Hot Seat #79: 7wo F with sickle cell disease and fever
Posted on: August 29, 2016, by : Sam Zhao
Sam Zhao, Children’s National Medical Center
with Alex Rucker, Children’s National Medical Center
The Case
A 7wk ex-FT girl with sickle cell disease diagnosed on newborn screen presents to the ED with a fever of 100.5 F taken at home. Mom has also noticed some cough and runny nose. She continues to eat well and has not been in any distress. Of note, she had just received her two-month immunizations the previous day.
ROS: As per HPI, otherwise negative
PMH: Hgb FS on newborn screen, not yet evaluated by Hematology
BH: born via C-section for maternal hypotension and fetal bradycardia; post-delivery course uncomplicated
FH: unremarkable
SH: parent’s first child; lives with mother and father; family is originally from Nigeria
PE:
T 38.3, HR 144, BP 96/44, RR 41, O2 sat 99% on room air
The patient is alert, well-appearing, and developmentally appropriate. She has significant congestion and rhinorrhea and moist mucous membranes. Her cardiac, pulmonary, and abdominal exams are unrevealing.
Question 1:
Because of the patient’s age, a workup for serious bacterial infection is initiated including bloodwork and urine. Results show:
UA: pH 6.0; no blood, ketones, nitrites, or LEs; SG 1.004
CBC: WBC 10.0 (38% N, 53% L, 8% M, 1% E, 0% B, no bands), Hgb 8.5, Hct 24, Plt 485
Question 2:
How would you approach this case? Please share your opinions by clicking on “What do you think?” below.
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Given that she is a non-ill appearing ~49d old febrile infant, I would obtain blood and urine to better stratify her as high-risk or low-risk for SBI. Looking that published algorithms, with her CBC and U/A both low-risk, I’d move on to trying to identify other factors that would make her high-risk for SBI. She is FT, no hx of NICU, no systemic abx pre-evaluation. The only factor that jumps out is HbgSS as a “chronic medical condition”. However, my understanding is that HgbSS patients are only at higher risk of infection in the setting of complications of HgbSS, like spenic dysfunction. I also remember reading that infants experience little to no complications of HgbSS given their high levels of HgbF. Therefore, I wouldn’t be inclined to consider HgbSS in this patient to be a “chronic medical condition” that would change her risk stratification.
Therefore, I voted to defer LP and abx. As far as admission for obs vs. discharge, if I follow my same argument above, I should be risk-tolerant enough to discharge with follow-up. However, as a young clinician that is quite risk-averse, I probably would admit for observation.
I would agree this HbSS infant is well appearing. Despite HbSS Monica pointed out she would still have plenty of hemoglobin F and she does not have worrisome vitals. This infant would have had the full septic workup in the ’90s.
in 2016, at 49 days, and one set of immunizations, I agree with blood and urine, and a respiratory panel. I generally make my decision whether to admit or not on follow up and parental reliability. In this case, since she has a co-morbidity but stable presentation, I would also admit for observation and hold antibiotics. Since she hasn’t been seen by hematology yet, maybe they could stop by.
Out of curiosity, if a procalcitonin and CRP were available, would that change any opinions?
Four questions needed to be answered for this infant.
1) Why is she 28-90 days in my emergency department with a fever? Why is she persecuting me? Why does she hate me?
While this may be more of an existential than a medical question, we all know we tremble a bit with a child this age with fever.
According to UptoDate:
Infants at high risk for infection should get a full sepsis workup (ugh. Leading us to question #2…..)
Infants with a procalcitonin >0.3 or CRP > 20 should get a full sepsis workup
Infants with families with social barriers to outpatient management (transportation, no insurance, no PMD) should be admitted and observed
Interestingly:
Patients with rectal temperatures > 38.6 places them at higher risk and a full sepsis workup should be considered.
The latest evidence on infants this age is the recent article by Gomez et al. This was a multicenter study at 11 European pediatric emergency departments. All febrile infants were less than 90 days and none had chronic illness. It compared the Step-by-Step Approach (see below), the Lab-score approach and the Rochester criteria in predicting isolation of a bacterial pathogen in blood or CSF.
What’s the Step-by-Step approach?
Step 1: Look at clinic appearance—if they appear well, proceed to step 2
Step 2: If age is ≥ 21 days, proceed to step 3 (bold, huh? Idk—see below)
Step 3: Look at the UA – if no WBCs in the urine, proceed to step 4 (they did not comment on what their cutoff was for leukocyturia)
Step 4: Check the procalcitonin level – if ≤0.5, proceed to step 5
Step 5: Check the CRP and ANC – if the CRP < 20 and ANC 20 – LP
With the information given – no LP and admit off antibiotics. Why? At some point if she reaches 38.5, she will need ceftriaxone, and if she does, she needs an LP (yes, dear residents, on the floor. It can be done). To me (her non-parent), being in the hospital for that rather than schlepping all the way back would be preferable.
Often I’ll test kids that are this age for RSV if they have any sort of viral illness, given the studies that put their incidence of meningitis very low. These studies were only conducted in kids who actually had symptoms of bronchiolitis, so a positive RSV result in this patient would not help me decide.
Hey Big Bear—where’s my rapid procalcitonin??? Stop trying to pretend it doesn’t exist and get it for us in the ED!!!