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Management of Fever in Pediatric Sickle Cell Disease Patients

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Management of Fever in Pediatric Sickle Cell Disease Patients Management of Fever in Pediatric Sickle Cell Disease Patients Background Sickle cell disease (SCD) is a genetic blood disorder that results infections, particularly with encapsulated organisms (S. pneumo- in the formation of abnormal hemoglobin S within the red blood niae, N. meningitides, H. influenzae type b, S. typhi). Serious bac- cell. This abnormal hemoglobin results in stasis of flow in the terial infections carry the risk of progression to sepsis, shock and spleen, resulting in hypoxic damage, fibrosis and eventual auto- death. A rapid diagnostic workup and the rapid administration of splenectomy, typically by age 5. Autosplenectomy results in com- empiric broad-spectrum antibiotics are critical to prevent serious promised immune function and increased risk of serious bacterial infections in this patient population. Definitions Fever: a temperature of > 38.5 C (>101 F). NOTE: A subjective Sickle Cell Disease: A group of inherited red blood cell disorders (tactile) fever is acceptable for the definition of fever. including HgSS, HgSC, HgSE and Hgb Sβ-thalassemia types that predispose patients to anemia, infection and other complications. Target Population and Inclusion Criteria This pathway should be used for patients > 6 months of age with Patients eligible for outpatient management: SS, SC, SE or S -thalassemia sickle cell disease who have a tem- β ■ Non-toxic appearing perature ≥ 38.5°C. ■ < 40° C ■ No history of sepsis ■ No infiltrate on chest radiograph ■ No hypoxia ■ Labs at baseline ■ WBC < 30,000 ■ No social concerns (reliable follow up, access to transportation, at least 1 functioning telephone number) 1 Evaluation 1. General: Assess stability 4. Diagnostic Evaluation: a. Vital signs, mental status, respiratory and circulatory status a. CBC with differential and reticulocyte count b. Place on cardiorespiratory monitor with pulse oximetry b. Blood culture c. Electrolytes 2. History d. Liver function tests a. Duration of fever e. Type and Screen b. Presence of other symptoms i. If suspected splenic sequestration, acute chest c. Hydration status syndrome pale, persistent tachycardia, ill appearing, d. Contact hematologist on call: Baseline hemoglobin aplastic crisis (reticulocyte count < 1%) e. Medical history: SCD complications, previous admits/ICU admits, splenic sequestration/splenectomy, last transfu- f. Chest Radiograph sion, last antibiotics i. A CXR is indicated if the patient has any of the following f. Medications: Penicillin prophylaxis, hydroxyurea, immuni- signs or symptoms to rule out acute chest syndrome zation status (ACS). ACS syndrome is the leading cause of death in SCD. Diagnosing and treating quickly is imperative. 3. Physical Exam 1. Chest pain a. Vital signs 2. Shortness of breath b. General appearance: jaundice 3. Cough c. Respiratory 4. Hypoxia d. Circulatory e. Abdomen (spleen size) g. Respiratory Viral Panel f. Neurologic i. If suspected viral illness g. Signs of focal infection 1. Patients testing positive for influenza should be treated with Oseltamivir h. Urinalysis and Culture i. If clinical suspicion, history of urinary tract infection or renal anomaly or appropriate age range Treatment Recommendations Goal of antibiotic administration within 1 hour of presentation. 3. In suspected ACS, add azithromycin 10 mg/kg to the empiric All sickle cell patients with fever should be discussed with the treatment. pediatric hematology attending on call (617-636-5114). 4. Antibiotics are to be given to all SCD patients who present 1. Administer ceftriaxone (50 mg/kg, max 1 g) as soon as the with fever even if they are “well appearing.” blood cultures and labs are obtained. Do NOT wait for the lab 5. Administer IV fluids and other supportive medications as results to administer the antibiotic. indicated by the patient’s clinical presentation. 2. In septic appearing patients, add Vancomycin (15 mg/kg/ 6. Use caution with IVF administration if suspected acute chest dose) to the empiric treatment. syndrome or splenic sequestration. Admission Criteria Consideration for admission should be a dynamic process taking 1. Toxic, ill appearing or clinically suspected ACS into account reassessment of patient over time and in response 2. Child is under 6 months old or not up to date on vaccinations to intervention. However, there are some instances in which admission is necessary or strongly encouraged: 3. Hemoglobin and /or reticulocyte count well below baseline or WBC > 30,000 2 Follow Up 1. Follow up at 24 hours is necessary, even if patient has b. Options for in-person follow up: defervesced i. Primary care physician with hematologist on-call a. Follow up may be via telephone or in person contacted b. Clinician to ensure reliable phone number on record prior ii. Hematology-Oncology department at Floating to patient discharge Hospital for Children 2. For patients with central venous access, next day follow up iii. Emergency room with hematologist on-call in person is necessary for antibiotic administration contacted (i.e. weekend, holiday) a. Ceftriaxone (50 mg/kg IV/IM, max 1g) to be administered every 24 hours until blood cultures are negative for 48 hours. References 1. Baskin M, Goh X, Heeney M, Harper M. Bacteremia risk and outpatient management of febrile patients with sickle cell disease. Pediatrics. 2013;131(6);1035-1041 2. Norris CF, Smith-Whitley K, McGowan KL. Positive blood cultures in sickle cell disease: time to positivity and clinical outcome. J Pediatric Hematology Oncology. 2003; 25(5); 390-395 3. National Institutes of Health; National Heart, Lung and Blood Institute Guidelines for the management of sickle cell disease; 2014. 4. New England Pediatric Sickle Cell Consortium. Evaluation and management of fever in pediatric patients with sickle cell disease; 2005. Practice guidelines do not necessarily apply to every patient. A provider’s clinical judgment is essential. As always, clinicians are urged to document management strategies. Floating Hospital For Children at Tufts Medical Center contacts: Michael Kelly, M.D., Norah Emara, M.D., Dan Hale, M.D. ([email protected]) 3 FEVER TO > 38.5 IN SICKLE CELL Disease PatieNT FEVER TO > 38.5 IN SICKLE CELL Disease PatieNT Discuss WITH Pediatric HEMatOLOGY/ONCOLOGY ATTENDING ON CALL FLOatiNG HOspitaL: 617-636-5114 RAPID INitiatiON OF DIAGNOstic WORK UP CBC WITH DIFFERENTIAL, reticuLOCYTE COUNT, BLOOD cuLTURE, TYPE AND screen—oTHER Labs BY SYMptOM/preseNtatiON TOXIC APPEARING, HistORY OF Ye s OFF Ceftriaxone 50 mg/kg IV/IM bacteriaL sepsis OR ICU ADMIT, +/- Vancomycin 15 mg/kg IV PATHWAY T> 40 ? Call Heme/Onc or PICU for admit No * UR CONCERN FOR Acute CHest Chest X-ray Ye s OFF SYNDROME?1 Ceftriaxone 50 mg/kg IV/IM 1 HO PATHWAY + Azithromycin 10 mg/kg IV/PO Call Heme/Onc or PICU for admit No ELIGIBLE FOR OutpatieNT MANAGEMENT?2 Ye s No CEFTRIAXONE 50 MG/KG CEFTRIAXONE 50 MG/KG Call hematologist to ADMIT (MAX 1 G) (MAX 1 G) discuss disposition DOes patieNT HAVE 1. Acute Chest Syndrome: New infiltrate on chest x-ray associated with >1 A CENtraL VENOus of the following: fever, cough, sputum production, dyspnea, hypoxia catHETER? 2. Eligibility for outpatient management criteria includes: Non toxic Ye s No appearing, T< 40 C, no h/o sepsis, no infiltrate on CXR, no hypoxia, labwork at baseline, WBC < 30,000, no social concerns (i.e. reliable follow up, access to transportation, at least 1 functioning phone) DiscHARGE WITH IN- DiscHARGE 3. Patients with central venous catheters should receive two doses of persON WITH ceftriaxone until their blood culture is negative for 48 hours; this can NEXT DAY PHONE occur in outpatient setting. FOLLOW FOLLOW UP * Rapidly administer empiric antibiotics. Do not wait for the results of the UP3 labs/diagnostic work up. 4 Update 030315 | 15-0028 PD-HS LAST UPDATED: 19 MARCH 2015.
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