonization of encapsulated organisms, related to decreased serum concentrations of opsonic antibodies. Asplenic patients requirehigher concentrations of antibody to effectively eliminate organisms, placing SCD patients at even higher risk for sepsis. Streptococcus pneumoniae is the most common cause of sepsis in SCD patients. Early detection with newborn screening programs, allowing for early institution of penicillin prophylaxis, and widespread availability of immunizations against pneumococcus and Haemophilus influenzae type b have dramatically reduced the incidence of bacterial sepsis. Nonetheless, SCD patients with fever demand special consideration and treatment to prevent adverse clinical outcomes.

With the development of third-generation cephalosporins, most febrile SCD patients can now be managed as outpatients. However, certain patients are considered high risk and continue to require inpatient admission. The presenting signs and symptoms, coupled with diagnostic studies can help to determine if a patient is standard or high risk. Each febrile sickle cell patient should have a thorough history and physical examination performed. Blood cultures, urinalysis, complete blood count with white blood cell (WBC) differential and reticulocyte count should be obtained. All patients, even thosewithout respiratory signsorsymptoms, should have a chest radiograph(CXR).Patientsareconsideredhigh riskiftheyareill-appearing, had a fever >40°C, WBC >30,000/µL or <5,000/µL, a new pulmonary infiltrate on CXR, history of pneumococcal sepsis, penicillin noncompliant, platelet count <100,000/µL, rapidly enlarging spleen, or younger than age 1 year. High-risk patients need to be admitted to the hospital for intravenous antibiotics and observation for at least 48 hours. Standard-risk patients can safely receive 50 to 75 mg/kg of ceftriaxone and be discharged home. These patients need to return the next day to receive a second dose of ceftriaxone.

Pneumonia in a sickle cell patient can lead to acute chest syndrome (ACS), defined as a new pulmonary infiltrate on CXR accompanied by fever, chest pain, tachypnea, or hypoxia. ACS is the leading cause of death in sickle cell patients and requires aggressive treatment to prevent serious sequelae. One recent study found that 60% of pulmonary infiltrates found in febrile sickle cell patients were not suspected by the treating clinician. This study failed to demonstrate a constellation of symptoms to accurately predict new pulmonary infiltrates in febrile SCD patients. Therefore, all patients should have a CXR as part of the diagnostic workup.

Suggested Readings

Morris C, Vichinsky E, Styles L. Clinician assessment for acute chest syndrome in febrile patients with sickle cell disease: is it accurate enough? Ann Emerg Med. 1999;34:64–69.
West DC, Andrada E, Azari R, et al. Predictors of bacteremia in febrile children with sickle cell disease. J Pediatr Hematol Oncol. 2002;24:279–283.
Wilimas JA, Flynn PM, Harris S, et al. A randomized study of outpatient treatment with ceftriaxoneforselectedfebrilechildrenwithsicklecelldisease. NEnglJMed.1993;329:472– 476.

Book Source Details

• Book Title: Avoiding Common Pediatric Errors
• Author(s): Anthony D Slonim MD, DrPH; Lisa Marcucci MD
• Year of Publication: 2008
• Copyright Details: Avoiding Common Pediatric Errors, Copyright © 2008 Lippincott Williams & Wilkins.

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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.

More About This Book: Title: Avoiding Common Pediatric Errors Authors: Anthony D Slonim MD, DrPH; Lisa Marcucci MD Publisher: Lippincott Williams & Wilkins Copyright: 2008 ISBN: 0-7817-7489-6

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