A Systematic Search for Pediatric Sickle Cell Fever Guidelines Reveals the Need for Update
Elspeth Bittle, Tea Reljic, Ambuj Kumar, Jane S Hankins, Sheena MukkadaAbstract
Background
Infection/sepsis remains the number one cause of death among children with sickle cell disease (SCD) under the age of 5 years. The splenic autoinfarction and high inflammatory state characteristic of SCD limit the successful clearance of pathologic organisms, placing children at increased risk. Significant improvements in disease-modifying therapies and broader strain pneumococcal vaccines in the past ten years necessitate revisiting our clinical practice guidelines (CPG). The Adapted Resource and Implementation Application (ARIA) model for guideline development facilitates adaptions to pediatric cancer care in settings with varied resources but has not been applied to non-malignant catastrophic diseases. We hypothesize that the ARIA model can be employed in pediatric hematology, specifically acute fever management in SCD. We aim to assess the feasibility of using the ARIA guideline development and adaptation process to develop resource-adapted pediatric hematology care guidelines readily accessible to providers (https://aria.stjude.org). Here, we describe the results from Phase I, which involves a systematic search and critical appraisal of existing fever guidelines. Phase II, guideline development with content experts, and Phase III, guideline adaptation with context experts, will be described in subsequent publications.
Methods
A systematic search for guidelines was conducted in the PubMed database using established search filters for CPG, pediatrics, fever, and SCD. Any guideline published in the last ten years matching these terms was eligible for inclusion. The literature search was supplemented by inquiries into websites of professional societies and consortia and with experts in the field. Each guideline underwent methodological appraisal with the AGREE II instrument by two independent reviewers.
Results
The systematic search yielded 827 articles. None of these articles were unique fever guidelines, but seven referenced CPG. Ultimately, nine guidelines met the inclusion criteria. The mean overall score across these guidelines was 45% (range 25-67%). Table 1 demonstrates the AGREE-II results for the nine guidelines across the various domains; zero of these scored an acceptable rating (5 or greater out of 7 total possible points) in all categories.
Conclusions
Phase I of the ARIA methodology utilized established search filters to identify nine CPG for managing fever in pediatric sickle cell patients, most of which are not CPG informed by a systematic literature review. This work highlights the need for a contemporaneous pediatric sickle cell fever guideline that is easily identifiable via a simple internet search, is publicly available, and is rooted in an evidence base pertinent to the region of practice.