Systematic Review of l-glutamine for Prevention of Vaso-occlusive Pain Crisis in Patients with Sickle Cell Disease.

l-glutamine was approved by the U.S. Food and Drug Administration (FDA) for sickle cell disease (SCD) in 2017. A vaso-occlusive crisis (VOC) occurs in persons with SCD and is associated with acute pain episodes. This systematic review summarizes the evidence for l-glutamine in the prevention of VOC and associated pain in patients with SCD. Medline, Embase, and International Pharmaceutical Abstracts were searched for records reporting on l-glutamine use in persons with SCD. Eligibility criteria identified primary reports of investigations conducted in humans who were administered l-glutamine, reported on outcomes related to VOC or associated pain, published in English, and were available as full text. All relevant efficacy, safety, participant demographic data, and study method characteristics were extracted and documented. Risk-of-bias assessments were conducted using the Risk of Bias in Non-Randomized Studies-of Interventions (ROBINS-I) tool and the revised Cochrane risk-of-bias tool for randomized studies. Three studies assessing the effect of exogenous l-glutamine administration in patients with SCD met eligibility criteria: one prospective nonrandomized controlled study and two prospective randomized controlled trials. Rate of VOC and related hospitalizations were reduced in patients receiving l-glutamine, although some conflicting results were noted between studies. l-glutamine was generally well tolerated. Limitations of one or more of the eligible studies included small sample size, nonblinding, and study groups that differed at baseline. l-glutamine has limited high-quality evidence supporting its use. Although l-glutamine is FDA approved for the prevention of frequent episodes of VOC pain, only one randomized controlled trial has strong evidence to support this indication. Based on the results of a systematic review, l-glutamine may be considered for patients unable to receive hydroxyurea or in addition to hydroxyurea for reduction in VOC and associated pain.

Using Quality Improvement to Introduce and Standardize the National Early Warning Score (NEWS) for Adult Inpatients at a Children's Hospital.

INTRODUCTION: The population of adults with childhood-onset chronic illness is growing across children's hospitals and constitutes a high risk population. National Early Warning Score (NEWS) is among the most recently validated adult early warning scores (EWSs) for early recognition of and response to clinical deterioration. Our aim was to implement and standardize NEWS scoring in 80% of patients age 21 and older admitted to a children's hospital. METHODS: Our intervention was tested on a single unit of our children's hospital. The primary process measure was the percentage of NEWS documented within 1 hour of routine nursing assessments, and was tracked using a run chart. Improvement activities focused on effective training, key stakeholder buy-in, increased awareness, real-time mitigation of failures, accountability for adherence, and action-oriented response. We also tracked the distribution of NEWS values and medical emergency team calls. RESULTS: The percentage of NEWS documented with routine nursing assessments for patients age 21 and over increased from 0% to 90% within 15 weeks and remained at 77% or greater for 17 weeks. Our distribution of NEWS values was similar to previously reported NEWS distribution. CONCLUSIONS: A nurse-driven adult early warning system for inpatients age 21 and older at a children's hospital can be achieved through a standardized EWS assessment process, incorporation into the electronic health record, and charge nurse and key stakeholder oversight. Furthermore, implementation of an adult EWS being used at a pediatric institution and our distribution of NEWS values were comparable to distribution published from adult hospitals.